When it comes to PME, people basically shake their heads and think it’s a dead end, but there are still some PMEs that are on the benign side and don’t even qualify as PMEs, such as some types of RHS, also known as PMA. In an article entitled “Myoclonic cerebellar coordination failure – primary dentate nucleus atrophy ” describes four disseminated cases and one twin. The twin brothers had a presentation similar to Friedreich’s ataxia in early childhood and developed myoclonus and epilepsy around the age of 30. One of them died at the age of 36, and autopsy showed degeneration of the spinal cerebellar tract and posterior cord, atrophy of the dentate nucleus, and thinning of the superior cerebellar peduncle. As a result, Hunt suggested a specific relationship between myoclonus and cerebellar ataxia and postulated that primary atrophy of the cerebellar dentate nucleus system was the pathological basis of myoclonus and cerebellar ataxia, and subsequently, many similar cases named after Ramsay Hunt were reported. However, the question of whether RHS is a disease entity or a syndrome has been the subject of recent debate, and it has been clinically difficult to distinguish it from one of the types of progressive myoclonic epilepsy (PME), the In 1990, the Marseille Collaborative Group divided RHS into two main categories, PME and progressive myoclonic ataxia (PMA). PMA refers to myoclonic, progressive myoclonic ataxia with infrequent seizures.