Imaging In A Case Of Lhermitte Duclos Disease - Dysplastic Cerebellar Gangliocytoma.

"Lhermitte-Duclos disease (LDD) is a rare cerebellar lesion of uncertain origin. It is linked to an autosomal- dominant phakomatosis known as Cowden's disease in 40% of patients. The MRI features of LDD are almost unique and can be considered diagnostic. (1) We report on a case of 48 year old female patient who presented to us with history of seizures and bilateral lower limb weakness with the typical MRI features of the above disease. We also discuss the pathology and genetics of this rare disease"

Prosopagnosia: a rare presenting manifestation of frontotemporal lobar degeneration.

Frontotemporal dementia is an important neurodegenerative disorder accounting for a significant proportion of dementia cases with onset before 60 years of age. Apart from the well recognized behavioral changes the disease has many other distinctive features like predominant language involvement alone or associated features of motor neuron disease or parkinsonism etc. which at times may be the presenting manifestation itself. In the following article we describe a rare presenting manifestation; prosopagnosia, in the setting of frontotemporal degeneration.