CLINICAL CASE CONFERENCE

Psychiatric Comorbidity in Moyamoya Disease and
Preliminary Guidelines For Treatment
Misty Richards, M.S., M.D., Adrienne Grzenda, M.D., Ph.D., Evelyn Nelson, M.D., Michael Gitlin, M.D.

“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a coherent history and appeared withdrawn, selectively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.

Moyamoya disease is a cerebrovascular disorder of unknown
etiology characterized by progressive unilateral or bilateral
stenosis of the distal internal carotid arteries, frequently

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 multifocal diffusion restriction in the left and right centrum
semiovale in a watershed distribution. Corresponding
T2-weighted fluid-attenuated inversion recovery hyperintense 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.

extending to the anterior and middle cerebral arteries.
Cerebral angiogram reveals fragile compensatory collateral
vessels forming along the base of the brain, resembling a

See related feature: CME course (p. 325)

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FIGURE 1. MRI and cerebral angiogram of the patient after admission to the inpatient psychiatric unita

a

Panel A is an image from brain MRI with contrast, T2-weighted fluid-attenuated inversion recovery, axial section. Hyperintense signal is noted along
the left superior frontal gyrus and the left and right centrum semiovale in areas of diffusion restriction. Panel B is a cerebral angiogram of the right
common carotid artery. Severe narrowing of the internal carotid artery above the ophthalmic artery origin is observed as well as a network of
developing collateral vessels (arrow).

