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The Peripheral Neuropathy Program

Within The Peripheral Neuropathy Solution, you will discover a breakthrough 6-step proven extensive treatment program that can help you finally heal your ruined nerves and end your own case of neuropathy. Irrespective of your age, background and gender and the cause of your peripheral neuropathy, this program can meet your needs. Neuropathy Solution Program consists of only natural elements, with absolutely no substance additives. Believed to help your complete circulation of blood. These types of herbal products function in mix to boost restricted circulation of blood, enhance syndication, reduce high blood pressure, and control all the cardio exercise system. With six easy steps that include changes in diet, exercise and lifestyle habits, a peripheral neuropathy sufferer can have permanent relief from the many painful, debilitating symptoms in as little as a month, often times even less. Continue reading...

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Overview Of Peripheral Nervous System Myelination And Electrophysiology

The peripheral nervous system (PNS) comprises less than 0.1 of all nerve tissue. The somatic PNS, when defined anatomically by the presence of Schwann cells, includes the primary roots, dorsal root ganglia, mixed spinal nerves, plexuses, nerve trunks, autonomic nervous system, and the third to twelfth cranial nerves. Peripheral nerve trunks are composed of a great number of axons, which are grouped into individual bundles or fascicles. These structures vary in number, depending on the nerve, its size, and the site at which it is studied. Schwann cells ensheath every PNS axon nearly throughout their extent. Peripheral nerve fibers are divided into two groups, either myelinated and unmyelinated, depending on whether they have one, or several, axons per Schwann cell. In the human sural nerve, the ratio of unmyelinated to myelinated axons is approximately 3.7 to 1. '6 A myelinated peripheral nerve fiber consists of an axon and a series of Schwann cells located sequentially on its external...

Lesions in the Retroparotid or Retropharyngeal Spaces and Distal Peripheral Nerve Lesions

CN XI and XII may be affected by a number of distal peripheral nerve lesions resulting from surgical trauma, '32' y local infections, stretch,y , y neck irradiation,'41' or local tumors.y y A dissecting carotid aneurysm may result in hypoglossal nerve palsy. y A self-limited idiopathic hypoglossal nerve palsy also has been reported. y , '49' The resultant findings (i.e., weakness and atrophy) in peripheral nerve lesions have been described earlier. Occasionally, a lesion may affect CN IX through XII, with a resultant Collet-Sicard syndrome, characterized by the same features as Vernet's syndrome described earlier, with the addition of weakness of the ipsilateral tongue.y , '12'

Electrical Characteristics Of Peripheral Nerve Stimulators Currently Available In Clinical Practice

Electrical Nerve Stimulator Anesthesia

To determine which electrical characteristics of the PNS contributed to the localization of a peripheral nerve, the electrical properties of eight commercially available peripheral nerve stimulators and a Grass S-88 stimulator were measured. The PNS was then used in the laboratory to locate a peripheral nerve in an anesthetized cat. The controlled environment in the laboratory allowed each PNS to be used under nearly identical conditions. Experiment I Determination of Output Characteristics of Peripheral Nerve Stimulators To determine the shape and duration of the stimulating pulse, the linearity of the output, and the change in the output in response to a change in load resistance, the peripheral nerve stimulators were connected to a variable resistance and an oscilloscope (Tektronix Type 564B) as shown in Figure 17-6 . The shape, duration, and amplitude of the output were measured on the oscilloscope screen. The linearity of the output was determined by correlating the current...

Efficacy Of Clinically Used Peripheral Nerve Stimulators

Kaiser and colleagues123 produced a list of desirable properties in peripheral nerve stimulators, which included adjustable constant current at resistances of 500 Q to 10k Q a monophasic square wave initial impulse selectable impulse duration of 0.1 and 1.0 ms unequivocal scale graduation or current indicator, especially over the range of 0.5 to 1.0 mA and impulse frequency of 1 to 2 Hz. They also made recommendations on self-test and safety features for nerve stimulators, which included an alarm at high impedance and check on electrical circuit battery test unequivocal assignment of load end high-quality connections and cable instructions for use and design operating parameters. The general procedure of nerve localization involves producing a small evoked muscle twitch with the PNS, moving the needle in a direction that causes an increase in the twitch strength, and then reducing the stimulus strength to restore the twitch to its previous tension. This procedure is repeated until...

Demyelinating Disorders of the Peripheral Nervous System

Overview of Peripheral Nervous System Myelination and Electrophysiology Waldrop, in 1834, reported a single patient who probably had acute inflammatory demyelinating polyradiculoneuropathy (AIDP), thereby providing the first clinical description of a demyelinating polyneuropathy. Landry, in 1859, described 10 patients who very likely had the same disorder he recounted most of the clinical features now considered typical for AIDP. Guillain, Barre, and Strohl, in 1916, reported two soldiers who were recovering from rapidly evolving, principally motor polyneuropathies. They emphasized the loss of the deep tendon reflexes (DTRs) and the presence, on cerebrospinal fluid (CSF) examination, of an elevated protein level with a normal cell count (albuminocytological dissociation). Their paper probably has been the most influential of all the articles concerned with demyelinating neuropathies from it the commonly used eponymic title for AIDP derives, that is, the Guillain-Barre syndrome, or...

Amyloidoma of the Peripheral Nerve

Amyloidoma or amyloid tumor consists of a localized deposit of amyloid material, which is composed of extracellular fibril-lar proteins with a b-pleated sheet secondary structure. Amy-loidomas are rare and usually located in the lung and upper respiratory tract. Occasionally, these tumors have been detected in the PNS, most commonly in the trigeminal or gasserian ganglion or one of its three branches.96,129 The etiology and patho-genesis of amyloidomas in peripheral nerves are unknown. Some studies have suggested that amyloidoma formation is due to an abnormal inflammatory response, where elevated numbers of plasma and other inflammatory cells are present surrounding the amyloid deposits. Treatment includes surgical excision of these abnormal masses.

Peripheral Nerve Blocks

In a nonrandomized prospective study of regional anesthetic blocks reported by Arner and associates,112 38 consecutive patients with neuralgias were treated with various local anesthetic peripheral nerve blocks. Etiologies included entrapment syndromes as well as surgical and traumatic nerve damage, and all patients had demonstrable sensory dysfunction. The peripheral nerves blocked included ilioinguinal, iliohypogastric, saphenous, superficial peroneal, intercostal, suprascapular, and lateral femoral cutaneous nerves. Each patient underwent a series of blocks (median number of 5) with 0.5 bupivacaine. The outcomes assessed included the magnitude of pain relief and its time course. All patients reported complete pain remission immediately after the blocks were performed. After a single block, 18 patients (47 ) reported complete relief lasting from 12 hours to 6 days, which were durations longer than the period of conduction block. Twenty patients (53 ) reported initial complete relief...

Malignant Peripheral Nerve Sheath Tumors

MPNSTs include all malignancies arising from the connective tissues of the peripheral nerves. Attempts to subdivide these tumors based on cell of origin have not yielded consistent results. They are extremely rare in the general population, with an incidence of 0.001 2 however, they are a major cause of mortality in individuals with NF1. These patients exhibit a lifetime risk of 8 to 13 .3 In patients with NF1, MPNSTs are thought to arise from preexisting plexiform neurofibromas and occasionally neurofibromas. MPNSTs are aggressive lesions with a poor prognosis that exhibit progressive enlargement, infiltration of surrounding tissues, and frequent metastases.

Inflammatory Pseudotumor of the Peripheral Nerve

Localized or diffuse swelling of a peripheral nerve may also be due to a number of inflammatory or infectious diseases. Inflammatory pseudotumor, also described as nodular lymphoid hyperplasia, plasma cell granuloma, and fibrous xanthoma, are all reactive and non-neoplastic processes that can mimic a peripheral nerve mass lesion. Inflammatory pseudotumors are characterized by chronic infiltration of inflammatory cells, primarily the T-cell population, as well as extensive fibrosis and collagen deposition. The etiology is unclear, but the inflammation may be triggered by various stimuli such as physical, biologic, or chemical factors. Five cases of inflammatory pseudotumor involving peripheral nerves (median nerve, facial nerve, sciatic nerve, radial nerve, and greater auricular nerve) have been reported in which the inflammatory cells penetrate the nerve fascicles.95 The differential diagnosis often includes other non-neoplastic processes such as amyloidoma of the peripheral nerve and...

Hiv1associated Peripheral Neuropathy Syndromes

Acute demyelinating polyneuropathy, brachial plexopathy, and mononeuritides may occur at the time of acute infection or seroconversion. Acute inflammatory demyelinating polyneuropathy (AIDP) and chronic inflammatory demyelinating neuropathy (CIDP), although rare, are the most common form of peripheral neuropathy during the latent, asymptomatic, or mildly symptomatic stage of HIV- 1 disease when CD4+ cell counts are greater than or equal to 500 cells mm. 3 As immunodeficiency progresses and as CD4+ cell counts decline to the 200 to 500 cells mm3 range, the most frequent neuropathies encountered are mononeuritis multiplex and herpes zoster neuropathy. With HIV-1 disease progression (CD4+ cell counts are less than 200 cells mm 3 ), the occurrence of distal symmetrical polyneuropathy increases, as does the prevalence of other types of neuropathies such as autonomic neuropathy, mononeuritis multiplex, cranial mononeuropathies, mononeuropathies-radiculopathies associated with neoplasms, and...

