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2017 Case 1

Case 1

E. McGinnis1 and J.A. Maguire1,2

1Department of Pathology and Laboratory Medicine, Faculty of Medicine, 2Division of Neuropathology, Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC Canada

Case Summary:

A 63-year-old man presented with reduced level of consciousness, right-sided motor abnormalities, mixed aphasia, visual blurriness, slight memory loss and a transient right-sided gaze. There was no history of headache, cough, rashes, chest or abdominal symptoms, or diarrhea. His past medical history included asthma, gout, gastroesophageal reflux disease. No recent illnesses. Travel history revealed that one month prior to admission he had engaged in camping; and swimming and kayaking in a fresh water lake. He had worn a contact lens in the right eye for days at a time without changing it. The last lens he wore was removed 4 days after hospitalization.

Investigations and course: CT head and CSF analyzed early in his admission were unremarkable. Over subsequent days he developed T2 hyperintense foci in left parietal and temporal cortex and leptomeningeal enhancement by magnetic resonance imaging. He developed CSF lymphocytic pleocytosis with elevated protein and low glucose, with no identifiable microorganisms. CSF VDRL was negative. Bacterial, viral, and fungal cultures and serologies were negative (Lyme disease, West Nile virus, viral hepatitis, arboviruses, enteroviruses, cryptococcus, HHB-6/7, Rickettsia, Bartonella, Brucella, Coxiella, HIV, Syphilis, mycobacteria, HSV, VZV). No paraneoplastic autoantibodies were identified. Blood cultures were negative. CT chest, abdomen, and pelvis revealed multiple hyperdense foci in the liver identified on ultrasound as cysts but no intraabdominal or intrathoracic masses. He developed more prominent decompensation of his dense right hemiplegia. An EEG showed left-sided epileptiform activity. He had intermittent fevers and was treated with ceftriaxone, ampicillin, vancomycin, acyclovir, and dexamethasone for presumed meningoencephalitis of undetermined etiology. Following neurologic decline requiring intubation a repeat CT demonstrated widespread left hemispheric injury extending through the basal ganglia to the midbrain and pons. He was not a neurosurgical candidate and died twenty-two days after admission.

Materials submitted: 2 MRI images, 2 gross autopsy photos, 1 H&E stained scanned slide



1. What diagnosis was reached at autopsy?

2. How can you confirm the diagnosis?

3. Is there any treatment?