Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Search research articles

Related Experiment Videos

[Cadasil--a new model for subcortical dementia]

P Davous¹, D Bequet

¹Service de Neurologie, Centre Hospitalier, Argenteuil.

Revue Neurologique

|November 1, 1995

Summary

This study presents three cases of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) presenting with subcortical dementia. Dementia and associated psychoaffective disturbances are proposed as key diagnostic criteria for CADASIL.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

A prospective study of depression in French patients with Parkinson's disease. The Depar study.

European journal of neurology·2013

Same author

[Indication of neuro-imaging for the initial management and the follow-up of acute community-acquired bacterial meningitis].

Medecine et maladies infectieuses·2009

Same author

[Facial diplegia and acute inflammatory demyelinating neuropathy secondary to varicella].

Revue neurologique·2008

Same author

[Fragile X premutation presenting as postural tremor and ataxia (FXTAS syndrome)].

Revue neurologique·2007

Same author

[Subacute deterioration of imbalance associated with headaches in an 81-year-old male].

Revue neurologique·2006

Same author

[Crossed anarthria and a dissociated lateralisation of language].

Revue neurologique·2005

Area of Science:

Neurology
Neuroscience
Genetics

Background:

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare genetic disorder.
Understanding the full spectrum of CADASIL's neurological manifestations is crucial for diagnosis and management.

Observation:

Three patients with CADASIL exhibited dementia with a distinct subcortical and frontal presentation.
Symptoms included behavioral changes, amnesia, executive dysfunction, bradyphrenia, and slowed information processing, without aphasia, apraxia, or agnosia.
One patient developed overt dementia prior to any focal neurological signs.

Findings:

Brain imaging revealed subcortical infarcts and leukoencephalopathy, sparing the cerebral cortex.
The observed dementia pattern aligns with the concept of subcortical dementia.
Psychoaffective disturbances were noted alongside cognitive decline.

Implications:

Dementia and psychoaffective disturbances may serve as major diagnostic criteria for CADASIL.
CADASIL offers a valuable model for studying subcortical dementia and its underlying pathophysiology.
This research highlights the importance of considering cognitive and psychiatric symptoms in CADASIL diagnosis.

Related Experiment Videos