Other Peripheral Nerve Tumors Not Included In The Who Classification System

We have described benign peripheral nerve tumors arising primarily from Schwann cells, in the case of schwannomas and neurofibromas, and perineural cells in the case of perineuri-omas. Other tumors arising from cells normally found in peripheral nerves that will be discussed include mucosal neuroma, neurothekeoma, granule cell tumor, lipoma, vascular tumors, and neuromuscular hamartoma. Peripheral nerve tumors can also originate from cells not normally found in nerves by local invasion, as in the case of desmoid tumors (i.e., from adjacent muscle tissue) or by metastatic spread in the case of primary tumors located at a distance. For example, a variety of primary tumors, such as breast and lung tumors, can metas-tasize to peripheral nerves and their plexi. More rarely, thyroid tumors osteochondromas bladder tumors extracranial meningiomas and malignant Ewing's tumors, which include Ewing's sarcoma, extraosseous Ewing's sarcoma, primitive neuroecto-dermal tumors (PNETs) or peripheral...

Peripheral Nerve Blockade

Peripheral nerve blockade is an essential tool in the management of acute and chronic pain. It is useful in the treatment of procedure-related pain (e.g., laceration repair, fracture reduction, IV catheter insertion), traumatic pain (e.g., postoperative pain, femur fracture), and in the diagnosis and management of complex regional pain syndromes (e.g., sympathetic nerve blocks). Additionally, regional anesthetic techniques are very useful when general anesthesia or systemic analgesics cannot be easily used because of their potential to exacerbate a patient's underlying medical condition. For example, nerve blocks may offer the best analgesic alternative for patients with neuromuscular, metabolic, cardiac, or chronic lung disease because regional blockade provides intense (total) analgesia and produces minimal changes in cardiac and pulmonary physiology. Thus, these blocks can provide intense, unparalleled analgesia in patients with minimal reserve who cannot tolerate opioid-induced...

Peripheral Nerve Tumors In Children

Peripheral nerve tumors (PNTs) are not common lesions. Although it is widely recognized that these lesions do occur in the pediatric population, PNTs occur with less frequency there than in the adult population. The differential diagnosis of a suspected PNT in a child follows the same general framework as for adult patients (Table 107-1), but there are several key differences between PNTs in the pediatric population and their adult counterparts. For instance, as will be discussed in detail later, lesions with a neural histogenesis, such as neuroblastoma, occur in a much larger proportion of pediatric patients with PNTs than in adults, whereas nerve sheath tumors are relatively less common. This chapter highlights the specific nuances of PNTs that are unique to the pediatric population.

Sympathetic Blockade For Neuropathic Pain

Sympathetic nerve blocks have been widely used in the treatment of multiple forms of neuropathic pain, and their role has been reviewed extensively by Boas.- Despite its historical role in the treatment of complex regional pain syndromes (CRPS), convincing evidence is lacking to support the use of sympathetic blockade in other neuropathic pain states. For example, although evidence exists that sympathetic blocks can effectively treat acute herpes zoster pain, there are no randomized controlled studies supporting belief by some that they can prevent PHN or effectively treat long-standing PHN pain- Advances in the understanding of neuropathic pain, CRPS, and sympathetically maintained pain, clinical pain states that have significant overlap in both mechanism and presentation, will require further basic science inquiry and well-designed clinical trials. Future Directions in Neuropathic Pain Management Rapid advances are being made in our understanding of chronic pain mechanisms and novel...

Table 501 Etiological Classification Of Neuropathic Pain Syndromes

Peripheral nerve injury Trauma Surgical Meralgia paresthetica (lateral femoral cutaneous nerve) Thoracic outlet syndrome Metabolic diseases Diabetic neuropathy Uremia Amyloidosis Multiple myeloma Hypothyroidism nociceptors. These signals are transmitted to the central nervous system where they undergo modulation by local and descending control systems and finally reach the level of perception at the sensory cortex. In contrast to this physiologic pain, neuropathic pain can be associated with spontaneous pain in the absence of an external stimulus, with pain evoked by a normally innocuous stimulus, or with both. The plastic changes in the nervous system that result in the neuropathic pain state have been an area of considerable interest for researchers. Neuronal plasticity refers to the process by which neurons involved in pain transmission are altered from a state of normal sensitivity to a hypersensitive states A number of peripheral and central mechanisms contribute to the...

Peripheral Nerve Metastases

Cancer can affect peripheral nerves either by compression or direct invasion. Direct invasion occurs either from hematogenous spread of tumor to peripheral nerves or dorsal root ganglia or by direct extension to nerve from surrounding structures. Typically, head and neck malignancies, melanoma, lung and breast cancer, and abdominal and pelvic tumors cause either cranial or peripheral nerve dysfunction. Occurring either early or late in the disease process, the clinical signs and symptoms are reflective of the anatomical sites and nerves involved. Frequently pain, sensory symptoms, or weakness are manifested by involvement of either single or multiple cranial nerves, spinal roots, nerve plexi, or peripheral nerves. Complications of therapy including radiation fibrosis and chemotherapy-induced neuropathy can mimic peripheral nerve metastases. Acute ischemic or inflammatory neuropathies and complications of paraneoplastic neurological syndromes should also be considered. Diagnosis may be...

Other Peripheral Nerve Tumors

Benign tumors of the peripheral nerves are often localized to single sites and are related to pressure or trauma. For example, Morton's neuroma is commonly associated with increased pressure or tight-fitting shoes and represents fibrotic swelling of the nerve in response to repeated insult. Located on the plantar surface of the foot, it is treated with excision or observation. Symptoms include localized pain, tenderness, and weakness of the distally affected muscles innervated by the nerve. Ganglion cysts may result from repeated trauma, leading to undue inflammation and cystic degeneration. In other cases, transected stump nerves undergo unorganized sprouting, leading to a large clump of disorganized cells and their processes.

Neuropathic Pain In The P Sto P E Rat Iv E Peri O D

The possibility of the development of neuropathic pain should be borne in mind after surgery, as it is often missed in patients with acute pain and may require specific therapy (see Ch. 61 for the management of chronic pain). A useful definition of neuropathic pain is 'pain associated with injury, disease or surgical section of the peripheral or central nervous system'. One diagnostic clue after surgery is an unexpected increase in opioid consumption, as neuropathic pain often responds poorly to opioids. Features suggestive of neuropathic pain include

Malignant Peripheral Nerve Tumors

I Peripheral nerve tumors are rare lesions that can arise anywhere on the body and as a result have a wide differential diagnosis. They commonly occur as a nonspecific mass lesion that is diagnosed as a peripheral nerve tumor at surgery. Although these tumors may initially be seen by a wide variety of surgeons, early recognition of the nature of the lesion and appropriate surgical treatment by a surgeon with expertise in peripheral nerve surgery is essential to minimize postoperative neurologic deficits. Even more rare are the malignant neoplasms of the peripheral nerves, with an incidence of 1 in 10,000 in the general population. This chapter focuses on the latter and aims to provide a classification and a management scheme for malignant peripheral nerve tumors.

Mechanisms Of Neuropathic Pain

Many of these cortical and subcortical pain pathway nodes are visualized during functional neuroimaging activation studies by PET.80,81 Indeed, event-related fMRI reveals regions of the anterior cingulate cortex that are related to stimulus perception, stimulus intensity, or to pain itself.82 Motor-related activations are immediately posterior to the pain-related and stimulus-related cingulate activations in the supplementary motor area. Within these systems, rehabilitationists can find solid and hypothetical rationales for physical, psychologic, pharmacologic, and surgical interventions for neuropathic pain. Pain can arise from primary afferents in peripheral nerves and dorsal and ventral roots with, for example, a diabetic neuropathy or root compression by a vertebral disk protrusion. The high thresholds of the peripheral terminals of C-fiber nociceptors are decreased by prior activation (autosensitization) or by an increase in membrane excitability by stimuli that do not activate...

Peripheral Nerve Injury

Pain following peripheral nerve injury may be exacerbated by sympathetic activity. In these cases, the structures distal to the injury (almost invariably in a limb) are cold, discoloured, demonstrate marked allodynia and have worse pain in a cold environment. Symptoms are improved with a sympathetic block. In these cases, a-adrenoceptors are expressed on the damaged nerve and, in the presence of norepinephrine from local sympathetic nerves or circulating epinephrine, induce peripheral sensitization of nociceptors and even Ap terminals. Stimulation of the latter may, in the presence of central sensitization, cause pain. However, many patients with persistent pain following trauma, with or without a peripheral nerve injury, do not have these symptoms, do not have sympathetically mediated pain, and so do not respond to a sympathetic block.

Electrophysiology Of Peripheral Nerve Stimulators

Before the middle of the 19th century, nerve fiber conduction was thought to be instantaneous. In 1850, von Helmholz,8 in a classic series of experiments with an isolated nerve muscle preparation, demonstrated the temporal nature of nerve fiber conduction and paved the way for the elucidation of most of the relevant physiology of peripheral nerve stimulation. Of particular importance is the relationship between the strength and duration of the current and the polarity of the stimulus.

