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 Table of Contents    
Year : 2018  |  Volume : 60  |  Issue : 1  |  Page : 149-151
Dyke-Davidoff-Masson syndrome presenting with bipolar I mania with psychosis

1 Psychiatry Department, Liaquat National Hospital & Medical College, Karachi, Pakistan
2 Psychiatry Department, Cavan and Monaghan Mental Health Services, Ireland
3 Department of Psychiatry and Behavioral Sciences, George Washington University School of Medicine and Health Sciences, Washington, USA

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Date of Web Publication12-Apr-2018

How to cite this article:
Kumari P, Mohsin H, Koola MM. Dyke-Davidoff-Masson syndrome presenting with bipolar I mania with psychosis. Indian J Psychiatry 2018;60:149-51

How to cite this URL:
Kumari P, Mohsin H, Koola MM. Dyke-Davidoff-Masson syndrome presenting with bipolar I mania with psychosis. Indian J Psychiatry [serial online] 2018 [cited 2020 May 29];60:149-51. Available from:


 Dyke-Davidoff-Masson syndrome More Details (DDMS) is characterized by cerebral hemiatrophy, seizures, facial asymmetry, contralateral hemiparesis, shortening of the extremities on the ipsilateral side, skull vault thickening, and intellectual deficiency.[1] Most cases present in neurology or pediatrics. Only a few patients with DDMS have presented with psychiatric symptoms. To our knowledge, this is the first case of a patient presenting with mania who was eventually diagnosed with DDMS.

A 25-year-old man presented to the emergency room (ER) with physical aggression, irritability, increased energy, pressured speech, and sleeping only 3 h for 3 days. Three days earlier, he was taken to another psychiatric hospital for the same complaints, but he was brought to the ER for slurring of speech and limping gait (suggestive of hemiparesis) that he had developed a few hours before. At the previous hospital, he was started on oral haloperidol 30 mg/day and was given a depot injection of fluphenazine (dose unknown) for mania because he was out of control.

After presenting to the ER, he was admitted to the psychiatric inpatient unit for bipolar I mania and presumptive extrapyramidal symptoms according to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Editioncriteria. There was no family history of bipolar disorder or psychosis, and urine toxicology results were negative. He had mild intellectual deficiency since birth, and with great difficulty, he had completed grade 10 in his early 20s. He had not received IQ or imaging testing. He is right handed and was diagnosed with epilepsy at the age of 14 years after he was involved in a road traffic accident. He sustained a concussion injury according to the family, but no records were available regarding the accident. The patient was maintained on carbamazepine 400 mg/day since the age of 14 years (his carbamazepine level was not obtained). He was adherent with carbamazepine, and his last seizure was 7 years ago; this finding ruled out postictal psychosis and forced normalization. However, electroencephalography was not performed because it was not indicated.

After admission to the psychiatric inpatient unit, procyclidine (anticholinergic) 5 mg 3 times/day was started, and the dose of haloperidol was gradually decreased from 30 mg/day to 5 mg/day. Carbamazepine was continued at the same dose. After having mania for 6 days, he developed persecutory delusions and thought that the ward had done some black magic on him.

To address the on-going slurring of speech and limping gait, a neurologic consultation was performed on the 4th day of hospitalization. Magnetic resonance imaging findings are shown in [Figure 1]. There was dilatation of ipsilateral lateral ventricle, cortical sulci, and extra-axial cerebrospinal fluid spaces. The right lateral and third ventricle were also mildly dilated. Small patchy areas of high-signal intensity were seen in both cerebral hemispheres, representing ischemic infarctions. There was no midline shift. A diagnosis of DDMS was made on the basis of history and magnetic resonance imaging findings after ruling out common differential diagnoses such as Sturge– Weber syndrome More Details, basal ganglia germinoma, Silver– Russell syndrome More Details, Rasmussen's encephalitis, and Fishman syndrome.
Figure 1: Magnetic resonance imaging of the brain showing atrophy of the left cerebral hemisphere and ipsilateral calvarial thickening

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The patient's manic and psychotic symptoms improved, and he was discharged on quetiapine 200 mg/day after slowly tapering and stopping haloperidol. He was followed up for regular physiotherapy and behavioral management exercises. The carbamazepine dose was not changed considering his response since the age of 14 years.

DDMS has presented with childhood schizophrenia,[2] treatment-resistant schizophrenia,[3] schizoaffective disorder depressive type,[4],[5] schizophrenia-like psychosis,[6] and psychosis.[7] In all these cases, DDMS was diagnosed after psychiatric presentation. Of the six cases, two were female,[5],[7] and two had right cerebral hemiatrophy.[2],[7] To our knowledge, this is the first case report of DDMS presenting with bipolar I mania with psychosis. In patients with intellectual deficiency, early-onset seizures, and hemiparesis or hemiplegia who present with mood and/or psychotic symptoms, DDMS should be kept in the differential diagnosis.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Dyke CG, Davidoff IM, Masson CB. Cerebral hemiatrophy and homolateral hypertrophy of skull and sinuses. Surg Gynecol Obstet 1933;57:588-600.  Back to cited text no. 1
White JH, Rust JB. Davidoff-Dyke-Masson syndrome presenting as childhood schizophrenia. J Autism Dev Disord 1979;9:37-40.  Back to cited text no. 2
Puri BK, Hall AD, Lewis SW. Cerebral hemiatrophy and schizophrenia. Br J Psychiatry 1994;165:403-5.  Back to cited text no. 3
Amann B, García de la Iglesia C, McKenna P, Pomarol-Clotet E, Sanchez-Guerra M, Orth M, et al. Treatment-refractory schizoaffective disorder in a patient with Dyke-Davidoff-Masson syndrome. CNS Spectr 2009;14:36-9.  Back to cited text no. 4
Tatlidede AD, Yalcin AD, Canpolat TG. Neurodevelopmental influences in psychosis: A case of left cerebral hemiatrophy and schizoaffective disorder. Bull Clin Psychopharm 2013;23:368-72.  Back to cited text no. 5
Honer WG, Kopala LC, Locke JJ, Lapointe JS. Left cerebral hemiatrophy and schizophrenia-like psychosis in an adolescent. Schizophr Res 1996;20:231-4.  Back to cited text no. 6
Hegde D, Guru N, Krishna PM, Raghuraj U, Rao S. Psychosis in a patient with Davidoff -Dyke-Masson syndrome. Clin Schizophr Relat Psychoses 2015;8:1-8.  Back to cited text no. 7

Correspondence Address:
Maju Mathew Koola
Department of Psychiatry and Behavioral Sciences, George Washington University School of Medicine and Health Sciences, Washington
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/psychiatry.IndianJPsychiatry_229_17

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  [Figure 1]