Altered Sexuality Syndromes

Altered sexuality may take many forms. The loss of libido is the most common form and can present as inhibited excitement or orgasm. Hypoactive sexual desire may be due to a number of psychiatric, medical, and neurological disorders. Typically, the cause is functional and the condition occurs in the presence of anxiety or depression. Hyposexuality associated with hypergraphia, hyper-religiosity, irritability, and elation comprise the main features of the Gastaut-Geschwind syndrome. This...

Aseptic Meningitis

Retrospective studies have suggested that up to 10 percent of patients with HIV-1 infection and AIDS develop clinically apparent aseptic meningitis during the course of their illness. Additionally, HIV has been isolated in blood and CSF of patients with an acute self-limited aseptic meningitis at the time of HIV seroconversion. The clinical features in these cases are similar to those present in patients with aseptic meningitis owing to other viruses, outside of the setting of HIV infection....

Oligodendroglioma and Oligoastrocytoma

Because these tumors arise from oligodendrocytes, they usually occur in the white matter of the cerebrum, and because there are relatively more oligodendrocytes in the frontotemporal area, oligodendrogliomas have a predilection for these areas. Usually surgical specimens contain areas that are identifiable as astrocytoma. Increasing difficulty in the diagnosis of these tumors centers on the percentage of oligodendro-glioma cells needed to categorize these...

Subdural Hematomas

A subdural hematoma (SDH) results from venous bleeding after blunt head trauma, which causes brain motion within the skull, shearing off the bridging veins between the surface of the brain and adjacent dural venous sinuses. The blood leaks and collects slowly, forming a hematoma in the subdural space.y An SDH may be absorbed spontaneously or may form an encapsulated and liquefied hematoma. After about 2 weeks, membranes form around the hematoma. The outer...

Table 162 Ototoxic Medications

Aspirin and sodium salicylate (cochleotoxic, reversible) Chemotherapy (mainly mixed toxicity) including lack of normal gesturing and spontaneous movements. The face is hypomimetic, with lack of expression, and there is reduced rate of blinking. Rest tremor, if present, is elicited when the hands and feet are completely relaxed. In hyperkinesias, key information is obtained by observing the patient at rest in complete repose without talking. Relaxing these patients and finding the best rest...

Trigeminal Neuralgia

Trigeminal neuralgia (TN) probably results from the focal demyelination of the trigeminal nerve or ganglia. y , '57' Symptomatic causes include intrinsic and extrinsic tumors near the gasserian ganglia and multiple sclerosis (MS) plaques located around the root entry zone of the trigeminal nerve. In the past, dental disease or dental procedures were thought to occasionally cause TN. However, the delineation of the syndrome of pretrigeminal neuralgia, which mimics dental illness, casts doubt on...

REM Sleep

The anatomical substrates for the different components of REM sleep are as follows 1. An important substrate is cortical desynchronization. The origin of the mixed frequency activity is the mesencephalic reticular formation. The reticular cells fire about 15 seconds before activation of cortex, and their projections extend to the intralaminar nuclei of the thalamus with widespread projections to cortex. 2. Hippocampal theta activity is highly synchronous activity with a frequency of 5 to 10 Hz,...

Retina

Retinal processing of visual information occurs before its transmission through the optic nerve and remainder of the afferent visual pathway. The cone and rod photoreceptors TABLE 8-1 -- CLINICOANATOMICAL CORRELATION OF DISORDERS OF CRANIAL NERVE II AND AFFERENT Other Neurological and Medical Findings Carotid disease, giant cell arteritis, migraine, vasospasm, cardiac emboli Retinal hemorrhage, cotton wool spots, dilated retinal veins Central serous chorioretinopathy, macular degeneration...

Sex Chromosomal Abnormalities

This syndrome, characterized by a 47XXY chromosomal karyotype, is the most common human sex chromosomal aberration and is associated with the sex chromatin-positive form of seminiferous tubular dysgenesis. The frequency of the 47XXY karyotype is reported to be 0.9 in 1000 0.15 in 1000 have a mosaic form. About half of patients with a 47XXY disorder die in utero. Affected children have small, firm testes, and adult patients have azoospermia. y This disorder is a common cause of primary...

Affective Disorder Syndromes

Major depression has a lifetime prevalence of about 15 percent and is about 15 times as common as bipolar disorder (manic-depressive disorder). Major depression is about twice as common in women as in men in all countries and cultures and does not vary in occurrence among different races. It may occur at any age, but the majority of cases occur in adulthood. Studies suggest a genetic predisposition because there is an increased incidence of major depression and alcoholism in relatives of...

Tension Type Headache

Although many patients with TTHs have muscle tenderness, TTH is not the result of sustained contraction of the pericranial muscles with subsequent ischemic pain in response to emotion or stress. Muscle ischemia is not present during headache. Electromyography activity is increased in some muscles, independent of tenderness and pain. Reduced CNS 5-HT levels may be responsible for abnormal pain modulation, producing the decreased pain thresholds that are observed...

Table 383 Major Causes Of Hypocalcemia

Normal or increased parathyroid function (renal failure, vitamin D deficiency) Protein-binding and anion-chelation of ionized ealeium Medications (anticalcemic and antineoplastic agents) Multifactorial (gram negative sepsis) Modified from Spiegel AM The parathyroid glands, hypercalcemia, and hvpocalcemia. In Bennett JC, Plum F (eds) Cecil Textbook ,of Medicine, 20th ed. Philadelphia, WB. Saunders, 1995, pp 1364-1373 . may be caused by PTH or 1,25-DHCC deficiency, as well as by end-organ...

Strychnine

Excessive and spontaneous release of acetylcholine acetylcholinesterase and pseudoCholinesterase Central analeptic agent GABA antagonist postsynaptic inhibitor Blood low Cholinesterase activity (70 of baseline) Pulmonary and dermal irritant renal effects pulmonary effects cardiac effects GI distress garlic or rotten fish odor Metallic taste thirst burning eyes GI distress excessive sweating and salivation hypothermia liver dysfunction Mania hallucinations delinum headache vertigo cognitive...

