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Uplifting news for frontotemporal dementia

In this issue of Neurology ®, Wicklund et al.1 report and discuss an extraordinary series of individuals with clinical features of behavior variant frontotemporal dementia (bvFTD)2 caused by frontotemporal brain sagging syndrome (FBSS) (spontaneous intracranial hypotension). In FBSS, structural imaging shows downward displacement or “sagging” of the brain,3 which may have an iatrogenic or traumatic etiology, or occur spontaneously.4 Some cases of spontaneous FBSS may occur with dural rupture and CSF leakage along nerve root sleeves.5 Other times, however, CSF leakage is not demonstrated. The precise mechanism thus remains to be established. This syndrome nevertheless is of interest to clinicians because there are specific treatments available for some forms of FBSS such as spontaneous intracranial hypotension, as opposed to the symptomatic management strategies that are used …