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Abstract Details

Slowly Progressive, Acquired Hemicerebral Atrophy Secondary to Chronic Internal Carotid Vascular Disease Mimicking Neurodegenerative Disease
Cerebrovascular Disease and Interventional Neurology
P1 - Poster Session 1 (5:30 PM-6:30 PM)
3-065

Case series of three patients with slowly progressive cerebral hemiatrophy associated with chronic carotid vascular disease.

Cerebral atrophy is a common finding in elderly patients, however, cerebrovascular disease causing very slowly progressive focal cerebral atrophy and focal cerebral dysfunction is unusual. In this report we present three cases of hemicerebral atrophy due to ipsilateral internal carotid artery (ICA) stenosis or occlusion mimicking neurodegenerative conditions.

Case series of three patients with symptoms suggestive of a primary neurogenerative disease, but found to have hemicerebral atrophy with ipsilateral ICA chronic stenosis or occlusion.
Patient 1 had a frontal dysexecutive syndrome potentially consistent with a diagnosis of behavioral variant frontotemporal dementia, however, neuroimaging revealed a chronically occluded left ICA and a pattern of atrophy restricted to the left MCA territory, demonstrating a stark demarcation between the ACA and PCA territories, suggestive of a vascular etiology. Patient 2 presented with progressively worsening seizures and right sided weakness consistent with left hemispheric dysfunction, with radiographic evidence of left hemicerebral atrophy. Angiography revealed a chronic dissection of the left ICA leading to left cerebral hypoperfusion. Patient 3 had asymmetric parkinsonism, alien limb, and cognitive impairment consistent with a diagnosis of corticobasal syndrome. His imaging however, revealed atrophy and encephalomalacia within the right MCA/ACA watershed territories with chronic, severe stenosis of the left ICA suggestive of a chronic hypoperfused state.
In this case series we report three examples of hemicerebral atrophy secondary to chronic ipsilateral ICA vascular disease with diverse progressive clinical symptoms mimicking primary neurodegenerative conditions. This case series highlights the importance of considering chronic hypoperfusion and large vessel severe stenosis or occlusion in patients with evidence of asymmetric brain atrophy. In addition to symptomatic treatment, the management of vascular risk factors including treatment with antiplatelet agents, statins, and revascularization procedures in non-occluded cases may be beneficial.
Authors/Disclosures
Jeffrey R. Vitt, MD (University of California, Davis)
PRESENTER 		Dr. Vitt has nothing to disclose.
Ali G. Hamedani, MD, MHS (Hospital of the University of Pennsylvania) Dr. Hamedani has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ArgenX. Dr. Hamedani has received personal compensation in the range of $0-$499 for serving as a Consultant for LoQus23. The institution of Dr. Hamedani has received research support from NIH. The institution of Dr. Hamedani has received research support from Biogen. The institution of Dr. Hamedani has received research support from Biohaven.
Sarah Horn, MD (UT Health San Antonio) Dr. Horn has received personal compensation in the range of $500-$4,999 for serving as a Consultant for AbbVie. The institution of Dr. Horn has received research support from Alzheimer's Association.
Kimberly Gannon, MD, PhD (Chrisiana Care Health Services - Vascular Neurology) No disclosure on file
Raymond Price, MD, FAAN (University of Pennsylvania) Dr. Price has received personal compensation in the range of $50,000-$99,999 for serving as an Expert Witness for Price Lakeside. Dr. Price has received publishing royalties from a publication relating to health care.
Maxwell A. Greene, MD (Stanford Neurology) Dr. Greene has received personal compensation in the range of $500-$4,999 for serving as a Advisory Board Member with Dysimmune Diseases Foundation .