Partial tentorial hypoplasia with ipsilateral focal brain herniation is an extremely rare cause of idiopathic intracranial herniation with very few
cases reported in the literature. This is an essentially benign condition which may mimic a sinister pathology to a radiologist unaware of this rare
entity.We present a case of 58 year old male presenting with giddiness and headaches. An MRI brain was performed which revealed this rare
incidental nding which on the rst look simulated a mass.
Creutzfeld -Jacob Disease (CJD) is a rare type of neurodegenerative disease caused due to prion disease and is rapidly progressive and fatal. It is usually suspected based on the typical presenting symptoms, most important symptom being rapidly progressive dementia. Cases with atypical presentation are usually missed due to low clinical suspicion.
We present a case report of a 64-year-old male who presented with worsening cerebellar ataxia and only mild dementia.MRI done to rule out any organic cause, revealed classic findings of CJD.Following this EEG was performed. MRI helped in clinching the diagnosis in this case of low clinical suspicion. This was an extremely rare variant of CJD-the brownell openheimer variant.(1)
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