Abstract

Background: Lhermitte-Duclos disease (LDD), also referred to as dysplastic gangliocytoma of the cerebellum, is a rare hamartomatous benign lesion of the cerebellum. Its etiology is yet unknown; some research classifies it as a neoplastic tumor, while others identify it as a malformation or hamartomatous illness. The symptoms of increased intracranial pressure are the main presentation of LDD. The exact correlation between LDD and Cowden syndrome (CS) is still uncertain. However, evidence shows that LDD is strongly associated with CS and considers it part of this condition. Although histological biopsy is used to confirm the diagnosis, knowledge of the characteristic radiological findings helps diagnose this rare disease and spares the need for biopsy in most cases. T2-weighted and fluid-attenuated inversion recovery sequences in magnetic resonance imaging (MRI) demonstrate hyper-intense lesions, while T1-weighted sequences display hypo-intense lesions. As a result, the lesion surface shows parallel linear striae widening of the cerebellar folia. This pattern appears frequently and is referred to as "striated cerebellum" or "tiger stripes" in LDD patients.
Case Presentation: We report a 15-year-old girl presented to our emergency department complaining of progressive headache, vomiting, and gait ataxia for 2 weeks. With characteristic images finding "tiger stripes" of LDD, she underwent suboccipital craniotomy with total resection of the tumor. Our patient was followed up, and she is healthy with no recurrence.
Conclusion: Lhermitte-Duclos's disease is an exceedingly rare atypical lesion of uncertain cause that has been linked to Cowden disease and PTEN hamartoma tumor syndrome path mutations. As a result, a histological investigation should be undertaken if the MRI is compatible. We propose that peripheral enhancement of LDD is most likely due to cerebellar venous draining system vascular proliferation and, therefore, should be regarded as one of the imaging hallmarks of LDD.

Key words: Lhermitte-Duclos disease, dysplastic cerebellar gangliocytoma, Cowden syndrome, cerebellar mass.