Behavioral Variant Frontotemporal Dementia-like Syndrome–Atypical Presentation of Intracranial Hypotension.

Abstract

Spontaneous intracranial hypotension (SIH) is an uncommon syndrome which may result from a cerebrospinal fluid (CSF) leak. Atypical presentation of SIH may include neurobehavioral symptoms similar to what is observed in behavioral variant Frontotemporal Dementia.We present the case of a 56 yo Male with postural headache and behavioral symptoms, initially diagnosed with bvFTD. On re-evaluation in our clinic imaging revealed caudal displacement of the midline structures suggestive of CSF hypotension. We hypothesized that with unmitigated CSF loss over a long period of time, the patient developed brain sagging clinically resulting in neurobehavioral, frontal lobe dysfunction. After multitude of investigative procedures CSF venous fistula, originating from a T7 nerve root cyst was found and repaired. Surgical intervention and restoration of CSF volume resulted in resolution of cognitive as well as neurobehavioral symptoms.