1/25. Chronic demyelinating hypertrophic brachial plexus neuropathy.A patient with unilateral, painless, chronic progressive upper limb sensorimotor deficit showed electrophysiological evidence of a focal demyelinating neuropathy with almost complete conduction block across the brachial plexus. magnetic resonance imaging disclosed marked brachial plexus hypertrophy. Intravenous immunoglobulin led to fast and complete recovery, maintained by intermittent perfusions. Hypertrophic brachial plexus neuropathy can be a presentation of focal chronic inflammatory demyelinating polyradiculoneuropathy. Objective and quantitative assessment of hand function is useful to evaluate treatment results and to optimize treatment regimens.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, polyradiculoneuropathy, demyelinating (Clic here for more details about this article) |
2/25. Trovafloxacin-induced weakness due to a demyelinating polyneuropathy.fluoroquinolones have been associated with peripheral sensory disorders and weakness, especially in patients with underlying myasthenia gravis or myasthenia-like Eaton-Lambert syndrome. Trovafloxacin is a relatively new quinolone for which these side effects have not been described. We report a case of diffuse weakness due to a demyelinating polyneuropathy that began after initiation of trovafloxacin in a patient without an underlying neurologic disorder.- - - - - - - - - - ranking = 0.069713539033526keywords = demyelinating (Clic here for more details about this article) |
3/25. Predominant arm weakness in acute idiopathic polyneuritis: a distinct regional variant.Acute idiopathic polyneuritis is a heterogeneous disorder with a number of clearly established variants. We describe four patients who present with an acute neuropathy predominantly affecting the arms which we believe should be considered as a distinct variant of acute polyneuritis. In two cases a primary demyelinating process affecting both motor and sensory nerves is suggested whereas in the other two the features were of a pure motor axonal degeneration. The relationship between these neuropathies, other variants of acute idiopathic polyneuritis and multifocal motor neuropathy is considered.- - - - - - - - - - ranking = 0.013942707806705keywords = demyelinating (Clic here for more details about this article) |
4/25. Massive intravenous immunoglobulin treatment in pregnancy complicated by guillain-barre syndrome.A pregnant woman developed acute demyelinating poly-neuropathy (guillain-barre syndrome (GBS)) in the 28th week of gestation (GW) after flu-like infection. hypertension, liver dysfunction, and a decrease in consciousness level developed at 29GW. blood chemical analysis revealed increased levels of liver enzymes GOT 247 IU/l and GPT 624 IU/l. Viral serological study showed a positive test for Epstein-Barr virus IgM. Weakness of bilateral facial muscles and limbs, a loss of tendon reflexes, and generalized paresthesia were detected by neurologic examinations. Over the course of 5 days, a massive dose (100g) of intravenous immunoglobulin (MIVIg) was infused in 30GW. An average manual muscle testing score by the Medical research Council method and peak flow value began to be significantly restored during and after MIVIg infusions. Values of the liver enzymes gradually decreased, and improvement of the muscle weakness and dysbasia was observed. Her pregnancy normally ended in spontaneous vaginal delivery of a healthy infant in 37GW. This is the first report confirming the efficacy of MIVIg, without plasmapheresis, in GBS-complicated pregnancy.- - - - - - - - - - ranking = 0.013942707806705keywords = demyelinating (Clic here for more details about this article) |
5/25. Neurolymphomatosis associated with muscle and cerebral involvement caused by natural killer cell lymphoma: a case report and review of literature.We report a biopsy-proven case of neurolymphomatosis (NL) presenting with sensory motor axonal polyneuropathy, polymyositis, and cerebral involvement. Ours is the second reported case of NL caused by natural killer-cell lymphoma defined by morphology and immunophenotyping. For 3 months, the patient developed stocking-glove distribution of hypesthesia, subacute progressive weakness and mental deterioration. EMG showed severe sensorimotor mixed axonal-demyelinating polyradiculoneuropathy. Lumbar puncture revealed mildly high protein level with normal glucose and cell count. sural nerve biopsy demonstrated lymphomatous axonal neuropathy and muscle biopsy was indicative of lymphomatous polymyositis. brain MRI revealed multiple white matter lesions, consistent either with progressive multifocal leukoencephalopathy or cerebral lymphoma. bone marrow biopsy showed neoplastic infiltrates. The patient died of multiple organ failure prior to initiation of chemotherapy.- - - - - - - - - - ranking = 0.1696804369931keywords = polyradiculoneuropathy, demyelinating (Clic here for more details about this article) |
6/25. Motor dominant neuropathy in sjogren's syndrome: report of two cases.Most of the peripheral neuropathies in sjogren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of guillain-barre syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating polyradiculoneuropathy. These patients received i.v. immunoglobulin therapy. To our knowledge, this is the first report indicating that i.v. immunoglobulin has beneficial effects on motor dominant neuropathy in SS.- - - - - - - - - - ranking = 0.35756208530429keywords = inflammatory demyelinating, polyradiculoneuropathy, demyelinating (Clic here for more details about this article) |
7/25. The "spray can" sign: validation of a clinical observation in chronic inflammatory demyelinating polyneuropathy.BACKGROUND: The presentation of chronic inflammatory neuropathies is variable. The decision regarding when to intervene with treatment is ideally determined by identifying early markers of loss of function. OBJECTIVE: To test the hypothesis that an observation of functional impairment, defined by a patient with demyelinating neuropathy, can be used as a reproducible and reliable measure of improvement with intravenous immune globulin. DESIGN: A 28-year-old woman presented with a chronic inflammatory demyelinating polyneuropathy. Her first complaint was the inability to use her deodorant spray because of hand weakness. A calibrated pincer gauge fixed on top of her usual spray can was used to objectively test finger flexion. Tip grip and lateral pinch were also measured. A calibrated dynamometer was used to measure grip strength. RESULTS: Power and precision grip force were reproducible in normal control subjects by means of the spray can test. This test proved to be a reliable indicator of reduced muscle strength in the patient and improved after treatment with intravenous immune globulin. CONCLUSIONS: The spray can test objectively quantified the daily function, nominated by the patient, of operating an aerosol can. This measurement, drawn from a functional loss observed by the patient, proved to be a portable and reliable indicator of decline and recovery in chronic inflammatory demyelinating polyneuropathy.- - - - - - - - - - ranking = 4.6612350984872keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
8/25. 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.- - - - - - - - - - ranking = 0.77454873178008keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
9/25. Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement.A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.- - - - - - - - - - ranking = 5.5817187657988keywords = chronic inflammatory demyelinating, inflammatory demyelinating, polyradiculoneuropathy, demyelinating (Clic here for more details about this article) |
10/25. Inflammatory demyelination in a patient with CMT1A.We report a case of charcot-marie-tooth disease (CMT), with identified PMP22 gene duplication (CMT type 1A), and with evidence of an inflammatory demyelinating process superimposed on the course of the chronic genetic disease. Macrophage-associated demyelination was observed on the peripheral nerve biopsy. This observation supports some experimental data from the literature and shows that there may be a genetic susceptibility to inflammatory demyelinating processes in certain CMT kindreds.- - - - - - - - - - ranking = 0.40364871223579keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
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