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...

Reviews And Selected Updates

Berg BO Principles of Child Neurology. New York, McGraw-Hill, 1996. De Grouchy J, Turleau C Clinical Atlas of Human Chromosomes, 2nd ed. New York, John Wiley, 1984. Rosenberg RN, Pettegrew JW Genetic Neurological Diseases. In Rosenberg RN (ed) Comprehensive Neurology. New York, Raven Press, 1991. Rosenberg RN, Prusiner SB, DiMauro S, et al The Molecular and Genetic Basis of Neurological Disease. 2nd ed. Boston, Butterworth- Heinemann, 1997. Schinzel A Catalogue of Unbalanced Chromosome...

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...

Basic Principles and Techniques

The advent of CT was one of the most exciting developments in the history of neuroimaging. For the first time, direct visualization of the brain became possible. Although MRI has surpassed CT in displaying neuroanatomy and pathology, CT remains a mainstay for several reasons. Wider availability and lower costs of CT are considerations in today's managed care environment. CT is easier to perform in the setting of acute trauma and in the ventilated patient. Owing to the speed with which CT images...

Pseudobulbar Palsy

Patients with pseudobulbar palsy demonstrate pathological laughing, crying, and grimacing, often following little or no stimulation. Spastic weakness of the lower cranial nerve muscles (cranial nerves V, VII, and IX through XII) may be noticed on examination with accompanying dysarthria. This syndrome may be seen in patients with upper motor neuron dysfunction including those with amyotrophic lateral sclerosis, multiple sclerosis, or bilateral cerebral infarction. Patients with progressive...

Clinical History

Obtaining a detailed clinical history is especially important when diagnosing a sleep disturbance because routine physical examination is often not revealing during the waking hours. From the history, age of onset, duration and progression of the sleep complaint, and the general classification of the type of sleep disturbance is usually obtained. The International Classification of Sleep Disorders categorizes sleep disturbances as (1) dyssomnias or disorders that result in insomnia or excessive...

Etiology Percent

Jejunal diverticula 2 Dietary cobalamin malabsorption Intrinsic factor antibodies present, or, correction of abnormal Part I Schilling test with intrinsic factor Evidence of achlorhydria (elevated serum gastrin) with abnormal Part I Schilling test in some, but evaluation incomplete. Evidence of achlorhydria (elevated serum gastrin) with abnormal Part I Schilling test. Reprinted with permission from Healton EV, Savage DG, Brust JCN, et al Neurologic aspects of cobalamin deficiency. Medicine...

Intraparenchymal Metastases

To establish a metastatic colony, tumor cells must grow within the primary site of malignancy, escape from the primary tumor, penetrate the circulatory system either as single cells or small tumor emboli, survive while circulating, arrest in the microvasculature of other organs, extravasate into the organ parenchyma, and efficiently grow and compress or invade tissue at the secondary site. Solid tumors or metastases larger than 1 or 2 mm in diameter must be...

Degenerative and Compressive Structural Disorders

History and Definitions Degenerative Structural Disorders Degenerative Disc Disease Cervical Spondylosis Ankylosing Spondylitis Compressive Structural Disorders Fibrous Dysplasia Paget's Disease Other Compressive Disorders Compressive Neuropathies Reviews and Selected Updates References A variety of neurological disorders result from abnormalities of bones, ligaments, muscles, and other mesenchymal tissue that compress the nervous system. In many instances the result is a focal disorder of...

Degenerative Disc Disease

The vertebral disc is composed of the inner gelatinous nucleus pulposus and the surrounding annulus fibrosus. Vertebral disc herniation refers to rupture of the annulus with displacement of the central nucleus. In youth, the disc is highly elastic. With the passage of time, the direct vascular supply to the vertebrae and discs decreases, and they undergo the accumulated effects of axial loading. The resulting decreases in water and oxygen content, and metabolic...

Efferent Autonomic Pathways

The autonomic output to effector organs consists of a two-neuron pathway with one synapse in the peripheral autonomic ganglia. The central neuron is called preganglionic, and the ganglionic neuron is called (illogically) postganglionic. Sympathetic preganglionic neurons are located in the intermediolateral cell column of the gray matter of the spinal cord at all thoracic and high lumbar levels (T1-L3) (thoracolumbar outflow) ( F.i9, . 1 3 A). Parasympathetic preganglionic neurons are located...

Neuronal Migration Defects

Anomalous cerebral cortical development is generally characterized as a cortical migrational abnormality, although an actual defect in migration has yet to be conclusively demonstrated. Rorke y has hypothesized a variety of factors that must be considered in the pathogenesis of cortical migrational abnormalities. Among the various components of development, cellular proliferation, cell death, postmigrational intracortical growth and development, and...