Cortical Versus Subcortical Dementing Syndromes

A distinction has been noted between the cortical dementia typically seen in Alzheimer's or Pick's disease and the subcortical dementia typified by Huntington's and Parkinson's disease but noted also in progressive supranuclear palsy, multiple sclerosis, Wilson's disease, and human immunodeficiency virus (HIV) infection. 48 Dementia seen in diseases with primarily subcortical neuropathology feature slowed movement (bradykinesia) and thought (bradyphrenia), disproportionate problems in the...

Eosinophilic Myositis and Related Syndromes

True eosinophilic myositis occurs in the setting of a subacute or chronic unexplained peripheral blood eosinophilia. Patients with hypereosinophilia may be asymptomatic, or may have end- organ damage including myositis. The skeletal muscle in such patients resembles polymyositis with an eosinophil- predominant infiltrate. W Eosinophilic fasciitis is likely a separate condition with eosinophilia, elevated ESR, and hypergammaglobulinemia. Clinically, these...

Headache And Facial Pain Sleep Disorders

DRUG-INDUCED AND IATROGENIC DISORDERS Meningitis encephalitis neuritis, myelitis Acute bacterial meningitis recurrent bacterial meningitis abscess (bacterial, fungal, paracytic) intracranial thrombophlebitis subdural empyema (bacterial, fungal, paracytic) extradural abscess (cranial and spinal epidural) myelitis (Lyme disease) neuritis (Lyme disease) syphilitic dorsal root ganglion disease cyst formation (echinococcus), myopathy (parasites) tick paralysis Sporadic disease familial disease,...

Speech Related Disorders

Miscellaneous speech-related disturbances not classified as dysarthrias or dysphonias include phonic tics, vocalizations, coprolalia, and reiterative speech disorders. PHONIC TICS AND VOCALIZATIONS Phonic tics are either simple or complex vocal tics. Simple vocal tics are similar in character to motor tics and are expressed as inarticulate noises and sounds (throat clearing, grunts, coughs, shouts, snorts, word accentuation). Complex vocal tics include articulate words, phrases, or sentences...

Syndromes of Intracerebral Hemorrhage

There are general features of the clinical syndrome of intracerebral hemorrhage that may help to characterize it. Historical features include a presentation that is maximum at the onset in one third of patients and gradual with smooth progression over 30 minutes in two thirds of patients. Interestingly, most hemorrhages occur during activity rather than during sleep. y , y A headache is present in approximately one half of patients. Nausea and vomiting are present in over 50 percent of...

MHexane and MethylMButyl Ketone

N-hexane is a component of a number of glues. Therefore, exposure is likely to come from inhalant abuse. With acute exposure, N-hexane causes euphoric effects. Pronounced peripheral polyneuropathy may occur after chronic intoxication. Hexane is metabolized to 2,5-hexanedione (2,5-HD), which is responsible for much of the neurotoxicity related to this compound. Unlike toluene, hexane does not produce significant central neurological symptoms. Lightheadedness, headache, decreased appetite, and...

Multiple Myeloma

Multiple myeloma is a hematological disorder characterized by a proliferation of a single clone of plasma cells that produces a specific immunoglobulin. This disorder may affect patients between the ages of 40 and 70, but the peak incidence is in the seventh decade. Multiple myeloma typically causes bone pain, general fatigue, and weakness, and is often accompanied by anemia, chronic renal failure, and hypercalcemia. Over 99 percent of patients will have an...

Epidemiology and Risk Factors HIV1 is transmitted

By either sexual contact, parenteral exposure to blood, blood products or body fluids, or from mother to infant. In the United States, western Europe, Australia, and in some Latin American countries, the majority of AIDS cases were initially among men infected through sex with other men. In the United States, the proportion that this group now represents has declined from approximately 72 percent in 1986 to 53 percent. '37' It is estimated that 75 percent of HIV-1 infection worldwide is the...

Stiff Person Syndrome

Stiff-person syndrome (SPS) is believed to be an idiopathic disorder in some patients and an autoimmune disorder with functional impairment of spinal neurons due to antibodies directed against the enzyme glutamic acid decarboxylase (GAD) in others. y This enzyme is essential for the conversion of glutamic acid to GABA, an inhibitory neurotransmitter found throughout the central nervous system. y In a paraneoplastic form of SPS, amphiphysin, a protein associated...

Vertebrobasilar System

Depressed consciousness, global aphasia Emotional lability, aphasia (left), apraxia (right) Lower altitudinal defect saccade pursuit defects Ipsilateral amaurosis fugax, contralateral HH Transient CN findings-diplopia, dysarthria Retinal emboli, contralateral HH, conjugate eye deviation Transient uni- or bilateral quadriparesis Transient contralateral weakness or clumsiness Transient bilateral weakness or clumsiness Ipsilateral weak palate, Horner's syndrome, decreased p t ipsilateral face...

Autosomal Dominant Cerebellar Ataxia

Spinocerebellar ataxia type I (SCA1) Calcium channel (CAG repeat expansion) Dentatorubral-pallidoluysian atrophy (DRPLA) that reduced frataxin levels are the primary cause of the neurodegeneration and cardiomyopathy characteristic of FRDA. 3 The first pathological changes in FRDA are thought to occur in the dorsal root ganglia with the loss of large sensory neurons. In advanced cases, the neuropathological abnormalities include axonal sensory and motor neuropathies, degeneration of spinal...

Reviews And Selected Updates

Philadelphia, J.B. Lippincott, 1990. Miller NR Walsh and Hoyt's Clinical Neuro-ophthalmology. Vol. 1, 4th ed. Baltimore, Williams & Wilkins, 1985. Zeki S A Vision of the Brain. Oxford, Blackwell Scientific, 1993. 1. Liu GT Disorders of the eyes and eyelids. In Samuels MA, Feske S (eds) The Office Practice of Neurology. New York, Churchill Livingstone, 1996, p 0. 2. Hart WM Acquired dyschromatopsias. Surv Ophthalmol 1987 32 10 3. Tessier-Lavigne M The...

