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

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Spontaneous Intracranial Hypotension (SIH) is an uncommon syndrome which may result from a Cerebrospinal Fluid (CSF) leak. Atypical SIH may present with neurobehavioral symptoms similar to those observed in behavioral variant Frontotemporal Dementia (bvFTD). We present a case of a 55-year-old male with postural headache and behavioral symptoms who was initially diagnosed with bvFTD. On re-evaluation in our clinic, imaging revealed caudal displacement of the midline structures suggestive of Cerebrospinal Fluid (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, a 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.