Acute dysphasia and reversible cognitive decline in a patient with probable cerebral amyloid angiopathy-related inflammation.

Cerebral amyloid angiopathy related inflammation (CAAri) is becoming increasingly recognised as a subset of cerebral amyloid angiopathy (CAA). CAAri generally presents with subacute cognitive decline, headaches, seizures, behavioral changes, and focal neurological deficits. We describe a patient who developed acute dysphasia and reversible cognitive decline due to probable CAAri. CT brain showed bilateral vasogenic edema in the cerebral hemispheres, predominantly involving the parietal and temporal lobes, left greater than right without enhancement. Magnetic resonance brain imaging showed extensive multifocal areas of subcortical white matter T2 hyperintensity in the frontal and temporal regions with associated mass effect, negligible enhancement, and multiple foci of microhemorrhage on susceptibility weighted imaging sequences consistent with a diagnosis of probable CAAri. She responded dramatically to a course of intravenous methylprednisolone followed by further immunosuppression with pulse intravenous cyclophosphamide. Her dysphasia resolved within 5 days of intravenous methylprednisolone therapy. Her MMSE improved from 11/30 at day 5 of admission to 28/30 at 6-month follow-up. The notable features of our case were the unusual CT findings, which were inconsistent with stroke and diagnostic utility of susceptibility-weighted magnetic resonance imaging in confirming the diagnosis which allowed for prompt institution of immunosuppression.

Nontraumatic spinal cord ischaemic syndrome.

This study presents the clinical features and functional outcomes of eight consecutive patients who were admitted to our institution between 2012 and 2014 with nontraumatic spinal cord infarction (SCI), a rare and devastating condition. We also present a literature review of aetiologies and prognostic factors relevant to our case series. The mean age of our cohort was 64 years and five patients were female. Aortic disease was causative in three, including one patient with biopsy confirmed giant cell arteritis. Fibrocartilaginous embolism was a possible aetiology in two others, anterior spinal artery aneurysm in one, and the cause was undetermined in two patients. American Spinal Injury Association impairment scale (ASIA) scores at nadir (time of maximum severity of signs) were B in three, C in three and D in two patients (all were wheelchair dependent). At last follow-up, ASIA scores were C in one, D in five and E in one patient. One patient died, two remained wheelchair dependent, four required a walking aid or frame and one was mobilising independently. A literature review of 11 patient series of nontraumatic SCI found that prognosis is primarily determined by the severity of motor or sensory involvement, in particular, initial and nadir ASIA A/B scores which strongly correlate with poor outcome. In the majority of series, 40-60% of patients had initial ASIA A/B scores with a similar proportion remaining wheelchair dependent on follow-up. Most patients in our cohort had nadir ASIA C/D scores, which may explain their better outcomes.