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1/53. role of neurophysiologic evaluation in diagnosis.

    The electrodiagnostic evaluation assesses the integrity of the lower-motor-neuron unit (i.e., peripheral nerves, neuromuscular junction, and muscle). Sensory- and motor-nerve conduction studies measure compound action potentials from nerve or muscle and are useful for assessing possible axon loss and/or demyelination. Needle electromyography measures electrical activity directly from muscle and provides information about the integrity of the motor unit; it can be used to detect loss of axons (denervation) as well as reinnervation. The electrodiagnostic examination is a useful tool for first detecting abnormalities and then distinguishing problems that affect the peripheral nervous system. In evaluating the patient with extremity trauma, it can differentiate neurapraxia from axonal transection and can be helpful in following the clinical course. In patients with complex physical findings, it is a useful adjunct that can help discriminate motor neuron disease from polyneuropathy or myeloradiculopathy due to spondylosis.
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2/53. Detection of cytomegalovirus antigens in phagocytosed serum complexes from a patient with rheumatoid arthritis, vasculitis, peripheral neuropathy, cutaneous ulceration, and digital gangrene.

    A patient with rheumatoid arthritis, vasculitis, peripheral neuropathy, cutaneous ulceration, and digital gangrene was studied. Circulating immune complexes were detected by C1q binding although serum complement levels were within the normal range. Immunofluorescent staining of buffy coat cells with specific antisera showed the presence of IgG and IgM in phagocytosed inclusions but complement c3 was not detected. A monoclonal antibody specific for cytomegalovirus detected antigens in phagocytosed inclusions on one occasion. These results may suggest that cytomegalovirus antigens are a hitherto unidentified component of serum complexes in patients with rheumatoid arthritis and may contribute to the pathogenesis of the vasculitic complications of rheumatoid arthritis by participating in immune complex formation.
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3/53. Immune-mediated neuropathy and myopathy in post-streptococcal disease: electron-microscopical, morphometrical and immunohistochemical studies.

    A 22-year-old man suffered from a complete flaccid tetraparesis and an immune complex-mediated rapid progressive glomerulonephritis after group A streptococcal infection. serum creatine kinase was excessively elevated and myoglobinuria occurred. Nerve conduction studies revealed evidence of axonal neuropathy. Recovery was satisfactory within 18 months. sural nerve and peroneus muscle biopsies were performed in the 4th and 14th week of the disease. light microscopy of the sural nerve showed an incipient axonal type of neuropathy in the first biopsy. Ultrastructurally, wallerian degeneration and endoneurial inflammatory cells were present. In the muscle biopsy, few atrophic fibers and altered blood vessels without further anomalies were found. In the second sural nerve biopsy, macrophages were numerous, some of which were immunoreactive for HLA-DR, and only a few myelinated and some unmyelinated nerve fibers remained. Muscle fibers in the second biopsy showed high-grade atrophy and myofibrillar abnormalities. immunohistochemistry revealed diffuse endoneurial immunoglobulin deposition in the first sample, while in the later biopsy specimen, deposits of IgG, and kappa and lambda light chains were visible in circumscribed endoneurial areas. Immune-mediated neuropathy and myopathy are not well-known complications of streptococcal disease. This is, to our knowledge, the first detailed report on morphological findings in muscle and nerve in such a disorder.
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4/53. Hypertrophic neuropathy of the cauda equina: case report.

    OBJECTIVE AND IMPORTANCE: Hypertrophic neuropathy of the cauda equina (HNCE) is a rare form of peripheral neuropathy. The diagnosis is complicated by an insidious clinical presentation and complex radiographic images. We present a case of HNCE caused by chronic inflammatory demyelinating polyneuropathy with symptomatic improvement after decompressive lumbar laminectomy and dural expansion. CLINICAL PRESENTATION: A 54-year-old woman with a history of back pain since she was in her 20s presented with low back and radicular pain that had increased during a period of 6 months, bilateral lower-extremity weakness, and sensory loss in the right thigh. magnetic resonance imaging of the lumbosacral spine revealed multiple, poorly enhancing mass lesions and apparent intrathecal nerve root thickening from L1 to L5. INTERVENTION: An L1-L5 decompressive laminectomy, performed with continuous somatosensory evoked potential and electromyographic monitoring, revealed multiple segmentally enlarged nerve roots. One nerve root that did not respond to high levels of stimulation was identified. This root was resected and submitted for pathological analysis. The dura was expanded with an 11-cm-long dural patch. The pathological examination revealed hypertrophic neuropathy, with extensive S-100-positive "onion bulb" formation. The patient's symptoms improved postoperatively. CONCLUSION: HNCE is a rare disorder that can cause radicular pain and lower-extremity weakness, sensory loss, and hyporeflexia. One possible cause is demyelinating polyneuropathy. Although medical management is typically effective in the treatment of demyelinating polyneuropathy, it has little effect on compressive symptoms caused by intradural nerve root enlargement. As this case demonstrates, surgical management of symptomatic radiculopathy by lumbar laminectomy is a reasonable and effective approach to the treatment of HNCE.
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5/53. Pharmacologic management part 2: lesser-studied neuropathic pain diseases.

