[X chromosome-linked bulbospinal neuronopathy in adults].

Two cases of the X-chromosome-linked adult bulbospinal neuronopathy are reported. Patients displayed major signs of the disease: the onset at adult age, slowly progressing involvement of limb, facial and tongue muscles, light bulbar disorders, generalized fasciculation and neuronal signs in EMG investigation. Observed were also bilateral gynecomastia, fascicular tremor. Clinical differences in the 2 patients could be accounted for by different expression of the pathological gene.