M Maia

"Myoclonic encephalopathy of infants" (Kinsbourne 1962), "Dancing eyes, dancing feet and polymyoclonia" (Dyken and Kolar 1968 ), "Rapid irregular movements of eyes and limbs" ( Pampiglione and M. Maia 1972 ), designations which has been used to describe an acute encephalopathy of young infants affected by involuntary movements of eyes and limbs with marked ataxia and disturbance of development. Recently in Macau I have seen a four years old chinese girl who, at 11 months of age had an acute illness with "shaking of eyes and limbs" and became unable to sit. Admitted to hospital in China, CSF, CT and MRI were normal. EEG was slightly abnormal and Phenobarbital and Clonazepam were prescribed. After three months the abnormal eyes movements disappear but the girl remained ataxic ever since.

She was examined last June with a quite marked ataxia, able to stand but still unable to walking. She was alert and cooperative but her expressive language was limited to "mummy and daddy". Vanillmandelic acid was asked for (as some proportion of cases of Dancing eyes syndrome have neuroblastoma) and it was normal. She was admitted in our hospital for treatment with ACTH.

Rather high dosages were given for 47 days monitoring particularly blood pressure. The girl had some transitory side effects of ACTH but began to walk and so far no relapses have occurred. A review of literature of this peculiar encephalopathy is presented and discussed.