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

Abstract BACKGROUND Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. OBJECTIVE To describe detailed characteristics of a large cohort of patients with SIH and symptoms of bvFTD. METHODS We identified patients with SIH who met clinical criteria for bvFTD. Patients were compared to a cohort of SIH patients without bvFTD. RESULTS The mean age for the 21 men and 8 women was 52.9 yr (range, 37–65 yr). All 29 patients with bvFTD symptoms had hypersomnolence. Magnetic resonance imaging showed brain sagging in all patients, cerebrospinal fluid (CSF) opening pressure low in about half of patients, but a spinal CSF leak could not be detected in any patient. All patients underwent epidural blood patching, but 26 patients eventually underwent 1 or more surgical procedures. Overall, a good outcome was obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to 1 (14%) of 7 patients who did undergo Chiari surgery (P < .003). Compared to SIH patients without symptoms of bvFTD (n = 547), those with bvFTD symptoms were older, more often male, less often demonstrated CSF leak on spinal imaging, and more often underwent surgery (P < .02). CONCLUSION 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.

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Coma

Neurology ◽

10.1212/wnl.0000000000005477 ◽

2018 ◽

Vol 90 (19) ◽

pp. e1638-e1645 ◽

Cited By ~ 14

Author(s):

Wouter I. Schievink ◽

M. Marcel Maya ◽

Franklin G. Moser ◽

Stacey Jean-Pierre ◽

Miriam Nuño

Keyword(s):

Glasgow Outcome Scale ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Surgical Closure ◽

Percutaneous Procedures ◽

The Mean ◽

Brain Sagging ◽

Comprehensive Study

ObjectiveTo review our experience with patients with spontaneous intracranial hypotension (SIH) and coma because, although disorders of consciousness may complicate SIH, no comprehensive study of such patients has been reported.MethodsUsing a prospectively maintained registry, we identified all patients with SIH in whom coma developed. Patients or their caregivers/families were contacted for follow-up. Patients were compared to a cohort of patients with SIH without coma.ResultsThe mean age of the 12 men and 3 women with SIH was 56.2 years (range 34–72 years) at the time of onset of coma. In one-third of patients, coma developed after craniotomy for subdural hematomas or for an unrelated intracranial pathology. Imaging showed brain sagging, including bilateral temporal lobe herniation, in all 15 patients and brainstem edema in 8 patients (53%). Overall, coma was reversible in 7 of 15 patients treated with epidural blood patches, in 2 of 4 treated with percutaneous glue injections, and in 6 of 6 treated surgically. Only 1 patient had residual neurologic deficit related to coma (Glasgow Outcome Scale score 4 [moderate disability]). Compared to patients with SIH without coma (n = 568), those with coma were older, more often were male, and more often underwent surgery.ConclusionsComa in SIH is rare, reversible, and invariably associated with brain sagging. Coma due to SIH may be refractory to the usual percutaneous procedures, and surgical closure of the CSF leak may be required to regain consciousness.

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Spontaneous Spinal Cerebrospinal Fluid Leaks

Cephalalgia ◽

10.1111/j.1468-2982.2008.01776.x ◽

2008 ◽

Vol 28 (12) ◽

pp. 1345-1356 ◽

Cited By ~ 109

Author(s):

WI Schievink

Keyword(s):

Cerebrospinal Fluid ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Epidural Blood Patching ◽

Delay In Diagnosis ◽

Facial Numbness ◽

Cerebrospinal Fluid Leaks ◽

Brain Sagging ◽

Glue Injection ◽

Fluid Collections

Spontaneous intracranial hypotension is an uncommon but not rare cause of new onset daily persistent headaches. A delay in diagnosis is the norm. Women are affected more commonly than men and most are in the fifth or sixth decade of life. The underlying cause is a spontaneous spinal cerebrospinal fluid (CSF) leak. Typically the headache is orthostatic in nature but other headache patterns occur as well. Associated symptoms are common and include neck pain, a change in hearing, diplopia, facial numbness, cognitive abnormalities and even coma. Typical imaging findings consist of subdural fluid collections, pachymeningeal enhancement, pituitary hyperaemia and brain sagging, but magnetic resonance imaging may be normal. Myelography is the study of choice to identify the CSF leak but is not always necessary to make the diagnosis. Treatment consists of bedrest, abdominal binder, epidural blood patching, percutaneous fibrin glue injection or surgical CSF leak repair. Outcomes have been poorly studied.

