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 Table of Contents  
Year : 2021  |  Volume : 5  |  Issue : 1  |  Page : 83-85

Type-II Sturge-Weber Syndrome and Comorbidity with Bipolar Disorder

1 Department of Psychiatry, Jawaharlal Nehru Medical College, DMIMS, Wardha, Maharashtra, India
2 Department of Psychiatry, AIIMS, Raipur, Chhattisgarh, India
3 Department of Clinical Psychology, AIIMS, Raipur, Chhattisgarh, India

Date of Submission17-Jun-2020
Date of Decision05-Aug-2020
Date of Acceptance28-Aug-2020
Date of Web Publication18-Jun-2021

Correspondence Address:
Dr. Sangha Mitra Godi
Department of Psychiatry, AIIMS, Raipur, Chhattisgarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aip.aip_50_20

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Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder which was known to occur in 1 in 50,000 births with a triad of features including the port-wine stain on the face, glaucoma, and leptomeningeal angiomas. It is further divided into three types based on the regions involved. Here, we present the case of an 18-year-old male, Type II SWS with facial involvement since childhood who was presented with affective symptoms. The psychiatric manifestations ranging from symptomatic to a diagnostic presentation to be kept in mind which are often missed in the patients presented with SWS.

Keywords: Bipolar disorder, port-wine stain, Sturge-Weber syndrome

How to cite this article:
Mamidipalli SS, Godi SM, Mahant S, Soni PK. Type-II Sturge-Weber Syndrome and Comorbidity with Bipolar Disorder. Ann Indian Psychiatry 2021;5:83-5

How to cite this URL:
Mamidipalli SS, Godi SM, Mahant S, Soni PK. Type-II Sturge-Weber Syndrome and Comorbidity with Bipolar Disorder. Ann Indian Psychiatry [serial online] 2021 [cited 2022 Sep 30];5:83-5. Available from: https://www.anip.co.in/text.asp?2021/5/1/90/318681

  Introduction Top

Sturge-Weber syndrome (SWS) comprises of a triad of features including the port-wine stain on the face, glaucoma and leptomeningeal angiomas. It is a rare neurocutaneous disorder and is known to occur in 1 in 50,000 births. The port-wine stain of the face mostly in the region of trigeminal nerve innervation. Commonly, it is unilateral but can also be bilateral. It is subdivided into three types, depending on involved regions. Type-I have all the three characteristic features, Type-II shows the involvement of skin but does not involve leptomeningeal angiomas, Type-III is the rarest of the three with leptomeningeal involvement only.[1] Most frequent comorbidity noted in patients with these disorders is a seizure disorder with onset in early childhood. However, psychiatric disorders/symptoms comorbid with the syndrome is not uncommon. There are few reports which showed the presentation of bipolar disorder in patients with SWS.[2] We hereby report a case of Type-II SWS in which the patient had developed the bipolar disorder in his adolescence after obtaining informed consent.

  Case Report Top

The patient was an 18-year-old male, 12th pass, unmarried, student, belonging to the middle socioeconomic status from Hindu joint family hailing from an urban background presented with the illness of 5 months' duration with complaints of depressed mood, decreased interest in work, decreased interaction, suicidal ideations, and disturbed sleep. The above symptoms lasted for 1½ months. The patient showed improvement in the above symptoms after receiving treatment with escitalopram 20 mg, aripiprazole 10 mg, and clonazepam 0.5 mg for 1 month; however, the family members noticed a change in his symptoms. The patient was found to be overtalkative, appeared more cheerful, had increased sociability, inflated self-esteem, racing of thoughts, with an increase in goal-directed activities. These symptoms lasted for 20 days–1 months and his medications were adjusted by consulting psychiatrist (tablet valproate 400 mg+ escitalopram 20 mg+ aripiprazole 10 mg). By October 2019, the symptoms were subsided, and medications were stopped by the patient and his family members. After 2 months of discontinuing medication, the patient was presented to our outpatient department with depressed mood, decreased interest in work, decreased interaction, suicidal ideations, disturbed sleep of 10–15 days' duration in December 2019. The patient has been admitted due to the severity of depressive symptoms and suicidal ideation.

