Diffuse non-aneurysmal SAH in spontaneous intracranial hypotension: Sequela of ventral CSF leak?

Cephalalgia ◽  
2015 ◽  
Vol 36 (6) ◽  
pp. 589-592 ◽  
Author(s):  
Wouter I Schievink ◽  
M Marcel Maya
Keyword(s):  
Cerebral Angiography ◽  
Intracranial Hypotension ◽  
Aneurysmal Subarachnoid Hemorrhage ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Superficial Siderosis ◽  
Long Duration ◽  
Spine Mri ◽  
Lateral Sclerosis

Background Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak has become a well-recognized cause of headaches. Recently, various unusual neurological syndromes have been described in such patients with chronic ventral CSF leaks, including superficial siderosis and an amyotrophic lateral sclerosis-like syndrome. The authors now report two patients with spontaneous intracranial hypotension due to a chronic ventral CSF leak who suffered a diffuse non-aneurysmal subarachnoid hemorrhage (SAH). Description of cases A 62-year-old woman underwent uneventful microsurgical repair of a ventral thoracic CSF leak that had been present for 13 years. Seventeen months after surgery, she was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and spine and brain MRI did not reveal a source of the SAH. A 73-year-old woman was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and brain MRI did not reveal a source of the SAH, although superficial siderosis was detected. Spine MRI showed a ventral thoracic CSF leak that by history had been present for 41 years. She underwent uneventful microsurgical repair of the CSF leak. Discussion The authors suggest that patients with a ventral spinal CSF leak of long duration may be at risk of diffuse non-aneurysmal SAH.

Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: association with ventral spinal cerebrospinal fluid leaks

2011 ◽  
Vol 15 (4) ◽  
pp. 433-440 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Miriam Nuño
Keyword(s):  
Mr Imaging ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Superficial Siderosis ◽  
Cerebellar Hemorrhage ◽  
Spinal Imaging ◽  
Time Period ◽  
Long Duration ◽  
Persistent Headache

Object Spontaneous intracranial hypotension is an important cause of new-onset daily persistent headache. Cerebellar hemorrhage has been identified as a possible feature of spontaneous intracranial hypotension. The authors reviewed the MR imaging studies from a group of patients with spontaneous intracranial hypotension to assess the presence of cerebellar hemorrhage. Methods Medical records and radiological images were reviewed in 262 cases involving patients with spontaneous intracranial hypotension who had undergone MR imaging of the brain as well as spinal imaging. Results Chronic cerebellar hemorrhages were found in 7 (2.7%) of the 262 patients with spontaneous intracranial hypotension. These hemorrhages were found in 7 (19.4%) of the 36 patients with a ventral spinal CSF leak and in none of the 226 patients who did not have such a CSF leak (p < 0.0001). The degree of hemosiderin deposits was variable, ranging from mild involvement of the cerebellar folia to widespread superficial siderosis. Only the 1 patient with superficial siderosis had symptoms due to the hemorrhages. The time period between the onset of symptoms due to spontaneous intracranial hypotension and MR imaging examination was significantly longer in those patients with cerebellar hemorrhage than in those with a ventral spinal CSF leak and no evidence for cerebellar hemorrhage (mean 19.6 years vs 2.3 months, p < 0.0001). Conclusions Chronic cerebellar hemorrhage should be included among the manifestations of spontaneous intracranial hypotension. The severity is variable, but the hemorrhage generally is asymptomatic. The underlying spinal CSF leak is ventral and mostly of long duration.

Postural Headache In A Patient With Marfan's Syndrome

Cephalalgia ◽  
2003 ◽  
Vol 23 (7) ◽  
pp. 552-555 ◽  
Author(s):  
E Ferrante ◽  
A Citterio ◽  
A Savino ◽  
P Santalucia
Keyword(s):  
Intracranial Hypotension ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Postural Headache ◽  
Intracranial Hypotension Syndrome ◽  
Increased Risk ◽  
Diffuse Pachymeningeal Enhancement

