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Adult-onset, autosomal dominant myopathy with limb-girdle distribution in a Chinese family is reported. Nine members were affected with characteristic proximal weakness and pelvifemoral-preceded scapulohumeral involvement. The facial, extraocular and bulbar muscles were spared. Rimmed vacuoles were the most obvious characteristics in the myopathologic study of the biopsy specimens from four patients. In a review of the previous literature, we found three reported families with clinical and myopathologic features identical to ours, and these families are viewed as a distinct subgroup of myopathy.

Cryptococcal infection presenting primarily as lumbosacral polyradiculopathy is rare. We report on a 57-year-old man with lumbosacral polyradiculopathy, and in which a culture from the cerebrospinal fluid grew Cryptococcus neoformans. A serum cryptococcal antigen study showed a positive reaction. Biopsy specimens from the spinal nerve rootlet showed evidence of arachnoiditis and direct involvement of the nerve root by Cryptococcus neoformans. It is important to remember that localized lumbosacral polyradiculopathy can be the sole initial manifestation of cryptococcosis.

Migration disorders are congenital brain malformations. These anomalies may be overlooked in CT studies. We present four cases with migration disorders. All have both CT and MRI studies. Comparison of the images of CT and MRI at corresponding levels is demonstrated. The superiority of MR images to CT in delineating the migration and sulcation disorders is discussed.

We report the case of a 27-year-old woman who has had mirror movements in both hands since childhood. These synkinetic involuntary movements impair her performance in bimanual activities such as buttoning clothes, tying shoes, and washing dishes. Cranial magnetic resonance imaging disclosed only mild diffuse cortical atrophy. Although congenital mirror movements have been well-documented in the English literature, we herein report the first case of a Chinese patient in Taiwan.

We report a patient who developed a delayed onset of oculopalatal myoclonus, characterized by synchronous oscillatory movements of the eyeballs, soft palate and the orbicularis oculi muscle, 5 months after a pontine hemorrhage. The involuntary oscillations did not affect the speech or swallowing but continued in sleep with decreased amplitudes. These oscillations also failed to respond to clonazepam therapy and the patient was unable to voluntarily suppress them for several minutes. A temporary association in synchronous titubation of the head was also observed. A characteristic vertical ocular oscillation was demonstrated in an oculogram. This is in accordance with the midline form of oculopalatal myoclonus.