Sturge—Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome with an estimated frequency of one per 50,000 live births Reference Thomas-Sohl, Waslow and Marin[5]. Although mental retardation is a well-studied sequelae of SWS Reference Thomas-Sohl, Waslow and Marin[5], little attention has drawn on the psychological well being of SWS patients. SWS is devastating, especially when children experience recurrent seizures, pervasive learning and behavioral problems, and disabling visual impairment Reference Thomas-Sohl, Waslow and Marin[5], all predisposing to problems in behavioral functioning Reference Chapieski, Friedman and Lachar[1].

Despite the above, literature on psychotic symptoms in these patients is scarce.

We report on the case of a 33-year-old male with an extended nevus covering his whole face except for a small area surrounding his right eye and vascular deformities extending to the left temporo-parietal lobes. He was suffering from glaucoma and was on carbamazepine for complicated seizures. The patient had no personal or family history of major psychiatric disorder and had a normal psychosocial development. He was working as a freelancing software engineer but had little social life. He presented with psychomotor agitation and persecutory delusion, which had persisted for about 10 days. He was diagnosed as brief psychotic disorder and was prescribed penfluridol 20 mg/week, as he refused daily schemes. Despite the resolution of symptoms, the patient did not return to the prior level of functioning. After 3 months, and despite pharmacotherapy, the patient relapsed again, only to improve again with an increase in penfluridol dosage. Fortunately, the antipsychotic caused no worsening of the seizures.

The young people with SWS who appear to be at most risk for a broad range of behavioral and emotional problems are those with lower levels of intellectual functioning, seizure disorders and more frequent seizures Reference Chapieski, Friedman and Lachar[1]. As the area of the face covered with a port-wine stain increased, so did parent and teacher reports of emotional distress and teacher reports of social problems but only for children who were at least 10 years old Reference Chapieski, Friedman and Lachar[1].

Sing Lee reported Reference Lee[2] on three SWS patients. The first one was first diagnosed with paranoid disorder, a diagnosis that changed to obsessive–compulsive disorder 2 years later Reference Lee[3]. The second one was mentally retarded with a commorbid paranoid disorder and the third patient, also mentally retarded, suffered from a mood disorder Reference Lee[2]. Of these patients, the last two had left hemisphere lesions (same lateralization of lesion as our patient). The small number of reported cases prohibits safe conclusions on the existence of any relationship between lateralization of the lesions and the emergence of psyhcopathology.

The high incidence of schizophrenia permits the possibility of an accidental comorbidity of SWS and schizophrenic disorder. Nevertheless, we feel that psychosis is underreported in this group of patients. This could be due to the atypical presentation of psychiatric symptoms in patients with mental retardation, who represent more than 50% of SWS patients. Reference Reid[4] The clinician must keep in mind that, in the mentally retarded, the line between behavioral and psychotic symptoms is rather thin, depending heavily on the ability of the patient to express oneself fluently.