Cerebellar dysfunction

Cerebellar dysfunction: dyssynergia cerebellaris myoclonica is a clinical syndrome characterized by myoclonus, epilepsy, and cerebellar ataxia. It was first reported by Ramsay-Hunt in 1921, so it is also called Ramsay-Hunt syndrome. Myoclonic cerebellar coordination disorder is an autosomal dominant hereditary disease, and it is common in siblings, but there are also sporadic cases, OMIM: 159700. Gilbert observed that the genetic pattern in the families of patients with myoclonic cerebellar coordination disorder cannot be fully explained by the rule of autosomal dominant inheritance, so it is thought that it may be caused by autosomal dominant inheritance and apparent incompleteness. In recent years, there has been much controversy about whether myoclonic cerebellar coordination disorder is a disease entity or a syndrome. In 1990, the Marseille collaboration group divided myoclonic cerebellar coordination disorder into two categories, namely progressive myoclonus Progressive myoclonic epilepsy (PME) and progressive myoclonie ataxia (PMA). PME refers to myoclonus with seizures and progressive neurological decline, such as mild ataxia and dementia; PMA refers to myoclonus, progressive cerebellar ataxia, and seizures are not frequent. Its clinical manifestations are impairment of cerebellar function.