Brain Spontaneous Intracranial Hypotension Score for Treatment Monitoring After Surgical Closure of the Underlying Spinal Dural Leak

Spontaneous intracranial hypotension is a rare clinical entity caused in most cases by a cerebrospinal fluid leak occurring at the level of the spinal cord. Cranial dural leaks have been previously reported as a cause of orthostatic headaches but, as opposed to spinal dural leaks, were not associated with other findings characteristic of spontaneous intracranial hypotension. We present the case of a male admitted for severe orthostatic headache. The patient had a history of intermittent postural headaches, dizziness, and symptoms consistent with post-nasal drip, which appeared several years after head trauma. Brain imaging showed signs consistent with intracranial hypotension: bilateral hygromas, subarachnoid hemorrhage, superficial siderosis, diffuse contrast enhancement of the pachymeninges, and superior sagittal sinus engorgement. No spinal leak could be identified by magnetic resonance imaging, and the patient had a rapid remission of symptoms with conservative management. Further work-up identified an old temporal bone fracture which created a route of egress between the posterior fossa and the mastoid cells. Otorhinolaryngology examination showed pulsatile bloody discharge and liquorrhea at the level of the left pharyngeal opening of the Eustachian tube. The orthostatic character of the headache, as well as the brain imaging findings, were consistent with intracranial hypotension syndrome caused by a cranial dural leak. Clinical signs and imaging findings consistent with the diagnosis of apparently “spontaneous” intracranial hypotension should prompt the search for a cranial dural leak if a spinal leak is not identified.

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Headache due to Bilateral Subacute Subdural Hematomas following Intracranial Hypotension Caused by Cervical Disc Herniation

Case Reports in Neurology ◽

10.1159/000511428 ◽

2021 ◽

pp. 246-250

Author(s):

Iris Steenstraten ◽

Stephanie Mohamed ◽

Hille Koppen

Keyword(s):

Disc Herniation ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Cervical Disc Herniation ◽

Cervical Disc ◽

Resonance Imaging ◽

Recumbent Position ◽

Subdural Hematomas ◽

Dural Leak ◽

The Brain

Spontaneous intracranial hypotension (SIH) is caused by spinal leakage of cerebrospinal fluid (CSF) and typically causes orthostatic headache which is relieved by lying in a recumbent position. We describe the case of a 44-year-old male suffering from orthostatic headaches accompanied by symptomatic cervical disc herniation, for which he had an anterior cervical discectomy and fusion (ACDF). Computerized tomography of the brain at the emergency department 1 week after this procedure showed bilateral subacute subdural hematomas. In retrospect, the positional headache had been present for 3 months prior to the ACDF, and magnetic resonance imaging of the cervical spine prior to the ACDF already showed signs of an extradural CSF collection indicating intracranial hypotension. This case highlights the possibility of SIH caused by a spinal dural leak due to cervical disc herniation.

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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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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: diagnostic and therapeutic workup

Neuroradiology ◽

10.1007/s00234-021-02766-z ◽

2021 ◽

Author(s):

Niklas Luetzen ◽

Philippe Dovi-Akue ◽

Christian Fung ◽

Juergen Beck ◽

Horst Urbach

Keyword(s):

Nerve Root ◽

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Digital Subtraction ◽

Mri Findings ◽

Ct Myelography ◽

Surgical Closure ◽

Dural Tears ◽

Type 3

AbstractSpontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with typical MRI findings among which engorgement of the venous sinuses, pachymeningeal enhancement, and effacement of the suprasellar cistern have the highest diagnostic sensitivity. SIH is in almost all cases caused by spinal CSF leaks. Spinal MRI scans showing so-called spinal longitudinal extradural fluid (SLEC) are suggestive of ventral dural tears (type 1 leak) which are located with prone dynamic (digital subtraction) myelography. As around half of the ventral dural tears are located in the upper thoracic spine, additional prone dynamic CT myelography is often needed. Leaking nerve root sleeves typically associated with meningeal diverticulae (type 2 leaks) and CSF-venous fistulas (type 3 leaks) are proven via lateral decubitus dynamic digital subtraction or CT myelography: type 2 leaks are SLEC-positive if the tear is proximal and SLEC-negative if it is distal, and type 3 leaks are always SLEC-negative. Although 30–70% of SIH patients show marked improvement following epidural blood patches applied via various techniques definite cure mostly requires surgical closure of ventral dural tears and surgical ligations of leaking nerve root sleeves associated with meningeal diverticulae or CSF-venous fistulas. For the latter, transvenous embolization with liquid embolic agents via the azygos vein system is a novel and valuable therapeutic alternative.

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Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and a novel patch method in a series of 28 consecutive patients

Yearbook of Neurology and Neurosurgery ◽

10.1016/s0513-5117(10)79271-1 ◽

2010 ◽

Vol 2010 ◽

pp. 248-250

Author(s):

P. Klimo,

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Intracranial Hypotension Syndrome

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Postural headache due to spontaneous intracranial hypotension in an adolescent

Neuropediatrics ◽

10.1055/s-0033-1337801 ◽

2013 ◽

Vol 44 (02) ◽

Author(s):

J Schönberger ◽

M Möhlenbruch ◽

KS Kang ◽

A Seitz ◽

H Bächli ◽

...

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Postural Headache

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Radiologic Assessment of Spinal CSF Leakage in Spontaneous Intracranial Hypotension

Journal of the Korean Radiological Society ◽

10.3348/jkrs.1999.41.6.1091 ◽

1999 ◽

Vol 41 (6) ◽

pp. 1091

Author(s):

Chang Jin Han ◽

Ji Hyung Kim ◽

Jang Sung Kim ◽

Sun Yong Kim ◽

Jung Ho Suh

Keyword(s):

Intracranial Hypotension ◽

Spontaneous Intracranial Hypotension ◽

Radiologic Assessment ◽

Csf Leakage

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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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