Which anticonvulsant is most commonly used for neuropathic pain

Gabapentin (Neurontin) is currently used most often, although its use for pain is off-label. It may be useful for neuropathic extremity pain due to iatrogenic nerve injury, arachnoiditis, prolonged neural compression, and peripheral neuropathy. It has been shown to be useful in some patients with leg pain due to spinal stenosis. Gabapentin is started at 100 to 300 mg at night and then increased to 300 mg every 8 hours over the days to weeks, and then gradually titrated upward until there is good pain relief or significant side effects. Pain relief may occur at 900 mg per day, but often 1800 mg to 3600 mg per day are necessary. Side effects include dizziness, somnolence, ataxia, and headaches, but these are usually seen at the higher dose levels.

Efficacy Of Peripheral Nerve Block

By interrupting neural input into the CNS, peripheral nerve blocks may also minimize the surgical stress response after surgery. For instance, patients receiving regional anesthesia (interscalene block) for shoulder surgery have significantly lower serum epinephrine levels in the immediate postoperative period.122 Unlike those receiving general anesthesia, patients receiving regional anesthesia (retrobulbar block) for cataract surgery do not have a significant increase in catecholamines, glucose, and cortisol.112 112 Peripheral blocks of the sympathetic nervous system (paravertebral and celiac plexus block) may also blunt but not abolish the stress response.112 112 Systemic absorption of local anesthetic is not likely to be responsible for any suppression of the stress response in the immediate postoperative period.112 Intercostal, intraperitoneal, or intrapleural administration of local anesthetics is not effective in attenuating the stress response.122 1521 152 152 152 Although...

Neuropathic Pain

Neuropathic Pain and the Sympathetic Nervous System The sympathetic nervous system has been shown to contribute to the evolution and maintenance of certain chronic pain states through a variety of mechanisms. Numerous studies document sympathetic involvement in protective reflexes during acute and impending pain as well as reorganization and sensitization of spinal cord neuronal connections in response to persistent noxious stimulation, through inflammatory induction of hyperalgesia and in association with visceral pain. Obviously, many pain syndromes are sympathetically mediated, either partially or primarily, yet are not considered to be sympathetically maintained. The taxonomy is continually evolving. Fig. 27-2 illustrates the medical protocols associated with neuropathic pain. The development of CRPS can be expected in 5 of all trauma cases.11 Burning, aching pain, initially localized and then spreading proximally cold sensitivity and allodynia characterize the clinical syndrome....

Peripheral Nerve

The reported incidence of peripheral nerve injury is about 1 in every 1000 anaesthetics. Poor positioning is a common underlying factor. The brachial plexus and superficial nerves of the limbs (ulnar, radial and common peroneal) are the most frequently affected nerves. The usual mechanism of injury to superficial nerves is ischaemia from compression of the vasa vasorum by surgical retractors, leg stirrups or contact with other equipment. Nerve injury can be part of a compartment syndrome of a limb after ischaemia from poor positioning. Ischaemic injury is more likely to occur during periods of poor peripheral perfusion associated with hypotension or hypothermia. Nerves can also be injured by traction, e.g. the brachial plexus during excessive shoulder abduction. Needlestick or chemical injury can also occur during regional anaesthesia. Meticulous care is necessary when positioning the patient. Padding should be used beneath tourniquets and to protect pressure points. Extreme joint...

Diabetic Neuropathy

All components of the peripheral nervous system are susceptible, but nerves in the feet are often the first structures to signal the impact of DM. Typically, onset of symptoms occurs in the evening or at night, suggesting nerve dysfunction because capillary peripheral perfusion diminishes with reclining or sleeping. Paresthesias are perceived as numbness, burning, or pins and needles in the toes or the bottom of the foot. Massaging the foot, pushing against a bed board, or walking may help as the microcirculation improves. Remarkably, some patients demonstrate these classic symptoms of diabetic neuropathy even before hyperglycemia occurs in the blood.

Intrinsic Risk Factors

Intrinsic risk factors for pressure ulcer development include age, conditions that impair mobility, malnutrition, and sensory impairment. Skin changes associated with aging (e.g., epidermal thinning, diminished vascularity) increase the susceptibility of older persons to shearing forces, pressure, and friction. Immobility can cause infrequent position changes, thus exposing an older person to prolonged pressure. Malnutrition, specifically an inadequate intake of calories or protein, has been associated with the development of pressure sores (Thomas, 2001). AHCPR (1994) defines clinically significant malnutrition as a serum albumin level of less than 3.5 mg dL, a total lymphocyte count of less than 1800 cells mm3, or body weight less than 80 of ideal weight. Supplementation of micronutrients involved in skin healing, such as ascorbic acid and zinc, has not been shown to prevent pressure sores or improve rates of healing. Sensory impairment, such as in diabetic neuropathy, can prevent...

Pathologic Features

Malignant melanomas present a significant challenge to the surgical neuropathologist because they can show extreme variation in both morphologic and immunohistochemical features. Some tumors can easily be mistaken for glioblastoma, meta-static carcinoma, or even malignant peripheral nerve sheath tumor.40 Histopathologic features other than location and multifocality cannot distinguish primary from metastatic melanomas. The only reliable method for the diagnosis of a primary melanoma is to exclude conclusively a primary site elsewhere in the body. In large series of melanomas, 60 to 80 of tumors occur as solitary lesions, whereas the remainder occur as multiple lesions at diagnosis.9

Associated Neurological Findings

Motor power can be assessed and the distribution of any weakness found noted, such as the distal symmetrical weakness of peripheral neuropathy, which aids in localization of the deficit found on sensory examination. The examiner should look for atrophy of muscles and fasciculations that might suggest a peripheral nervous system process. Muscle tone should be assessed. Deep tendon reflexes are extremely important in the assessment of patients with sensory complaints because they are frequently abnormal in the patient with a lesion in the sensory axis. One should look for the antalgic gait of a painful limb, such as in lumbosacral radiculopathy, or the slapping gait associated with a proprioceptive deficit. Ataxia or pseudoathetosis of the extremities may be associated with a proprioceptive loss in the affected limb. Associated Medical Findings. A good general medical examination of all systems is necessary to aid in generating a differential diagnosis...

Reviews And Selected Updates

McDonald WI Physiological consequences of demyelination. In Sumner A (ed) The Physiology of Peripheral Nerve Disease. Philadelphia, W. B. Saunders, 1980, pp 265-286. Ochoa JL Positive sensory symptoms in neuropathy Mechanisms and aspects of treatment. In Asbury AK, Thomas PK (eds) Peripheral Nerve Disorders, Vol 2. Oxford, Butterworth Heinemann, 1995, pp 44-58. Olesen LL, Jensen TS Prevention and management of drug-induced peripheral neuropathy. Drug Safety 1991 6 302-314. Thomas PK, Griffin JW Neuropathies predominantly affecting sensory or motor function. In Asbury AK, Thomas PK (eds) Peripheral Nerve Disorders, Vol 2. Oxford, Butterworth Heinemann, 1995, pp 59-94.

History And Definitions

Pain is the consequence of stimulation of pain-sensitive structures and is often an early sign of disease or tissue damage. When pain is the result of physiological activity in the normal pain receptors, and there is no primary dysfunction of the nervous system, it is called nociceptive pain. Nociceptive pain may indicate a disorder in any other system or organ, and its diagnosis and treatment involve different medical specialties. Pain resulting from the dysfunction of the central or peripheral nervous system is called neuropathic pain, and its treatment usually involves the neurological team. Neuropathic pain actually involves different kinds of pain and reflects different pathophysiological mechanisms (see later discussion).

Baseline Fetal Heart Rate

Normal baseline FHR ranges from 110 to 160 beats min. A baseline change is interpreted as one that persists for 10 minutes or more and occurs between or in the absence of contractions. An FHR of less than 110 beats min is considered bradycardia. FHR is a function of the autonomic nervous system. The vagus nerve provides an inhibitory affect, whereas the sympathetic nervous system provides an excitatory influence. As the gestation advances, the vagal system gains dominance, resulting in a gradual decrease in the baseline. Stressful events such as hypoxia, uterine contractions, and head compression evoke a baroreceptor reflex, with resulting peripheral vasoconstriction and hypertension causing bradycardia. Stimulation of peripheral nerve receptors can cause acceleration of FHR (Fig. 21-10). An FHR baseline greater than 160 beats min is defined as tachycardia. This is

Thomas W Gould and Ronald W Oppenheim

One of the most counter-intuitive events during the normal development of the nervous system is the massive loss of neurons that characterizes virtually all populations in the central and peripheral nervous system (CNS and PNS) (Oppenheim, 1991 Pettmann and Henderson, 1998 Oppenheim and Johnson, 2003) counter-intuitive, because as a general rule, development is a progressive process whereby new cells, tissues and organs are gradually built-up over time, whereas cell death is a prototypical regressive process. Although precise numbers are not available, as adults we are in the seemingly unenviable position of having many fewer nerve cells (several millions less ) than were present during fetal and early postnatal development. Since most of this loss occurs prior to birth,1 it cannot be attributed to aging, pathology or other life history events such as puberty, although, as we discuss below, pathologic neuronal loss by contrast can occur at virtually any stage in the life cycle....

