1/169. Chronic motor axonal neuropathy: pathological evidence of inflammatory polyradiculoneuropathy.Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/169. AAEM case report 4: guillain-barre syndrome. American association of Electrodiagnostic medicine.A 57-year-old woman developed rapidly progressive, symmetric, extremity weakness, facial diplegia, ophthalmoplegia, respiratory insufficiency, and sensory ataxia over a 3-week period. Electrodiagnostic studies were performed on days 6, 13, and 50 following the onset of weakness. Motor nerve conduction abnormalities were the predominant findings. Prolonged motor distal latencies, prolonged or absent F waves, and partial motor conduction blocks were present and form the diagnostic features of an acquired, demyelinating polyneuropathy. Abnormalities in sensory nerve conductions and blink reflexes were also present. guillain-barre syndrome was diagnosed prompting the initiation of therapeutic plasma exchange. The patient's clinical status continued to worsen over the next 10 days before stabilizing. Considerable improvement in extremity strength, ocular motility, and respiratory function occurred in the subsequent weeks. Well-planned and well-executed electrodiagnostic studies generate key adjunctive data to the clinical diagnosis of guillain-barre syndrome.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
3/169. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
4/169. guillain-barre syndrome following malaria.Two adult males were admitted with acute are flexic quadriplegia and bifacial and bulbar weakness 2 weeks after an acute episode of malaria, one due to Plasmodium falciparum infection (patient 1) and the other due to plasmodium vivax (patient 2). cerebrospinal fluid analysis and nerve conduction studies confirmed the diagnosis of guillain-barre syndrome (GBS). Patient 1 progressed to develop respiratory paralysis and required mechanical ventilation. He received intravenous immunoglobulins for the GBS and made a complete recovery in 6 weeks. A review of 11 cases of GBS (nine previously reported and the present two) revealed that eight patients had preceding falciparum malaria and three had vivax infection. All but two patients had distal symmetric sensory deficits. Paralysis was mild in seven cases (three due to P. vivax and four due to P. falciparum) and recovered completely in 2-6 weeks without any specific treatment. Four patients with falciparum malaria developed severe paralysis with respiratory failure, and three patients died. One patient who received intravenous immunoglobulins recovered completely (patient 1 in this report).- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
5/169. polyradiculopathy in sarcoidosis.We present three new and 14 retrospective cases of polyradiculopathy in sarcoidosis. Of these, 71% had weakness and 59% areflexia of the lower extremities, and 35% had sphincter dysfunction. Cases often were associated with central nervous system sarcoidosis. All cases involved thoracolumbar or lumbosacral roots, except a single case of cervical polyradiculopathy. Of 14 treated patients, nine improved with corticosteroids, laminectomy, or both. polyradiculopathy complicating sarcoidosis: (1) is uncommon; (2) primarily involves thoracic and lumbar roots; (3) may arise from contiguous, hematogenous, or gravitational nerve root sleeve seeding; (4) may be asymptomatic; and (5) may improve with corticosteroids. Differential diagnosis of weakness in patients with sarcoidosis should include nerve root involvement from the primary process by direct sarcoid involvement.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
6/169. Multifocal polyradiculoneuropathy and carcinoma of the thymus.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.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
7/169. 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.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
8/169. Polyradiculoneuritis with myelitis: a rare differential diagnosis of guillain-barre syndrome.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.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
9/169. 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.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
10/169. Polyneuritis cranialis with contrast enhancement of cranial nerves on magnetic resonance imaging.The disorder of multiple cranial nerve palsies without spinal cord involvement is referred to as polyneuritis cranialis (PC) and is rare. It is thought to be an acute post-infective polyneuropathy or a variant of guillain-barre syndrome. Electrophysiological evidence of demyelination has been reported, but no radiological abnormalities of the affected cranial nerves have been noted. We report a case of PC where contrast enhanced magnetic resonance imaging (MRI) showed enhancement of the peripheral segments of the oculomotor and abducens nerves. This case illustrates the utility of MRI in the assessment of cranial nerve palsies.- - - - - - - - - - ranking = 2.6666666666667keywords = nerve (Clic here for more details about this article) |
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