The aim of this study is to describe a case of Lhermitte-Duclos disease (LDD), which is an extremely rare cause of cerebellar ataxia. LDD is an extremely rare type of benign cerebellar mass and usually manifest as features of raised intracranial pressure and cerebellar dysfunction. Patients may remain asymptomatic for many years, and detection of tumor may be by chance on routine magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS). A 29-year-old female presented to the outdoor patient department with 18 months history of a headache, which was moderate to severe in intensity, throbbing in nature, localized to the right side of the head, and associated with nausea and photophobia. On examination, she had mild cerebellar dysfunction, and MRI/MRS finding was consistent with LDD. LDD is a rare cause of headache and cerebellar dysfunction in younger age group. Treatment may be individualized, and conservative management with regular follow-up may be a better approach if symptoms are mild and nonprogressive in nature.
Keywords: Ataxia; cerebellum; magnetic resonance imaging.