Abnormal Findings and Clinical Uses of the SEP

Somatosensory evoked potentials are helpful in detecting and localizing lesions of the somatosensory pathways within the CNS but provide no indication about the nature of the underlying pathological processes. They are of little value in evaluating the peripheral nervous system, except when there is concern about the functional integrity of nerves

Nerve Selection and Techniques

Biopsy of peripheral nerve is usually completed either as a full-thickness nerve biopsy (i.e., a complete transection of the nerve to remove a segment), or as a fascicular biopsy (i.e., a longitudinal dissection of the nerve to remove segments of only one or several fascicles), sparing at least a portion of the nerve. Full-thickness nerve biopsy is considered technically easier to perform and is preferable when the pathological evaluation should include both nerve fibers and surrounding connective tissue and vascular structures. Fascicular nerve biopsy usually produces a smaller deficit and is favored when larger nerves are biopsied. The choice of peripheral nerve biopsy site is limited by the potential deficits arising from nerve transection. The most common sites are the sural nerve or the superficial peroneal nerve, both subcutaneous sensory nerves in the lower extremity. In occasional cases, the superficial radial nerve in the upper extremity or the greater auricular nerve is also...

Neurological Symptoms

The OLF at the lower thoracic spine causes various neurological symptoms, sometimes mimicking those caused by lumbar spinal disease, motor neuron disease, or peripheral neuropathy because the epiconus, conus, and cauda equina are located at the lower thoracic and thoracolumbar levels and because their localization often varies among individuals (Fig. 3) 11 . For example, OLF at the lower thoracic spine from T10 to T12 usually compresses the epiconus, which consists of spinal cord segments L4-S2, causing the epiconus syndrome. For example, patients with OLF at T11-T12 may have muscle weakness and sometimes atrophy of the quadriceps muscles, anterior tibial muscles, and gastrocnemius muscles (Fig. 4). Although the pattern of abnormalities of the deep tendon reflexes differs among patients, the patellar tendon reflex (PTR) is frequently normal or diminished, whereas the Achilles tendon reflex (ATR) is exaggerated. Babinski's reflex may be present. Sensory disturbances are often observed...

Metachromatic Leukodystrophies

The metachromatic leukodystrophies (see TabJe.,.30-6 ), also known as sulfatide lipidoses, are a group of lysosomal storage disorders recognized by the accumulation of excessive amounts of sulfatide. The term metachromatic, as a description of the diseases, derives from the staining properties of the stored lipid sulfatides, which develop a brown or gold hue with toluidine blue rather than the usual blue of myelin. The enzymatic defect involves arylsulfatase-A or cerebroside sulfatase-A. A heat-stable nonenzymatic protein activator is also necessary for the hydrolysis of the sulfatide. The sulfatide is stored in lysosomes of neuronal white matter as well as in other somatic tissues, giving rise not only to signs of the disease in the central and peripheral nervous systems but also to disease in other organs and tissues such as the kidneys, pancreas, adrenal glands, liver, and gallbladder. y A peripheral neuropathy may exist even in the early stages of this disease when absence of...

Regional Anesthetic Techniques

Both peripheral and neuraxial blocks avoid the systemic effects of opioids. In contradistinction to neuraxial blocks, hypotension and interference with bowel and bowel functions are not consequences of peripheral nerve blocks. Also, the sensory and motor blockades are unilateral. Ambulation, early mobilization, and assessment of limb function are easier to achieve with peripheral nerve blocks. Several regional anesthesia techniques can be done in the emergency room. These techniques can decrease the amount of analgesics required by the patient. Infiltration of an intermediate or long-acting local anesthetic into a wound can give pain relief that lasts several hours. Suturing of lacerations can be performed under infiltration local anesthesia. A field block extends the area of anesthesia to a wider area the addition of epinephrine to the local anesthetic prolongs the duration of anesthesia. The irrigation of wounds with local anesthetic solutions can be performed with a catheter left...

Continuous Techniques

Continuous peripheral nerve blocks allow the maintenance of analgesia either by repeated injections or by continuous infusion of local anesthetics. Most of the studies on the use of this technique are in the postoperative setting rather than in the setting of trauma. The advantages of this technique in trauma, however, are quite obvious. With a catheter in place, the duration of the analgesia or anesthesia can be extended, and the concentration of the local anesthetic can be changed to provide sympathetic block, sensory block, or motor block with muscle relaxation, as desired.

Reflex Sympathetic Dystrophy

Sympathetically maintained pain, usually of the hand or foot, has been associated with stroke, MS, SCI, radiculopathies, and peripheral neuropathies. Trauma to bone, soft tissue, a major nerve or a plexus, and immobilization are other predisposing causes. Approximately 1 of every 2000 traumatic injuries leads to chronic reflex sympathetic dystrophy (RSD), also called complex regional pain syndrome type 1 (CRPS).88 Following stroke, the incidence is less than 2 for patients who are closely monitored during their rehabilitations.89 Symptoms include poorly localized burning, boring pain, hyper-esthesia, mechanical or thermal allodynia, patchy bone demineralization, and autonomic dysfunction with hyperhydrosis, abnormal skin color, intermittent warm and cold sensations, increased nail and hair growth, and edema. Atrophy is a late feature. Weakness, tremor, muscle spasms, and dystonia may precede the pain or occur on the side contralateral to the pain. After a peripheral nerve injury, the...

Overview Of Management

For postherpetic neuralgia, clinical trials show that lidocaine patches, gabapentin, and tricyclics work best. For HIV-associated distal sensory neuropathy, tricyclics and lamotrigine were shown in controlled trials not to be useful. Unfortunately, the efficacy of the drugs used to treat neuropathic pain is low. The clinician will have to treat from 3 to 6 patients with any one drug to see a partial benefit in 1

Tim Spencer Marco Domeniconi and Marie T Filbin

The adult mammalian central nervous system (CNS) is one of the most sophisticated and intricate structures found in nature. It is also, however, one of the most delicate. Injuries to the mammalian brain or spinal cord often result in a loss of sensory and or motor function which is invariably considered to be untreatable. The ancient Egyptians were the first to recognize this fact and, since then, doctors and scientists have sought to understand why, following injury, neurons of the mammalian CNS fail to regrow cut or damaged axons whereas their peripheral nervous system (PNS) counterparts often do. Research conducted in many laboratories over the past 25 years, have resulted in the elucidation of some of the causes and mechanisms of this regenerative failure and, indeed, have also suggested potential targets for therapeutic intervention thus indicating that these ailments may, in fact, not be untreatable. Why do injured CNS neurons fail to regenerate Do these neurons lose their...

Nerve Sheath Tumors Of The Spine

Nerve sheath tumors of the spine are relatively common entities. Except in instances of rare malignant tumors, these lesions have a favorable prognosis. They arise from the supportive tissue of the peripheral nerves that begin just beyond the Obersteiner-Redlich zone that demarcates the transition from the central nervous system (CNS) to the peripheral nervous system (PNS).8 These tumors may be intradural and extra-medullary in location, may extend with the nerve root through the neural foramen to form a transdural dumbbell tumor, or may be purely extradural. Nerve sheath tumors consist predominantly of two benign lesions, the schwannoma and the neurofibroma, and the more rare malignant peripheral nerve sheath tumor (MPNST). The prognosis for benign nerve sheath tumors is favorable. The main therapeutic challenge for benign nerve sheath tumors is preservation of the involved nerve while attempting to achieve complete resection. MPNSTs follow an aggressive course and have a poor...

Nerve Sheath Tumors Neurofibroma

Neurofibromas are benign lesions that arise from the connective tissue elements of peripheral nerves. They are found throughout the peripheral nervous system, involving the spinal nerve roots in 2 of cases.10 They occur primarily in patients with NF1 but may occasionally occur sporadically. They are globular lesions surrounded by a loose capsule and contain a mixture of Schwann cells, perineural-like cells, fibroblasts, and various intermediates embedded in an extracellular matrix.20 Unlike schwannomas, neurofibromas often ensheathe or incorporate nerve fascicles within their capsule, making their dissection surgically challenging. When present in the spine, they typically arise from the sensory nerve root. They grow at variable rates, often develop during adolescence, may progress during pregnancy, and affect nearly all adults with NF1 at

Adequacy of muscle relaxation

Clinical signs of return of muscle tone include retraction of the wound edges during abdominal operations and abdominal muscle, diaphragmatic or facial movement. An increase in airway pressure (with a time- or volume-cycled ventilator) may indicate a return of muscle tone. Quantitative estimation of neuromuscular status may be obtained with a peripheral nerve stimulator (see Ch. 38). Small increments (e.g. 25-35 of the original dose) of muscle relaxant may be given to maintain relaxation alternatively, an i.v. infusion may be a more convenient method of administration, but the use of a peripheral nerve stimulator is mandatory with this technique.

Indications for Surgery

Because high parathormone (PTH) levels may worsen many disorders associated with chronic renal failure (CRF), other potential indications include hypertension, anemia, deranged myocardial function, and peripheral neuropathy.5 High intact PTH (iPTH) (1-84) levels and a proven highturnover bone disease are prerequisites for considering a CRF patient with SHPT for surgery.58 Calciphylaxis is an absolute indication for immediate PTX.5 9

Intraspinal Drug Therapy

The decision to start intraspinal drug therapy is based on pain location, pain mechanism, and life expectancy. In contrast to neurolysis, intraspinal drug therapy may be effective in controlling more generalized pain. This is because the drug diffuses throughout the intrathecal space to reach receptors located at multiple levels of the spinal cord. As discussed earlier, neurolysis is less effective for neuropathic pain. Therefore, intraspinal drug therapy may be more effective in these cases. The location of the pain influences the technique of delivery. Pain that is localized and unilateral is often better managed with the epidural delivery of opioid and local anesthetic combinations. However, if the life expectancy is longer than 3 months, epidural delivery may be more costly and less effective over time because of development of epidural fibrosis. Pain that is more diffuse is better managed with intrathecal drug delivery. Because neuropathic pain is often less responsive to the...