Multiple System Atrophy

This is an uncommon and heterogeneous group of disorders generally characterized by atypical, relative dopamine-unresponsive parkinsonism with variable degrees of cognitive impairment. MSA includes olivopontocerebellar atrophy (OPCA), striatonigral degeneration, and parkinsonism with orthostatic hypotension and dysautonomia (Shy- Drager syndrome). Pathogenesis and Pathophysiology. Neuropsychological studies in both sporadic and familial cases of OPCA have demonstrated frontostriatal...

Strachans Syndrome

In 1888, Henry Strachan, a British medical officer stationed in Jamaica, described a syndrome of painful peripheral neuropathy, ataxia, optic neuropathy, and stomatitis among sugar cane workers. Denny-Brown and others found similar ailments among allied troops liberated from prisoner-of-war camps after World War II. 12 In these patients, other symptoms included sensorineural deafness, dizziness, confusion, spastic leg weakness, foot drop, Wernicke's encephalopathy, and rare cases of neck...

Neurocysticercosis

Neurocysticercosis is considered the most common parasitic disease of the CNS. In countries where this infection is endemic, it is the most common cause of late-onset epilepsy. Humans acquire cysticercosis by the ingestion of food contaminated with Taenia solium eggs, most often undercooked pork but also water and vegetables contaminated with human feces. '1241 Pathogenesis and Pathophysiology. After food contaminated with T. solium eggs is ingested, the eggs...

Episodic Weakness Syndrome

There are a number of unusual syndromes in which weakness is intermittent or fleeting. These are listed in Table 15-15 . Because of the phenomenon of fatigability and recovery after rest the patient with myasthenia gravis may complain of episodic weakness. Myasthenia gravis is seldom confused with these other disorders because of the features mentioned earlier and the predilection of the weakness for the ocular and cranial muscles. An episodic hemiparesis is more apt to lead to confusion. The...

Special Autonomic Function Testing

Many laboratory tests are used to diagnose autonomic disorders, quantify autonomic function, and evaluate the efficacy of treatment. Disorders for which autonomic testing provides useful information include those involving generalized autonomic failure such as the Shy-Drager syndrome, Parkinson's disease, and pure autonomic failure, suspected distal small-fiber and other peripheral neuropathies, orthostatic intolerance of uncertain etiology, syncope, localized autonomic disorders, unexplained...

Other Disorders of the Skull and Spine Dwarfism

Although dwarfism is primarily a defect of the musculoskeletal system, central and peripheral nervous system manifestations are encountered in a variety of these disorders. The most frequent neurological manifestations are a result of impingement of the nervous system as a result of narrowing of bony foramina. For example, in some dwarfism syndromes, such as achondroplasia, narrowing of the foramen magnum can obstruct CSF flow and result in hydrocephalus. In achondroplasia, the development of...

Dermatomyositis and Polymyositis

Dermatomyositis (DM) and polymyositis (PM) have been thought to have an autoimmune origin for many years. This concept is based on indirect associations with other autoimmune disorders, mononuclear inflammatory cells in affected muscles, and responsiveness to immunosuppressive agents. Genetic factors play a role, as evidenced by reports of identical twins with DM, families with other connective tissue diseases and DM or PM, and increased associations with...

Various Transporter Defects Hartnups Disease

Hartnup's disease is an autosomal recessive transport disorder of neutral amino acids, affecting the kidney and small intestine and leading to excessive neutral aminoaciduria 5 to 20 times normal.1 These monoamino monocarboxylic amino acids include alanine, glutamine, histidine, serine, phenylalanine, tyrosine, tryptophan, asparagine, leucine, isoleucine, and valine but not proline. Plasma levels are normal. These same amino acids, particularly tryptophan, are retained in the gut and broken...

Nonhereditary Idiopathic Cerebellar Ataxia

Idiopathic cerebellar ataxia (IDCA) refers to a heterogeneous group of nonhereditary degenerative ataxias that begin after the age of 25 years.y Ihe etiology of IDCA is unknown. Clinically and pathologically, there are two major types. Ihe cerebellar type, IDCA-C, is characterized by a purely cerebellar syndrome and an almost exclusive degeneration of the cerebellar cortex. Ihe plus type, IDCA-P, often has additional clinical features that suggest involvement...

