Multimodal evoked potentials in three siblings with mitochondrial disease.
Mitochondrial diseases are heterogeneous disorders affecting multiple systems. Here, we presented the findings of multimodal evoked potential (EP) studies of three siblings with a specific A8344G mutation of mitochondrial DNA. One of them had DM and another two had a history of encephalopathy. Visual EPs were abnormal in one patient and motor, somatosensory and brainstem auditory EPs were observed in all three patients. Our EP studies showed that the A8344G mutation of mitochondrial DNA involved multiple levels of the central nervous system even though there were no correlated symptoms. Therefore EP is an adjunct of methods to detect the functional disturbance and to screen the distribution of the involvement of the nervous system in mitochondrial diseases.
Lai
Kuan-Lin
KL
Department of Neurology, Taipei Veterans General Hospital, Taipei, Taiwan.
Liu
Chih-Yang
CY
Liu
Yo-Chuen
YC
Lin
Chia-Yi
CY
Chen
Jen-Tse
JT
Liao
Kwong-Kum
KK
Wu
Zin-An
ZA
D000328:Adult; D004272:DNA, Mitochondrial / Q000235:genetics; D016057:Evoked Potentials, Auditory, Brain Stem; D005073:Evoked Potentials, Somatosensory; D005074:Evoked Potentials, Visual; D006801:Humans; D008297:Male; D028361:Mitochondrial Diseases / Q000235:genetics* / Q000503:physiopathology*; D009154:Mutation; D011930:Reaction Time
D004272:DNA, Mitochondrial