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Question Date: 19/09/2023

Question: 58 year old male presented with subacute onset rapidly progressive behavioral disturbances, headache, right upper limb weakness and right upper limb focal motor seizures with preserved awareness. MRI Brain showed patchy confluent asymmetric T2 weighted hyperintense signals and GRE showed multiple tiny area of blooming in the same areas. CSF routine showed 10 Wbc and 76 mgdl proteins, sugar was normal. CSF bio fire was negative and neoplastic cells were negative. What is the most likely diagnosis?

Options:

CAARI Cerebral amyloid angiopathy related inflammation
CAA Cerebral amyloid angiopathy
Superficial siderosis
Tumefactive demyelination

Correct Answer: CAARI Cerebral amyloid angiopathy related inflammation

Explaination: CAARI is characterized by a subacute onset of cognitive or behavioral changes associated with headache, seizures, and stroke-like signs, in contrast to the acute intraparenchymal hemorrhages typically seen on presentation in patients with CAA. CAARI is associated not only with the characteristic microbleeds present on MRI gradient echo sequences as in CAA but also with large areas of inflammation or edema on MRI. Diagnostic criteria (1) acute or subacute onset of symptoms (2) age 40 years (3) at least one of the following symptoms headache, mental or behavioral change, focal neurological signs, or seizures (4) MRI showing patchy or confluent T2 signal (5) evidence of CAA by gradient echo MRI or previous lobar hemorrhage and (6) no neoplastic or infectious cause of this picture

Reference: Kirshner HS, Bradshaw M. The Inflammatory Form of Cerebral Amyloid Angiopathy or "Cerebral Amyloid Angiopathy-Related Inflammation" (CAARI). Curr Neurol Neurosci Rep. 2015 Aug15(8)54. doi 10.1007s11910-015-0572-y. PMID 26096511.