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About:
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According to Orphanet is “a potentially fatal neurological disease characterised by neuropathological lesions principally involving the brainstem, thalamus and putamen. Onset occurs during early childhood, typically a few days after a febrile illness (these include chicken pox, rubella and HSV6, to name a few). Manifestations include vomiting, seizures, spasticity, language regression, rigidity and abnormal posturing of the head. Residual neurologic impairment (muscle weakness, speech disturbance, intellectual deficit and mood disorders) persists in some patients. The disease is chronic in one out of two cases.” |
