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Although neuropsychiatric manifestations are prominent in some patients with Wilson disease, there is little published information regarding the efficacy of liver transplantation for these patients. A 22-year-old male with advanced neurological impairment and prominent psychiatric manifestations due to Wilson disease who underwent liver transplantation is presented. After transplantation, the ceruloplasmin and copper studies normalized and eventually the Kayser-Fleischer rings disappeared. Neurological recovery was very slow and incomplete, and his behavioural and personality disorder was entirely unaffected. He committed suicide 43 months post-transplantation. A review of the small number of related published cases in the English language literature shows variable neurological recovery post-transplantation, but the course of psychiatric manifestations is virtually never described. This case suggests that one must be cautious regarding liver transplantation for Wilson disease in patients with prior psychiatric manifestations. Aggressive medical management is likely to be preferable in most cases.

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Patients with pathologically confirmed diagnoses of meningioma were examined to investigate the hypothesis that left-hemisphere lesions are smaller or declared earlier than right-hemisphere lesions. 77 patients were examined with regard to age at presentation and nature of symptoms. In addition CAT scan films of 47 patients were obtained for measurement of lesion size. There were no differences between right and left lesion groups with regard to size of lesion or age at presentation. Subgroups of patients with lesions in frontal association areas also did not differ. Some relationships were discovered between lesion location and nature of symptoms.

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We describe a patient who had asymmetrical atrophy of limb muscles and myasthenic weakness of neck, facial, and bulbar muscles. Electrophysiological tests indicated myasthenia gravis of facial muscles and changes consistent with an asymmetrical motor neuropathy as a cause of the muscle atrophy. Both conditions occurred as complications of a locally invasive thymoma, and both failed to improve after surgery and radiation but substantially improved with subsequent treatment by corticosteroids, azathioprine, and plasmapheresis. Review of the literature disclosed that several neuromuscular conditions may be associated with thymoma, the commonest being myasthenia gravis. Muscle atrophy may occur in 10% of patients who have myasthenia gravis, whether associated with or without thymoma. Its mechanism is debated, and further studies are needed, but observations in our patient suggest the atrophy is due to a motor neuropathy. Primary treatment of the thymoma by surgery, irradiation if the tumor is invasive, and immunosuppressive therapy for neuromuscular complications offers a relatively good prognosis for this group of patients.