Behavioral Variant Frontotemporal Dementia as a Serious Complication of Spontaneous Intracranial Hypotension

Schievink, Wouter I.; Maya, M. Marcel; Barnard, Zachary R.; Moser, Franklin G.; Jean-Pierre, Stacey; Nuño, Miriam A

doi:10.1093/ons/opy029

Cited by 43 publications

(86 citation statements)

References 28 publications

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“…The etiology of FTBSS is usually attributed to a spontaneous spinal CSF leak, however in the largest clinical series, 12/29 (41%) cases had normal spine imaging. [ 15 ] In our case, a CSF-venous leak was meticulously identified. They seem to occur in the thoracic spine and the thoracolumbar junction, are more frequently paravertebral, and most of them are associated with a nerve root sleeve diverticulum.…”

Section: Discussionmentioning

confidence: 80%

“…[ 22 ] coined the term FTBSS, characterized by behavioral disinhibition, apathy/inertia, loss of empathy, perseverative behavior, hyperorality, memory impairment, auditory and/or visual hallucinations, hypersomnolence, orthostatic headache, neck pain, tinnitus, dysarthria, dysphagia, tremors, orofacial dyskinesias, nausea, and unsteady lurching gait. [ 15 ]…”

Section: Discussionmentioning

confidence: 99%

“…Although SIH, also called craniospinal hypovolemia, is caused by leakage of CSF volume from the spine, CSF pressure is normal in the majority of patients and it only decreases when the rate of CSF volume loss cannot be furthered compensated. [ 8 - 10 , 14 ] The most important intracranial complications are in the short-term subdural hematomas or hygromas and in the long-term FTBSS,[ 15 , 17 ] which leads to a mechanical disruption of the frontotemporal, deep midline, and brainstem tracts. [ 22 ] Orthostatic headaches are secondary to a CSF depletion, leading to undue traction to vascular structures[ 11 ] and brain displacement toward the skull base meninges.…”

Section: Discussionmentioning

confidence: 99%

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Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula

Ortega-Porcayo

Ortega

Quiroz-Castro

et al. 2020

Surgical Neurology International

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Background: The frontotemporal brain sagging syndrome (FTBSS) is defined as an insidious/progressive decline in behavior and executive functions, hypersomnolence, and orthostatic headaches attributed to cerebrospinal fluid (CSF) hypovolemia. Here, a T6 CSF-venous fistula (e.g., between the subarachnoid CSF and a paraspinal vein) resulted in a CSF leak responsible for craniospinal hypovolemia. Case Description: A 56-year-old male started with orthostatic headaches and fatigue after scuba diving. His symptoms included progressive, vertigo, tinnitus, nausea, lack of judgment, inappropriate behavior, memory dysfunction, apathy, tremor, orofacial dyskinesia, dysarthria, dysphagia, and hypersomnolence. The lumbar puncture revealed an opening pressure of 0 cm H2O. Magnetic resonance imaging (MRI) findings included brain sagging, bilateral temporal lobe herniation, and pachymeningeal enhancement. The computed tomography (CT) myelogram showed a thoracic diverticulum and a CSF-venous leak at the T6-T7 level. Surgery, which comprised a T6-T7 laminotomy, allowed for dissecting, clipping, and ligating the diverticulum/fistula. The patient improved postoperatively (e.g., cognitive, behavioral, and brainstem symptoms). The follow-up MRI’s showed the reversion of the sagging index/uncal herniation. Conclusion: The FTBSS should be considered in the differential diagnosis of an early onset frontotemporal dementia. Establishing the diagnosis and localizing the site of a spinal CSF/venous leak warrant both MRI and myelogram CT studies, to pinpoint the CSF leak site for proper surgical clipping/ligation of these thoracic diverticulum/CSF-venous leaks.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula

Ortega-Porcayo

Ortega

Quiroz-Castro

et al. 2020

Surgical Neurology International

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show abstract

“…Schievink et al more recently described 29 cases with a long follow-up (average 7.85 years). 2 Interestingly, 21/29 patients had a good long-term outcome, though a high proportion (18/29) underwent multiple surgical procedures. Also of note, recovery of this syndrome has been reported after head injury, where the authors propose that the patient's fall created a contusion and auto-blood patch.…”

Section: Case Presentationmentioning

confidence: 98%

“…This is perhaps most convincingly illustrated in the Schievink series where intrathecal saline infusion helped 7/8 patients. 2 A proposed mechanism for this syndrome is that intracranial hypotension causes brain sagging, with distortion of the midline and brainstem anatomy. This is thought to disrupt the frontotemporal cortices or their networks, causing a clinical syndrome of bvFTD.…”

Section: Case Presentationmentioning

confidence: 99%

Lessons of the month 3: Spontaneous resolution of frontotemporal brain sagging syndrome

2019

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Skull defect – Frontotemporal dementia sagging brain syndrome

Schievink,

Maya,

Babadjouni

et al. 2024

Ann Clin Transl Neurol

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ObjectiveFrontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non‐directed treatments is low. Some of these patients have a remote history of craniectomy/cranioplasty and we report a positive response to custom implant cranioplasty revision many years after their initial cranioplasty.MethodsWe reviewed medical records and imaging studies of 61 consecutive patients with FTD sagging brain syndrome. A SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms before and after custom implant cranioplasty. Pre‐ and post‐operative brain MRI was obtained to assess degree of brain sagging.ResultsEight (13.1%) of the 61 patients had a history of craniectomy/cranioplasty 1.5–13.5 years prior to onset of symptoms of FTD sagging brain syndrome. The mean age of the one woman and seven men at the time of presentation to our medical center was 50 years (range, 26–68 years). None had sinking scalp flap syndrome. Prior treatments included epidural blood patching and dural reduction surgery. Custom cranial implant surgery was performed in four patients and resulted in prompt and remarkable improvement of symptoms in three patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in one patient. Brain MRI showed improvement of brain sagging.InterpretationA disproportionate number of patients with FTD sagging brain syndrome have a remote history of supratentorial craniectomy/cranioplasty and revision cranioplasty should be considered.

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Behavioral Variant Frontotemporal Dementia as a Serious Complication of Spontaneous Intracranial Hypotension

Abstract: bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured.

Cited by 43 publications

References 28 publications

Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula

Frontotemporal brain sagging syndrome: Craniospinal hypovolemia secondary to a T6-T7 cerebrospinal fluid-venous fistula

Lessons of the month 3: Spontaneous resolution of frontotemporal brain sagging syndrome

Skull defect – Frontotemporal dementia sagging brain syndrome

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