He had a history of depressive episode in 2017–2018 (recovered spontaneously) and had no family history of psychiatric illness, neurological illness. The patient has been of easy temperament since childhood, and there was no history of antenatal complications. The patient was first in birth order and was born out of forceps delivery (exact reason not known) but had no other natal or postnatal complications. There was no history of any focal neurological events such as transient ischemic attacks, migraine, vertigo, and seizures. There was no delay in the attainment of milestones as reported by parents.

The general physical examination revealed red-colored patch over the right side of face-covering anterior 1/3 of the temple and upper 2/3 of the face and upper one-third of right upper ear, continuing up to right labial mucosa (not blanching over pressing), adherent earlobe, high arched palate, multiple buccal frenula, the wide gap between 1st and 2nd toes, soft pliable ears, and Stahl's ear [Figure 1] and [Figure 2]. The systemic evaluation revealed no abnormality in the central nervous system, cardiovascular system, gastrointestinal system, and respiratory system.
Figure 1: Right-sided port-wine stain in the ophthalmic and maxillary area

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Figure 2: Multiple frenula

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Mental state examination revealed nonspontaneous, monotonous speech with sad affect and thought content revealed ideas of hopelessness, helplessness, suicidal ideations, and insight of Grade 4. Cognitive functions such as attention, concentration, memory, abstract thinking, intelligence, and judgment were found to be intact.

The patient was diagnosed to be a case of bipolar affective disorder current episode severe depression without psychotic symptoms as per the International Classification of Diseases-10, and co-morbid SWS was considered. All routine investigations were carried out (thyroid-stimulating hormone, complete blood count liver function test, and renal function test), which were within normal limits. Neuroimaging (magnetic resonance imaging brain) done previously was crosschecked to look for any leptomeningeal involvement. However, no abnormality was found. The patient was started on tablet sodium valproate, and the dose was optimized to 1000 mg/day over a week. High-risk management was carried out with the patient. The Hamilton depression rating scale was used and revealed a score of 22 suggestive of moderate-severe depression.[3]

Ophthalmology consultation was taken, and the correction of myopia was advised. The Intraocular pressure was found to 28.0 mm of Hg in the right eye and 14.7 mm of Hg in the left eye. No abnormality was found in fundus examination in both the eyes. The psychological assessment was also done by a clinical psychologist.

Wechsler Adult Intelligence Scale®-Fourth Edition (India) which is a comprehensive and advanced intelligence test designed to measure cognitive ability in adolescents and adults was applied. Based on this test, Full-Scale Intelligence Quotient was found to be 95, which indicate that he has an average level of intellectual functioning.

The patient was discharged after 10 days of hospital stay and is in regular follow-up with us. He is currently asymptomatic and has joined back his studies [Table 1].
Table 1: Summary of the Wechsler Intelligence Scale for Children IV scale scores

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  Discussion Top

Intellectual disability or mental retardation is commonly seen in patients with SWS. About 50% of patients with the syndrome have mental retardation. It has been well studied and is frequently co-morbid with seizures, which are difficult to control.[4] However, psychiatric disorders concerning the syndrome are relatively understudied but not infrequent.