Headache or facial pain associated with disorders of cranium neck eyes ears nose sinuses teeth mouth or other facial or

During the aura of classic migraine, a wave of decreased CBF spreads forward from the occipital cortex, preceding the aura symptoms and persisting into the headache phase. This change in CBF may be caused by cortical spreading depression (a short-lasting wave of neuronal activation, followed by inhibition), which may also produce the aura symptoms and activate trigeminal nerve endings. In the headache of migraine with aura (classic migraine), increased CBF occurs after the headache...

Compressive Neuropathies

COMPRESSIVE DISORDERS OF THE MEDIAN NERVE Pathogenesis and Pathophysiology. The median nerve is subject to compression at the wrist, where it accompanies the flexor tendons as they pass beneath the volar carpal ligament. This region, the carpal tunnel, is a closed space within which pressure may rise. Studies with recording wicks placed in the canal show that flexion or extension elevate the canal pressure and that thickening of tendon sheaths or encroachment by other structures leads to a...

Intracranial Granulomatous Arteritis or Primary Angiitis of the Central Nervous System

This disorder has carried a number of names, including noninfectious granulomatous angiitis with a predilection for the CNS, granulomatous angiitis of the CNS (GACNS), primary angiitis of the CNS (PACNS), and vasculitis confined to the CNS. First described in 1959, very few cases were described antemortem until the widespread availability of angiography in the 1960s. The term PACNS is most appropriate because it requires the absence of a systemic disease, but...

Brachial And Lumbosacral Plexopathies

Trauma can result in variable injury to the myelin, axon, and connective tissue covering of plexus elements. Mild injuries may result in focal demyelination with conduction slowing. More severe injuries may cause demyelinating conduction block (neurapraxia), which can occur alone or in association with axon degeneration. Axonotmesis, which is loss of the relative continuity of the axon and myelin but preservation of the connective tissue framework, leads to...

Lower Motor Neuron Syndromes

Diseases of the lower motor neuron may affect the cell body itself in the anterior spinal gray or its axon as it leaves the spinal cord in the spinal root and becomes a peripheral nerve. Signs of disease of the lower motor neuron include muscular weakness, atrophy, fasciculations, and loss of tendon reflexes. PROGRESSIVE SPINAL MUSCULAR ATROPHY SYNDROMES There may be selective degeneration of the anterior horn cells of the spinal cord or brain stem or both. This results in a progressive...

Localizationrelated Focal Epilepsies and Syndromes

BENIGN FOCAL EPILEPSIES OF CHILDHOOD Benign focal epilepsies of childhood are idiopathic localization-related epilepsies characterized by partial seizures, focal EEG abnormalities, and absence of a known etiology. These are age-related syndromes occurring in neurologically normal children that have a tendency for spontaneous remission. BCECT is the most common, comprising 15 to 20 percent of all childhood epilepsies. y In comparison, benign epilepsy of childhood with occipital paroxysms...

Grade Description

I Subependymal hemorrhage into one or both germinal matrices II Germinal matrix hemorrhage with intraventricular extension, no hydrocephalus III Germinal matrix hemorrhage with intraventricular extension and hydrocephalus IV Germinal matrix hemorrhage with intraparenchymal extension infants are evaluated for neurological disorders secondary to prematurity. This coupled with primary neurological diseases has led to wide use of US as a leading diagnostic imaging tool in the neurological...

Limbic System

The term limbic lobe was coined by Broca in 1878 to describe a series of structures that envelop the brain stem. The word limbic is derived from limbus, meaning border in Latin, 5 and the limbic system encompasses the amygdala, hippocampus, septum, cingulate gyrus, cingulate cortex, hypothalamus, epithalamus, anterior thalamus, mammillary bodies, and fornix. The limbic system has rich connections throughout the brain, particularly with the primary sensory cortices, including the rhinencephalon...

Pharmacological Neuroanatomy

A number of neurotransmitters, specifically the catecholamines (norepinephrine and dopamine) and serotonin, are closely related to mood, emotion, and thought disorders.y Catecholamines are formed in brain, chromaffin cells, sympathetic nerves, and sympathetic ganglia from tyrosine, their amino acid precursor (Fig. 3-1 (Figure Not Available) ). Tyrosine is taken up from the bloodstream and concentrated within the brain and other sympathetically innervated tissue via active transport. In the...

Testing Methods

The usual morphological techniques for examination of peripheral nerve include standard light microscopy and electron microscopy, supplemented by morphometric studies, examination of teased nerve fibers, and examination of frozen sections when special studies such as immunohistochemistry, are needed (.TabJe25 6 ). '13' For light and electron microscopy and teased nerve studies, specimens are usually fixed in glutaraldehyde solution. Cross sections are standard and useful for morphometric...