Joel M Levine and Lorne M Mendell

Developing neurons in both the central and peripheral nervous system face many challenges. They must migrate to their proper location, extend an axon towards their correct post-synaptic targets and survive a period of naturally occurring cell death. The neurotrophic hypothesis, a basic tenet of developmental neurobiology, states that as developing neurons extend axons to their target tissues, their survival becomes dependent on soluble factors released from the target tissues in limiting amounts. After injuries that sever axons, the damaged neurons face many of the same problems as developing axons, that is they must survive, extend an axon, and if function is to be restored, make synapses with correct target cells. Consequently, it has often been suggested that supplying exogenous neurotrophins (NTs) might have therapeutic value after traumatic injury to the nervous system. In this chapter, we review the biology of NTs and their receptors with an emphasis on their use to encourage...

Vitamins and Minerals

While there's nothing wrong with taking a daily multivitamin, megadoses aren't a good idea because their long-term safety remains in question. Over the years, various supplements have been recommended for an assortment of ailments. B-vitamins, for instance, were used to treat diabetic neuropathy, but their beneficial role was never proven and they're not recommended as a therapeutic option. Although chromium piccolinate was reported to have a good effect on blood-sugar control, it was never conclusively demonstrated. One antioxidant, alphalipoic acid, is a prescription drug in Germany that has been used to treat diabetic neuropathy. Although studies are underway in the United States, results will not be available for years.

Elliot S Krames Francis S Pecoraro

Galen (c. 129 ad-c. 200 ad), a Greek physician who lived in Rome, believed strongly in the link between mind and body. Through a series of physiologic experiments, he was able to elucidate a connection between the peripheral nervous system and the central nervous system. The understanding of this connection was helpful in turning the treatment of pain from external or nonmedical therapy, such as physical manipulation or spiritual remedies, to more internal medical management.121 These early internal remedies came mostly in the form of herbal concoctions for the relief of pain. Since then, our understanding of pain and the remedies for its relief has developed significantly. Melzack's and Wall's gate control theory3 led the way for a less empiric and more scientific approach to treating pain. In 1965, it was known that large-diameter nerve fibers inhibit nervous transmission and that small-diameter nerve fibers encourage nervous transmission. Melzack and Wall hypothesized that...

Dermatomal Distribution

The dermatomal innervation is from multiple peripheral nerves that carry the same spinal levels (Figure 29-5C). For example, the spinal root of C6 provides cutaneous innervation to the lateral part of the forearm and hand. In other words, all sensory neurons originating in this region will terminate at the C6 spinal nerve level (Figure 29-5D).

Clinical Presentation

Extrapolating data from previous studies50,69 reveals that the annual incidence rate for peripheral nerve tumors that are operated on is approximately 6 cases per 1 million people, for a total of several thousand cases in the United States. The great majority, more than 90 , of peripheral nerve tumors are benign. They can arise from any of the cellular components that make up a peripheral nerve. The 2000 World Health Organization (WHO) classification of tumors subdivides benign peripheral nerve tumors into three major types on the basis of their clinical course, histologic appearance, immunophenotypic features, and molecular or cytogenetic profile schwannoma, neurofibroma, and perineurioma.13,43,72 Forty-five percent of all peripheral nerve tumors occur in the head and neck region.125 Peripheral nerve tumors arising in the limbs are relatively rare. It has been reported that these tumors account for less than 5 of all neoplasms found in the upper extremities.76,115 The most common...

Diagnostic Evaluation Clinical Examination

Peripheral nerve tumors are not common and are usually found incidentally during evaluation of a soft tissue mass. The most common indications are new detection or growth of a mass associated with neurologic symptoms such as tingling, pain, paresthesias, numbness, or weakness. To successfully manage these lesions, the clinical evaluation should start with a thorough clinical history and physical examination. A positive family history can help diagnose one of the genetic syndromes associated with peripheral nerve tumors, such as neurofibromatosis types 1 (NF1) and 2 (NF2). Other clinical manifestations of these genetic syndromes should be looked for, such as superficial skin masses, cafe-au-lait spots, axillary or inguinal freckling, Lisch nodules (melanotic pigmented hamartomas of the iris), and bone abnormalities in the case of NF1. Physical characteristics such as skin color, texture, and temperature dif ferences, as well as the presence of pain or tingling dysesthesias upon...

Radiologic Imaging Studies

Radiologic studies have become increasingly more important in the preoperative evaluation of peripheral nerve mass lesions. Imaging tools such as computed tomography (CT), ultra-sonography, and especially magnetic resonance imaging (MRI) are widely used. CT imaging, especially following contrast administration, can visualize abnormal masses such as tumors.98,128 Ultrasound is also capable of visualizing masses and is particularly good for distinguishing cystic from solid masses, as well as for resolving vascular structures with blood flow.10,57,71,83 MRI is particularly useful because of its higher spatial resolution and its ability to visualize and distinguish more subtle differences in soft-tissue structures. These charac teristics permit MRI to visualize and distinguish the typical nerve fascicular anatomy from most mass lesions, such as tumors and cysts. Magnetic resonance neurography (MRN) techniques, employing phased-array coils in combination with particular pulse sequences and...

Clinical Presentation and Evaluation

Schwannomas of all types are among the most common type of peripheral nerve tumor in adults. Most patients with schwannomas are between the ages of 20 and 50, with only 10 of schwannomas found in those younger than 21 years. Both sexes are equally affected.3,20,27,48 In the United States, the exact prevalence of benign schwannomas is unknown. However, based on a review of 3364 cases of neurogenic neoplasms of the intercostal nerves and posterior mediastinum in 1971, 141 cases (4.2 ) were schwannomas. The frequency of this tumor was ranked third, following neurofibromas and ganglioneuro-mas.55 Overall, schwannomas represent 35 of peripheral nerve tumors, 35 of all head and neck tumors,125 29 of primary spinal tumors, and 8 of intracranial tumors.120 There is a tendency for schwannomas to arise from sensory nerves.93,120 They may sporadically involve the eighth cranial nerve (as in acoustic schwannomas) at the cerebellopontine angle, dorsal roots arising from the spinal cord, and...

Treatment and Prognosis

Of cases.3 The clinical outcome following complete resection of a solitary neurofibroma associated with NF1 is satisfactory or better in 68 of cases.62 The clinical results following resection of a plexiform neurofibroma associated with NF1 are relatively poor because complete resection, especially of a large lesion involving a functionally important nerve, is rarely possible, whereas sparing function and recurrence is more common.3,48,62 Therefore surgical resection of plexiform neu-rofibromas involving functionally important nerves is usually partial and often results in some loss of neurologic function.76 To minimize postoperative functional deficits, surgical resection of neurofibromas, especially of the plexiform type, should be performed carefully by peripheral nerve surgeons with the aid of intraoperative electrophysiologic monitoring.31,109110 In contrast to neurofibromas, schwannomas can more easily be completely resected with little if any functional loss caused by...

Neurofibromatosis Syndromes and Association with Tumor Types

Autosomal dominant disorders that can arise from either familial inheritance ( 50 ) or spontaneous mutation ( 50 ). They can affect any peripheral nerve throughout the body. Most tumors caused by neurofibromatosis are benign. Neurofibromas, especially the plexiform type, are predominantly associated with nF type 1, or von Recklinghausen's disease, whereas schwannomas of the spinal and cranial nerve roots, especially of the vestibular cranial nerve, are often associated with NF type 2.3,51 Schwannomas can also be associated with NF1, but they are much less common than neurofibromas. The diagnostic criteria of NF1 and NF2 are based on the National Institutes of Health (NIH) Consensus Development Conference on Neurofibromatosis in 1987 (see Although aspects of this Consensus Statement have been superceded in view of continued research, these criteria are primarily valid and valuable in the diagnostic evaluation for NF.

Neurofibromatosis 1

Neurofibromatosis type 1 showing numerous skin cafe-au-lait spots and pigmented neurofibromas. (From Stout AP Tumors of the peripheral nervous system. In National Research Council Committee on Pathology (eds) Atlas of Tumor Pathology, Section II, Fascicle, 6, Washington DC, Armed Forces Institute of Pathology, 1949.) neurofibromatosis type 1 showing numerous skin cafe-au-lait spots and pigmented neurofibromas. (From Stout AP Tumors of the peripheral nervous system. In National Research Council Committee on Pathology (eds) Atlas of Tumor Pathology, Section II, Fascicle, 6, Washington DC, Armed Forces Institute of Pathology, 1949.)

Neurofibromatosis 2

Molecular Genetics and Clinical Presentation. Neurofibromatosis type 2, also known as bilateral acoustic or central neurofibromatosis, is less common than NF1. NF2 occurs in 1 out of every 40,000 to 100,000 live births.351 Isolated in 1993, the NF2 gene is located on chromosome 22 (22q12.2 locus) and codes for a protein called merlin, or schwannomin.17-18,48 Merlin, or schwannomin, is expressed in fetal brain, kidney, lung, breast, and ovary. Its function is unclear but possibly involved with membrane stabilization of a cytoskeleton-associated protein and intercellular communication. The loss of merlin may lead to the elimination of intercellular contact inhibition, which could lead to tumor development.102 In 10 of cases, NF2 patients become symptomatic before the age of 10. Fifty percent of NF2 patients become symptomatic by age 30 and 90 by age 50.3 NF2 is characterized by schwannomas of the cranial nerve roots, particularly the vestibular portion of the eighth cranial nerve, as...