Clinical Uses of Nerve Conduction Studies

Motor conduction studies are helpful in indicating that weakness is due to pathology of the peripheral nerves rather than other parts of the motor unit. Sensory conduction studies may indicate that sensory symptoms are due to an impairment of peripheral nerve function or, when normal, to a lesion proximal to the dorsal root ganglia. Motor and sensory conduction studies are important in determining the presence and extent of a peripheral neuropathy, distinguishing between a polyneuropathy and...

Extracranial Granulomatous Arteritis Giant Cell Temporal Arteritis

Giant cells are the pathological hallmark of this large-vessel, T-cell mediated vasculitis. CD4 cells aggregate with a response centered on the internal elastic lamina. These cells may contain elastic fiber fragments. Giant cell arteritis (GCA) affects the aorta and its branches, including the cervicocephalic arteries (carotid and vertebral), reflecting its predilection for the internal elastic lamina. Intracranial arteries are usually not involved, although...

First Order Neuron

Beyond the mechanoreceptors, the sensory impulses are propagated centrally by a population of afferent nerve fibers. The cross-sectional diameter, degree of myelination, and conduction velocity of peripheral nerve fibers are useful categories that relate directly to the modality subserved by certain mechanoreceptors and nerve fiber populations. The accepted composition of the sensory nerve compound action potential includes Aalphabeta myelinated fibers (30-72 m sec) Adelta lightly myelinated...

References

Jennette JC, Falk RJ Clinical and pathological classification of ANCA-associated vasculitis What are the controversies Clin Exp Immunol 1995 ioi(Suppi i) i8-2 2. Roitt IM Principles of autoimmunity. In Brostoff J, Scadding GK, Male DK, Roitt IM (eds) Clinical Immunology. London, Gower Medical Publishing, 1991, pp 401-' 12. 3. Huston KA, Hunder GG, Lie JT Temporal arteritis. A 25-year epidemiologic, clinical, and pathologic study. Ann Intern Med 1978 88 162-167 4. Turnbull J Temporal arteritis...

Intrastriatal Structure

Within the striatum, there is a large neuronal matrix compartment, comprising approximately 80 of the striatum, and a smaller compartment interdigitated with the matrix and known as striosomes or patches. Both the matrix and striasomal neurons receive inputs from dopamine cells coming from the pars compacta of the substantia nigra. Both cell types send efferent projections to the GP e , and these cells all contain enkephalin. In contrast, however, the matrix population also sends efferents to...

Rocky Mountain Spotted Fever

Rocky Mountain spotted fever is a rickettsial disease caused by Rickettsia rickettsi, a small intracellular parasite. Infection is acquired by a tick bite. The majority of infections are acquired in the south Atlantic coastal and the western and southern central states with the highest incidence in Virginia, North Carolina, South Carolina, Oklahoma, and Tennessee. The disease typically occurs during spring and summer. Fever, rash, and a history of exposure to ticks is the classic triad of Rocky...

Associated Neurological Findings

Because the basal ganglia circuits include the cortex and because the caudate nucleus is particularly involved with cognition, the cerebral examination can have important contributory findings in patients with hypokinesia or hyperkinesia. Bedside screening tests of dementia and depression are very useful, and an assessment of aphasia and apraxia indicates likely cortical lesions. Cranial Nerves. Most cranial nerve functions are retained in hypokinesia and hyperkinesia. Ocular saccades...

Patients with specific complications

Patients on levodopa who develop motor fluctuations More frequent, small doses of levodopa or liquid Sinemet Selegiline agonists (or COMT inhibitors) Consider neurotransplantation or pallidal surgery Thalamic surgery (ablative or deep brain stimulation) Reduce medications. Stop all drugs except Sinemet Consider Clozapine or other neuroleptic with few extrapyramidal side effects Modified from Hughes AJ, Calasimo C, Kleedorfer B, et al The dopaminergic response in multiple system atrophy. J...

Nuclear Brain Stem Syndromes

Trauma, ischemic injury, and tumors may affect the trigeminal system within the brain stem. These syndromes are characterized by sensory paresthesias, numbness, or pain in the distribution of the V1 to V3, along with combinations of cranial nerve palsies and distinct sensory and cerebellar system signs. Reflecting the lateral circumferential TABLE 10-3 -- SELECTED ETIOLOGIES ASSOCIATED WITH TRIGEMINAL NERVE DISORDERS TABLE 10-3 -- SELECTED ETIOLOGIES ASSOCIATED WITH TRIGEMINAL NERVE DISORDERS...

Basal Gangliar Connections

Anatomical discussions of the basal ganglia usually consider structures in afferent and efferent relationship with the striatum. The caudate and putamen complex contains several different neuronal types, the most abundant population being the medium spiny neuron which uses gamma-aminobutyric acid (GABA) as its neurotransmitter. This neuron sends its axonal projection out of the striatum and also has several recurrent axon collaterals that are distributed primarily within its own intrastriatal...

Abnormal Findings and Clinical Uses of Polysomnography

Polysomnography is important in the investigation of patients with excessive daytime somnolence, disorders of initiating and maintaining sleep, disorders of the sleep-wake cycle, and disorders associated with certain sleep stages (parasomnias). It is important in confirming the existence of insomnia and characterizing its nature by determining, for example, whether it is associated with nocturnal myoclonus or periodic leg movements. Some patients complain of insomnia but, in fact, have a normal...

Evaluation Guidelines Table82

Most patients with suspected optic neuropathies should undergo neuroimaging to exclude a compressive RDERS OF CRANIAL NERVE II AND AFFERENT VISUAL PATHWAYS MRA carotid occlusion, stenosis, or dissection electroretinogram if photoreceptors are affected Elevated ESR in giant cell arteritis Vascular occlusion or leakage on fluorescein angiography Thromboembolic source on cardiac echography or carotid ultrasound MRI with gadolinium and fat saturation, coronal views optic nerve...