diagnostic “puff of smoke”—moyamoya in Japanese (1, 2). The
prevalence of moyamoya is highest in Korea, Japan, and
China, although awareness of the disease has resulted in its
increased characterization in other countries. In Japan, the
incidence rate is estimated at 0.54 per 100,000 individuals,
with a female-to-male ratio of 1.98 (3). Illness onset follows a
bimodal distribution with a large peak centered at approximately age 5 and a smaller peak at age 40 (3). A family history
of the disease is present in up to 15% of identified patients, and
in this group the mean age at symptomatic onset is lower than
in sporadic cases of moyamoya disease, indicating genetic anticipation (3, 4). Moyamoya disease may also occur secondary
to atherosclerosis, autoimmune illness, neoplasms, Down’s
syndrome, or irradiation, among other conditions, referred to
as moyamoya syndrome (2).
Ischemia secondary to hypoperfusion is the primary
clinical feature, resulting in repeated transient ischemic
attacks or stroke, particularly in the cerebral cortex (e.g.,
frontal, parietal, and temporal lobes) (2, 5). Hemorrhagic
stroke may also occur from rupture of fragile collateral vessels or aneurysms with intraventricular, intraparenchymal,
and subarachnoid distributions (6). Embolism is uncommon.
Disruption of the anterior circulation results in hemiparesis, speech disturbance, and hemisensory impairments (1).
Headache is a common presenting and persisting symptom
even with treatment (7). Involvement of the posterior circulation is more infrequent and is associated with greater
illness severity (8). Cognitive dysfunction is common, with
impairments observed in processing speed, verbal memory,
verbal fluency, and executive function (9, 10). Ischemic symptoms are more common in children, and hemorrhagic
symptoms predominate in older adults (11, 12).
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Studies have suggested that moyamoya disease may
manifest with a different phenotype in non–East Asian
countries (13). In California and Washington, the incidence is
estimated at 0.086 per 100,000 persons (14). An analysis of
2,280 admissions for moyamoya disease from the Nationwide
Inpatient Sample, the largest inpatient care database in the
United States, calculated a hospital admission rate of 0.57
per 100,000 persons per year (15). Predominantly females
were affected (72%), with a mean and median age of 31.6 years
at presentation. Overall, 49% were white, 24% black, 11%
Hispanic, 11% Asian, and 5% other. Children and adults were
more likely to present initially with ischemic rather than
hemorrhagic illness. The authors of that report acknowledge,
however, that these phenotypic differences may be due to
an increased number of cases with moyamoya syndrome. In
the Mayo Clinic moyamoya disease/moyamoya syndrome
cohort, individuals are also primarily female (72.3%) and
Caucasian (85.1%), with higher incidences of autoimmune
diseases in comparison with the general population (22.3%
and 3.2%, respectively), including type 1 diabetes and Graves’
disease (16). Failure to recognize moyamoya syndrome may
falsely lower incidence rates in the United States.
Moyamoya disease is a chronic illness with a highly variable natural course ranging from a slow, asymptomatic illness to rapid decompensation. A Japanese prospective study
estimated a natural progression rate of approximately 20%
over 6 years (17). In symptomatic patients, surgical revascularization is the treatment of choice to prevent cerebral
infarction and restore perfusion and reserve capacity (18).
Revascularization occurs either via direct artery-to-artery
anastomosis or indirect arteriosynangiosis. Conservative
treatment utilizes long-term aspirin, antiplatelet, or statin
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therapy, which has been shown to be insufficient to stop
disease progression compared with surgery, after which the
rate of symptomatic progression may be as low as 2.3% (18,
19). The role of surgical intervention in hemorrhagic disease
is controversial, with rates of rebleeding in up to 18% of
patients regardless of treatment modality (20, 21). Rebleeding is associated with extremely poor prognosis (6). In children and adults, functional status at the time of surgical
intervention is a better predictor of favorable outcome and
quality of life than age at presentation (12, 22). Early intervention is critical to reducing the risk of permanent neurological and cognitive deficits and improving long-term
prognosis and life expectancy.
PSYCHIATRIC MANIFESTATIONS OF
MOYAMOYA DISEASE
Exclusively psychiatric presentations of moyamoya disease
are exceedingly rare in the literature. In adults, coincident
complaint of depression or anxiety in assessment of new focal
neurological symptoms is more common (23, 24). In children,
months to years of mood lability, hyperactivity, impaired
concentration, and poor academic performance have also
been described (25, 26). Of those cases identified where psychiatric symptoms preceded neurological deficits, none of
the patients reported a prior history of mental illness. The
clinical presentations are highly variable, although psychosis
appears to be a more frequent manifestation in children and
young adults. One case report (27) describes an 11-year-old
Indian boy who developed insomnia, disorganization, agitation, and internal preoccupation after a febrile illness,
treated as presumptive schizophrenia for 1 week until he
developed extrapyramidal symptoms. EEG revealed slow
waves in the right frontal and temporal region, and angiography demonstrated bilateral moyamoya disease. The patient’s symptoms improved with surgical treatment and
antipsychotic medication, albeit with continued impulsivity
and restlessness. In a similar case (28), a 23-year-old Vietnamese man with auditory hallucinations and paranoid delusions received antipsychotic treatment for 2 years before
an MRI for a research study revealed bilateral moyamoya
disease. His symptoms resolved with antipsychotic treatment
and conservative management.
The degree of psychosis can be profound in some instances. One case report (29) describes a 12-year-old Caucasian boy who developed acute-onset insomnia, hyperverbal
speech, tangential thought processes, severe mood lability, auditory hallucinations of laughter and babies crying, visual hallucinations of dolls, Capgras delusion regarding his
mother, paranoid delusions of being observed and hypnotized, and the belief that he could receive messages through
the television. Symptoms started 9 days after he participated
in a 1500-meter run. The patient’s EEG was normal, but MRI
and MR angiography showed unilateral moyamoya disease.
His psychotic symptoms resolved with antipsychotic treatment without surgical revascularization.
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Acute psychiatric symptoms in the setting of known
moyamoya disease may also signal new ischemia. One report
(30) describes an 8-year-old boy with moyamoya disease
who presented to outpatient psychiatry with irritability,
paranoia, internal preoccupation, poor self-care, insomnia,
and hallucinations of cartoon characters accusing or scolding him throughout the day. MRI subsequently revealed new
ischemic changes in the left middle cerebral artery distribution. His symptoms resolved with antipsychotic treatment.
In adults, refractory depression and psychosis prompting
expanded neurological investigation have revealed moyamoya
disease, sometimes years after symptom onset (24, 31). A
report from Korea (32) describes the case of a 45-year-old
woman with depression and transient episodes of confabulation and inappropriate behavior provoked by arguments with
her husband. The authors initially thought her symptoms
were secondary to depression until she developed right-sided
weakness and MRI showed bilateral moyamoya disease. She
received treatment with antiplatelet agents, statins, and a
mood stabilizer, which partially resolved her mood lability.
PSYCHIATRIC COMORBIDITY
Most large studies of moyamoya disease have excluded psychological measures in examination of long-term outcomes.
