Abstract Details

Title One Side of the Story: Unilateral Gyriform Enhancement in Wilson's Disease

Topic Movement Disorders

Presentation(s) P8 - Poster Session 8 (8:00 AM-9:00 AM)

Poster/Presentation
Number 5-024

Objective
To report a case of untreated Wilson’s disease presenting with unilateral gyriform enhancement on Brain MRI.

Background
Gyral enhancement, or gyriform enhancement, is a contrast enhancement pattern in the superficial brain that follows the shape of the cerebral gyri. In Wilson's disease, abnormalities often appear in the gray matter of the lentiform, caudate, and thalamic nuclei, along with cerebral atrophy in the frontal lobes and cerebellum. We present a case of Wilson’s disease exhibiting a notable finding of unilateral gyral contrast enhancement on brain MRI.

Design/Methods N/A

Results
This case involves a 66-year-old woman with a medical history of Wilson’s disease, bipolar disorder, and squamous cell carcinoma of the tongue post-glossectomy. She was diagnosed with Wilson's disease at age 24 during an ophthalmology appointment, where Kayser-Fleischer rings were observed. Initially treated with penicillamine, she discontinued it due to concerns about long-term side effects and taste aversion. Subsequent zinc preparations were intolerable due to gastrointestinal issues, and she struggled with compliance to a low-copper diet.

In early 2024, she presented to the hospital with decompensated Wilson's disease after stopping chelation therapy, reporting frequent falls. She had multiple admissions for weakness, difficulty ambulating, and failure to thrive. Video EEG showed multiple episodes without ictal correlation. Imaging revealed restricted diffusion in the right thalamus and significant cortical enhancement in the right frontal lobe and basal ganglia. CSF analysis showed no signs of inflammation, suggesting disease progression rather than an inflammatory process.

Conclusions

Gyral enhancement in Wilson's disease is a noteworthy and rare imaging finding, resulting from copper accumulation in the brain and leading to patchy or gyriform areas of increased signal on MRI. This pattern is rarely seen in untreated or advanced cases. Recognizing gyral enhancement is crucial for timely diagnosis and treatment, highlighting the severity of copper toxicity and the need for effective management strategies.

Authors/Disclosures
Nikita Srinivasan, MBBS PRESENTER	Dr. Srinivasan has nothing to disclose.
Rafail A. Chionatos, MD (Tufts Medical Center)	Dr. Chionatos has nothing to disclose.
Camelia Valhuerdi Porto, MD (Tufts Medical Center)	Dr. Valhuerdi Porto has nothing to disclose.
Naman Bareja, MBBS (Naman Bareja)	Dr. Bareja has nothing to disclose.
Illya Tolokh, MD (Massachusetts General Hospital, Brigham, Harvard)	Dr. Tolokh has nothing to disclose.

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