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Abstract Details

Inflammatory Variant of Cerebral Amyloid Angiopthy Mimicking Herpes Encephalitis
Cerebrovascular Disease and Interventional Neurology
P02 - (-)
043
BACKGROUND: The acute occurrence of epileptic seizures, focal neurological signs and reduced vigilance point to the diagnosis of herpes simplex encephalitis. CSF can be normal in about 5% of the cases. This clinical appearance can be mimicked by the inflammatory variant of cerebral amyloid angiopathy (CAA).
DESIGN/METHODS: Case report: A female patient aged 76 with chronic lymphatic leukemia in remission and hypertension was admitted to the ICU because of acute global aphasia and series of generalized tonic-clonic seizures. CCT scans showed bilateral hypodensities in the temporo-parietal white matter with small bleeding spots. EEG rhythm was slowed moderately, and there was a left temporal delta focus. CSF showed an elevated protein content of 77 mg/dl. Immediately, antiepileptic medication with 2 x 500 mg levitirazetam was started and an antiviral therapy using intravenous acyclovir. Within 2 days, the patient regained consciousness, he showed mild cognitive slowing and gait ataxia, but no more focal neurological signs and an improvement of focal slowing in the EEG.
RESULTS: MRI on day 3 exhibited multifocal confluent edema in the left temporo-occipital, right temporal and right parietal white matter, with multiple microbleeds in susceptibility-weighted imaging, without any gadolinium enhancement. PCR and antibodies for HSV were negative. Cerebral angiography was normal. An oral medication of prednisolon lead to a slow, but good alleviation of neurological symptoms, in parallel with disappearance of subcortical edema.
CONCLUSIONS: The typical MRI appearance and the good response to steroids point to the by the inflammatory variant of cerebral amyloid angiopathy as correct diagnosis, which is a clinical, neuroradiological, histological and genetical disease entitiy of its own (Kinnecom et al., Neurology 2007). In such cases, its diagnosis does not necessarily require a brain biopsy.
Authors/Disclosures
Wolfgang Heide, MD, FEAN (AKH Celle)
PRESENTER
No disclosure on file
No disclosure on file
Val J. Lowe, MD (Mayo Clinic) Dr. Lowe has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AVID Radiopharmaceutical. Dr. Lowe has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai Inc. The institution of Dr. Lowe has received research support from AVID Radiopharmaceuticals.