Recently, Berkovic et al. (1) criticized the concept of the Ramsay Hunt syndrome. In their excellent review of progressive myoclonus epilepsies, they concluded that the Ramsay Hunt syndrome "does not represent a specific disease, and its use should now be abandoned." They were led to this view by the belief that this entity "has no agreed-upon definition" and by their experience that, with modem investigations, a specific diagnosis may be achieved in all such cases. More recently, the same authors expanded on this theme under the provocative title Mitochondrial Encephalomyopathies: A Solution to the Enigma of the Ramsay Hunt Syndrome (2). They described 11 cases previously diagnosed as having the Ramsay Hunt syndrome (among over 70 patients with progressive myoclonus epilepsy). Two had died; the remaining nine patients included three sporadic cases and a family with six affected members. Reevaluation of these nine living patients showed evidence of mitochondrial abnormalities in all (ragged-red fibers on muscle biopsy in eight and abnormal mitochondria in a skin biopsy in the remaining case). Berkovic et al. (2) again concluded that the "Ramsay Hunt syndrome is no longer a useful diagnostic category. It is largely, and in our experience, completely accounted for by the mitochondria1 encephalomyopathies."
However, we believe that the burial of the Ramsay Hunt syndrome proposed by Berkovic et al. is premature, and that their funeral oration may have led to the loss of a useful clinical concept. Here we state the alternative view.
MITOCHONDRWL ENCEPHALOMYOPATHIES MAY NOT ACCOUNT FOR ALL CASES OF RAMSAY HUNT SYNDROME
We have evaluated 10 cases of the Ramsay Hunt syndrome (for definition, see below) in which there was no evidence of mitochondrial disease. Seven had mus-Address correspondence and reprint requests to C.