[Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (Melas) associated with a Fahr disease and cerebellar calcifications].
BACKGROUND
Melas syndrome is a mitochondrial disease which corresponds to the association of mitochondrial encephalopathy, lactic acidosis and stroke-like espisodes.


METHODS
The authors report the case of a 39 year-old woman presenting with hearing loss, seizures, visual field deficit, three stroke-like episodes and calcifications of the basal ganglia and cerebellar dentate nuclei. Melas syndrome was suspected and confirmed by muscle biopsy, showing ragged red fibers and the presence of an A3243G mutation of mitochondrial DNA.


CONCLUSIONS
This clinical, pathological and radiological observation shows that intracerebral calcifications may involve the dentate nuclei of the cerebellum in the Melas syndrome.
Younes-Mhenni
S
S
Service de neurologie D (Pr G. Chazot), hôpital neurologique Pierre-Wertheimer, 59 Bd Pinel 69003 Lyon, France.
Thobois
S
S
Streichenberger
N
N
Giraud
P
P
Mousson-de-Camaret
B
B
Montelescaut
M E
ME
Broussolle
E
E
Chazot
G
G
D000328:Adult; D001927:Brain Diseases / Q000150:complications; D002114:Calcinosis / Q000150:complications*; D002531:Cerebellum; D005260:Female; D006801:Humans; D017241:MELAS Syndrome / Q000150:complications* / Q000175:diagnosis