According to a study by Turin et al., mood symptoms (94%) have been the most frequently reported psychobehavioral symptoms with SWS. However, the diagnosis of mood disorder was present in only 31%, which was found to be the most frequent diagnosis associated with SWS followed by disruptive behavior disorder (25%) and adjustment disorder (25%).[5] According to another author, port-wine stains were associated with increased mood and social problems, particularly in older children.[6] In congenital conditions such as SWS, port-wine stain influence child's self-image because of the timing of onset of skin disease. This discrepancy between child's perceived body and idealized body creates cutaneous body image dissatisfaction which further lowers the self-esteem.[7]

The index patient had a port-wine stain on the right side of the face from birth; however, did not have any history of seizures and cognitive impairment. A detailed assessment of cognitive functions also did not show any impairment. There was no meningeal involvement as per the neuroimaging; however, on examination, the patient had raised intraocular pressure unilaterally (right). This suggests that our patient might be having Type-II SWS, according to Roach classification.[8] The patient's history and examination showed that he had a comorbid diagnosis of bipolar disorder too.

Lee in 1990, have reported the association of psychosis and depression with the syndrome in three patients.[9] A recent case report also suggested the association of postpartum psychosis with the syndrome.[10] To the best of our knowledge, there is only one case report on co-existence of SWS and bipolar disorder.[2]

More than half of these patients have intellectual disability, mood symptoms, and psychotic symptoms which are often considered to be the behavioral problems secondary to intellectual disability and diagnosis is delayed.[11] This leads to “diagnostic overshadowing,” the negative bias which impacts the clinician's judgment regarding co-occurring disorders as rightly suggested by Lee, in 1990.[9],[11] He also put forward several hypotheses leading to psychiatric illnesses in these patients. They include psychosocial effects, biological effects of the lesions, laterality of cerebral hemisphere involved. In chronic skin disease such as SWS, failure of skin as an organ of physical expression (“skin failure”) leads to shame, isolation, and psychosocial impairment. S. Kellet described the responses of these patients to disfigurement as “dermatological shame” which might have a psychosocial effect and this poor cutaneous body image also affects the treatment adherence.[7] Hence, the neurologists and psychiatrists involved in treating these patients should be aware of this presentation to assess and address the multiple aspects of this condition. As the proper evaluation, diagnosis and management of psychiatric disorders in these patients can improve the functioning and quality of life.

Declaration of patient consent

The patient consent statement was taken from the patient as per institutional ethics committee approval along with consent taken for participation in the study and publication of the scientific results/clinical information/image without revealing their identity, name, or initials. The patient is aware that though confidentiality would be maintained anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Sudarsanam A, Ardern-Holmes SL. Sturge-Weber syndrome: from the past to the present. Eur J Paediatr Neurol 2014;18:257-66.  Back to cited text no. 1
Mirsepassi Z, Mohammadian F, ElhamHakki, Shadloo B. Sturge-Weber syndrome with bipolar presentation: A case report. Iran J Psychiatry Behav Sci 2017;11:e6232.  Back to cited text no. 2
Hamilton M. A rating scale for depression. J Neurol Neurosurg Psychiatry 1960;23:56-62.  Back to cited text no. 3
Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatr Neurol 2004;30:303-10.  Back to cited text no. 4
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. 5
Chapieski L, Friedman A, Lachar D. psychological functioning in children and adolescents with Sturge-Weber syndrome. J Child Neurol 2000;15:660-5.  Back to cited text no. 6
Sadock BJ, Sadock VA, Ruiz P, Kaplan HI, editors. Kaplan & Sadock's Comprehensive Textbook of Psychiatry. 9th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2009. p. 2425-26.  Back to cited text no. 7
Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am 1992;39:591-620.  Back to cited text no. 8
Lee S. Psychopathology in Sturge-Weber syndrome. Can J Psychiatry 1990;35:674-8.  Back to cited text no. 9
Saraf AS, Babhulkar SS, Joge VP. Postpartum psychosis in Sturge-Weber syndrome: A case report. Indian J Psychiatry 2019;61:649-50.  Back to cited text no. 10
[PUBMED]  [Full text]  
Reiss S, Levitan GW, Szyszko J. Emotional disturbance and mental retardation: diagnostic overshadowing. Am J Ment Defic 1982;86:567-74.  Back to cited text no. 11


  [Figure 1], [Figure 2]

  [Table 1]


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