From the case literature and the limited number of small
studies, depression and anxiety appear to be the most common
psychiatric sequelae of illness (23, 33–36). A sample of Korean
pediatric cases reported comorbid depression, anxiety, impulsivity, impaired concentration, and social adaptivity issues
(37). In a study of American adults with moyamoya disease who
were evaluated before surgical revascularization, 36% were
found to have mild depression and 28% to have moderate to
severe depression, as measured by the Beck Depression Inventory (BDI) (9). A large percentage of participants reported
a history of stroke (83%), although degree of neurocognitive
dysfunction did not differ between the stroke and nonstroke
groups and showed no correlation with the depression. Another study of American adults with moyamoya disease (38)
revealed a 20% rate of at least mild depression in a cohort
with a mixed history of stroke. Among the portion of the
cohort without evidence of ischemic stroke on MRI, 29%
reported suffering clinically significant depression and 28%
clinically significant anxiety (10). Patients and families indicated apathy as the predominant behavioral issue (10).
Anxiety also appears to be a common comorbidity. A
Chinese study of adults with moyamoya disease (39) compared quality of life with intraventricular hemorrhagic
moyamoya disease managed conservatively to matched
healthy control subjects and patients with spontaneous intraventricular hemorrhage. One year after initial stroke, those
with hemorrhagic moyamoya disease reported higher rates
of sensitivity, poor stress tolerance, poor interpersonal relationships, and pessimism compared with both control groups,
and 73% reported depression. The patients with moyamoya
disease also scored higher for obsessive-compulsive, depressive,
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and phobic anxiety symptoms. Baseline measures of mood and
personality were not available for comparison. A study (40) of
Korean males (59.5% ischemic presentation, 2% hemorrhagic)
with a mixture of moyamoya disease management types
(70.2% surgical and 29.8% antiplatelet or no treatment)
showed significantly increased anxiety, depression, and somatization compared with healthy control subjects. An association
was additionally observed between degree of encephalomalacic
changes in the frontal lobe and paranoia. Only one study has
directly examined the impact of revascularization on depression (41). As measured by the BDI in a group of U.S.
adults with moyamoya disease, 19% indicated improved depression, 11% worsened depression, and 70% no change in
depression at 6-month follow-up after revascularization.
Mood lability, irritability, psychomotor agitation, insomnia, and impulsivity resembling bipolar disorder developing days to years after illness onset has also been
described, although less commonly (35, 42, 43). Rarer are
hallucinations and paranoia (23, 42, 44). Psychic akinesia
has also been described (45, 46). Typically associated with
lesions in the prefrontal cortex or basal ganglia, the condition is characterized by severely flattened affect, apathy,
and amotivation. Similar symptoms may be seen in schizophrenia and Parkinson’s disease. Unlike depression, psychic
akinesia occurs without subjective sad or negative thinking
and may be accompanied by obsessive-compulsive behaviors and motor tics (47).
PSYCHOPHARMACOLOGIC TREATMENT FOR
PSYCHIATRIC SYMPTOMS IN MOYAMOYA DISEASE
Although patients with moyamoya disease are frequently
treated with a variety of psychotropic medications, no controlled trials have examined the efficacy of specific
treatments in the management of associated psychiatric
symptoms (9). Review of the case literature indicates that
several features of a psychotropic medication should be
considered prior to initiation in patients with moyamoya
disease, including a medication’s ability to alter vascular
hemodynamics and affect cerebral perfusion, induce confusion or paradoxical disinhibition, lower the seizure threshold, worsen mood lability, and block dopamine D2 receptors.
As psychiatric phenotypes are highly heterogeneous in
moyamoya disease, no absolute contraindications exist for
any individual medication. Medication selection must also
consider the individual’s specific disease characteristics—
degree and location of occlusions, prior ischemic or hemorrhagic injuries, and seizure history. Specific recommendations
evidenced in the literature are summarized below by medication class.
Mood Stabilizers
Although less common than depression and anxiety, secondary
mania is associated with moyamoya disease, characterized
by mood lability (typically irritability, although euphoria is
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also described), psychomotor agitation, impulsivity, insomnia,
and/or psychosis (26, 32, 35, 42, 43). Use of carbamazepine
(43), divalproex sodium (26, 33), and oxcarbazepine (25) has
been reported, with diminished irritability and impulsivity in
moyamoya disease (32). Given evidence that older agents
produce cognitive and behavioral side effects (48), preference would be for medications that have more established
mood stabilization benefits, such as lamotrigine and divalproex sodium.
Antidepressants
Moyamoya disease–associated depression, anxiety, and
psychic akinesia respond variably to antidepressants (9, 28,
35, 36). Given the risk for depression secondary to severe
medical illness, comorbid anxiety, or obsessive-compulsive
symptoms, early antidepressant treatment is recommended,
particularly given the relatively safe side effect profile of
these medications. Furthermore, anxiety carries a risk for
panic attacks, including crying and hyperventilation, which
may result in repeated hypoperfusion stress on collateral or
anastomotic vessels, raising risk for new ischemia. Use of
amitriptyline (9, 23) and sertraline (9, 28, 36) is commonly
reported. Selective serotonin reuptake inhibitors are favored
because of data on improved motor coordination following
ischemic injury, a relative lack of anticholinergic side effects compared with tricyclic antidepressants, and a low
impact on blood pressure compared with some serotoninnorepinephrine reuptake inhibitors (49). Atypical agents
such as trazodone and mirtazapine appear to be effective in
targeting mood and insomnia.
Antipsychotics
Patients with moyamoya disease display high neuroleptic
sensitivity, with frequent reports of extrapyramidal symptoms and neuroleptic malignant syndrome, especially with
first-generation agents (26–29). Impaired basal ganglia function due to abnormal vessel collateralization or ischemia
may explain this sensitivity, which is similar in Lewy body
dementia. Antipsychotics, however, are effective in reducing hallucinations and paranoia (23, 27–30, 44). Secondgeneration agents, such as quetiapine, are preferred given
a lower risk for extrapyramidal complications. Trial reduction after prolonged resolution of symptoms appears
reasonable, particularly if symptoms occurred prior to surgical intervention. Antipsychotic treatment may also assist
with motor tics, if present (23).
Benzodiazepines
Treatment with benzodiazepines is commonly described
in the treatment of moyamoya disease–related anxiety and
agitation, but their efficacy as a monotherapy has been reported to be poor, owing to rapid tolerance and possible
paradoxical disinhibition (9, 23, 29, 33, 43). For short-term
management of anxiety or emergent agitation, however,
benzodiazepines appear to be a safe option.
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Stimulants
Stimulants or stimulant-like medications (e.g., modafinil)
offer theoretical benefit in depression, apathy, impulsivity,
and neurocognition in moyamoya disease. An Internet search
reveals frequent anecdotal reports of patients with moyamoya
disease receiving stimulants to assist with cognitive difficulties.
Given the potential for hypertension, vasoconstriction, and
irritability, conservative management would restrict stimulant
trials to medically and psychiatrically stable patients after
surgical revascularization.

