Repetitive Nerve Stimulation Test in Myotonic Patients

In 17 myotonic cases (16 of myotonic muscular dystrophy and 1 of congenital myotonia), 13 cases showed significant decremental responses on RNS study. The decremental responses had the tendency of more prominence on faster stimulating rate, and in the proximal limb muscles. This result suggests that the RNS test is useful but not specific for the disorder of neuromuscular junction.

Two Cases of Chronic Idiopathic Ataxic Sensory Neuropathy

Sensory neuropathy is characterized by pure sensory dysfunction, ultimately evolving into areflexia and severe sensory ataxia in spite of the preservation of the other functions in the nervous system such as motor function. This disorder is known to be commonly found in cancer patients as a paraneoplastic syndrome, but nowadays many reports are made that sensory neuropathy is not merely related to malignancy but also with autoimmune disorders such as Sjogren's syndrome or polyclonal gammopathy, etc. Hence we report two patients who have been diagnosed as chronic idiopathic ataxic sensory neuropathy through electrophysiological and histopathological studies without any evidence of cancer or any other known cause.