Miscellaneous Agents

2 mg IM 2-4 mg PO 50-150 mg PO IM IV 5-10 mg PO PR IM IV 25-50 mg IV IM 25-100 mg PR 5-10 mg IV IM 25 mg PR 5 mg PO 4 mg IM 8 mg IN 4 mg IM 16 mg PO 400 mg PO IM IV 60 mg PO PR IM IV 400 mg IV IM 400 mg PR 40 mg IV IM 75 mg PR 20 mg PO 20 mg IM M, Migraine C, cluster headache T, tension-type headache IN, intranasal SC, subcutaneous IM, intramuscular PO, per mouth IV, intravenous SL, sublingual per rectum. migraine headaches, oral medications may be unsuitable, and alternative formulations, as...

Cluster Headache

The pathogenesis of cluster headaches has not been fully determined. Cluster events may be related to alterations in the circadian pacemaker, which may be due to hypothalamic dysfunction. Attacks increase following the beginning and end of daylight savings time, and there is a loss of circadian rhythm for blood pressure, temperature, and hormones, including prolactin, melatonin, cortisol, and beta endorphins. Neurogenic inflammation, carotid body chemoreceptor...

Comments

Indistinguishable clinically from AIDP and CIDP in Most common HIV-l-related form of neuropathy Prominent lower extremity syndrome resembling spinal cord compression Laryngeal nerve involvement can ocour and lead to hoarseness with vocal cord paralysis Modified from So YT Holtzman DM Abrams Dl Olney RK Penpheral neuropathy assoeiated with acquired immunodeflciency syndrome prevalence and clinical features from a population-based survey Arch Neurol 1988 45 945 948. AIDP Acute inflammatory...

Transient Ischemic Attacks and Strokes

Miller Fisher first described the phenomenology of TIAs as prodromal fleeting attacks of paralysis, numbness, tingling, speechlessness, unilateral blindness or dizziness, which nearly always preceded cerebral infarction in patients with occlusion of the internal carotid artery (ICA). '6 TIA is a strong indicator of a subsequent stroke. The first year after a TIA carries the greatest stroke risk (5 percent). y TIA is arbitrarily defined as a focal neurological deficit lasting less than 24 hours,...

Myopathic Syndromes

Note that myasthenia gravis commonly affects ocular muscles (diplopia) and eyelids (ptosis), whereas ALS does not Autoantibodies against calcium channel Autoimmune or inflammatory (polymyositis) muscular dystrophy in the limbs with normal function in the cranial muscles and a normal jaw jerk. In the presence of diffuse hyperreflexia of the tendon jerks, normal cerebral function and normal speech increase the probability that the lesion is at the level of the cervical cord. A normal jaw jerk...

Reyes Syndrome

Reye's syndrome is defined by the Centers for Disease Control and Prevention as an acute, noninflammatory encephalopathy with altered levels of consciousness and liver dysfunction. y The liver disease must include either fatty metamorphosis of the liver or at least a threefold increase in alanine aminotransferase, aspartate aminotransferase, or serum ammonia. Pathogenesis and Pathophysiology. A growing body of evidence suggests that Reye's syndrome may be a multiorgan disease due to diffuse...

Migraine Headache

Although the exact pathophysiology of migraine is unknown, several theories exist. Any migraine theory must explain the prodrome, the aura, the headache with its associated symptoms, and the postdrome. The traditional vascular theory proposed that the migraine aura was caused by intracerebral vasoconstriction and the headache by painful reactive vasodilation. This theory cannot explain the prodromal features of migraine or the reason some antimigraine drugs...

Multiple Sclerosis

Multiple sclerosis is an inflammatory relapsing or progressive disorder of CNS white matter and a major cause of disability in young adults. Pathologically, it is characterized by multifocal areas of demyelination with relative preservation of axons, loss of oligodendrocytes, and astrogliosis. Although certain clinical features are characteristic of MS, investigative studies are often needed to confirm the clinical suspicion and exclude other possibilities. These tests may be useful in...

Suspected

LMN signs only in at least two regions POSSIBLE ALS UMN and LMN signs in only one region, or UMN signs only in at least two regions, or LMN signs rostral to UMN signs Special cases monomelic ALS, progressive bulbar palsy without spinal UMN and or LMN signs, primary lateral sclerosis without spinal LMN signs PROBABLE ALS UMN signs in at least two regions, with some UMN signs above LMN slgns DEFINITE ALS UMN signs and LMN signs in bulbar region and at least two spinal regions, or UMN and LMN...

Sleep Disordered Breathing and Neuromuscular Diseases

Breathing disturbances in neuromuscular diseases are due to weakness of respiratory muscles or upper airway muscles, or both. Respiratory muscle weakness is usually severe in Duchenne's muscular dystrophy and also occurs in myotonic dystrophy, limb-girdle dystrophy, polymyositis, poliomyelitis, amyotrophic lateral sclerosis, myasthenia gravis, and congenital myopathies. Weakness of the diaphragm is the most important cause of respiratory disturbance during sleep. Diaphragmatic workload...

Neuromuscular Junction And Muscle

Cachectic myopathy Stiff-person syndrome Data from Posner JB Neurologic Complications of Cancer. Philadelphia, F. A. Davis, 1995. neuronal phenotype. The mechanism for the inflammatory infiltrates, which consist of B cells, CD4+ and CD8+ lymphocytes, and macrophages, remains to be elucidated. Epidemiology and Risk Factors. All small cell lung cancers with or without paraneoplastic symptoms express the Hu antigen, and 16 percent of patients with small cell lung cancer but lacking neurological...

Parkinsonism Plus Syndromes

In addition to Parkinson's disease, parkinsonism is one of the major clinical features in several other primary neurodegenerative conditions. However, because they all have additional features not typical of Parkinson's disease and share an overall worse prognosis and poorer response to antiparkinsonian therapy, they are often grouped together under the conglomerate term parkinsonism-plus syndromes. Within this group, each condition has distinctive characteristics that must be recognized and...