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

Operative Neurosurgery ◽

10.1093/ons/opz126 ◽

2019 ◽

Vol 17 (2) ◽

pp. E90-E90

Author(s):

Kaveh Barami

Keyword(s):

Frontotemporal Dementia ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Behavioral Variant Frontotemporal Dementia

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[P3-276]: BEHAVIORAL VARIANT FRONTOTEMPORAL DEMENTIA AS A SERIOUS COMPLICATION OF SPONTANEOUS INTRACRANIAL HYPOTENSION

Alzheimer s & Dementia ◽

10.1016/j.jalz.2017.06.1490 ◽

2017 ◽

Vol 13 (7S_Part_22) ◽

pp. P1048-P1049 ◽

Cited By ~ 1

Author(s):

Wouter Schievink ◽

Marcel Maya

Keyword(s):

Frontotemporal Dementia ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Behavioral Variant Frontotemporal Dementia

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Intraspinal hemorrhage in spontaneous intracranial hypotension: link to superficial siderosis? Report of 2 cases

Journal of Neurosurgery Spine ◽

10.3171/2015.6.spine15428 ◽

2016 ◽

Vol 24 (3) ◽

pp. 454-456 ◽

Cited By ~ 10

Author(s):

Wouter I. Schievink ◽

Philip Wasserstein ◽

M. Marcel Maya

Keyword(s):

Differential Diagnosis ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Superficial Siderosis ◽

Dural Tear ◽

Epidural Blood Patching ◽

Intraspinal Hemorrhage ◽

Csf Leaks

Spontaneous intracranial hypotension due to a spinal CSF leak has become a well-recognized cause of headaches, but such spinal CSF leaks also are found in approximately half of patients with superficial siderosis of the CNS. It has been hypothesized that friable vessels at the site of the spinal CSF leak are the likely source of chronic bleeding in these patients, but such an intraspinal hemorrhage has never been visualized. The authors report on 2 patients with spontaneous intracranial hypotension and intraspinal hemorrhage, offering support for this hypothesis. A 33-year-old man and a 62-year-old woman with spontaneous intracranial hypotension were found to have a hemorrhage within the ventral spinal CSF collection and within the thecal sac, respectively. Treatment consisted of microsurgical repair of a ventral dural tear in the first patient and epidural blood patching in the second patient. The authors suggest that spontaneous intracranial hypotension should be included in the differential diagnosis of spontaneous intraspinal hemorrhage, and that the intraspinal hemorrhage can account for the finding of superficial siderosis when the CSF leak remains untreated.

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Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant

Journal of Neurosurgery ◽

10.3171/jns.2004.100.6.1098 ◽

2004 ◽

Vol 100 (6) ◽

pp. 1098-1100 ◽

Cited By ~ 58

Author(s):

Wouter I. Schievink ◽

M. Marcel Maya ◽

Franklin M. Moser

Keyword(s):

Fibrin Sealant ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Invasive Treatment ◽

Content Type ◽

Epidural Blood Patching ◽

Lower Thoracic Spine ◽

Fine Print ◽

Csf Leaks

✓ Spontaneous intracranial hypotension due to a cerebrospinal fluid (CSF) leak in the spine is an important cause of new, daily persistent headaches. Most patients respond well to conservative treatments including epidural blood patching. Limited options for effective treatment are available for patients in whom these treatments fail. The authors treated four patients (mean age 38 years; range 26–43 years) with percutaneous placement of a fibrin sealant. All these patients presented with intractable positional headaches. The CSF leak was located in the lower cervical spine in three patients and in the lower thoracic spine in one patient. Four to 20 milliliters of fibrin sealant was injected at the site of the CSF leak. Two of the four patients became asymptomatic within days of the procedure and thus avoided surgery. There were no complications of this procedure. Percutaneous placement of a fibrin sealant is a safe, minimally invasive treatment for spontaneous spinal CSF leaks and should be considered in patients in whom conservative treatment has failed.

