CADASIL: underdiagnosed in psychiatric patients?

Objective:  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is exclusively related to symptoms of the central nervous system. Retrospectively in up to 15% the initial presentation is psychiatric disturbances. In these cases the diagnosis often is...

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Bibliographic Details
Published in:Acta psychiatrica Scandinavica 2005-05, Vol.111 (5), p.392-396
Main Authors: Leyhe, T., Wiendl, H., Buchkremer, G., Wormstall, H.
Format: Article
Language:English
Subjects:
Adult and adolescent clinical studies
Aged
Biological and medical sciences
Brain
Brain - pathology
CADASIL
CADASIL - diagnosis
CADASIL - epidemiology
CADASIL - pathology
cognitive impairment
Comorbidity
dementia
depression
Diagnosis, Differential
Female
Humans
Magnetic Resonance Imaging
Male
Medical diagnosis
Medical sciences
Mental Disorders - epidemiology
Mental Disorders - pathology
Mental health care
Microscopy, Electron, Transmission
Middle Aged
mood disorder
Nervous system
NMR
Nuclear magnetic resonance
Organic mental disorders. Neuropsychology
psychiatric disturbances
Psychiatry
Psychology. Psychoanalysis. Psychiatry
Psychopathology. Psychiatry
Skin - blood supply
Skin - pathology
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Summary:Objective:  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is exclusively related to symptoms of the central nervous system. Retrospectively in up to 15% the initial presentation is psychiatric disturbances. In these cases the diagnosis often is delayed or missed. Method:  Two cases of CADASIL diagnosed in a psychiatric hospital are presented. Results:  Both patients were admitted to the gerontopsychiatric department (one because of a suicidal attempt and a depressive episode, the other because of cognitive decline and progressive personal neglect). Brain magnetic resonance imaging (MRI) showed severe leukoencephalopathy in the absence of cardiovascular risk factors. In both cases, diagnosis of CADASIL was made by the identification of specific granular osmiophilic material in skin biopsies. Conclusion:  Brain MRI should be performed in all cases of late onset of severe psychiatric symptoms. CADASIL should be considered as a possible differential diagnosis whenever a marked leukoencephalopathy is detectable. Diagnosis can be verified by taking a skin biopsy or by specific genetic testing.
ISSN:0001-690X
1600-0447
DOI:10.1111/j.1600-0447.2004.00452.x