Perineurioma Pathology

Perineuriomas have a different pattern grossly, histologically, and ultrastructurally as compared with both neurofibromas and schwannomas (Figure 73-6). They can be found in dermal, subcutaneous, or more deeply situated tissues. In gross appearance, they are usually well circumscribed and may or may not have a hypocellular fibrous pseudocapsule. Perineuriomas are of variable size, ranging from 1 to 20 cm. These tumors comprise only perineural cells. Histologically, these cells do not stain positively with S-100 protein or Leu-7. However, they are immunoreactive to EMA, in contrast to schwannomas and neurofibromas, which, if they do stain, do so only lightly. Expression of low-affinity NGFr has also been documented in some cases of perineuri-omas.105 Perineuriomas are characterized by bland fusiform tumor cells arranged in whorls or interweaving fascicles, or they may show a storiform pattern of tumor growth in which spindle cells radiate from a central point. EM reveals an abundant...

Tumors Arising From Neurons

A group of tumors arises from neurons. These tumors may differ considerably in terms of their histologic appearance, state of differentiation, location, clinical presentation, and recommended treatments. Most of these tumors are benign and therefore often a surgical cure can be achieved. Those that are malignant require adjuvant therapies such as chemotherapy and radiation treatment. Although usually not included in chapters on peripheral nerve tumors, these tumors are described briefly in this chapter for the sake of being comprehensive.

Metastatic and Other Miscellaneous Tumors

True metastatic tumors involving peripheral nerves are most commonly seen in patients with breast (24 ) and pulmonary carcinoma (19 ). They may also be seen in the clinical setting of lymphoma, bladder cancer, and melanoma.61 Breast and pulmonary carcinomas often affect the brachial plexus through direct invasion and infiltration of peripheral nerves.73 Apical pulmonary tumors, characteristic of Pancoast's syndrome, often involve the lower spinal nerve roots and trunks of the brachial plexus.74,133 Other tumors such as pelvic tumors often infiltrate the lumbar and sacral plexus, whereas head and neck tumors often invade the cranial nerves.6 Definitive diagnosis of these carcinomas requires multiple biopsy samples. Invasion by carcinomas can appear as a mass on CT or MRI. These tumors can cause severe neuropathic pain that often precedes weakness or sensory loss. Metastatic tumors involving the brachial plexus or other peripheral nerve elements are often approached surgically with the...

Variants of Guillain Barr Syndrome

Any number of clinical syndromes have been attributed to variants of acute inflammatory demyelinating polyneuropathy. The most robust candidates are the Miller Fisher syndrome and the pharyngealcervical-brachial variant. The Miller Fisher syndrome consists of the triad of ataxia, ophthalmoplegia and areflexia, without weakness.34 Ventilatory support is not usually required but typical Miller Fisher patients may evolve into acute inflammatory demyelinating polyneuropathy syndromes and thus lead to respiratory failure. The presence of electrophysiological evidence of a demyelinating peripheral neuropathy in Miller Fisher syndrome supports the association with acute inflammatory demyelinating polyneuropathy. So too does the fact that such patients often have the antiGMQ1b ganglioside antibody29 that is also found in the 5 of patients with otherwise classic acute inflammatory demyelinating polyneuropathy who have an opthalmoplegia. In the pharyngeal-cervical-brachial variant, leg reflexes...

Radiation Induced Neuropathy Plexopathy

Peripheral nerves are relatively resistant to radiation. However, a slowly progressive neuropathy may develop locally many years after radiation therapy. It is often a diagnosis of exclusion, and therefore it is important to rule out the possibility of residual or recurrent tumor.1718 On MRI, the involved peripheral nerves usually have a smaller than normal diameter and increased signal on T2 and short tau inversion recovery (STIR) pulse sequences (Figure 73-8). Rarely, the involved nerves can have an enlarged appearance, though in a more uniform pattern than the irregular enlargements seen with peripheral nerve tumors and other mass lesions. Radiation-induced neuropathy or plexopathy involving the brachial plexus, often a complication following radiation therapy to the axillary and supraclav-icular regions, has been most commonly reported.8,86 The incidence of radiation-induced plexopathy is usually related to the total dose and to the dose per fraction.97,119 The clinical features...

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), also known as chronic relapsing polyneuropathy, is an immune-mediated inflammatory neuropathy. It is a rare autoimmune disorder in which there is a swelling of nerve roots and destruction of the myelin sheath over the nerves. Histologically, the damaged nerve has a well-developed onion bulb-like morphology.7 Clinically, CIDP is characterized by slowly progressive weakness and sensory loss involving the legs and arms. Although it can occur at any age and in both sexes, CIDP is more common in young adult males. Symptoms include tingling or numbness beginning in the toes and fingers, weakness of the arms and legs, aching pain in the involved muscles, loss of deep tendon reflexes, fatigue, and abnormal sensations.14 To diagnose CIDP, a peripheral nerve biopsy is sometimes performed. Treatment consists of a course of intravenous immunoglobulin (IV Ig), plasmapheresis, or a corticosteroid such as prednisone, which is typical...

Triggered Electromyography

A peripheral nerve lesion involving motor fibers can be monitored while recording EMG activity from innervated muscles (Figure 73-10). Visible or recordable muscle contraction can be observed when motor nerves entering the proximal or distal pole of a lesion are electrically stimulated. The surface of a tumor can then be mapped delivering stimulation with a fine-tipped monopolar electrode and recording of compound motor action potentials (CMAPs) from target muscles to identify motor axons such that they can be avoided as the surgeon begins to dissect and remove the tumor. Direct electrical stimulation of the peripheral nerve lesion or mass with the use of a fine-tipped monopolar electrode permits the precise localization and mapping of the distribution of functioning nerve fibers within a mass lesion site. Direct electrical stimulation is highly advantageous in the resection of benign peripheral nerve tumors such as schwannomas, which tend to displace neighboring functional nerve...

Nomenclature And Classification

The classification scheme of malignant peripheral nerve tumors in this chapter will be based on the World Health Organization (WHO) classification.52 As defined in the WHO system, malignant peripheral nerve sheath tumors (MPNST) are malignant tumors arising from a peripheral nerve or showing a nerve sheath differentiation, with the exception of tumors originating from epineurium or the peripheral nerve vasculature. In addition to their rarity, further confusion and hence uncertainty about the management of malignant peripheral nerve tumors stems from the diagnostic difficulties in differentiating these tumors from the larger group of other soft-tissue sarcomas. This has led to a large number of terms used to describe these tumors. These include malignant schwannoma, MPNST, malignant neurilemoma, and neurofibrosarcoma, all implying a known cell or structure of origin, which is an unresolved issue. The terms neurogenic sarcoma and MPNST are the most widely used. In this chapter we will...

Secondary Malignant Nonneural Tumors

Peripheral nerves may be secondarily compressed by adjacent tumors or their metastasis to lymph nodes. Rarely, there may be direct longitudinal invasion of epineurium of the nerve by a carcinoma or sarcoma, resulting in pain or neurologic symptoms. Furthermore, there are a few reports of isolated primary neurolymphomatosis.39 Pulmonary or breast carcinomas are the most common secondary tumors to involve the brachial plexus.12,28-30,34 In women, breast carcinomas, either directly or by involvement of the lymphatics, should be suspected with an infraclavicular brachial plexopathy. A relatively common scenario is someone with brachial plexus pain and progressive Peripheral nerve (i.e., brachial plexus) surgery is warranted to make a diagnosis, if the diagnosis has not been determined, and is also warranted for treating secondary malignancies as well as intractable pain or rapidly worsening neurologic deficts that are unresponsive to radiation therapy or chemotherapy. Diagnostic...

Mary Bartlett Bunge and Patrick M Wood

The goal of this chapter is to provide an overview of the efficacy of Schwann cell (SC) and olfactory ensheathing cell (OEC) transplantation to repair the central nervous system (CNS). A transplanted bridge of cells to span the site of injury is a promising strategy to provide a permissive scaffold for axonal growth (reviewed in Bunge, 2001 Geller and Fawcett, 2002). Both cell types have been shown to be effective both cell types offer advantages. SCs may be easily extricated from peripheral nerve and placed into culture to generate far larger numbers than OECs they effectively myelinate regenerated fibers or remyelinate denuded axons in vivo. Because they do not invade astrocyte territory, they are not as migratory as OECs and additional strategies are needed to lure the regenerated fibers from the SC implant, unlike OECs. OECs are less accessible and are not yet available in large numbers, but they have been demonstrated to be reparative, including improving functional outcome, in...

Synopsis of Neurological Diseases

This synopsis is restricted to neurological diseases affecting the brain. This excludes disturbances affecting peripheral nerves and neural transmission within the spinal cord. Neurological diseases affecting the brain may be distinguished by their class of etiology. The disease may be (1) degenerative, (2) due to failures of blood supply, (3) due to inflammation and problems within the immune system,

Cognitive Functioning

Risk of brain atrophy and infarction exists particularly in those patients with associated coagulation disorders and in those with a history of hypertensive crises. Reports of nonspecific electroencephalo-graphic abnormalities, delayed myelination of the somatosensory cortex, and slower peripheral nerve conduction velocities have also been published. Furthermore, Gipson et al. (2007) pointed out that general cognitive functioning scores are lower than normal among children with CKD and that attention, executive function, and memory are common areas of concern.