considered, to fully characterize a patient’s psychiatric and
cognitive deficits. Classical psychotropic medications appear to be effective but underutilized. Dopamine precursors,
dopamine agonists, and acetylcholinesterase inhibitors are
virtually unexplored in treating the illness. Because untreated
or poorly treated psychiatric symptoms may have a negative
impact on quality of life, disability, and prognosis, a teambased multidisciplinary approach is highly recommended,
with collaboration between psychiatry and neurology early
in diagnosis.
AUTHOR AND ARTICLE INFORMATION

CONCLUSIONS
Moyamoya disease and moyamoya syndrome carry a unique
risk for misdiagnosis as an affective or psychotic disorder.
Transient ischemic events may be easily mistaken for anxiety
and panic disorder, emphasizing the need for thorough
screening of precipitating triggers and characterization of
symptoms. In the case presented here, the proximity of the
patient’s father’s death confounded diagnosis. The absence of
a family history, particularly of psychotic illness, in combination with atypical illness features (e.g., age at onset, visual
hallucinations) should prompt neurological investigation,
with a strong argument in favor of MRI or MR angiography
rather than screening CT. In cases of new psychiatric
symptoms in patients with known moyamoya disease, repeat
neuroimaging is justified to rule out new ischemia.
The pathogenesis of psychiatric symptoms in moyamoya
disease appears to be multifactorial, with both genetic and
environmental factors influencing specific phenotypes.
Cellular destruction of internal carotid artery distribution
territories from ischemic stroke, repeated transient ischemic
attacks, or hemorrhage of aneurysms and collateral vascular
networks may manifest immediately or progress with time
and new vascular insults. However, patients without ischemic or hemorrhagic stroke also suffer from depression,
anxiety, and other symptoms, indicating a possible linkage
with the disease pathogenesis, likely related to chronic hemodynamic insufficiency of small cortical vessels. Finally,
psychological precipitants, related to prognosis, decrements
in quality of life, and frustration from cognitive impairment,
likely contribute (34). Early engagement in psychotherapy
is highly recommended. One study (8) suggested that the
regions of the brain that are susceptible to hemodynamic
insufficiency in moyamoya disease change as the disease
progresses, shifting from the inner to the outer cortical
structures and from the anterior to the posterior territories.
Thus, even in stable moyamoya disease, new psychiatric
symptoms may develop over time.
Psychiatric comorbidity in moyamoya disease appears to
be a heterogeneous and dynamic phenomenon that affects at
least one-quarter of patients, although the prevalence is likely
significantly underestimated. Treatment choice should be
guided by the patient’s symptoms and neuroimaging of affected brain territories. Neuropsychological testing may be
Am J Psychiatry 176:4, April 2019

The Department of Psychiatry, Semel Institute for Neuroscience and
Human Behavior, University of California, Los Angeles.
Send correspondence to Dr. Gitlin (mgitlin@mednet.ucla.edu).
Drs. Richards and Grzenda contributed equally to this article.
Dr. Grzenda is partly funded by a fellowship from the American Psychiatric
Association Foundation.
The authors report no financial relationships with commercial interests.
Received April 10, 2018; revisions received August 1 and September 20,
2018; accepted October 1, 2018.
Am J Psychiatry 2019; 176:269–274; doi: 10.1176/appi.ajp.2018.18040404

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Am J Psychiatry 176:4, April 2019