Lhermitte-Duclos syndrome

Last edited and medically reviewed by Alaric Steinmetz on 04.03.2023

Synonyms: dysplatisches cerebelläres Gangliozytom

Lhermitte-Duclos syndrome describes a rare mass lesion of the posterior fossa, also referred to as dysplastic cerebellar gangliocytoma (WHO Grade I).

Epidemiology

This is a very rare disease that is frequently associated with autosomal dominant Cowden syndrome. Approximately 200 cases have been described in the literature worldwide¹.

Imaging

Imaging typically shows a tiger-stripe pattern resulting from enlarged folia.

Treatment

Particularly in young patients, a biopsy should be considered to rule out a medulloblastoma in the differential diagnosis. Surgical resection should be considered in the presence of significant mass effect².

Imaging

Lhermitte-Duclos syndrome MRI — Axial T1-weighted MRI image of Lhermitte-Duclos syndrome. Figure adapted from Robinson and Cohen. Figure [A] shows the characteristic cerebellar “tiger-stripe” pattern of Lhermitte-Duclos disease without mass effect in a 17-year-old female patient. Figure [B] shows an advanced finding with mass effect already present.

References

Robinson S, Cohen AR. Cowden disease and Lhermitte–Duclos disease: an update. FOC. January 2006:1-6. ↩ ↩
Greenberg MS, Abel N, Agazzi S. Handbook of Neurosurgery. Georg Thieme Verlag; 2020. ↩ ↩

Article navigation