The diagnostic sensitivity of three laboratory tests [serum antiacetylcholine receptor antibody (AChR-ab) assay, the repetitive nerve stimulation (RNS) test, and, the single fiber EMG (SFEMG)] for myasthenia gravis (MG) was compared in 120 patients. In all cases, at least one of the tests was abnormal. SFEMG was the most sensitive test, being abnormal in 92% of cases, followed by the RNS test (77%) and the AChR-ab assay (73%). SFEMG was abnormal in all cases with negative AChR-ab and RNS tests, in 97% of cases with negative AChR-ab assay, in 89% of cases with negative RNS test, and in 89% of cases with mild MG. We conclude that one of these three tests is abnormal in all cases of MG, and that the SFEMG is most sensitive in the diagnosis of MG.
The clinical electrophysiological and histological features of 10 cases of "chronic sensory demyelinating neuropathy" (CSDN) are reported. This entity is characterised by: 1) subacute or chronic progression; 2) pure sensory neuropathy; 3) high spinal fluid protein in the majority of cases; 4) electrophysiological evidence of demyelination affecting motor as well as sensory nerve fibres; 5) demyelination on sural nerve biopsy and 6) good response to immunotherapy in progressive phase. It is believed that this entity represents chronic inflammatory demyelinating polyneuropathy (CIDP) presenting as pure sensory neuropathy. (7 Neurol Neurosurg Psychiatry 1992;55:677-680) the distal value (fig 1). Abnormal temporal dispersion was considered to be present when the CMAP shape was abnormal with multiple phases (more than four) and total duration longer than three standard deviations above the normal mean value (fig 1).3 In five patients, the plantar nerves were studied using the nearnerve needle sensory nerve conduction technique.4 Needle EMG examination was carried out in all cases.Sural nerve biopsy was performed in eight cases. Frozen sections were stained with modified trichrome, cresyl-fast violet, hematoxylin and eosin, and crystal violet stains. Paraffin sections were stained with hematoxylin and eosin and congo-red stains. Semithin EM sections in three cases were stained with toluidine blue. Teased nerve preparations were studied in four cases.To compare the degree of sensory impair-
The existence of chronic "limb-girdle" form of myasthenia gravis (MG) has been questioned. We report here 12 such patients (10 women and two men) who constituted 3.8% of 314 MG patients in our study. The duration of disease ranged from 4 months to 7 years before the diagnosis. In almost all cases, the initial diagnosis was other than MG. None of the patients had any oculobulbar weakness. Acetylcholine receptor antibody was positive in five cases, although not all in the first assay. Repetitive nerve stimulation test was positive in all cases, although not necessarily the first time. Single-fiber EMG was positive in 11 cases. All patients responded to acetylcholinesterase inhibitors, and two thirds underwent immunotherapy. Diagnosis of limb-girdle MG requires a strong index of suspicion.
Although the reinnervation capacity was notably higher in the ADM of patients with ALS, the lack of a significant difference in CutSP measurements between ADM and FDI argues against spinal excitability changes in the development of split-hand syndrome. Muscle Nerve 58: 503-508, 2018.
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