Intracranial Hypotension Causing Reversible Frontotemporal Dementia and Coma

Frontotemporal brain sagging syndrome

“…2,4,5,8 The lack of typical MRI or pathologic findings suggests a different etiology of the clinical bvFTDlike phenotype in FBSS. [12][13][14][15][16] In fact, CSF leaks have been previously implicated in the presentations of FTD [17][18][19] and in patients with deep brain swelling with few neurologic symptoms and scarce pachymeningeal enhancement. Decreased salient network connectivity within the cortices, deep midline, and brainstem structures with enhanced cortical default mode network connectivity has been associated with bvFTD.…”

“…10 The marked distortion of the deep midline and brainstem structures in FBSS may disrupt these networks in a similar pattern. Nevertheless, a variety of presentations of CSF leaks are becoming increasingly recognized [17][18][19][24][25][26][27][28][29][30][31][32][33] : pachymeningeal enhancement can be absent, opening pressure can be normal in a significant minority, [34][35][36] CT myelography may only show meningeal diverticula as leaks can be difficult to visualize, 22 early bladder activity on cisternography occurs in high frequency in active leaks, 22,37,38 and lack of sustained improvement with epidural blood patch is not uncommon. 11 The sagging brain in FBSS may be due to a CSF leak or spontaneous intracranial hypotension, where brain sag is also a common imaging finding.…”

Atypical Presentations of Intracranial Hypotension: Comparison with Classic Spontaneous Intracranial Hypotension

“…Atypical clinical presentations of SIH have been sparsely reported in the literature, mostly in case report format, and include the following: obtundation, 3 stupor, 4 sensorineural deafness, 5 short-term memory deficit, 5 dementia with frontotemporal features, [6][7][8] and parkinsonism and ataxia.…”

Clinical Reasoning: A 50-year-old man with headache and cognitive decline

“…7,8 The mechanism of the headache in intracranial hypotension is postulated to be caused by downward displacement of the brain due to lack of buoyancy normally provided by CSF, stretching pain-sensitive intracranial structures such as the dura. 2 Because the brain sags and becomes compressed against the skull base, patients may present with cognitive changes similar to those seen in the behavioral variant of frontotemporal dementia, a condition described as the frontotemporal brain sagging syndrome.…”