1/19. Fisher syndrome with tetraparesis and antibody to GQ1b: evidence for motor nerve terminal block.A Fisher syndrome (FS) patient with antibody to tetrasyaloganglioside GQ1b (GQ1b) developed late limb weakness. Serial motor conduction velocities (MCVs) showed a marked reduction of distal compound muscle action potential (CMAP) amplitudes, worse at 2-3 weeks, followed by a dramatic increase in week 5. Motor conduction velocities were always in the normal range, distal motor latencies changed only slightly, and conduction block in intermediate nerve segments was absent. These electrophysiological data might suggest an axonal neuropathy or a distal demyelinating conduction block. However, the dramatic increase of distal CMAP amplitudes over a short time without significant changes of distal motor latencies, CMAP duration, and morphology indicate that weakness in this FS patient might be due to a block of acetylcholine release from motor terminals, possibly mediated by anti-GQ1b antibodies.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/19. IgG anti-GQ1b positive acute ataxia without ophthalmoplegia.IgG anti-GQ1b antibody was present in a patient with acute ataxia and areflexia without ophthalmoplegia or elementary sensory loss. Sensory nerve conduction studies and somatosensory evoked potentials were normal, but postural body sway analysis showed dysfunction of the proprioceptive afferent system. The clinical presentation and laboratory results for this patient resemble those of miller fisher syndrome, except for the lack of ophthalmoplegia. This case may represent part of an IgG anti-GQ1b syndrome.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
3/19. magnetic resonance imaging enhancement of cranial nerves in inflammatory bulbar polyneuropathy.A patient with generalized inflammatory polyneuropathy and facial diplegia was studied with magnetic resonance imaging. Multiple cranial nerves showed signal enhancement without sensory or motor dysfunction.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/19. Acute onset of a bilateral areflexical mydriasis in Miller-Fisher syndrome: a rare neuro-ophthalmologic disease.Miller-Fisher syndrome (MFS) is characterized by variable ophthalmoplegia, ataxia, and tendon areflexia. It seems to be a variant of guillain-barre syndrome (GBS), but unlike in GBS, there is a primitive involvement of the ocular motor nerves, and in some cases there is brainstem or cerebellum direct damage. The unusual case of MFS in the current study started with a bilateral areflexical mydriasis and a slight failure of accommodative-convergence. Ocular-movement abnormalities developed progressively with a palsy of the upward gaze and a bilateral internuclear ophthalmoplegia to a complete ophthalmoplegia. In the serum of this patient, high titers of an IgG anti-GQ1b ganglioside and IgG anti-cerebellum. anti-purkinje cells in particular, were found. The former autoantibody has been connected to cases of MFS, of GBS with associated ophthalmoplegia, and with other acute ocular nerve palsies. The anti-cerebellum autoantibody could explain central nervous system involvement in MFS. The role of these findings and clinical implications in MFS and in other neuro-ophthalmologic diseases are discussed.- - - - - - - - - - ranking = 0.4keywords = nerve (Clic here for more details about this article) |
5/19. Acute ophthalmoparesis (without ataxia) associated with anti-GQ1b IgG antibody: clinical features.OBJECTIVE: To examine the clinical features of acute ophthalmoparesis (AO) (without ataxia) associated with anti-GQ1b immunoglobulin g (IgG) antibody. DESIGN: Retrospective observational case series. PARTICIPANTS: Twenty-one subjects with AO (without ataxia) who had anti-GQ1b IgG. methods: Clinical features of 21 subjects with AO were analyzed. RESULTS: Seventeen had symptoms of antecedent infection. Gaze limitation was bilateral in 16 subjects and unilateral in five, indicative that laterality does not always negate AO. Nine subjects showed abducens paresis, and two limitation of abduction and adduction. Eight, who initially had bilateral abducens palsy, subsequently had impairment of adduction and vertical movement. These showed that bilateral abducens palsy followed by oculomotor nerve involvement is characteristic of AO. Muscle stretch reflexes were normal in nine subjects, hypoactive in eight, absent in three, and brisk in one. Distal paresthesias were present in seven subjects. Acellular cerebrospinal fluid (CSF) associated with raised protein concentration was detected in three. CONCLUSIONS: Antecedent infectious symptoms, characteristic limitation of ocular movement, areflexia, distal paresthesias, and CSF albuminocytologic dissociation are useful markers for diagnosing AO as well as anti-GQ1b IgG. AO can be considered a mild form of miller fisher syndrome or a regional variant of guillain-barre syndrome.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