    This second part of a review of the pharmacologic management of neuropathic pain diseases describes the current treatment options for three lesser-studied neuropathic syndromes: Central poststroke pain, spinal cord injury, and complex regional pain syndrome II. diagnosis can be difficult in patients with these syndromes, because the pain experienced is much greater and of a different type than would normally be expected following a stroke or injury to the spinal cord or a peripheral nerve. Even when an accurate and timely diagnosis is made, treatment options are limited and frequently suboptimal. However, the results of published trials do support the use of anticonvulsants and/or tricyclic antidepressants as first-line pharmacotherapy in these three neuropathic pain syndromes. To maximize treatment outcomes, future research must: Continue to more fully elucidate the relationship between the signs and symptoms of pain and the underlying pathophysiology; Delineate the natural history of central poststroke pain, spinal cord injury, and complex regional pain syndrome; Identify patient-related factors that may indicate an increased risk of developing neuropathic pain following stroke or nerve injury; Investigate emerging treatments that target underlying pain mechanisms.
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6/53. Intraoperative somatosensory evoked potentials to facilitate peripheral nerve release.

    PURPOSE: The significance of intraoperative somatosensory evoked potentials (SSEP) monitoring is well known during spinal surgery. This technology could be beneficial during peripheral nerve surgery as well. In order to illustrate potential applications, two cases of successful peripheral nerve release demonstrated by on-line, intraoperative, SSEP are reported.Clinical and technical features: The first case presents a complex brachial plexus lesion involving two mixed sensory-motor nerves: median and ulnar. The second case involved an entrapment neuropathy of the lateral femoral cutaneous nerve, a pure sensory nerve (meralgia paresthetica). For each patient we elicited specific peripheral nerve SSEP (recorded using bipolar cephalic montage) by stimulating each nerve independently. In each case, during difficult nerve dissection and after having excluded other possible factors of intraoperative SSEP variations, an increase of the SSEP amplitude was observed, and later correlated with favourable patient clinical outcome. CONCLUSIONS: Two cases demonstrate that intraoperative SSEP monitoring may provide an effective tool to guide surgical dissection during peripheral nerve release. This technique has potentially beneficial clinical applications and warrants further investigation.
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7/53. Peripheral neuropathy associated with sjogren's syndrome: pathologic and immunologic study of two patients.

    Two cases of sjogren's syndrome accompanied by peripheral neuropathy are reported. The level of anti-endothelial cell antibody was increased in both patients. Immunofluorescent deposits of immunoglobulin and C3 component were detected in the vasa nervorum of both cases. The pathological findings showed damage to the endothelial cells in the same vessels. The findings suggest that injury from immune complex and anti-endothelial cell antibody may be the immunological factor in the induction of peripheral neuropathy.
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8/53. Cryoprotein complexes and peripheral neuropathy in a patient with chronic active hepatitis.

    A cryoprotein complex was isolated and characterized from a patient with chronic active hepatitis and a severe peripheral neuropathy. This cryoprotein was composed of IgM, IgG, and hepatitis b surface antigen (HBsAg) and had a concentration of approximately 36 mg per 100 ml of serum. Electron microscopic examination of the cryoprotein demonstrated aggregates of Dane particles in close association with the antigenically related tubular and spherical forms of HBsAg. HBsAg, IgM, and IgG were detected by immunofluorescent staining in the intima of small arteries and veins. The association of a high serum level of cryoprotein and deposition of the cryoprotein components in small blood vessels suggests a role for the cryoprotein in the pathogenesis of peripheral neuropathy in this patient with chronic active hepatitis.
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9/53. Peripheral neuropathy caused by methaqualone.

    Three patients are described who each received methaqualone and developed signs and symptoms of peripheral neuropathy. The subsequent improvement after cessation of methaqualone was highly suggestive or a direct toxic action of the drug or one of its metabolites. In one patient methaqualone was recommended with reappearance of signs and symptoms of peripheral neuropathy. Again cessation of the drug caused disappearance of these signs. There was no evidence whatsoever of any electrolyte or metabolic disturbance or any other pathology which might have given rise to this symptom complex. In addition, no other drugs were prescribed besides methaqualone.
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10/53. Superior orbital fissure syndrome. review of 130 cases.

    Superior orbital fissure syndrome is a symptomatologic complex, consisting of retroorbital pain, paralysis of extraocular muscles, impairment of first trigeminal branches and frequent involvement of the optic nerve. From a review of 130 published cases including two personal observations, it appears that the clinical subdivisions and the several eponymic differentiations of this syndrome are unjustified on the basis of etiologic, therapeutic and prognostic elements. Only the presence--or absence--of optic signs may allow to hold the clinically useful distinction between the often 'benign' superior orbital fissure syndrome without optic nerve involvement, and the orbital apex syndrome where orbital exploration may be recommended. This review concludes that repeated neurosurgical and neuroradiologic investigations--with the exceptions of carotid angiography and orbital phlebography--can be avoided in such cases.
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