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1/34. Idiopathic small-fiber sensory neuropathy in childhood: A diagnosis based on objective findings on punch skin biopsy specimens.

    Idiopathic small-fiber sensory neuropathy (SFSN) has not previously been reported in children. Although affected patients complain of neuropathic pain, this condition is often difficult to diagnose because of the few objective physical signs and normal nerve conduction studies. We report a girl with idiopathic SFSN in whom the results of a sural nerve biopsy were normal, but punch skin biopsy revealed reduced intraepidermal nerve fiber density and established the diagnosis. Idiopathic SFSN should be considered in the differential diagnosis of children who have burning limb pain with no routine electrophysiologic or pathologic abnormalities.
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2/34. Posterior interosseous nerve palsy in a patient with rheumatoid synovitis of the elbow: a case report and review of the literature.

    A 54-year-old woman with rheumatoid arthritis developed loss of finger extension in the left hand. history, physical examination, and electromyography led to the diagnosis of posterior interosseous nerve palsy secondary to synovitis of the elbow. Anterior decompression and synovectomy resulted in a complete recovery. A literature review describes similar cases and compares outcomes.
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keywords = physical examination, physical
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3/34. Bilateral phrenic neuropathy as a presenting feature of multifocal motor neuropathy with conduction block.

    Diaphragmatic paralysis has previously been reported as a result of diverse pathologic processes involving the peripheral nervous system. We report the clinical history, physical findings, and antibody profile of an atypical case of multifocal motor neuropathy with conduction block initially presenting with respiratory failure secondary to bilateral phrenic neuropathy.
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4/34. 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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5/34. Anti-myelin-associated glycoprotein antibodies and endoneurial cryoglobulin deposits responsible for a severe neuropathy.

    A 73-year-old man was investigated for a peripheral neuropathy which occurred in the course of a Waldenstrom's macroglobulinemia. serum immuno-fixation electrophoresis demonstrated two IgM monoclonal gammopathies of the kappa and lambda chain isotypes, and one had the physical characteristics of cryoglobulin. Immunoblot studies on the patient's serum revealed antibodies which reacted with peripheral nervous system proteins of different molecular weights including the myelin-associated glycoprotein (MAG). An immunofluorescence study of a superficial peroneal nerve biopsy revealed not only a binding of IgM and kappa light chain on several myelin sheaths but also the presence of IgM and kappa light chain deposits in the endoneurium. On electron microscopic examination, numerous fibres presented a widely spaced myelin and the endoneurial deposits had the ultrastructure of cryoglobulin. This is the first case presenting features of widely spaced myelin related to serum anti-MAG activity associated with monoclonal cryoglobulin deposits in the endoneurium.
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6/34. thallium and arsenic poisoning in a small midwestern town.

    thallium and arsenic have been used as a means of criminal poisoning. Although both manifest characteristically with peripheral neuropathies, thallium is associated with alopecia and arsenic with gastrointestinal symptoms. We describe the symptoms, physical findings, diagnostic test results, and outcomes in a group of men poisoned with thallium and arsenic. Seven patients had evidence of elevated thallium levels, and 2 patients had elevated arsenic and thallium levels. The most commonly reported symptoms included myalgias, arthralgias, paresthesias, and dysesthesias. Five patients developed alopecia. All patients with symptoms and peripheral neuropathies had characteristic blackening of their hair roots. Initially treated with dimercaptosuccinic acid, patients were switched to multiple-dose activated charcoal after testing revealed thallium poisoning. By 6 months, all patients' symptoms and peripheral neuropathies improved, but 5 patients had ongoing psychiatric problems. thallium remains a means of criminal poisoning and should be considered in any patient with a rapidly progressing peripheral neuropathy with or without alopecia.
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7/34. vincristine-induced neuropathy as the initial presentation of charcot-marie-tooth disease in acute lymphoblastic leukemia: a Pediatric Oncology Group study.

    PURPOSE: After profound peripheral neurotoxicity during induction chemotherapy for acute lymphoblastic leukemia (ALL) in the index patient with Charcot-Marie-Tooth hereditary neuropathy (CMT), study coordinators of the Pediatric Oncology Group (POG) front-line ALL protocols reviewed patient registrations to identify any other patients with possible CMT. The goal was to provide preliminary information about patients with undiagnosed CMT who develop ALL. patients AND methods: Five children with ALL who were enrolled in POG B-precursor or T-cell ALL protocols from 1994 to 1999 subsequently were determined to have CMT hereditary neuropathy. Their clinical presentations and treatment records were reviewed in detail. Records of all patients entered on POG 9201 (lesser-risk ALL) were reviewed to identify all cases of significant vincristine toxicity noted in the first 6 months of treatment. RESULTS: The five identified patients all had substantial peripheral neurotoxicity that required alteration in treatment and/or orthopedic/physical therapy evaluation and follow-up. The POG 9201 review identified 25 of 686 patients (3.6%) with significant peripheral neuropathy. Three of 25 were diagnosed with CMT; the others have had no testing reported. CONCLUSIONS: A family history of CMT or other peripheral neuropathy should be sought at the time of diagnosis of ALL. Testing for CMT should be considered in any child with substantial vincristine-induced peripheral neurotoxicity. Treatment of such patients must be individualized. Testing of all patients with significant peripheral neuropathy would be necessary to determine the percentage of such neuropathy explained by underlying CMT.
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8/34. Peripheral neuropathies in clinical practice.

    Based on the history and physical examination, it is often possible to arrive at a relatively specific diagnosis of peripheral neuropathy. The clinical history and examination are often sufficient to create a more focused and appropriate laboratory evaluation, counseling for the patient as to long-term prognosis, and options for clinical management.
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keywords = physical examination, physical
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9/34. Electrodiagnostic medicine.

    Electrodiagnostic testing examines the physiologic integrity of the peripheral nervous system. However, such testing should represent only one part of an electrodiagnostic consultation in which the entire clinical context, including the history, physical examination, laboratory studies, and electrodiagnostic testing, is considered as a whole. Although each electrodiagnostic laboratory establishes its own normal values for nerve conduction studies and needle EMG, these values should not be used in isolation. The electrodiagnostic consultation can help narrow an otherwise broad differential diagnosis, confirm a suspected diagnosis, or help define a confusing clinical picture.
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keywords = physical examination, physical
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10/34. brachial plexus injury caused by impalement.

    Open injuries of the brachial plexus are rare. One such case, that of a 68-year-old impaled on a fence spike, is presented here. Certain principles to guide evaluation and treatment are discussed. Concomitant injury to the pleura or to vascular structures requires immediate attention; the extent and type of plexus damage may be determined from physical findings and the nature of injury. The results of plexus reconstruction are variable and routine exploration may be detrimental. The Brooks classification is reviewed.
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