To the Editor: Churg-Strauss syndrome is a systemic small-vessel necrotizing vasculitis characterized by asthma, hypereosinophilia, granulomas, and pathological involvement of the heart, kidneys, and CNS.1 Various neurological manifestations have been described, with peripheral neuropathy being the most common.2 Central nervous system involvement has been reported in 13% of Churg-Strauss syndrome patients.3 However an extensive literature search did not reveal any case reports of psychiatric symptoms associated with Churg-Strauss syndrome. Here we present a case of Churg-Strauss syndrome with cognitive and psychotic symptoms, describe our management approach, and briefly discuss pathophysiology.
A 70-year-old man presented to the emergency department with an asthmatic attack and was also found to be acutely confused and agitated. He had a prior history of occasional asthma attacks over the past 2 years. For this admission, he was noted to have marked hypereosinophilia—absolute eosinophil count was 4.71×109(normal 0.04—0.44×109) and 28.3% (normal 0.6%). Antineutrophil cytoplasmic antibodies were positive. There was no renal impairment but the patient was noted to have dilated cardiomyopathy. Diagnosis of Churg-Strauss syndrome was made based on the American College of Rheumatology criteria. The psychiatric consultant noted paranoid delusions about visitors to the hospital following him around and debt collectors hounding him. Further history from the patient’s family revealed that he had been admitted to another hospital 2 years prior with a first episode of delirium which resolved spontaneously. Moreover they reported that he was markedly more forgetful over the preceding 2 years and that he had become increasingly paranoid about neighbors and debt collectors. They verified that he did not have outstanding debts.
Mental status examination revealed an alert, responsive man who was oriented to place and person but not to time. He denied feeling depressed and any past or current auditory hallucinations. He had fixed non-bizarre paranoid delusions. His Mini-Mental State Examination score was 22. He was unable to cooperate with further neuropsychological testing. There were no focal neurologic findings. A head CT showed bilateral cerebral lacunae consistent with thromboembolic disease.
He was treated with high dose prednisone, 30 mg/day, which was tapered during the hospitalization. His psychiatric symptoms predated the introduction of prednisone. The absolute eosinophil count decreased to 0.57×109 and 5.9% within 2 weeks of commencing steroids. Given the absence of literature on psychiatric manifestations of Churg-Strauss syndrome and treatment, our approach was symptom management. He was started on risperidone, 1 mg twice a day, and he became markedly less agitated. His paranoid delusions persisted but to much lesser degree. However his cognitive functioning remained unchanged. Eventually he was discharged in stable condition with prednisolone, 10 mg/day, and risperidone, 1 mg twice a day.
In consultation with his physicians, we decided that aggressive treatment using high dose steroids may modulate the primary disease process and perhaps arrest or slow down the cognitive deterioration while we closely monitored him for steroid-associated psychiatric symptoms. He was stable on outpatient follow-up with pulmonology, rheumatology, and psychiatry.
Churg-Strauss syndrome is known to affect the CNS, but psychiatric symptoms have not been specifically reported in the literature. Keogh4 suggested that CNS involvement was the one clinical manifestation that correlated closely with antineutrophil cytoplasmic antibody status. Like other vasculitides, damage is likely be a result of thromboembolic phenomenon; the hypereosinophilia itself could possibly be neurotoxic,5 or the underlying etiologic cause of Churg-Strauss syndrome could account for neuronal damage. Further investigation is warranted to elucidate the pathophysiology and management of neuropsychiatric sequelae in Churg-Strauss syndrome and similar vasculitic diseases.