(098) Conjuring Comprehension of Amyloid Spells: An Unexpected Presentation of Psychosis and Catatonic Symptoms in a Patient With Cerebral Amyloid Angiopathy

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Background: When elderly patients without a psychiatric history present with psychosis, catatonic symptoms, ‎and cognitive decline, a typical differential includes delirium, encephalitis, worsening neurocognitive disorder, ‎substance-induced disorder, and primary psychiatric disorder. Here, we present a case of Cerebral Amyloid Angiopathy ‎‎(CAA) in a patient with hallucinations, catatonic symptoms, and cognitive impairment. We also highlight the importance ‎of accurate diagnosis, especially in black persons who are disproportionately affected by CAA (Copenhaver, 2008) and ‎by misdiagnosis of psychotic illnesses (Schwartz, 2014).‎

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Case: X.X. is a 67-year-old black man with no psychiatric or substance use history, medical history of seizures, ‎chronic microhemorrhages, and hypertension who was admitted for worsening confusion and psychosis in setting of ‎social stressors and rapid cognitive decline. Consultation liaison psychiatry (C-L) was consulted for paranoia. On ‎evaluation, X.X. was dysphoric, paranoid, thought blocked, and disorganized. Initial differential included encephalitis, ‎dementia, delirium, and psychotic depression. He was started on olanzapine. He was later observed having ‎unresponsive episodes associated with staring, mutism, and immobility. The episodes initially responded to ‎lorazepam, raising concern for catatonia verses seizure. Video-electroencephalogram revealed no epileptiform ‎activity. Lorazepam was discontinued due to sedation on repeat low doses. CSF was largely unremarkable. Imaging ‎was remarkable for chronic small vessel infarctions and positive hemosiderin stain shifting diagnostic concern to CAA-‎Related Inflammation (CAARI), a CAA subset. Diagnostic brain biopsy revealed amyloid infiltrate confirming CAA ‎diagnosis. Unresponsive episodes were reconsidered "amyloid spells," transient focal neurologic episode seen in ‎‎~15% of CAA (Charidimou, 2014). X.X. was treated with high dose steroids. Aspirin was discontinued given increased ‎bleeding risk associated with CAA. Rheumatology was consulted, and he was started on cyclophosphamide. He was ‎discharged with multidisciplinary follow-up. X.X currently has improved cognition and his psychosis has resolved.‎

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Discussion: This case highlights the importance of considering a broad differential, and the complexity in ‎diagnosing and treating CAA given the overlapping features it shares with seizures, catatonia, and psychiatric ‎disorders, among other diagnoses. Moreover, psychiatric misdiagnosis may lead to undue social stigma (Schwartz, ‎‎2014) and inappropriate treatment (e.g., aspirin that further increases bleeding risk in CAA), especially in black ‎patients.‎

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Conclusion: Collaborative care involving C-L and other relevant specialties in cases like these is imperative. C-L ‎should remain vigilant to psychological sequelae of neurologic conditions such as CAARI. Obtaining swift imaging and ‎neurology input may alleviate prolonged suffering and reduce the risk of misdiagnosis.‎

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References: ‎
‎1. Charidimou A, et al. Spectrum of transient focal neurological episodes in cerebral amyloid angiopathy: multicentre ‎magnetic resonance imaging cohort study and meta-analysis. Stroke. 2012;43:2324-30.‎
‎2. Cooperman CS, Shah AK, Rajamani K. Amyloid spells. Neurol Clin Pract. 2019; 9:17-18.‎
‎3. Copenhaver, BR., et al. Racial Differences in Microbleed Prevalence in Primary Intracerebral Hemorrhage. ‎Neurology. 2008; 71:1176-1182.‎
‎4. Ghaffari L, Bal T. An Unusual Presentation of Acute Psychosis in an Elderly Patient with Inflammatory Cerebral ‎Amyloid Angiopathy. American Academy of Neurology. 2020; 94.‎
‎5. Schwartz RC, Blankenship DM. Racial disparities in psychotic disorder diagnosis: A review of empirical literature. ‎World J Psychiatry. 2014; 4:133-40.‎