Alcoholic Neuropathy

Alcoholic neuropathy is difficult to separate from nutritional, specifically thiamine-deficient, neuropathy. '92 The clinical pattern and pathology of axonal degeneration is virtually identical to beriberi. The incidence ranges from 9 to 30 percent among hospitalized alcoholics, y , y and up to 93 percent of ambulatory alcoholics may have electrophysiological evidence of neuropathy. y Victor and associates found signs of polyneuropathy in 82 percent of 230 patients with WKS, '8 and 84 percent...

Ocular Misalignment Syndromes

Lesions of the oculomotor nerve can involve the nucleus in the midbrain, or nerve fascicles within the ventral midbrain, subarachnoid space, cavernous sinus, superior orbital fissure, or orbit. As an example, in the setting of an isolated right nuclear third nerve lesion (see Fig. 9-7 (Figure Not Available) ), there is complete disruption of the outflow of that nerve. Additionally, fibers from the left subnuclei for the superior rectus (contralateral outflow) and levator palpebrae (bilateral...

Cranial and Spinal Epidural Abscess

Cranial epidural abscesses develop in the space between the dura and inner table of the skull and are usually caused by the spread of infection from the frontal sinuses, middle ear, mastoid, or orbit. y Epidural abscesses may also develop as a complication of a craniotomy or compound skull fracture. At present, the most common cause of a cranial epidural abscess is craniotomy that has been complicated by an infection of the wound, bone flap, or epidural space.y...

Diabetes

The neurological complications of diabetes were first recognized in 1798, and until 1864, diabetes was considered an effect of neuropathy. 123 For discussion purposes, the neurological complications of diabetes can be divided into CNS, cranial nerve, and PNS disturbances, of which PNS disturbances predominate. These disorders involve different parts of the nervous system, and therefore, produce different clinical manifestations. CENTRAL NERVOUS SYSTEM COMPLICATIONS OF DIABETES The brain,...

Postgastroplasty Polyneuropathy

Some patients undergoing bariatric, or weight reduction, surgery develop a syndrome of acute or subacute sensory loss, weakness, and areflexia in the limbs, usually following a period of dramatic weight loss and repeated bouts of protracted vomiting. y A few patients have also developed a type of encephalopathy that is clinically and pathologically identical to WKS, with or without an associated polyneuropathy. y Indeed, Wernicke and Korsakoff each described young women with intractable...

Hypokinetic Movement Disorders

Pathological findings of Parkinson's disease (PD) include depigmentation and neuronal loss in the substantia nigra (SN) and the presence of Lewy bodies and pale bodies( Fig 34-1). Because the degenerating cells in the SN normally synthesize the neurochemical dopamine, the pathophysiological hallmark of PD is dopaminergic underactivity at the site of these cells' axonal projection--that is, the striatum (caudate nucleus and putamen). Lewy bodies are eosinophilic...

Language Related Disorders

Aphemia was the term originally used by Broca to describe all language disturbances, but he later accepted the term aphasia promoted by Trousseau (1864). Bastian (1887) used aphemia to denote a specific syndrome described here others have termed the same language abnormality pure motor aphasia, cortical anarthria, or apraxia of speech. The patient presents acutely with mutism but is able to communicate using written language. When verbalization returns, the output is hypophonic, slow, and...

Hiv1associated Peripheral Neuropathy Syndromes

Distal symmetrical polyneuropathy Progressive polyradiculopathy Mononeuritis multiplex AUTONOMIC NEUROPATHY SENSORY GANGLIONITIS MOTOR NEURON DISEASE the time patients develop AIDS, clinical and electrophysiological evidence of neuropathy is present in up to one third of patients 137 and an even higher rate of nerve pathology is reported from autopsy studies. Data from the Multicenter AIDS Cohort Study indicate that the frequency of neuropathy in AIDS increased between 1985 and 1992. 98 Acute...

Evaluation Guidelines Table211

Magnetic resonance imaging (MRI) is useful for the evaluation of patients with focal or organ-specific autonomic syndromes. It can detect lesions that involve the frontal lobes (e.g., hydrocephalus) or the cervical, thoracic, or lumbar spine, producing different types of neurogenic bladder. It is also indicated for the detection of hypothalamic lesions, lateral medullary infarctions, syringomyelia, or lesions in the cavernous sinus that produce Horner's syndrome. MRI of the neck...

Table 305 Glycogenoses

Type 1 Von Gierke's disease (Cori) (glucose-6-phosphatase) Type 2 Pompe's disease (Cori) (acid maltase) Type 3 Smith's disease (late infantile) (acid maltase) y , y Type 4 Engel's disease (adult) (acid maltase) y Type 5 Forbes' disease (Cori) (debrancher- or amylo-1,6-glucosidase) Type 6 Anderson's disease (Cori) (debrancher- or amylo-transglucosidase) Type 7 MeArdle's disease (Cori) (myophosphorylase) Type 8 Hers' disease (Cori) (hepatophosphorylase) Type 9 Tarui's disease (Cori)...

Optic Nerve

Decreases in visual acuity and color vision, unilateral visual field loss, and an afferent pupillary defect suggest an optic neuropathy. Commonly associated field defects include arcuate, central, altitudinal scotomas, and constriction. The funduscopic examination may reveal an optic disc that is normal, swollen, or pale, depending on the etiology and temporal profile of the optic neuropathy. A glaucomatous disc has a normal rim, normal overall color, and an enlarged cup. Optic disc swelling,...

Pupillary Syndromes Anisocoria

It is commonly written that 20 percent of the population has anisocoria of at least 0.4 mm in dim light, although published series cite highly variable percentages from 2 to 60 percent. The most common sort of variable anisocoria among healthy people has been called see-saw anisocoria or simple central anisocoria whereas the mechanism is indeterminate, this benign condition may vary from one examination to the next or even reverse sides. Light stimulation of one or both eyes decreases the...

