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Abstract Details

Diagnosis and Treatment of Steroid Responsive Cerebral Amyloid Angiopathy Related Inflammation without Brain Biopsy
Cerebrovascular Disease and Interventional Neurology
P2 - Poster Session 2 (5:30 PM-6:30 PM)
1-008
To present a rare case of steroid responsive Cerebral Amyloid Angiopathy-related Inflammation (CAARI) where diagnosis and treatment were made without brain biopsy.
CAARI is a new subset of cerebral amyloid angiopathy. Clinically it presents with acute/subacute cognitive decline, focal neurologic deficits and headache without hemorrhage. The diagnosis and treatment of CAARI are still being elucidated, but diagnosis has previously been made with brain biopsy.
An 88 year old woman presented with word finding difficulty and headache. Non-contrast CT head showed large regions of vasogenic edema in the right frontal and left parietal lobes. Dexamethasone 4 mg every 6 hours was started for presumed cerebral metastasis with improvement in symptoms.  MRI brain was consistent with CAARI. Aphasia resolved within a day. The patient was discharged on a tapering dose of oral steroids but returned after 2 weeks with headache and worsening confusion despite improved MRI. Symptoms again improved with pulse dose steroids.

MRI brain with/without contrast showed large regions of T2/FLAIR hyperintensity within the right frontal, left parietal and left temporal lobes with hyperemic enhancement. There were innumerable cortical microhemorrhages consistent with amyloid angiopathy. The constellation of findings was compatible with a diagnosis of CAARI.

Repeat MRI brain after 3 weeks of treatment showed decreased size and extent of abnormal regions.

CSF analysis detected reduced A-beta 42 to Total-tau index with elevated phospho-tau, consistent with Alzheimer’s disease.

Based on the patient’s age, clinical and diagnostic features suggestive of CAARI, the decision was made not to pursue brain biopsy.

Our patient’s clinical and radiographic improvement with steroids confirmed the diagnosis without requiring invasive brain biopsy. On tapering steroids there was recurrence of symptoms; the duration of immunosuppressive treatment is yet to be determined. Unlike traditional amyloid angiopathy, this is a treatable condition.
Authors/Disclosures
Kavneet Kaur, MD
PRESENTER 		Dr. Kaur has nothing to disclose.
Vaibhav Goswami, MD (Tower Health) Dr. Goswami has nothing to disclose.
Rafia Shafqat, MD (OhioHealth Physician Group) No disclosure on file
Kristina Maselli, MD (Montefiore Headache Center) Dr. Maselli has nothing to disclose.
Anila Thomas, MD Dr. Thomas has nothing to disclose.