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 Table of Contents    
Year : 2019  |  Volume : 61  |  Issue : 6  |  Page : 649-650
Postpartum psychosis in Sturge–Weber syndrome: A case report

1 Department of Psychiatry, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India
2 Department of Psychiatry, BJMC, Pune, Maharashtra, India

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Date of Web Publication5-Nov-2019

How to cite this article:
Saraf AS, Babhulkar SS, Joge VP. Postpartum psychosis in Sturge–Weber syndrome: A case report. Indian J Psychiatry 2019;61:649-50

How to cite this URL:
Saraf AS, Babhulkar SS, Joge VP. Postpartum psychosis in Sturge–Weber syndrome: A case report. Indian J Psychiatry [serial online] 2019 [cited 2020 Feb 16];61:649-50. Available from:


Sturge–Weber syndrome (SWS), also known as leptomeningeal angiomatosis, is a rare vascular neurocutaneous disorder primarily involving capillary-venous malformations of the face, eyes, and leptomeninges.[1] Although frequently associated with neurological and ophthalmic disturbances; the literature regarding the behavioral manifestations of SWS is sparse.[1],[2] The most frequent associations found are mood disorders, disruptive behavior disorders, adjustment disorders, and substance-related disorders.[1] We highlight here a case (after obtaining informed consent) of SWS, presenting as postpartum psychosis, along with its management and review of available literature.

A 45-year-old female presented with acute onset of suspiciousness, irritability, aggression, and sleep disturbance, 1 day after she delivered her second child. She was fearful and afraid that people around her are trying to harm her baby and are talking about planning to kidnap her baby. She was refusing to cooperate with medical care and alleged that the staff was also trying to harm her by giving dangerous medications. Despite her apprehensions and fears, she was ignoring the care of her newborn and trying to flee away from the hospital. She had a similar episode 4 years back, lasting about 3 weeks after giving birth to her first child. She also had a history of seizures and right-sided weakness in childhood, which remitted later.

On physical examination, she had large left-sided port-wine stain involving the ophthalmic and maxillary division of the trigeminal nerve [Figure 1]. Her ophthalmic examination revealed left-sided capillary hemangioma with proptosis, band-shaped keratopathy [Figure 2], and raised intraocular pressure in the left eye and amblyopia in the right eye. Her mental status examination revealed increased psychomotor activity, irritable affect, persecutory and referential delusions, and third-person auditory hallucinations with reference to herself and her newborn. She was also apprehensive that her baby will become disfigured and develop port-wine stain similar to her. Her thyroid function tests were within the normal limits, and computed tomography scan revealed mild cerebral atrophy but did not show any calcification.
Figure 1: Left-sided port-wine stain involving the ophthalmic and maxillary division of the trigeminal nerve

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Figure 2: Left eye capillary hemangioma with proptosis and band-shaped keratopathy

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A provisional diagnosis of SWS with postpartum psychosis was made, and after taking care of safety of the baby, she was started on tablet olanzapine 10 mg at night. She started responding within a week while the olanzapine was titrated to 15 mg at night. Her psychosis remitted within a month, and she is maintaining well with medications on subsequent follow-ups. Her child is also maintaining good health. Her prognosis seems good.

Although psychiatric manifestations have been previously reported in cases of SWS,[2],[3],[4],[5],[6] this is probably the first report of SWS presenting as postpartum psychosis. SWS affects about 1/20,000–50,000 live births, occurring sporadically due to somatic mutations disrupting local angiogenesis.[1],[3] It typically presents with neurological manifestations such as seizures, hemiparesis, migraine, glaucoma, mental retardation, and learning disabilities.[2],[3] Psychiatric symptoms may be a direct neurobiological consequence of intracranial lesions in SWS,[2] especially with left-sided brain involvement,[1] as in our case. Lee highlighted the influence of social factors on the development of psychopathology, especially negative evaluation of persons with facial disfigurement.[2] He explained the development of paranoia by “shame-humiliation theory,” in which the patient attempts to avoid the negative effect of humiliation by blaming someone else for a problem.[2] In our case, apprehensions of the mother regarding the development of similar disfigurement in her child might have contributed.

Such cases lie at the interface of neurology and psychiatry, but its psychiatric manifestations are often ignored.[6] We emphasize the need for special psychiatric care along with comprehensive interdisciplinary management in such cases, especially during peripartum period.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Turin E, Grados MA, Tierney E, Ferenc LM, Zabel A, Comi AM. Behavioral and psychiatric features of Sturge–Weber syndrome. J Nerv Ment Dis 2010;198:905-13.  Back to cited text no. 1
Lee S. Psychopathology in Sturge–Weber syndrome. Can J Psychiatry 1990;35:674-8.  Back to cited text no. 2
Madaan V, Dewan V, Ramaswamy S, Sharma A. Behavioral manifestations of Sturge–Weber syndrome: A case report. Prim Care Companion J Clin Psychiatry 2006;8:198-200.  Back to cited text no. 3
Kalaitzi CK, Sakkas D. Brief psychotic disorder associated with Sturge–Weber syndrome. Eur Psychiatry 2005;20:356-7.  Back to cited text no. 4
Mirsepassi Z, Mohammadian F, Hakki E, Shadloo B. Sturge-Weber syndrome with bipolar presentation: A case report. Iran J Psychiatry Behav Sci 2017;11:e6232. doi: 10.17795/ijpbs-6232.  Back to cited text no. 5
Gadit AA. Sturge-Weber syndrome: Neurology-psychiatry interface. BMJ Case Rep 2011;2011. pii: bcr0220113817.  Back to cited text no. 6

Correspondence Address:
Dr. Anantprakash Siddharthkumar Saraf
Department of Psychiatry, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/psychiatry.IndianJPsychiatry_560_18

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  [Figure 1], [Figure 2]