Tendon Reflex Amplitude

Present but decreased in amplitude and velocity from the normal range and elicited with reinforcement Normal amplitude and velocity without reinforcement Increased in amplitude and or velocity with spread to adjacent site Increased in amplitude and or velocity with spread to adjacent site and duplication of the jerk or clonus cutaneous reflexes are noted as present or absent. Tendon reflexes are elicited with a percussion hammer, of which many types are available. The two most commonly used are...

Directed Neurological Examination

The directed or focused neurological examination for the assessment of cranial nerve VIII includes an evaluation of the external ear, hearing, and vestibular responses. The examination begins with an assessment of gait and balance. Figure 12-4 (Figure Not Available) Arterial supply of the inner ea(From Schuknecht HF Pathology of the Ear. Philadelphia, Lea & Febiger, 1993, p 64.) Gait can often be observed as the patient walks into the examination room. The most useful test to quantify...

Occlusive Disease of Small Penetrating Arteries

The small penetrating arteries deep within the brain parenchyma are the sites of various occlusive processes that are different from those of the larger arteries. Pathogenesis and Pathophysiology. Lipohyalinosis, a destructive vasculopathy linked to severe hypertension, affects arteries 40 to 200 pm in diameter. The arterial lumen is compromised not by an intimal process but by thickening of the vessel wall itself. Subintimal lipid-laden foam cells and pink-staining fibrinoid material thicken...

Decompression Sickness

With descent, the partial pressure of the gases breathed by the diver increases proportionately, according to Dalton's law. Although oxygen is metabolized, tissues soak up inert nitrogen and become saturated. With ascent, the nitrogen moves from the tissues to the blood and is exhaled by the lungs, which is a process termed decompression. When ascent is too rapid and the tissues are supersaturated, dissolved gas changes to free gas, which creates bubbles. When...

Autosomal Recessive Inheritance

Ataxia and telangiectasias of the bulbar conjunctivae, malar eminences, ear lobes, and upper neck increased increased of respiratory infections, Iymphomas, Hodgkins disease, acute leukemia, and a variety of cancers thymus gland is hypoplastic or absent there is decreased IgA and IgG increased chromosomal breakage and increased sensitivity to ionizing radiation of fibroblasts and Iymphocytes Rare, partial oculocutaneous albinism, photophobia, neuropathy, and recurring infection giant cytoplasmic...

Developmental Hydrocephalus

In contrast to normal CSF physiology, which is discussed in Chapter 26 , hydrocephalus is the result of a disturbance in the normal CSF fluid dynamics. Two terms frequently used when discussing hydrocephalus are obstructive (noncommunicating) and nonobstructive (communicating) hydrocephalus. These terms were first coined by Harvey Cushing to describe the results of injection of air (pneumoencephalography) into the subarachnoid space or dye into the ventricles....

Pagets Disease

Paget's disease (osteitis deformans) is a metabolic bone disease of un known etiology characterized by increased osteoclast size and activity that results in resorption of bone followed by reactive new bone formation. This process produces areas of bone resorption with new bone laid down in an abnormally dense mosaic pattern. The osteoclasts in affected bone contain nuclear and cytoplasmic virus-like inclusions, and evidence has been presented of an association...

Osteosclerotic Myeloma

Osteosclerotic myeloma may be considered a rare variant of multiple myeloma, comprising only 3 percent of all patients with myeloma. Many of the typical features of multiple myeloma, including anemia, hypercalcemia, chronic renal failure, fatigue, weakness, and bone pain, are rare or at least uncommon in osteosclerotic myeloma. Furthermore, the level of M-protein is lower, and there are fewer plasma cells in the bone marrow. In contrast to multiple myeloma,...

Sensory Gait Syndrome

The adaptation of spinal and brain stem synergies to an individual's goals and limitations (i.e., context) requires a knowledge of the relations of the body segments to each other, the situation of the body in space and the gravitational field, and the presence of environmental hazards and aids to balance and locomotion. This information is largely derived from the somatosensory, vestibular, and visual senses. Generally, one sensory system is adequate to orient a person and permit normal...

Evaluation Guidelines j Table194

Plain radiographs may be helpful with peripheral localized nerve injury, such as identifying a humeral dislocation in the setting of an infraclavicular brachial plexopathy. Cervical spine or lumbar spine radiographs are useful in radicular complaints, and a chest radiograph is needed to evaluate lower trunk plexopathy of the brachial plexus to look for a cervical rib or an upper lobe structural lesion. If the clinical pattern of sensory loss suggests a brain stem or hemispheric...

Orthostatic Hypotension

Management of orthostatic hypotension requires patient education to avoid factors that precipitate a fall in blood pressure. Patients should be made aware of the hypotensive effects of certain drugs, large meals, environmental temperature increases, and physical activities. Other instructions include institution of a high-fiber diet to lessen straining resulting from constipation and the use of physical maneuvers that help to increase postural tolerance. These maneuvers include crossing the...

Chronic Cluster Headache

Divalproex 500-3000 mg day Same as episodic Lithium 300 mg bid or tid (0.4-0.8 Hypothyroidism and polyuria Methysergide 2-14 mg day Fibrotic reactions Verapamil 120-640 mg day Same as episodic *Begin early in the cluster period and continue until the patient has been headache-free for at least two weeks. Combinations are often required. bid, twice daily tid, three times daily. sumatriptan or DHE mesylate provide significant relief for about 80 percent of patients. An intranasal local anesthetic...

Cockaynes Syndrome

Cockayne's syndrome (see T.ab e,30-6 ) is sometimes classified as a variant of Pelizaeus-Merzbacher disease because of the similar pattern of patchy demyelination among preserved islands of myelin.y However, in this disease there is pronounced involvement of multiple systems, suggesting a different pathogenic mechanism. The brain is small, and the white matter is atrophic. Calcifications are frequently Proteolipid apoprotein in defect in gene Xq22 on X-chromosome Psychomotor retardation,...

