Neuropsychiatric aspects of the adult variant of Tay-Sachs disease

被引：49

作者：

MacQueen, GM ^{[1
]}

Rosebush, PI ^{[1
]}

Mazurek, MF ^{[1
]}

机构：

[1] McMaster Univ, Dept Psychiat, Ctr Med, Div Neurol & Biomed Sci, Hamilton, ON L8N 3Z5, Canada

来源：

JOURNAL OF NEUROPSYCHIATRY AND CLINICAL NEUROSCIENCES | 1998年 / 10卷 / 01期

关键词：

D O I：

10.1176/jnp.10.1.10

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Tay-Sachs disease (a G(M2) gangliosidosis) is an inherited neuronal storage disease that can affect individuals across the age spectrum. Psychosis is reported in 30% to 50% of adult-onset patients, and many are misdiagnosed with schizophrenia. Mood disorders are present in more than 25% and cognitive impairment in more than 20%. Treatment of psychosis with neuroleptics may not have a favorable risk/benefit ratio, but treatment with benzodiazepines or electroconvulsive therapy may be efficacious. Metabolic diseases such as gangliosidosis are probably under-recognized as causes of neuropsychiatric illness. Increased awareness of these disorders will lead to accurate diagnosis, appropriate treatment selection, and genetic counseling.

引用

页码：10 / 19

页数：10

共 78 条

[1]

ADAMS RD, 1993, PRINCIPLES NEUROLOGY, P799

[2]

ANDO S, 1983, NEUROCHEM INT, V5, P507, DOI 10.1016/0197-0186(83)90043-8

[3] METACHROMATIC LEUKODYSTROPHY (MLD) .8. MLD IN ADULTS - DIAGNOSIS AND PATHOGENESIS [J].

AUSTIN, J ;

ARMSTRON.D ;

FOUCH, S ;

MITCHELL, C ;

STUMPF, D ;

SHEARER, L ;

BRINER, O .

ARCHIVES OF NEUROLOGY, 1968, 18 (03) :225-&

[4] LYSOSOMAL-ENZYMES IN ATAXIA - DISCOVERY OF 2 NEW CASES OF LATE ONSET HEXOSAMINIDASE-A AND HEXOSAMINIDASE-B DEFICIENCY (ADULT SANDHOFF DISEASE) IN FRENCH CANADIANS [J].

BARBEAU, A ;

PLASSE, L ;

CLOUTIER, T ;

PARIS, S ;

ROY, M .

CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES, 1984, 11 (04) :601-606

[5]

BERGINER VM, 1988, AM J PSYCHIAT, V145, P354

[6]

Berkovic S F, 1988, Am J Med Genet Suppl, V5, P105

[7] KUFS DISEASE - A CRITICAL REAPPRAISAL [J].

BERKOVIC, SF ;

CARPENTER, S ;

ANDERMANN, F ;

ANDERMANN, E ;

WOLFE, LS .

BRAIN, 1988, 111 :27-62

[8] ADULT METACHROMATIC LEUKODYSTROPHY (SULPHATIDE LIPIDOSIS) SIMULATING ACUTE SCHIZOPHRENIA - REPORT OF A CASE [J].

BETTS, TA ;

SMITH, WT ;

KELLY, RE .

NEUROLOGY, 1968, 18 (11) :1140-&

[9] THE DIAGNOSIS OF CHILDHOOD NEURODEGENERATIVE DISORDERS PRESENTING AS DEMENTIA IN ADULTS [J].

COKER, SB .

NEUROLOGY, 1991, 41 (06) :794-798

[10] BIOCHEMICAL BASIS OF LATE-ONSET NEUROLIPIDOSES [J].

CONZELMANN, E ;

SANDHOFF, K .

DEVELOPMENTAL NEUROSCIENCE, 1991, 13 (4-5) :197-204

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