Signs And Symptoms

Symptoms associated with the spinal cord dysfunction syndrome include motor or sensory dysfunction. The motor dysfunction can occur insidiously21 in the upper or lower extremities and can include atrophy, hypotonia,25 spasticity, hemiparesis, quadriparesis,5 and hypoactive reflexes.9 Muscle disuse may lead to contractures25 and atrophy.9 Sensory findings can also involve both the upper or lower extremities and can include paresthesias, dysesthesias,21 and neuropathic pain.13 Patients can also have symptoms associated with increased intracranial pressure and hydrocephalus such as headache, nystagmus, and vomiting.25 Nausea and vomiting has also been reported to occur unassociated with increased intracranial pressure.19 Other findings that have been reported in children in association with cervicomedullary tumors include failure to thrive in infants and young children,19 clumsiness, syncope, weight gain, ataxia,25 and scoliosis.12 Cervicomedullary tumors have been reported to simulate...

Neurological examination

Neck pain may be the first clinical symptom of a slowly growing acusticus neurinoma, with absent corneal reflex being the first sign. Patients with referred pain in the region of trigeminus nerve pain commonly present an underlying pathology of the upper cervical spine, often observed in at-lanto-axial instability due to rheumatoid arthritis 38, 42 .

Psychosocial Adjustment

Children living with HIV commonly experience pain (Gaughan et al. 2002 Hirschfeld et al. 1996 Lolekha et al. 2004), including abdominal pain of unclear etiology, myositis, tension headaches, and neuropathic pain that is difficult to manage. Discomfort related to invasive procedures, toxicities and adverse drug reactions, invasive secondary infections, pancreatitis, and erosive esophagitis may be treated pharmacologically. Pain has been found to be associated with more severe immunosuppres-sion and increased likelihood of death (Gaughan et al. 2002). There are no published studies examining the relationship between chronic pain and psychological distress in HIV-infected children. Nevertheless, children fear pain, and pain is made worse by emotional distress, so attention at regular intervals to physical and emotional distress is warranted.

Neurophysiological investigation of the cervical spine

SEPs are generally recorded after electrical stimulation of peripheral nerves or skin. The nerves used are the posterior tibial, sural, or common peroneal nerves of the lower limbs, and the median radial and the ulnar nerve for the upper limbs. In radicular and spinal disease, several nerves, supplied by different segments, must be stimulated for a level diagnosis. SEPs from tibialis nerve are recommended for the diagnosis of cervical myelopathy 41 . A method of painless magnetoelectric transcranial stimulation of the cerebral cortex was introduced in 1985 by Barker et al. 2, 3 . They applied short magnetic pulses, designed to stimulate peripheral nerves, to the scalp, and For motor root stimulation over the cervical and lumbar spine, the intensity of the stimulator is adjusted so that a potential with a steep negative rise can be recorded. With this, the onset latency is not critically dependent on the positioning of the coil or the stimulation strength 6 . The excitation site of the...

Neuromuscular Stimulation

A lift system combined with treadmill stepping offers opportunities to add other elements for locomotor retraining. Investigators could make use of peripheral nerve stimulation to drive one aspect of stepping such as flexion155 (Fig. 1-6) or add a more sophisticated FNS compo-nent.156-158 This combined approach can become expensive and weigh on the expertise required of clinicians. Investigators will need to develop an optimal training paradigm, patient selection criteria, and practical ways to stimulate as few muscles, nerves or cutaneous regions as possible. To prove the efficacy of the combined approach, a clinical trial will have to randomize patients to each component, BW-STT and FNS, as well as to the two in concert. This undertaking ought to await the results of studies that compare BWSTT to conventional treatment after SCI.143 Robotic steppers continue to be developed to aid stepping on a treadmill159 or to move the legs through the step cycle with force feedback from the...

Risk Factors For Neuromuscular Injury Patient Factors

Several risk factors for peripheral nerve injury and rhabdomyolysis related to the patient have been cited, including patient age and body habitus (Table 1). Advanced age may be a risk factor for the development of peripheral neuropathy post-operatively. Wolf et al. found that those patients with motor nerve palsies were the oldest group and postulated a higher prevalence of preexisting subclinical nerve deterioration in the elderly (3).

The definition of a BCI

A BCI is a communication and control system that does not depend in any way on the brain's normal neuromuscular output channels. The user's intent is conveyed by brain signals (such as EEG) rather than by peripheral nerves and muscles, and these brain signals do not depend for their generation on neuromuscular activity. (Thus, e.g., a device that uses visual evoked potentials to determine eye-gaze direction is not a true BCI, for it relies on neuromuscular control of eye position, and simply uses the EEG as a measure of that position.)

The fundamental principle of BCI operation

This dependence on initial and continuing CNS adaptation is present whether the person's intent is accomplished in the normal fashion, that is, through peripheral nerves and muscles, or through an artificial interface, a BCI, that uses brain signals rather than nerves and muscles. BCI use depends on the interaction of two adaptive controllers the user, who must generate brain signals that encode intent and the BCI system, that must translate these signals into commands that accomplish the user's intent. Thus, BCI use is a skill that both user and system must acquire and maintain. The user must encode intent in signal features that the BCI system can measure and the BCI system must measure these features and translate them into device commands. This dependence, both initially and continually, on the adaptation of user to system and system to user is the fundamental principle of BCI

Traumatic Trigeminal Neuralgia

Traumatic trigeminal neuralgia is defined as continuous neuropathic pain following complete or partial peripheral nerve injury in the trigeminal nerve distribution. Treatment with topical therapies such as capsaicin may be of benefit. Neural blockade produces temporary relief only.

Anatomic Classification

The major anatomic patterns of peripheral nerve disease can be distinguished by the clinical presentation mononeu-ropathy, multiple mononeuropathy, and polyneuropathy. Mononeuropathies occur when there is damage to a single peripheral nerve or root. This is seen with local entrapment from causes such as trauma (acute or chronic), tumor infiltration, or infarction. Multiple mononeuropathies (mono-neuropathy multiplex) occur when several nerves are individually affected by a disease process. Involvement is usually asymmetric and noncontiguous. These mononeuropathies are much less common than other peripheral neuropathies and are more difficult to recognize and treat. Multiple mononeuropathies are usually seen with systemic diseases such as vasculitis, diabetes, and rheumatoid arthritis. Poly-neuropathies occur in a symmetric, diffuse, bilateral pattern and produce a characteristic stocking-glove pattern of sensory changes. However, most polyneuropathies affect both sensory and motor...

Meningeal and Ventricular Metastases

Leptomeningeal metastases can develop when malignant cells reach the cerebrospinal fluid (CSF). Tumor cells may enter through leptomeningeal veins, the choroid plexus, y or veins draining the bone marrow of the skull. Malignant cells may grow along cranial or peripheral nerve routes. Rarely, metastases to the parenchyma of brain or spinal cord seed into the CSF. y

Inflammatory Neuropathies

With inflammatory neuropathies, an inflammatory process is directed against the peripheral nerve the most common causes follow. Treatment is usually directed toward the causative agent. Several opportunistic infections of the PNS may result from HIV infection. The HIV itself, cytomegalovirus, and herpes zoster are most common. Painful sensorimotor polyneuropa-thies or demyelinating polyradiculoneuropathies occur during the early and late stages of HIV disease. Symptomatic relief of neuropathic pain may be achieved with antidepressants (e.g., amitriptyline) or anticonvulsants (e.g., carbamazepine). Although uncommon in the United States, leprosy is the most common worldwide treatable cause of neuropathy. Symptoms of neuropathy can occur before the systemic manifestations of disease are present. As leprosy progresses, with its characteristic skin lesions, the risk for peripheral nerve injury increases. Sensory loss of some degree is expected. Treatment is directed toward the disease...

Metabolic Neuropathies

Diabetic peripheral neuropathy has a widely variable presentation but usually is seen as a symmetric polyneuropathy, with predominant sensory signs and mild motor signs. Patients experience burning dysesthesias and pain in the soles of the feet. Impaired position sense leading to ataxia and arthropathy (Charcot's joints) implies the involvement of large, myelinated, sensory fibers. Patients with diabetic neuropathy experience bilateral symptoms that include burning pain in the back and thigh with proximal muscle weakness, decreased patellar DTRs, and normal sensory function. This is thought to be caused by microvascular ischemia of the proximal motor trunks. Diabetes is also associated with autonomic neuropathies. Symptoms may include postural hypotension, gastropare-sis, intestinal dysmotility, atonic bladder, impotence, and loss of pain fibers in the cardiac sympathetic system, permitting silent MI. Patients with chronic renal disease develop a...

Neuromedical Complications

Many neurologic, general medical, and orthopedic derangements can complicate and interfere with the early and late rehabilitation of patients after a moderate to severe head injury. Traumatic brain injury is often accompanied by injuries to bone, soft tissues, viscera, and to the spinal cord and peripheral nerves. Deep vein thrombosis has been detected by serial testing in over 50 of patients after major TBI within 3 weeks of onset.43 This incidence rose to over 75 with associated femoral or tibial fractures. In a study of 180 TBI patients admitted for rehabilitation, 56 had neurologic complications, 50 developed gastrointestinal compli

A3 The nervous system

Overall, the nervous system consists of the central nervous system (CNS), comprising the brain and spinal cord, and the peripheral nervous system (PNS), which is composed of nerves extending to and from the CNS. The major constituent systems of the central and peripheral nervous systems, shown in Box A2, are described in the following sections.

