A practical enzyme immunoassay (EIA) has been developed for the measurement of anti-acetylcholine receptor (AChR) antibodies in sera from patients with myasthenia gravis (MG). This system is based on the double antibody technique, using denervated rat muscle AChR labeled with horseradish peroxidase-linked alpha-bungarotoxin (HRP-alpha BGT). This method has the following advantages compared to conventional radioimmunoassay (RIA): (1) HRP-alpha BGT is more stable than [125I]alpha BGT and can be used for at least one year without any loss of the binding activity to AChR and enzymatic activity, (2) the procedure avoids the use of radioactive isotopes, and (3) the equipment for our EIA is more economical than that for RIA.

Adolescent↗

PubMedDate not supplied

Myasthenic neuromyopathy. An unusual neuromuscular disorder.

4 cases with an identical neuromuscular disorder are presented. The disorder was characterized by slowly progressive weakness and wasting of the proximal muscles together with either ocular or bulbar symptoms, fatigability, positive tensilon test, myasthenic response on repetitive nerve stimulations, elevated serum creatine phosphokinase, and neuropathic and myopathic muscle pathology. All the patients showed a fairly good response to anti-ChE medications and steroid administration. Serum creatine phosphokinase levels returned to normal values after steroid therapy. Myasthenia, polymyositis or other neuromuscular disorders were discussed in the differential diagnosis.

Adult↗

PubMedDate not supplied

Supranuclear paralysis preventing lid closure in amyotrophic lateral sclerosis.

We report a case of amyotrophic lateral sclerosis (ALS) in which the ability to close the eyes on command or voluntarily, was lost in spite of retention of reflex activity. A electrophysiological study of the blink reflex revealed a prominent R1 component with normal latency, which confirmed that the blink reflex was exactly preserved and also suggested a hemispherical lesion. Postmortem examination disclosed prominent cortical and subcortical lesions of the precentral areas on both sides. These lesions seem to be very closely related to the inability to initiate lid closing.

Adult↗