Lhermitte-Duclos disease: rare clinical case

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Abstract
Lhermitte-Duclos disease (LDD) is a rare pathological entity, histologically defined as dysplastic cerebellar gangliocytoma ‒ a benign cerebellar neoplasm (CNS WHO grade 1). Although its etiology remains unclear, cerebellar involvement may present either sporadically, predominantly in children, or as a component of Cowden syndrome, an autosomal dominant condition characterized by multiple hamartomas arising from all three germ layers. The prognosis of this slow-growing benign lesion is generally favorable, with surgical resection recommended only in symptomatic cases. We report a clinical case of successful neurosurgical management in a 3-year-old patient presenting with hypertensive-hydrocephalic syndrome. Magnetic resonance imaging revealed the characteristic “tigroid” striation pattern on T2-weighted images, while histopathological examination of the cerebellar cortex confirmed the presence of large dysplastic ganglionic cells. This case highlights the importance of an individualized therapeutic approach in neuro-oncology and underscores the key clinical and morphological features of Lhermitte-Duclos disease.

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