A321 Autonomic nervous system

The part of the peripheral nervous system that supplies smooth muscles (in contrast to the striated or skeletal muscles found in our limbs etc.) is termed the autonomic nervous system. The autonomic nervous system also controls the heart, the digestive and urinary systems, and the secreting glands such as sweat and salivary glands. In general, the auto-nomic nervous system is concerned with involuntary nerve impulses. The part of the peripheral nervous system that controls voluntary actions, such as movement, is known as the somatic or motor nervous system. The autonomic nervous system itself is subdivided into

A322 Parasympathetic nervous system

The neurotransmitter at ganglions (synapses), which are junctions between the preganglionic fibres and the post-ganglionic fibres, in both the parasympathetic nervous system and the sympathetic nervous system is acetylcholine (see Figure A4 of the peripheral nervous system). Acetylcholine is also the neurotransmitter at the post-ganglionic nerve endings of the parasympathetic nervous system, and acts as the neurotransmitter at some post-ganglionic nerve endings of the sympathetic nervous system.

Neuropeptide Medicines Still Waiting In The Wings

If one were currently to select a single neuropeptide that has had the most promising and most widely evaluated track record in humans, it would be the first item in the above list (i.e., Org-2766). This peptide emerged gradually from David de Wied's work on memory enhancing ACTH-related peptides (Kovacs and de Wied, 1994). It also proved to have various interesting neuroprotective effects after peripheral nerve injury as well as following damage to certain central systems such as DA pathways (Strand, 1999). Subsequent work on structure activity relations led to localization of activity in the ACTH-(4-10) fragment and to the synthesis of an array of orally active synthetic peptides, the most promising of which was called Organon-2766 This orally available, artificial peptide is about a thousand times as potent as the parent compound (ACTH 4-9), and has now been widely studied as a neuroprotective agent (e.g., van Rijzingen et al., 1996), and it has been reported to promote...

The complex regional pain syndrome Commentary

Complex regional pain syndrome (CRPS) Types I and II are important examples of neuropathic pain, which may affect a wide range of age groups. The condition is seen almost exclusively in the chronic pain management clinics and you may well have little direct experience of its main features and management. Neuropathic pain, however, complicates many disease states, is severe and difficult to treat, and remains incompletely understood. For this reason it continues to appear as a popular examination topic.

Sensation Testing Neural Problems

Problems of the cervical nerve roots or peripheral nerves may affect both muscular strength and sensation of the upper extremity. Major peripheral nerves and each neurological level should be tested. Use a sharp object to touch the areas of the skin supplied by the nerve or nerve root while the athlete looks away.

Relief of chronic pain

Although peripheral nerve blocks are normally performed in the pain clinic, severe intractable pain is sometimes relieved only by dorsal cordotomy or rhizotomy. Both techniques involve upper thoracic laminectomy to expose the spinal cord, with the patient prone as for decompressive surgery. Some patients are extremely frail, and positioning them to avoid pressure sores is particularly important. A number also have an element of autonomic neuropathy, with resultant cardiovascular instability. Neurological ablation produces intense temporary stimulation, and adequate anaesthesia and analgesia are particularly important during the process of nerve section.

Disorders Of The Motor Unit

The rehabilitation of patients with diseases of the anterior horn cell, peripheral nerve, neu-romuscular junction, and muscle depends especially on the temporal course of the specific illness, its natural history, and the distribution of weakness. Approaches to neuromuscular rehabilitation have far more similarities than differences, whether the disease affects primarily muscle or nerve.1,2 These approaches have not changed much over the past 25 years, but better evidence for their safety and efficacy has evolved. The prospects for gene therapies,3 molecular approaches,4 trophic factors,5,6 immune therapies, and approaches to regeneration of muscle7 have raised the hopes of physicians and patients for new interventions and cures for hereditary diseases (see Chapter 2).

Advantages of Multichannel Monitoring

The primary advantage of our multichannel monitoring is that spinal segments at the C5-T1 and L1-S4 levels, which are important to motor functions for activities of daily living, can be monitored simultaneously. In addition, control waveforms can be obtained from the same patient, which is useful for distinguishing actual spinal dysfunction from technical failures. For example, when potentials in the lower extremities diminish during thoracic spine surgery and similar decreases in potential are observed in the upper extremities, effects other than those due to surgical manipulations (primarily the effect of muscle relaxants) are suggested. When potentials from a few muscles diminish that do not correspond to the operated spinal level, technical failures are suspected and the electrodes should be checked from outside the surgical field. Indeed, in one case we found that the patient's arm had fallen off the surgical bed in another case, peripheral nerve compression was suspected and...

Restless Legs Syndrome and Periodic Limb Movement Disorder

RLS and PLMs may be induced or aggravated by a variety of conditions, including iron deficiency, anemia, and chronic renal failure. Peripheral neuropathy may be a factor in some cases, although peripheral nerve function is clinically normal in most affected patients. Symptoms occur in 10 to 20 percent of pregnant women and usually resolve postpartum. Other disorders that may be associated with PLMs or RLS include venous disease, degenerative CNS disorders, and vitamin deficiency. Differential Diagnosis. The diagnosis of RLS is based on the history. The essential elements are the urge to move associated with sensory phenomena in the legs, motor restlessness, and exacerbation at rest and in the evening and night. y The differential diagnosis may include akathisia, peripheral neuropathy, claudication, and leg cramps. With akathisia caused by phenothiazines, there is restlessness of the body and a compulsion to move, but the sensory component is less than with RLS and it is usually not...

Clinical Presentation And Differential Diagnosis

Often, specific features of the clinical history, findings on physical examination, or diagnostic investigations can help to identify the soft-tissue lesion associated with a peripheral nerve and hence a potential peripheral nerve neoplasm. Whereas exact numbers are difficult to determine, estimates suggest that 14 of all pediatric soft-tissue tumors arise from the peripheral nervous system.6 For instance, a family history or clinical stigmata of neurofibromatosis 1 (NF1) or neurofibromatosis 2 (NF2) raises suspicion of neurofibromas or schwannomas, respectively. NF1 and NF2 are autosomal dominant phako-matoses that arise because of germline mutations of chromosomes 17q and 22q, respectively, and are associated with the development of numerous tumors, including PNTs. Further detailed discussion of the diagnostic criteria, phenotype, and specific molecular genetic aberrations of NF1 and NF2 are found elsewhere in this text. Unfortunately, there are no pathognomonic features that permit...

Benign Nerve Sheath Tumors

The classification of peripheral nerve sheath tumors has traditionally been an area of great dispute, and a detailed nosologic discussion of schwannomas, neurofibromas, and perineuriomas is beyond the scope of this chapter. It should suffice to say that the nerve sheath itself consists of three interconnected and tubular compartments the epineurium, perineurium, and endoneurium with perineurial fibroblasts and Schwann cells within the sheath. Schwann cells are derived from neural crest cells, and perineurial fibroblasts are of mesenchymal origin. Although it is known that schwannomas arise from Schwann cells (which produce the peripheral nerve's myelin coating) and perineuriomas from perineurial fibroblast (which gives rise to the epineurium, perineurium, and endoneurium), the histogen-esis of the neurofibroma remains obscure and may be derived Tumors of nerve sheath origin are less common in the pediatric population, but as a group they continue to account for a significant portion...

Sporadic Non Neurofibroma 1 Neurofibromas

Pediatric neurofibromas that occur outside of NF1 are almost exclusively solitary, fusiform lesions and found in association with nerves ranging from small unidentifiable cutaneous nerves, to larger peripheral nerves, spinal nerve roots, and the brachial and lumbosacral plexi. As with schwannomas, a painless, palpable mass is the most common occurrence. However, neurofibromas are more likely to be associated with radicular pain than are other nerve sheath tumors.7 Children with neu-rofibromas may also complain of sensory loss, paresthesias, or weakness in the distribution of the involved nerve. Although demonstrable objective loss of function still occurs in only a minority of patients, it does occur more commonly with neu-rofibromas than with schwannomas.7

Antibiotic Antineoplastic Agents

The existence of pure bleomycin toxicity has not been established, because the drug is ordinarily used in conjunction with other chemotherapeutic agents. Mental status changes and peripheral neuropathy have been reported with bleomycin therapy, although no pathological changes have been associated in brain or peripheral nerves in such patients. In animals, cochlear damage has been demonstrated, although there is no comparable clinical evidence in humans. ii Adriamycin and actinomycin D produce no neurological side effects in humans in the usually prescribed doses, and when administered systemically, the drugs do not enter the CSF. Adriamycin can produce a severe form of myelopathy and encephalopathy after accidental intrathecal injection. Experimentally, it can produce cerebral damage after intracarotid injection, and anterior horn cell loss after direct injection into a peripheral nerve. yi

Peripheral Neuropathy Natural Treatment Options

Peripheral Neuropathy Natural Treatment Options

This guide will help millions of people understand this condition so that they can take control of their lives and make informed decisions. The ebook covers information on a vast number of different types of neuropathy. In addition, it will be a useful resource for their families, caregivers, and health care providers.

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