title: Case report: bipolar disorder as the first manifestation of CADASIL.
abstract: BACKGROUND
  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebrovascular disease, clinically characterized by variable manifestations of migraine, recurrent transient ischemic attack or lacunar strokes, cognitive decline, and mood disturbances. However, manic episodes have rarely been documented as an initial symptom of CADASIL and bipolar disorder presenting as the first manifestation in CADASIL has not been reported previously from evaluations by psychiatrists or psychological testing by psychologists.
  
  
  METHODS
  A 53 year old woman developed symptoms of mania in her 50s leading to a personality change involving a continuously labile mood and irritability over a number of years. Neuropsychological testing revealed an intact memory, but impairment in attention and executive function. In the Rorschach test, she showed a high level of cognitive rigidity. Magnetic resonance imaging findings were very consistent with a diagnosis of CADASIL, which was confirmed by genetic testing for NOTCH3 mutations. Atypical antipsychotics proved to be helpful in treating her manic symptoms and for behavior control.
  
  
  CONCLUSIONS
  We present a novel case of CADASIL that first presented as bipolar disorder. We contend that when patients show a late onset personality change or chronically irritable mood that deteriorates over many years, an organic cause such as CADASIL must be considered. Further studies are needed to better understand the exact impacts of cerebral tissue lesions and psychiatric symptoms in CADASIL patients.
authors.forename: Soyeon
authors.initials: S
authors.lastname: Park
authors.forename: Boram
authors.initials: B
authors.lastname: Park
authors.forename: Min Kyung
authors.initials: MK
authors.lastname: Koh
authors.affiliation: Department of Psychiatry, University of Ulsan College of Medicine, Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 136-736, Korea. jooyh@amc.seoul.kr.
authors.forename: Yeon Ho
authors.initials: YH
authors.lastname: Joo
mesh_terms: D001714:Bipolar Disorder / Q000209:etiology*; D046589:CADASIL / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009483:Neuropsychological Tests; D051883:Receptor, Notch2 / Q000235:genetics; D012392:Rorschach Test
chemical_list: C497764:NOTCH2 protein, human; D051883:Receptor, Notch2