Symptomatische infantile Polymyoklonie

Abstract

The chief symptom of the so called infantile polymyoclonic encephalopathy is ataxia in combination with myoclonie jerks generalized as well as localized in the orbital muscles ("opsoelonus" ; "dancing eyes"). 2 cases of infantile polymyoclonus are reported, one seen in tile course of an acute meningoencephalitis which recovered spontaneously within a few weeks; the other occured with a neuroblastoma of the mediastinum. In this child the myoelonie symptoms did not disappear with the surgical removal of the tumour, followed by cytostatic treatment and radiation. Amelioration was seen after ACTH and Mogadan, but mild ataxia was still present after 6 months' treatment. 1 year later the motor symptoms had disappeared completely but the static and language development were retarded.The aetiology of infantile polymyoclonus is still unknown. Immuno-pathological reactions to different functional disturbances of certain cerebellar and brain stem structures are discussed.

Polimioclonia-opsoclono: síndrome de Kinsbourne. Relato de um caso

“…É patologia de início agudo 1 » 2 » 4 » 8 ou subagudo 1 , precedida em um terço dos casos de infecção das vias aéreas superiores ^1 2 , gastrenterite 12 , doença infecciosa de qualquer natureza £ ou meningoencef alite 6 . Em 48% dos casos está relacionada a neuroblastoma 1 » 12 , sendo que 49% destes neuroblastomas são mediastínicos Quanto ao grupo etário, nosso paciente, com 9 anos de idade é exceção.…”