| We studied a patient with polyradiculoneuropathy with anaplastic carcinoma of the thymus. Motor manifestations dominated. Postmortem examinations indicated that the primary changes were in the spinal nerve roots, peripheral nerves and, possibly, the spinal anterior horn cells. The posterior funiculi and posterior root ganglia were also affected, implying multifocal and multiphasic degeneration. This unusual polyradiculoneuropathy is a form of carcinomatous neuropathy. ( info) |
12/552. Chronic hepatitis B exacerbated by guillain-barre syndrome: a report of two cases. Several neurologic complications involving both the central and peripheral nervous systems due to acute viral hepatitis have been described, but Guillain-Barre (G-B) syndrome occurring as a complication of chronic viral hepatitis is extremely rare. Although it is generally agreed that G-B syndrome develops as an immune-mediated reaction, its exact pathogenesis remains obscure. We report the cases of two patients with chronic hepatitis b virus (HBV) infection acutely exacerbated by the development of G-B syndrome. G-B syndrome was diagnosed by nerve conduction velocity studies, electromyographic studies and a rise in acellular total protein in the cerebrospinal fluid (albumino-cytologic dissociation). In these two patients, we were able to accurately define the relationship between the onset of acute exacerbation of chronic HBV infection and G-B syndrome. The neurologic symptoms of G-B syndrome resolved with the return of liver enzymes to normal. Interferon therapy may be beneficial in relieving neurologic symptoms in patients with HB infection-related G-B syndrome. ( info) |
13/552. Encephalomyeloradiculoneuropathy following exposure to an industrial solvent. A 19-year-old male developed complaints including weakness of the lower extremities and right hand, numbness, dysphagia and urinary difficulties following a 2 month exposure to an industrial solvent constituted mainly of 1-bromopropane, but also containing butylene oxide, 1,3 dioxolane, nitromethane, and other components. Nerve conduction studies revealed evidence of a primary, symmetric demyelinating polyneuropathy. Evidence of CNS involvement came from gadolinium enhanced MRI scans of the brain, showing patchy areas of increased T2 signal in the periventricular white matter, similar scans of the spinal cord revealing root enhancement at several lumbar levels, and SSEP studies. The patient's symptoms had started to resolve following the discontinuation of the exposure, before he was lost to follow-up. Similar findings have been reported following 1-bromopropane exposure in rats. I hypothesize that this patient's symptoms may have been due to 1-bromopropane-induced neurotoxicity. ( info) |
14/552. mixed connective tissue disease associated with chronic inflammatory demyelinating polyneuropathy. We present a patient with mixed connective tissue disease (MCTD) and slowly progressing demyelinating polyradiculoneuropathy (CIDP). To our knowledge, the case described is the first reported MCTD case associated with definite CIDP. ( info) |
15/552. Polyradiculoneuritis after botulinum toxin therapy for cervical dystonia. A 40-year-old man with cervical dystonia developed an acute inflammatory demyelinating polyradiculoneuritis after botulinum toxin type A treatment. Some cases of idiopathic brachial plexopathy and polyradiculoneuritis have been reported to date. Although a causal relationship is not firmly established, the clinical temporal profile suggests a pathogenic relationship. In patients with cervical dystonia, further use of type A botulinum toxin should be considered contraindicated, and the use of another type of botulinum toxin should be taken into consideration. ( info) |
| A 60-year-old woman who presented with quadriparesis, dysarthria and dysphagia after acute febrile illness was reported. Neurological examination and electrodiagnostic study were compatible with acute polyradiculoneuropathy. Lumbar puncture revealed cerebrospinal fluid eosinophilia. Her muscle power improved after supportive treatment. ( info) |
17/552. Discontinuous-flow plasmapheresis and patent foramen ovale: a possible cause of paradoxical embolism. A 68-year-old woman was admitted for a subacute polyradiculoneuropathy and submitted to PE. A central right jugular venous access was placed after the third PE procedure due to a failing peripheral blood access. During the fourth PE, performed with a discontinuous-flow system (continuous-flow used for 3 procedures), she developed multiple embolic ischemic lesions in the left middle cerebral artery (MCA) territory. A thorough cerebrovascular screening showed only a patent foramen ovale (PFO). In our case, a possible increase of the right atrium blood pressure induced by the positive flux of the discontinuous-flow PE could have been responsible for a paradoxical embolism through the PFO. plasma exchange (PE) is used in several neurologic disorders. It is commonly thought to be a relatively safe procedure. However, a number of adverse events may occur: paradoxical embolism has not been reported to occur. ( info) |
| AIM: To describe the symptoms, signs, findings and prognosis in children suffering simultaneously from polyradiculoneuritis and myelitis. methods: Retrospective review of eight patients aged 2 to 13 years out of 210 patients with polyradiculoneuritis reported from 70 hospitals. Diagnostic criteria for polyradiculoneuritis were: flaccid paresis with loss of tendon reflexes, increased CSF protein and slowing of nerve conduction velocity. Criteria for myelitis were: severe and persistent bladder dysfunction, a sharply defined sensory level and/or evolving spastic paresis, with or without myelitic changes in spinal MRI. RESULTS: In the disease's earliest stage it was difficult to differentiate polyradiculoneuritis with myelitis from classical GBS. However, onset was often unusually rapid compared to GBS. Five patients developed a sensory level and seven suffered from severe bladder dysfunction. Four of the six children studied showed focal myelitic changes in MRI. All seven children with sufficient follow-up remained with residual paresis and significant long-term motor deficits. CONCLUSION: Due to its severe long-term prognosis, polyradiculoneuritis with myelitis must be differentiated from classical GBS. In the disease's early stage, the detection of a sensory level, severe bladder dysfunction and an unusually rapid onset can be helpful. The effect of high-dose corticosteroids is not yet clear. After the acute phase, most children require extended rehabilitation. ( info) |
19/552. P-ANCA vasculitic neuropathy with 12-year latency between onset of neuropathy and systemic symptoms. BACKGROUND: The differential diagnosis of chronic progressive multifocal asymmetric neuropathies is challenging. Vasculitic neuropathies, multifocal forms of chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathies, and asymmetric lower motor neuron disorders are important considerations. CASE PRESENTATION: We report a patient with an unusually long 12-year course of nonsystemic vasculitic neuropathy prior to the development of systemic manifestations. CONCLUSION: We discuss some of the difficulties involved in the diagnosis of chronic progressive multifocal asymmetric neuropathies. ( info) |
20/552. Longstanding ataxic demyelinating polyneuronopathy with a novel autoantibody. The authors describe the clinical course, postmortem findings, and characterization of a novel autoantibody (anti-M-phase phosphoprotein-1 [anti-MPP-1]) in a patient with a longstanding acquired demyelinating polyneuropathy and neuronopathy. Postmortem examination identified active sensory neuron degeneration, sensory axon loss, and widespread peripheral nerve demyelination. A possible pathophysiologic role of anti-MPP-1 is not yet identified. ( info) |