Psychiatric comorbidity in moyamoya disease and preliminary guidelines for treatment

Abstract

“Ms. A” is a 54-year-old Chinese-American woman with no psychiatric history who presented to the emergency department in a “psychotic break.” She could not provide a coherent history and appeared withdrawn, selectively mute, and confused. Her husband reported that her father had died unexpectedly 2 weeks earlier from natural causes. When she returned to work after a few days off, she disappeared for several hours, without recollection of her activities. Ms. A’s physical examination was normal, with stable vital signs with mild systolic hypertension (143/86 mmHg). Fully oriented to person, place, time, and situation, she denied acute psychiatric concerns apart from sadness over her father’s death. Her medical, social, and family histories were unremarkable. Findings from routine altered mental status screening, including CT, serological studies, and lumbar puncture, were negative. Urinalysis, however, showed pyuria. A diagnosis of cystitis was made, for which cephalexin was prescribed, and Ms. A was discharged home with her husband. She returned to the emergency department 2 days later with worsening symptoms and new incontinence. Psychiatry was consulted and discharged her home to begin sertraline at 50 mg/day for observed depressive symptoms. At outpatient psychiatric follow-up 1 week later, her psychomotor retardation, mutism, and cognitive slowing were unchanged. She disclosed “feelings of inadequacy” and “sad” mood, describing her father’s death as “devastating.” She frequently repeated the clinician’s questions, but her orientation was intact. When asked to write her thoughts, she inscribed, “I am scared and excited,” over 100 times on a sheet of paper. She denied domestic violence when interviewed alone. Her sertraline dosage was increased to 100 mg/day, and lorazepam was started at 1 mg b.i.d. to treat presumptive catatonia. Ms. A showed minimal response after 2 weeks, and her incontinence worsened, resulting in inpatient psychiatric admission. Given acute stressors and negative medical findings, the differential diagnosis included depressive catatonia, psychosis, dissociative disorder, and conversion disorder. She reported a chief complaint of “confusion,” scoring 15/30 on the Mini-Mental State Examination. Flat affect, selective mutism, echolalia, insomnia, bizarre behaviors (e.g., smearing toothpaste on the wall), and significant cognitive and psychomotor retardation remained despite increases in her dosages of sertraline and lorazepam and the addition of olanzapine (2.5–5 mg h.s.). Subjectively, she disclosed feeling “not normal” and anxious. Orientation to time and place fluctuated in no discernible pattern, with preserved self-identification. Findings from additional workup, including an extensive autoimmune illness panel and EEG, were negative. MRI, however, demonstrated regions of diffusion restriction in the left superior frontal gyrus and in the anterior cerebral artery distribution, as well as small multi-focal diffusion restriction in the left and right centrum semiovale in a watershed distribution. Corresponding T 2 -weighted fluid-attenuated inversion recovery hyper-intense signal was observed in these areas (Figure 1A). Hemorrhagic transformation of the left basal ganglia was also noted. Magnetic resonance angiography and cerebral angiogram revealed bilateral stenosis of the internal carotid arteries with minimal filling of the anterior and middle cerebral vascular trees, as well as a network of collateral vessels at the terminal internal carotid arteries and posterior cerebral arteries, confirming bilateral moyamoya disease (Figure 1B). With blood pressure support, the patient’s mental status improved to a score of 29/30 on the Mini-Mental State Examination. She subsequently underwent cerebral artery bypass surgery.