A 48-year-old woman was admitted with a 3-year history of intermittent dizziness and unstable gait. Cranial MRI demonstrated a laminated lesion of T2 hyperintensity and T1 hypointensity involving the left cerebellar hemisphere, which appeared enlarged (figure, A–C). Secondary hydrocephalus and Chiari I malformation were observed. Because the striated appearance on MRI was characteristic of Lhermitte-Duclos disease,1 presurgical diagnosis was made. Total resection of this lesion was performed, and histopathologic evaluation confirmed the diagnosis (figure, D). Follow-up neuroradiologic studies revealed no recurrence. However, recent ultrasonography showed multiple nodules in the patient’s right breast, which was predictive of a confident clinical diagnosis of Cowden disease.2
Axial T2- and T1-weighted MRIs (A, B) show the typical striated pattern with alternating bands characterizing Lhermitte-Duclos disease. Mild hydrocephalus and the inferiorly displaced cerebellar tonsil were noted on the sagittal T1-weighted image (C). Histopathologic study (D) (hematoxylin & eosin stain; scale bar = 200 μm) confirmed the MRI diagnosis.
AUTHOR CONTRIBUTIONS
Dr. Wei: drafting and revising the manuscript, study concept or design, study supervision. Dr. Liu: drafting and revising the manuscript, analysis or interpretation of data, study supervision. Dr. Wu, Dr. Kang, Dr. Li: study concept or design, acquisition of data.
STUDY FUNDING
No targeted funding reported.
DISCLOSURE
The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- © 2013 American Academy of Neurology
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