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Abstract Details

Cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 positivity: A case report of a treatment dilemma and a possible relationship
Aging, Dementia, Cognitive, and Behavioral Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)

Cerebral amyloid angiopathy-related inflammation (CAARI) has been described as a distinct syndrome with subacute cognitive decline or seizure symptoms. Previous studies have established diagnostic criteria for this entity and proposed a treatment strategy. However, the precipitating factors behind developing CAARI in a subset of patients with CAA are unclear. We present a case of CAARI in a patient with no underlying diagnosis of dementia that was found to be positive for HHV-6 in serum and CSF and treated successfully with steroids.

 To examine the treatment dilemma utilizing high-dose steroids in a patient with cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 positivity. Additionally, to question a possible relationship between HHV-6 and CAARI.

An 83-year-old woman presented with a first-time generalized tonic-clonic seizure with postictal confusion. On presentation, her systolic blood pressure was elevated at 150-170, and she was tachycardic to 160 with a temperature of 38.4. A contrasted MRI brain showed multifocal vasogenic edema, sulcal FLAIR hyperintensity, and innumerable lobar microhemorrhages. Black blood contrast MRI brain angiogram was unremarkable, with no evidence of vasculitis. At this point, the patient had clinically improved but remained slightly encephalopathic with word-finding difficulties. Continuous EEG monitoring showed diffuse background slowing. Initial CSF studies revealed an isolated mildly elevated protein of 49 and negative cytology. Therefore, based on the clinical and radiographic evidence, a decision was made to undergo five days of high-dose methylprednisolone for probable CAARI. However, days later, HHV-6 in CSF resulted positive with 249 copies/ml, and a serum sample showed 1100 copies/ml. 


 The concurrence of cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 in serum and CSF might yield a diagnostic and therapeutic challenge. However, the treatment with high-dose pulsatile steroids with taper did improve the overall clinical picture. Further studies are needed to investigate a potential correlation.
Anas Alshawa, MD (NM)
Dr. Alshawa has nothing to disclose.
Muhammad Makkieh, DO (University of Arizona Tucson ) Dr. Makkieh has nothing to disclose.
Hanan Alsakka, MD Miss Alsakka has nothing to disclose.
Myrka Raquel Torres-Melendez, MD (Banner-University of Arizona) Dr. Torres-Melendez has nothing to disclose.