1/12. Can immunoadsorption plasmapheresis be used as the first choice therapy for neuroimmunological disorders?The subjects were 31 patients in whom immunoadsorption plasmapheresis (IAPP) was performed as the first choice therapy for primary or recurrent neuroimmunological disorders. The clinical manifestations before and after IAPP and the use of corticosteroids were investigated in the present study. IAPP was clinically effective in all patients. The corticosteroids (CSs) administration was begun or CSs were increased after completion of IAPP in 11 patients. IAPP was performed as the first choice therapy, and favorable results were obtained in patients with guillain-barre syndrome and Miller-Fisher syndrome. IAPP alone was also effective in a patient with lupoid sclerosis. When IAPP was used alone in 2 patients with chronic inflammatory demyelinating polyradiculoneuropathy, it completely eliminated the clinical manifestations, but the symptoms recurred about 2 months later. Therefore, although IAPP could be performed as the first choice therapy for many neuroimmunological disorders, subsequent therapies should be carefully investigated.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
2/12. 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 = neuropathy (Clic here for more details about this article) |
3/12. 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 = 5keywords = neuropathy (Clic here for more details about this article) |
4/12. miller fisher syndrome: an uncommon acute neuropathy.The syndrome of ophthalmoplegia, ataxia, and areflexia was first described in 1956 by Miller Fisher. This syndrome has long been believed to be a variant of guillain-barre syndrome (GBS), mainly because of its areflexia, cerebrospinal fluid findings, and its postinfectious presentation. The case of an 11-year-old male with miller fisher syndrome (MFS) is described. MFS differs from GBS in several key clinical features and presents an extensive and challenging differential diagnosis. It is useful to recognize MFS as both a variant of GBS and a distinct entity with its own therapeutic considerations.- - - - - - - - - - ranking = 4keywords = neuropathy (Clic here for more details about this article) |
5/12. Features of sensory ataxic neuropathy associated with anti-GD1b IgM antibody.Some reports have called sensory ataxic neuropathy (SAN) associated with IgM antibody against b-series gangliosides a chronic form of miller fisher syndrome (MFS), but this has yet to be established. We examined five patients with SAN and eight patients with IgG anti-GQ1b-positive MFS. Only one patient with SAN complained of diplopia, whose ocular movement was not limited. The other four patients had neither diplopia nor limitation of ocular movement. All the SAN patients had severe deep sense impairment, whereas one patient with MFS showed only mild vibratory sense impairment. All sera from the SAN patients had remarkably high IgM antibody titers to the b-series gangliosides GD3, GD2, GD1b, GT1b, GQ1b, GQ1b alpha, fucosyl-GD1b, and alpha galactosyl [alpha fucosyl] GD1b. An absorption study confirmed that the anti-GQ1b antibodies cross-reacted with GD3, GD2, GD1b, and GT1b. In contrast, only two samples from the MFS patients had IgG antibody to GD3, and no sample reacted with GD2, GD1b, or GT1b. SAN has different clinical or serological features from MFS, and therefore is not a chronic form of it.- - - - - - - - - - ranking = 5keywords = neuropathy (Clic here for more details about this article) |
6/12. Visual impairment in anti-GQ1b positive miller fisher syndrome.Autoimmune neuropathies such as the guillain-barre syndrome (GBS), the miller fisher syndrome (MFS), and chronic inflammatory demyelinating neuropathy (CIDP) have conventionally been considered diseases exclusively of the peripheral nervous system. In the last decades, however, several reports of CNS involvement in peripheral neuropathy have challenged this view. We describe a patient with anti-GQ1b positive MFS who--apart from the classical features--also presented with reversible loss of visual acuity suggesting CNS involvement.- - - - - - - - - - ranking = 2keywords = neuropathy (Clic here for more details about this article) |
7/12. 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 = 6keywords = neuropathy (Clic here for more details about this article) |
8/12. 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 = 6keywords = neuropathy (Clic here for more details about this article) |
9/12. Miller-Fisher syndrome mimicking intracranial hypertension following head trauma.INTRODUCTION: Miller-Fisher syndrome (MFS) is a polyneuropathy with benign outcome characterized by ophthalmoplegia, limb ataxia and tendon areflexia. Impaired consciousness level and intracranial hypertension are very rare symptoms in MFS. CASE REPORT: We describe the case of a 5-year-old girl who showed intracranial hypertension, transient coma and respiratory failure after mild head injury; moreover the patient showed mild ataxia, areflexia, ophthalmoplegia and autonomic disturbances. These symptoms were suggestive of MFS. Electrophysiologic studies and laboratory tests confirmed the diagnosis and immunoglobulins and steroids were given. The child showed a progressive clinical improvement and the final outcome was good. CONCLUSION: This case, initially managed as trauma injury due to the presence of suggestive signs and clinical history, maskered an atypical presentation of Miller-Fisher syndrome, a rare disorder of central nervous system.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
10/12. Electrophysiological evidence by single fibre electromyography of neuromuscular transmission impairment in a case of miller fisher syndrome.miller fisher syndrome is an autoimmune neuropathy characterised by ataxia, areflexia and ophthalmoplegia, with minimal if any limb weakness, and in the majority of cases by high titres of IgG anti-GQ1b ganglioside antibodies. in vitro electrophysiological experiments have demonstrated that these antibodies induce a transmission blockade at neuromuscular junction either pre- or post-synaptically. We report the case of a 63-year-old man with MFS that shows blood serum negative for anti-GQ1b but presents an impairment of neuromuscular transmission detected by single fibre electromyography. To the best of our knowledge, this represents the first case in the literature using jitter technique and suggests that other antibodies may be involved in the function of motor end plates by bindings to the synaptic membranes.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
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