Abstract Details

Title Imaging and Neuropsychological Findings in Chronic Wernicke-Korsakoff Syndrome with Isolated Involvement of the Periaqueductal Gray Matter

Topic General Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 6-003

Objective To describe a case of chronic Wernicke-Korsakoff Syndrome (WKS) with persistent but isolated signal abnormalities in the periaqueductal gray matter 12 months after onset.

Background WKS is a disorder of thiamine deficiency resulting in confusion, gait ataxia and ophthalmoparesis in the acute stage, with prominent memory loss in the chronic stage. Reported brain MRI findings in consist most often of T2 hyperintensities, diffusion restriction and/or enhancement of the mamillary bodies, medial thalami and periaqueductal gray matter (PAG). However, these findings are most often restricted to the acute stage, whereas imaging of the chronic phase is less well elucidated. Furthermore, isolated radiographical involvement of the PAG in WKS has not been described.

Design/Methods Case Report

Results A 69 year old man with 18 years education developed subacute encephalopathy and gait ataxia in the setting of cholangiocarcinoma, undergoing chemotherapy with rapid 75 pound weight loss. Brain MRI demonstrated T2/FLAIR hyperintensity isolated to the PAG while CSF was non-inflammatory. 12 months later, the signal change of the PAG persisted and the patient was noted to have difficulties with memory impairment, particularly temporal order and source memory, as well as confabulation, disorientation, and poor insight. Neuropsychological findings were notable for severe impairment in frontal-executive functions, which appeared to be interfering with learning and memory, suggestive of a frontal lobe memory disorder. He additionally demonstrated marked gait ataxia rendering him wheelchair bound.

Conclusions Isolated radiographical lesions of the PAG can occur in the context of WKS. Though the PAG is largely described to modulate pain/analgesia and fear/anxiety as well las autonomic responses, its role in anatomical and functional interface with the medial prefrontal regions can potentially lead to cognitive and behavioral deficits.

Authors/Disclosures
Meredith Wicklund, MD, FAAN (Mayo Clinic Arizona) PRESENTER	The institution of Dr. Wicklund has received research support from Arizona Alzheimer's Consortium. The institution of Dr. Wicklund has received research support from NIH.