Corticobasal Ganglionic Degeneration

The characteristic pathological changes in CBGD include asymmetrical frontoparietal cortical atrophy with corresponding neuronal loss and gliosis, substantia nigra degeneration, and swollen achromatic neurons. Wi Variable degrees of degeneration in other subcortical nuclei also occur. The achromatic neurons immunostain positively to neurofilaments (. Fig, 33-12 ). Some have regarded these neurons to be related to Pick cells, although immunohistochemical and...

Other Stereotypies

Stereotyped motor behavior can occur in normal children and in children with a variety of other primary neurodegenerative conditions. Repetitive head banging, an example of stereotypic activity, is seen in 15 percent of normal children. y In addition, thumb sucking, rocking, and other ritualistic behaviors are common during childhood. Most stereotypies, however, represent evidence of underlying brain dysfunction and hence require recognition. Stereotypic movements occur in at least a third of...

Contributors

Associate Professor of Neurology, Department of Neurology, University of Michigan Medical Center Director, Sleep Disorders Center, University of Michigan Medical Center, Ann Arbor, Michigan Sleep Disorders Professor of Neurology, School of Medicine, University of California Attending Physician and Director of the Clinical Neurophysiology Laboratories, University of California Medical Center, San Francisco, California Electrophysiology Instructor of Neurological Sciences, Rush University...

Spinal Cord Injury

Over the past decade there have been significant advances in transport, emergency management, pharmacological resuscitation, comprehensive acute care, and rehabilitation. Nevertheless, spinal cord injury (SCI) remains a physically and emotionally devastating problem. The neurologist may thus be confronted with the need for recognition of a potential spinal injury or SCI and institute appropriate stabilization procedures. Pathogenesis and Pathophysiology. The pathophysiology of SCI may be...

Vitamin E

Alpha-tocopherol is the most active form of vitamin E present in humans. Tocopherol is absorbed and incorporated into chylomicrons in the small intestine. It is carried in portal blood to the liver and alpha-tocopherol transfer protein (alpha-TTP) binds and recycles vitamin E in the liver for incorporation into low-density lipoproteins and very low density lipoproteins. Once it is delivered to the cells, alpha- tocopherol serves as an antioxidant, preventing free radical peroxidation and injury...

Polyarteritis Nodosa Churg Strauss and Overlap Syndromes

Classic polyarteritis nodosa (PAN) is a necrotizing, nodular vasculitis that preferentially affects medium and small arteries, and sometimes arterioles. The pathophysiology is believed to be deposition of immune complexes in the involved arteries. PAN is associated with a number of infections, particularly hepatitis B, tuberculosis, and streptococcus. In those cases associated with hepatitis B, hepatitis B surface antigen IgM, and complement can be demonstrated...

Sleep Disorders Associated with Degenerative Disorders of the Central Nervous System

Degenerative neurological diseases can lead to sleep disturbance through effects of the disease on neuronal systems involved in sleep-wake regulation, on systems involved with regulation of breathing during sleep, or on systems involved with motor control during sleep. Degenerative processes that affect the suprachiasmatic nucleus or the retina may produce circadian rhythm disturbances. Obstructive sleep apnea may occur if weakness, spasticity, or rigidity affect bulbar muscles, and central or...

Normal and Pathological Findings see Xables262 263 and 264

The general appearance of CSF is clear and colorless, because it is more than 99 percent water. Color of CSF is observed only in pathological circumstances. Whereas the term xanthochromia means yellow color, it has been used for the presence of other colors as well. Because of this practice, the actual color of the CSF should be stated and a gradation of its magnitude (from 1+ to 4+) should be noted. A yellowish tinge can be found with any cause of a markedly increased protein (greater than 200...

Evaluation Guidelines

The goals of the diagnostic evaluation are to establish the diagnosis of ischemic or hemorrhagic cerebrovascular disease as a cause of the patient's symptoms and to determine the underlying cause of the event. Early diagnosis and management in the first few hours after stroke are critical. Since proper management begins with an accurate diagnosis, it is paramount to differentiate between cerebral infarction and hemorrhage. The tests that may assist with diagnosis are detailed in Tabie.J.iiS ....

Alcoholic Myopathy

There are two forms of alcoholic muscle disease (1) an acute, painful myopathy associated with weakness, cramps, swollen and tender muscles, high creatine kinase, and rhabdomyolysis with or without myoglobinuria and (2) a chronic myopathy that is painless and often unnoticed by the patient, causing proximal weakness and type II fiber atrophy on nerve biopsy. In addition to skeletal muscle involvement, patients may have an associated cardiomyopathy. y Proposed mechanisms of alcoholic injury have...

Takayasus Arteritis

Takayasu's arteritis (TA), first described by Takayasu and Onishi in Asian patients with absent limb pulses and retinopathy, y is now well recognized in Western countries as well. The burden of disease is in the aorta and its main branches with symptoms attributable to vascular compromise or a systemic immune response. In tissue specimens, giant cells are seen invading the media and adventitia of affected vessels. There are a number of changes that suggest an...

Posterior Fossa Anomalies

The development of the brain stem and cerebellum has been extensively investigated at the anatomical, cellular, and molecular levels.y The cerebellum is entirely derived from the rhombic lip, a dorsal ridge of the developing neural tube located at the junction between the midbrain and hindbrain (rhombencephalon). Proliferation and migration at the rhombic lip results in caudal growth of the cerebellar anlagen, with the cerebellum forming the roof of the fourth...

Acute Bacterial Meningitis

There are approximately 25,000 cases of bacterial meningitis in the United States each year, but this disease is much more prevalent in developing countries. Group B streptococci and gram- negative enteric bacilli are the etiological organisms of the majority of cases of bacterial meningitis during the neonatal period in developed countries. In underdeveloped countries, gram-negative bacilli, predominantly Escherichia coli, are the most common pathogens. Risk...

