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Effect of d-penicillamine on neuromuscular junction in patients with Wilson disease

The clinical, serologic, and electrophysiologic features of myasthenia gravis (MG) associated with long-term use of d-penicillamine are indistinguishable from acquired autoimmune MG.1,2⇓ Most reports involve patients with rheumatoid arthritis (RA) who have underlying autoimmunity. We investigated the effect of d-penicillamine on the neuromuscular junction and the presence of acetylcholine receptor (AChR) antibody in the sera of patients with Wilson disease (WD).

Sixty prospective patients were recruited from a large cohort of WD patients from a university hospital of south India. These patients were on d-penicillamine for at least 6 months (Group I, n = 40) or drug-naive (Group II, n = 20). d-Penicillamine was started among Group II patients, and they were re-evaluated after 6 months. Patients on drug(s) that could induce or aggravate MG were excluded.

Details regarding history and clinical features were noted. Bedside fatigability tests (ptosis, diplopia, sit-ups, arm abduction, voice fatigability) were carried out in all patients. Repetitive nerve stimulation test study …