6/19. transcranial magnetic stimulation studies in the miller fisher syndrome: evidence of corticospinal tract abnormality.OBJECTIVES: To evaluate serial central motor conduction time in the miller fisher syndrome. METHOD: Three patients with classic miller fisher syndrome were evaluated clinically. They had serial central motor conduction times measured with transcranial magnetic stimulation and nerve conduction studies. Motor evoked potentials were recorded from the first dorsal interossei and abductor hallucis muscles. RESULTS: All three patients showed reduction in central motor conduction times in tandem with gradual clinical improvement at each review. CONCLUSIONS: There is electrophysiological evidence of a central reversible corticospinal tract conduction abnormality in the miller fisher syndrome.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
7/19. Autoimmune thyroiditis and acquired demyelinating polyradiculoneuropathy.guillain-barre syndrome (GBS) and Miller-Fisher syndrome (MFS) are variant forms of acquired demyelinating polyradiculoneuropathy. Their concurrence with immune disorders of the thyroid is infrequent. We report on a 7.5-year-old girl in whom a subclinical thyroiditis was concurrently detected to GBS and a 70-year-old woman with Hashimoto's thyroiditis (HT) who had recurrent MFS. Even though autoimmune thyroiditis is associated with many autoimmune disorders more often than would be expected by chance alone, its concurrence with immune disorders of the peripheral nerve is less frequently reported. The calculated coincidental concurrence of acquired demyelinating polyradiculoneuropathy (in both variants, MFS and GBS) and autoimmune thyroiditis (as in the present cases) was extremely low (0.0004%), thus suggesting common pathogenic mediators.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
8/19. miller fisher syndrome with transient coma: comparison with Bickerstaff brainstem encephalitis.We herein report a 4-year-old boy with miller fisher syndrome (MFS) who presented with transient coma in addition to the typical triad of internal and external ophthalmoplegia, cerebellar ataxia and areflexia after an influenza type B infection. The electroencephalogram findings revealed intermittently generalized slow wave bursts. The cerebrospinal fluid revealed high protein and a lack of any cellular response. The serum anti-GQ1b IgG antibody was elevated in the acute phase and disappeared in the convalescent phase. The transient coma with the triad of MFS in this patient indicated an extended brainstem lesion including a reticular formation, which is also the responsible lesion of Bickerstaff brainstem encephalitis (BBE), but the magnetic resonance imaging repeatedly showed no abnormal finding. Our patient suggested the involvement of central nervous system in addition to the peripheral nerve injury in MFS. He also suggested that MFS and BBE may belong to the same group of disorders as syndrome of ophthalmoplegia, ataxia and areflexia (SOAA).- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
9/19. Clinical, electrophysiological, and serological overlap between miller fisher syndrome and acute sensory ataxic neuropathy.We report a patient with severe sensory ataxia, areflexia, and ophthalmoplegia with preservation of limb muscle strength. Electrophysiological examinations revealed peripheral sensory nerve involvement. A serological examination showed the elevation of IgG antibodies to various b-series gangliosides as well as GT1a. These indicated that this case is an overlap between acute sensory ataxic neuropathy and miller fisher syndrome. Autoantibody is implicated as potential pathogenic agents in some cases of acute sensory ataxic neuropathy.- - - - - - - - - - ranking = 0.2keywords = nerve (Clic here for more details about this article) |
10/19. Involvement of the central nervous system in miller fisher syndrome: a case report.miller fisher syndrome (MFS) is characterised by ophthalmoplegia, ataxia and areflexia. Reports on cerebellar ataxia and supranuclear oculomotor derangement in MFS suggested an additional involvement of the central nervous system (CNS), resembling Bickerstaff's brainstem encephalitis (BBE). In the present report, a patient with a monophasic acute illness, early recovery and specific clinical-laboratory findings suggested both intrinsic brainstem and peripheral nerve disease (MFS and BBE). In pons and medulla oblangata, blurred to discrete T2-lesions were revealed by cranial MRI, while involvement of peripheral nerves was detected with EMG. The CSF showed no increase in protein or cell content, such as occurs in brainstem encephalitis.- - - - - - - - - - ranking = 0.4keywords = nerve (Clic here for more details about this article) |
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