A 26-year-old man with Marfan's syndrome had postural headache. Brain MRI with gadolinium showed diffuse pachymeningeal enhancement. MRI myelography revealed bilateral multiple large meningeal diverticula at sacral nerve roots level. He was suspected to have spontaneous intracranial hypotension syndrome. Eight days later headache improved with bed rest and hydration. One month after the onset he was asymptomatic and 3 months later brain MRI showed no evidence of diffuse pachymeningeal enhancement. The 1-year follow-up revealed no neurological abnormalities. The intracranial hypotension syndrome likely resulted from a CSF leak from one of the meningeal diverticula. In conclusion patients with spinal meningeal diverticula (frequently seen in Marfan's syndrome) might be at increased risk of developing CSF leaks, possibly secondary to Valsalva maneuver or minor unrecognizedtrauma.

scholarly journals Spontaneous Intracranial Hypotension and the durability of Epidural Blood Patch

Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Ali Hazama ◽  
Lori John ◽  
Alexander E Braley ◽  
Lawrence S Chin ◽  
Satish Krishnamurthy
Keyword(s):  
Intracranial Hypotension ◽  
Epidural Blood Patch ◽  
Symptomatic Relief ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Brain Magnetic Resonance Imaging ◽  
Definitive Treatment ◽  
Csf Leak ◽  
Persistent Symptoms ◽  
Blood Patch

Abstract INTRODUCTION Spontaneous Intracranial Hypotension (SIH) remains a rare and difficult clinical entity to diagnose and treat. Epidural blood patch (EBP) is the mainstay definitive treatment for refractory cases and has mixed efficacy. We sought to evaluate recent efficacy and outcomes of EBP for SIH at our institution. We also sought to explore the viability of repeat blood patches for patients whose symptoms persisted or recurred. METHODS A total of 23 patients (14 women, 9 men, mean age 49) were seen and treated for SIH between Summer 2009 and Spring 2018. All patients underwent brain magnetic resonance imaging (MRI) with and without gadolinium contrast and T2-weighted spine MRI. Targeted EBPs were placed at 1-2 vertebral levels below identified or suspected areas of leak. Patients were seen within a week following initial EBP and repeat EBP was offered to patients with persistent symptoms. Patients were followed if symptoms persisted or for 6 mo following clinical relief of symptoms. RESULTS 22/23 (95.7%) patients presented with complaints of orthostatic headache, 3 (13%) patients presented with altered mental status (AMS) or focal neurologic deficit. Brain MRI demonstrated pachymeningial enhancement in 16/23 (69.6%) patients, and 5/23 (21.7%) patients had subdural hematoma (SDH) present. Dural leaks were successfully identified in 18/23 (78.3%) patients. 12/23 (52.2%) patients had symptomatic relief with initial EBP, 5/23 (21.7%) patients received repeat EBPs for persistent symptoms will all achieving relief after repeat EBP. About 5/12 (41.7%) patients had recurrent symptoms after initial relief with EBP, and 4/5 (80%) were successfully treated with a second EBP. The mean initial EBP volume and number of EBPs per patient was 21.7 mL. In total, 18/23 (78.2%) patients are currently asymptomatic with regards to their SIH. Mean follow-up in this cohort was 2.6 yr. CONCLUSION EBP is a viable option for the treatment of SIH caused by CSF leak. Repeat epidural blood patch is reasonable in cases of recurrent symptoms.

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

2016 ◽  
Vol 24 (3) ◽  
pp. 454-456 ◽  
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.

Diagnostic Yield of Lateral Decubitus Digital Subtraction Myelogram Stratified by Brain MRI Findings

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000011522
Author(s):  
Dong Kun Kim ◽  
Carrie M. Carr ◽  
John C Benson ◽  
Felix E. Diehn ◽  
Vance T. Lehman ◽  
...  
Keyword(s):  
Diagnostic Yield ◽  
Brain Mri ◽  
Fluid Collection ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Diagnostic Study ◽  
Digital Subtraction ◽  
Mri Findings ◽  
Lateral Decubitus ◽  
Spine Mri