Neuromyelitis Optica

Neuromyelitis optica (NMO) is an uncommon neurological illness characterized by the occurrence of optic neuropathy and myelopathy in close temporal relationship. The separation from MS has become questioned, owing to the frequent dissemination of lesions outside the optic nerves and spinal cord. Part of the difficulty lies with the broad, imprecise, and inconsistent definition. The names Devic's syndrome, Devic's disease, and NMO are often used interchangeably, although the first name...

Evaluation Guidelines Table173

Immediate computerized tomography (CT) scanning or magnetic resonance imaging (MRI) has to be performed in the setting of acute and subacute cerebellar ataxia. Posterior fossa lesions (hemorrhage, ischemia, hydrocephalus, neoplasms, abscess, or parasitic infections) carry the risk of increased intracranial pressure and death because of the cerebellar tonsils moving downward and compressing the brain stem within the foramen magnum. Angiography of the vertebrobasilar vascular system...

Neuromuscular Junction Syndromes

The defect in neuromuscular transmission in myasthenia gravis (see Chapter50 ) produces a pure muscular weakness without the atrophy, fasciculations, or reflex changes seen in motor neuron disease. Myasthenia gravis also causes a pattern of weakness in the ocular and cranial muscles that is different from that seen in amyotrophic lateral sclerosis. A weakness of the extraocular muscles and eyelids producing diplopia and ptosis is common in patients with myasthenia gravis but rare in those with...

Niacin and Nicotinic Acid

Nicotinic acid deficiency was found to be the causative agent of pellagra in 1937, yet the name was changed to niacin in order to prevent confusion with the tobacco derivative, nicotine. Niacin includes both nicotinic acid and nicotinamide, which form the metabolically active nicotinamide adenine dinucleotide (NAD) and NAD phosphate (NADP), an end product of tryptophan metabolism. More than 200 enzymes are dependent on NAD and NADP to carry out oxidation and reduction reactions, and these...

Neurosyphilis

The origin of the name syphilis is from a poem titled Syphilis sive morbus Gallicus by an Italian physician, Girolamo Fracastoro. The poem is about a shepherd boy named Syphilus who lives in the time of King Alcithous of Haiti. When Syphilus loses his sheep because of a drought, he blames the Sun God and convinces the natives of Haiti to offer sacrifices to King Alcithous, rather than to the Sun God. The angered Sun God revenges himself by sending a plague to the island natives. The shepherd...

Cranial Nerve Xi Nucleus

The spinal accessory nerve (CN XI) is classified as a special visceral efferent (SVE) nerve because it innervates striated muscles that arise embryologically from the branchial arches. CN XI consists of two distinct portions, the cranial (or accessory) portion and the spinal portion ( .Fig 14-1 ). y These are sometimes referred to as the ramus internus and ramus externus, respectively, y each with distinctive embryological developmental The cranial and spinal portions of CN XI arise from...

Lower Motor Neuron Pool

Contraction of skeletal muscle can occur only when a nervous impulse is conducted down the axons of the motor neuron. The motor neurons lie in the ventral horn of the spinal cord and are known as lower motor neurons. There are two types of motor neurons that provide a parallel system of innervation of the muscle. The larger alpha motor neurons innervate the extrafusal muscle fibers, and the smaller gamma motor neurons innervate the intrafusal muscle fibers of the muscle spindle (Fig. 15-3...

Syndromes Primarily Involving Hearing

Hearing loss is highly prevalent, especially in the older population, and three types are commonly encountered conductive, sensorineural, and central hearing loss. In conductive hearing loss, sound is not transmitted into the inner ear. Diagnosis is ordinarily made via observation of an air-bone gap on audiometry, meaning that hearing is superior when sound is transmitted in such a way that it bypasses the middle ear ossicular chain. Causes include a buildup of ear wax, foreign body in the ear...

Acute Disseminated Encephalomyelitis

Acute disseminated encephalomyelitis is a monophasic inflammatory demyelinating disorder that characteristically begins within 6 weeks of an antigenic challenge such as infection or immunization. It occurs more often in the young and causes the rapid development of multifocal or focal neurological deficits. Perivenous inflammation, edema, and demyelination are the pathological hallmarks of ADEM, although these lesions commonly enlarge and coalesce, forming lesions pathologically...

Behcets Disease

In 1937, Hulusi Behcet (1889-1948), a Turkish dermatologist, described the syndrome that bears his name. Behcet's disease (BD) is a relapsing inflammatory disorder without a defined cause. It may be a viral or autoimmune disorder, although familial cases and an association with HLA-B5 and HLA- B1 support a genetic role. W , W Although it was previously thought to be a hypercoagulable state, elevation of procoagulant factors such as aPL is now known to be a...

Directed Neurological Examination Visual Acuity

Visual acuity is a measurement of the individual's capacity for visual discrimination of fine details of high contrast. Best corrected visual acuity should be tested for each eye. 1 Distance vision is assessed with a standard Snellen chart and near vision with a hand-held card. If the patient does not bring corrective lenses for the examination, a pinhole can correct most refractive errors. Acuity is most often recorded as, for example, 20 40, in which the numerator refers to the distance (in...

Table 122 Ototoxic Medications

Aspirin and sodium salicylate (cochleotoxic, reversible) Chemotherapy (mainly mixed toxicity) TABLE 12-3 -- CLINICOANATOMICAL CORRELATIONS OF DISORDERS OF CRANIAL NERVE Vlil-AUDITORY SYSTEM TABLE 12-3 -- CLINICOANATOMICAL CORRELATIONS OF DISORDERS OF CRANIAL NERVE Vlil-AUDITORY SYSTEM ossicular chain, ending at the stapes. Sound energy is then converted into changes in neural firing, which is passed more centrally through a complex cross-connected network of neurons. This neural network can be...

Arousal Disorders

The arousal disorders--confusional arousals, sleepwalking, and sleep terrors--are grouped together based on the hypothesis that impaired ability to arouse fully from slow-wave sleep is responsible for the phenomena. y The disorders, which are most common during childhood when the amount of slow- wave sleep is at its peak, probably exist on a continuum. Confusional arousals are associated with low levels of motoric and autonomic activation sleepwalking with...