ADULT ONSET MYOCLONIC HUNTINGTONS-DISEASE

被引：28

作者：

CARELLA, F

SCAIOLI, V

CIANO, C

BINELLI, S

OLIVA, D

GIROTTI, F

机构：

[1] IST NAZL NEUROL C BESTA,PRIMA DIV NEUROL,VIA CELORIA 11,I-20133 MILAN,ITALY

[2] IST NAZL NEUROL C BESTA,SERV NEUROFISIOL,I-20133 MILAN,ITALY

来源：

MOVEMENT DISORDERS | 1993年 / 8卷 / 02期

关键词：

HUNTINGTONS DISEASE; MYOCLONUS;

D O I：

10.1002/mds.870080216

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

A patient with adult onset Huntington's disease (HD) and prominent action myoclonus is described. Neither epileptiform activity nor electroencephalography (EEG) correlates of the movements was found. Unlike the case with most (nonmyoclonic) HD patients, centro-parietal components of somatosensory evoked potentials (SEPs) were well defined and a clear V2 response was found. Treatment with valproic acid greatly reduced myoclonus suggesting that the gamma-aminobutyric acid (GABA) system might be involved in the pathophysiology of myoclonus in HD.

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页码：201 / 205

页数：5

相关论文

共 30 条

[1] ABNORMALITIES OF PARIETAL AND PREROLANDIC SOMATOSENSORY EVOKED-POTENTIALS IN HUNTINGTONS-DISEASE [J].

ABBRUZZESE, G ;

DALLAGATA, D ;

MORENA, M ;

RENI, L ;

FAVALE, E .

ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY, 1990, 77 (05) :340-346

[2] PROGRESSIVE MYOCLONUS EPILEPSIES - SPECIFIC CAUSES AND DIAGNOSIS [J].

BERKOVIC, SF ;

ANDERMANN, F ;

CARPENTER, S ;

WOLFE, LS .

NEW ENGLAND JOURNAL OF MEDICINE, 1986, 315 (05) :296-305

[3]

BIRD ED, 1976, BIOCH NEUROLOGY, P83

[4] HUNTINGTONS DISEASE IN CHILDREN - NEUROPATHOLOGIC STUDY OF 4 CASES [J].

BYERS, RK ;

GILLES, FH ;

FUNG, C .

NEUROLOGY, 1973, 23 (06) :561-569

[5] CHOREA AND MYOCLONUS IN THE MONKEY INDUCED BY GAMMA-AMINOBUTYRIC ACID ANTAGONISM IN THE LENTIFORM COMPLEX - THE SITE OF DRUG-ACTION AND A HYPOTHESIS FOR THE NEURAL MECHANISMS OF CHOREA [J].

CROSSMAN, AR ;

MITCHELL, IJ ;

SAMBROOK, MA ;

JACKSON, A .

BRAIN, 1988, 111 :1211-1233

[6] THE EFFECT OF STIMULUS FREQUENCY ON POST-CENTRAL AND PRE-CENTRAL SHORT-LATENCY SOMATOSENSORY EVOKED-POTENTIALS (SEPS) [J].

DELBERGHE, X ;

MAVROUDAKIS, N ;

DEBEYL, DZ ;

BRUNKO, E .

ELECTROENCEPHALOGRAPHY AND CLINICAL NEUROPHYSIOLOGY, 1990, 77 (02) :86-92

[7] HAND MUSCLE REFLEXES FOLLOWING ELECTRICAL-STIMULATION IN CHOREATIC MOVEMENT-DISORDERS [J].

DEUSCHL, G ;

LUCKING, CH ;

SCHENCK, E .

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 1989, 52 (06) :755-762

[8]

FAU R, 1971, REV NEUROL-FRANCE, V124, P353

[9] THE DIAGNOSIS OF HUNTINGTONS-DISEASE [J].

FOLSTEIN, SE ;

LEIGH, RJ ;

PARHAD, IM ;

FOLSTEIN, MF .

NEUROLOGY, 1986, 36 (10) :1279-1283

[10] MYOCLONIC TYPE OF HUNTINGTON CHOREA [J].

GARREL, S ;

JOANNARD, A ;

FEUERSTEIN, C ;

SERRE, F .

REVUE D ELECTROENCEPHALOGRAPHIE ET DE NEUROPHYSIOLOGIE CLINIQUE, 1978, 8 (01) :123-128