Objective:Assess the diagnostic yield of lateral decubitus digital subtraction myelography (LDDSM) and stratify LDDSM diagnostic yield by the Bern spontaneous intracranial hypotension (SIH) score of the pre-procedure brain MRI.Methods:This retrospective diagnostic study included consecutive adult patients investigated for SIH who underwent LDDSM. Patients without pre-procedure brain and spine MRI, and patients with extradural fluid collection on spine MRI (type 1 leak) were excluded. LDDSM images and brain MRIs were assessed by two independent blinded readers; a third reader adjudicated any discrepancies. Diagnostic yield of LDDSM was assessed, both overall and stratified by Bern SIH scoring.Results:Of the 62 patients included in this study, 33(53.2%) had a CSF leak identified on LDDSM. Right-sided leaks were more common (70.6%), and the most commonly identified levels of leaks were at T6, T7, and T10. No leak was found in any of the 9 patients with Bern SIH score of 2 or less. Of the 11 patients with Bern SIH score of 3-4, 5(45.5%) had a CSF leak identified, while of the 42 patients with Bern SIH score of 5 or higher, 28(66.7%) had a CSF leak identified.Conclusions:LDDSM has a high diagnostic yield for finding the exact location of spinal CSF leak, and the diagnostic yield increases with higher Bern SIH score. No leaks were found in patients with Bern SIH score of 2 or less, suggesting that foregoing invasive testing such as LDDSM in these patients may be appropriate unless accompanied by high clinical suspicion.Classification of Evidence:This study provides Class II evidence that for patients with suspected SIH, higher Bern SIH scores are associated with a greater likelihood of LDDSM-identified CSF leaks.

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

2020 ◽  
Vol 32 (2) ◽  
pp. 305-310 ◽  
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.

scholarly journals Comparisons of clinical characteristics, brain MRI findings, and responses to epidural blood patch between spontaneous intracranial hypotension and post-dural puncture headache: retrospective study

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Gha-Hyun Lee ◽  
Jiyoung Kim ◽  
Hyun-Woo Kim ◽  
Jae Wook Cho
Keyword(s):  
Intracranial Hypotension ◽  
Dural Puncture ◽  
Epidural Blood Patch ◽  
Brain Mri ◽  
Spontaneous Intracranial Hypotension ◽  
Vein Of Galen ◽  
Mri Findings ◽  
Post Dural Puncture Headache ◽  
Straight Sinus ◽  
Blood Patch

Abstract Background Spontaneous intracranial hypotension and post-dural puncture headache are both caused by a loss of cerebrospinal fluid but present with different pathogeneses. We compared these two conditions concerning their clinical characteristics, brain imaging findings, and responses to epidural blood patch treatment. Methods We retrospectively reviewed the records of patients with intracranial hypotension admitted to the Neurology ward of the Pusan National University Hospital between January 1, 2011, and December 31, 2019, and collected information regarding age, sex, disease duration, hospital course, headache intensity, time to the appearance of a headache after sitting, associated phenomena (nausea, vomiting, auditory symptoms, dizziness), number of epidural blood patch treatments, and prognosis. The brain MRI signs of intracranial hypotension were recorded, including three qualitative signs (diffuse pachymeningeal enhancement, venous distention of the lateral sinus, subdural fluid collection), and six quantitative signs (pituitary height, suprasellar cistern, prepontine cistern, mamillopontine distance, the midbrain-pons angle, and the angle between the vein of Galen and the straight sinus). Results A total of 105 patients (61 spontaneous intracranial hypotension patients and 44 post-dural puncture headache patients) who met the inclusion criteria were reviewed. More patients with spontaneous intracranial hypotension required epidural blood patch treatment than those with post-dural puncture headache (70.5% (43/61) vs. 45.5% (20/44); p = 0.01) and the spontaneous intracranial hypotension group included a higher proportion of patients who underwent epidural blood patch treatment more than once (37.7% (23/61) vs. 13.6% (6/44); p = 0.007). Brain MRI showed signs of intracranial hypotension in both groups, although the angle between the vein of Galen and the straight sinus was greater in the post-dural puncture headache group (median [95% Confidence Interval]: 85° [68°-79°] vs. 74° [76°-96°], p = 0.02). Conclusions Patients with spontaneous intracranial hypotension received more epidural blood patch treatments and more often needed multiple epidural blood patch treatments. Although both groups showed similar brain MRI findings, the angle between the vein of Galen and the straight sinus differed significantly between the groups.

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

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.

Sign in / Sign up

Export Citation Format

Share Document