Myoclonic cerebellar coordination disorder

Myoclonic cerebellar coordination disorder (dyssynergia cerebellaris myoclonica) was first described by Ramsay Hunt (1921), so it is also called Ramsay Hunt syndrome (RHS). However, the clinical, pathological, and etiology of many cases reported by RHS in the future are very inconsistent. This disease is an autosomal recessive inheritance, which is classified according to the form of gene transmission in the hereditary ataxia of the spinal cord cerebellum type, and its etiology is unknown. The Marseille Collaboration Group (1990) has suggested that this name should not be used, and according to previous reports, the clinical manifestations of patients are divided into two categories: 1. Progressive myoclonic epilepsy (PME) manifested as myoclonic epilepsy with epilepsy Sexual convulsions, progressive exacerbations, especially ataxia and dementia. 2. Progressive myoclonic ataxia (PMA) is a group of clinical syndromes with myoclonus, progressive cerebellar ataxia, occasional or no seizures, and cognitive impairment. However, it was pointed out that the clinical symptoms between these two types of syndrome still partially overlap.