Case A 58 year old man was referred to the memory clinic with a 2 year history of deteriorating memory and altered behaviour. His wife reported that he has become obsessed with pornography and appeared apathetic and devoid of emotion. Reportedly, he has not been well for 10 years since developing a severe postural headache. He has reported intermittent incontinence, unsteadiness, fatigue and dysphagia. Though investigated extensively, no cause had been found. There was no family history of neurological or psychiatric illness.
Physical examination was unremarkable. He scored 91/100 on ACE-R testing, losing points for memory and fluency.
The history suggested behavioural variant frontotemporal dementia (bvFTD). Frontal deficits were confirmed on neuropsychological testing.
On review, a previous MRI demonstrated signs suggestive of intrancranial hypotension with distortion of the brainstem. A diagnosis of Frontotemporal brain sagging syndrome (FBSS) was proposed. An MRI myelogram failed to demonstrate a source of CSF leakage. Despite this, the patient is currently undergoing treatment with an epidural blood patch.
Discussion Intracranial hypotension with brain sagging has been reported in the context of Frontotemporal patterns of cognitive impairment. Attempts at treating FBSS have yielded varying results. We present our experiences in the diagnosis and management of this rare, but potentially reversible cause of dementia, alongside a review of the available literature.
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