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A case of schizencephaly associated with obsessive-compulsive disorder

Erica E. Coates, MA

Department of Psychology, University of South Florida, Tampa, FL, USA

Jason I. Chen, BA

Department of Psychology, University of South Florida, Tampa, FL, USA

Eric A. Storch, PhD, MS

Department of Pediatrics and Psychiatry, Rothman Center for Neuropsychiatry, University of South Florida, Tampa, FL, USA

KEYWORDS: schizencephaly, obsessive-compulsive disorder, children


TO THE EDITOR: chizencephaly is a rare developmental birth defect characterized by abnormal unilateral or bilateral clefts in the cerebral mantle formed during gestation. Few reports have examined the association between schizencephaly and psychiatric illness.1 There are no previous reports of its association with obsessive-compulsive disorder (OCD), which may provide insight into brain regions linked to OCD pathogenesis. We describe a pediatric patient diagnosed with parietal unilateral closed-lip schizencephaly who later developed OCD.

Case report

Noah, age 7, had a recent onset of obsessive-compulsive symptoms in the context of long-standing anxiety. He presented with obsessions related to contamination, needing to know or remember information, and fear of saying the wrong thing. He manifested washing, ordering, and repeating compulsions as well as rigid adherence to rules and routines. Noah’s total score on the Children’s Yale-Brown Obsessive Compulsive Scale was 28; 13 on the Obsessions Severity scale and 15 on the Compulsions Severity scale, suggesting severe obsessive-compulsive symptoms. The severity of his obsessive-compulsive symptoms interfered with his ability to attend school and develop friendships.

In addition to Noah’s obsessive-compulsive symptoms, hyperactivity, inattention, developmental delays, and generalized anxiety symptoms were present. Noah exhibited echolalia, difficulty with emotional reasoning, inability to develop age-appropriate social relationships, and repetitive speech and movements. Noah also reported pervasive general worries that were difficult to control and significantly interfered with his daily functioning. He had a family psychiatric history of depressive, bipolar, and anxiety disorders.

Noah was diagnosed with parietal unilateral closed-lip schizencephaly via MRI at age 10 months precipitated by premature signs of handedness. The MRI revealed a cleft in the left parietal lobe lined with gray matter. Noah exhibited congenital hemiparesis of the right side, and he achieved developmental milestones on-time with no speech delays. Genetic testing showed no abnormalities.


This is the first report of schizencephaly and comorbid OCD or anxiety. Although frontal-subcortical circuitry dysfunction has been implicated in the pathogenesis of OCD,2 the role of the parietal lobe in the pathophysiology of OCD has been suggested.3,4 Neuroimaging studies found OCD patients demonstrated decreased parietal activation relative to controls.5 Because inferior parietal activation is related to prefrontal activity, it is possible that parietal lobe dysfunction may indirectly be related to the development of obsessive-compulsive symptoms through interactions with the prefrontal cortex.3 Further attention to the relationship between parietal lobe dysfunction and obsessive-compulsive symptomology is needed. Studies assessing the presence of obsessive-compulsive symptoms in individuals with parietal lobe injury may further clarify its role in OCD.

DISCLOSURE: The authors report no financial relationship with any company whose products are mentioned in this article or with manufacturers of competing products.


  1. Arora M, Praharaj SK, Sinha VK, et al. Schizencephaly associated with bipolar II disorder. Singapore Med J. 2009;50:e79–e80.
  2. Lewin AB, Storch EA, Geffken GR, et al. A neuropsychiatric review of pediatric obsessive-compulsive disorder: etiology and efficacious treatments. Neuropsychiatr Dis Treat. 2006;2:21–31.
  3. den Braber A, Ent D, Blokland GA, et al. An fMRI study in monozygotic twins discordant for obsessive-compulsive symptoms. Biol Psychol. 2008;79:91–102.
  4. Roś LT, Podgórski JK. Obsessive-compulsive disorder secondary to a right parietal lobe haemorrhage: a case report. Acta Neurol Belg. 2007;107:94–95.
  5. Kwon JS, Kim JJ, Lee DW, et al. Neural correlates of clinical symptoms and cognitive dysfunctions in obsessive-compulsive disorder. Psychiatry Res. 2003;122:37–47.

CORRESPONDENCE: Erica E. Coates, MA, Department of Psychology, PCD 4118G, University of South Florida, Tampa, FL 33620 USA, E-MAIL: ecoates@mail.usf.edu