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The algorithm for diagnosis and management of intracranial hypotension with coma: Report of two cases

Surgical Neurology International ◽

10.25259/sni_460_2020 ◽

2020 ◽

Vol 11 ◽

pp. 267

Author(s):

Shintaro Arai ◽

Keisuke Takai ◽

Makoto Taniguchi

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Csf Leak ◽

Diagnosis And Management ◽

Blood Patch ◽

Dural Leak ◽

Brain Sagging ◽

Early Performance ◽

Dural Tears ◽

Ct Studies

Background: Spontaneous intracranial hypotension (SIH) is caused by spontaneous cerebrospinal fluid (CSF) leaks that can be treated in most cases with an epidural blood patch (EBP). However, some patients, who develop severe brain sagging, can neurologically deteriorate, and in occasional instances, which become comatose. Here, with the presentation of two cases, and a review of the literature, we have set guidelines for diagnosing SIH along with recommendations for its management. Case Description: We reviewed two cases of SIH. Both patients became comatose due to a CSF leak associated with a tear in the spinal dura diagnosed on myelo-CT studies. As targeted EBP failed to achieve sustained improvement, direct operative repair of the dural tears was warranted (video presentation). Conclusion: After reviewing two cases of SIH and the literature, we developed an algorithm for the diagnosis and management of SIH. To avoid deterioration to a comatose status, we recommend the early performance of myelo- CT studies to identify the location of the dural leak, followed by early dural repair.

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Spontaneous spinal CSF–venous fistulas associated with venous/venolymphatic vascular malformations: report of 3 cases

Journal of Neurosurgery Spine ◽

10.3171/2019.8.spine19716 ◽

2020 ◽

Vol 32 (2) ◽

pp. 305-310 ◽

Cited By ~ 3

Author(s):

Wouter I. Schievink ◽

Marcel M. Maya ◽

Franklin G. Moser ◽

Alexander Tuchman ◽

Rachelle B. Cruz ◽

...

Keyword(s):

Soft Tissue ◽

Intracranial Hypotension ◽

Vascular Malformations ◽

Spontaneous Intracranial Hypotension ◽

Proximal Portion ◽

Csf Leak ◽

Venous Fistula

Spontaneous CSF–venous fistulas may be present in up to one-fourth of patients with spontaneous intracranial hypotension. This is a recently discovered type of CSF leak, and much remains unknown about these fistulas. Spinal CSF–venous fistulas are usually seen in coexistence with a spinal meningeal diverticulum, suggesting the presence of an underlying structural dural weakness at the proximal portion of the fistula. The authors now report the presence of soft-tissue venous/venolymphatic malformations associated with spontaneous spinal CSF–venous fistulas in 2 patients with spontaneous intracranial hypotension, suggesting a role for distal venous pathology. In a third patient with spontaneous intracranial hypotension and a venolymphatic malformation, such a CSF–venous fistula is strongly suspected.

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690 Intracranial Hypotension Due to CSF Leak Secondary to Ventral Thoracic Osteophyte Complex: Resolution After Transdural Thoracic Discectomy and Dural Repair

British Journal of Surgery ◽

10.1093/bjs/znab134.313 ◽

2021 ◽

Vol 108 (Supplement_2) ◽

Author(s):

B Kewlani ◽

I Hussain ◽

J Greenfield

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Bed Rest ◽

Structural Defects ◽

Csf Leak ◽

History Of ◽

Surgical Cure ◽

Thoracic Discectomy ◽

Herniated Discs

Abstract The hallmark symptom of spontaneous intracranial hypotension (SIH) is orthostatic headaches which manifests secondary to cerebrospinal fluid (CSF) hypovolaemia. Well-recognised aetiologies include trauma which includes procedures such as lumbar punctures and spinal surgery. More recently, structural defects such as bony osteophytes and calcified or herniated discs have been attributed to mechanically compromising dural integrity consequently resulting in CSF leak and symptom manifestation. A thorough literature review noted only a handful of such cases. We report the case of a thirty-two-year-old Asian female who presented with a one-month history of new-onset progressively worsening orthostatic headaches. Workup included MRI of the thoracic spine which revealed an epidural collection of CSF consequently prompting a dynamic CT-myelogram of the spine which not only helped to confirm severe cerebral hypotension but also suggested the underlying cause as being a dorsally projecting osteophyte-complex at level T2-3. Conservative and medical management including bed rest, analgesia, mechanical compression, and epidural blood patches failed to alleviate symptoms and a permanent surgical cure was eventually sought. The surgery involved T2-T3 laminectomy and osteophytectomy and at a 3-month follow-up, complete resolution of symptoms was noted.

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