Lhermitte Duclos disease true is:

Ans. is 'a' i.e. Thickened cerebellar folli [Refwww.neurology india.com/article.asp; www.neuropath.org/orenJ Lhermitte Duclos disease was first described in 1920 and is quite rare with fewer than 100 cases having been previously reported. It is also known as Purkinjeoma, granular cell hypertrophy of the cerebellum, hamartoma of the cerebellum, dysplastic gangliocytoma, ganglioneuroma and angiomatosis of the cerebellum. Clinical features:- They are usually low-grade malignancy. They are usually diagnosed in the 3rd decade of life. Most patients show signs of increased intracranial tension or cerebellar dysfunction. Prognosis is good if the tumour is resected. Tonsillar herniation and hydrocephalus common. Macroscopically There is widening and thickening of the cerebellar folia which efface the sulci The thickening is usually quite homogenous which makes LDD look like "exaggerated" or "giant" folia on gross examination Histopathological findings Widening of the molecular layer Abnormal myelination that is occupied by abnormal ganglion cells. The absence of the Purkinje cell layer Hypertrophy of granular cell layer Atrophy of cerebellar white matter MRI appearance of Lhermitte Duclos disease Non enhancing unilateral lesion in the cerebellum with mass effect on surrounding structures. T1 images hyperintense and T2 weighted are hypointense with alternating parallel hyperintense and isointense stripes (tiger stripping laminations), pathognomonic of Lhermitte Duclos disease.