Spontaneous intracranial hypotension with brain sagging causing “prayer headache”

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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Spontaneous intracranial hypotension with chronic brain sagging causing foramen magnum CSF circulation disorder reversible after lumbar epidural blood patch

Neurological Sciences ◽

10.1007/s10072-018-3263-z ◽

2018 ◽

Vol 39 (6) ◽

pp. 1137-1138 ◽

Cited By ~ 3

Author(s):

Enrico Ferrante ◽

Valentina Prone ◽

Fabio Rubino ◽

Mirko Ferrante

Keyword(s):

Intracranial Hypotension ◽

Epidural Blood Patch ◽

Spontaneous Intracranial Hypotension ◽

Foramen Magnum ◽

Csf Circulation ◽

Blood Patch ◽

Brain Sagging

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Spontaneous Intracranial Hypotension With Brain Sagging Attributable to a Cerebrospinal Fluid–Venous Fistula

JAMA Neurology ◽

10.1001/jamaneurol.2020.2372 ◽

2020 ◽

Vol 77 (10) ◽

pp. 1320

Author(s):

Vinil N. Shah ◽

William P. Dillon

Keyword(s):

Cerebrospinal Fluid ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Brain Sagging ◽

Venous Fistula

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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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Hyperprolactinemia due to spontaneous intracranial hypotension

Journal of Neurosurgery ◽

10.3171/2014.9.jns132687 ◽

2015 ◽

Vol 122 (5) ◽

pp. 1020-1025 ◽

Cited By ~ 6

Author(s):

Wouter I. Schievink ◽

Miriam Nuño ◽

Todd D. Rozen ◽

M. Marcel Maya ◽

Adam N. Mamelak ◽

...

Keyword(s):

Intracranial Hypotension ◽

Brain Mri ◽

Age At Onset ◽

Spontaneous Intracranial Hypotension ◽

Pituitary Hormones ◽

Resonance Imaging ◽

Normal Range ◽

Mri Findings ◽

Pituitary Enlargement ◽

Brain Sagging

OBJECT Spontaneous intracranial hypotension is an increasingly recognized cause of headaches. Pituitary enlargement and brain sagging are common findings on MRI in patients with this disorder. The authors therefore investigated pituitary function in patients with spontaneous intracranial hypotension. METHODS Pituitary hormones were measured in a group of 42 consecutive patients with spontaneous intracranial hypotension. For patients with hyperprolactinemia, prolactin levels also were measured following treatment. Magnetic resonance imaging was performed prior to and following treatment. RESULTS The study group consisted of 27 women and 15 men with a mean age at onset of symptoms of 52.2 ± 10.7 years (mean ± SD; range 17–72 years). Hyperprolactinemia was detected in 10 patients (24%), ranging from 16 ng/ml to 96.6 ng/ml in men (normal range 3–14.7 ng/ml) and from 31.3 ng/ml to 102.5 ng/ml in women (normal range 3.8–23.2 ng/ml). In a multivariate analysis, only brain sagging on MRI was associated with hyperprolactinemia. Brain sagging was present in 60% of patients with hyperprolactinemia and in 19% of patients with normal prolactin levels (p = 0.02). Following successful treatment of the spontaneous intracranial hypotension, hyperprolactinemia resolved, along with normalization of brain MRI findings in all 10 patients. CONCLUSIONS Spontaneous intracranial hypotension is a previously undescribed cause of hyperprolactinemia. Brain sagging causing distortion of the pituitary stalk (stalk effect) may be responsible for the hyperprolactinemia.

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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 intracranial hypotension presenting with progressive cognitive decline

BMJ Case Reports ◽

10.1136/bcr-2020-241285 ◽

2021 ◽

Vol 14 (7) ◽

pp. e241285

Author(s):

Sanaz Shoja Gharehbagh ◽

Birthe Krogh Rasmussen ◽

Emil Smilkov ◽

Rigmor Højland Jensen

Keyword(s):

Intracranial Hypotension ◽

Brain Mri ◽

Fluid Collection ◽

Spontaneous Intracranial Hypotension ◽

Pituitary Tumour ◽

Normal Brain ◽

Thoracic Level ◽

Mri Scans ◽

Brain Sagging ◽

Venous Fistula

A 63-year-old woman presented with headache, progressive somnolence, neurocognitive decline and urinary incontinence through a year. Medical history was unremarkable except for hypertension and hypercholesterolaemia. Neurological examination was normal. Brain MRI showed findings typical for spontaneous intracranial hypotension (subdural fluid collection, pachymeningeal enhancement, brain sagging) and pituitary tumour. The patient’s complaints improved dramatically but temporarily after treatment with each of repeated targeted as well as non-targeted blood patches and a trial with continuous intrathecal saline infusion. Extensive work up including repeated MRI-scans, radioisotope cisternographies, CT and T2-weighted MR myelography could not localise the leakage, but showed minor root-cysts at three levels. Finally, lateral decubitus digital subtraction dynamic myelography with subsequent CT myelography identified a tiny dural venous fistula at the fourth thoracic level. After surgical venous ligation, the patient fully recovered. Awareness of spontaneous dural leaks and their heterogeneous clinical picture are important and demands an extensive workup.

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