Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts
and Leukoencephalopathy (CADASIL) is a small vessel disease. It is an
autosomal dominant inherited disease caused by a mutation in the
Notch3 gene. Clinically, it usually presents with recurrent transient
ischemic attacks, strokes, vascular dementia, migraine with aura,
cognitive impairments and psychiatric symptoms. Cranial MRI is the most
useful imaging modality to demonstrate the characteristic radiological
findings of CADASIL and gene analysis is the gold standard for diagnosis.
Although the clinical manifestations are mainly neurological, CADASIL
can also present with psychiatric disorders. Psychiatric disorders are one
of the main clinical manifestations of the disease, with a prevalence rate
ranging from 20 to 41%. Among psychiatric disorders, mood disorders are
the most commonly reported, and other psychiatric diagnoses include
psychotic disorders, anxiety disorders, adjustment disorder, personality
disorders, behavioral disorders, substance dependence and abuse. In
this case report, a paitent with CADASIL presenting with obsessivecompulsive
disorder at a relatively young age will be presented.
Keywords: CADASIL, neuropsychiatry, obsessive-compulsive disorder