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.

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.

Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks

2012 ◽  
Vol 116 (4) ◽  
pp. 749-754 ◽  
Author(s):  
Wouter I. Schievink ◽  
Marc S. Schwartz ◽  
M. Marcel Maya ◽  
Franklin G. Moser ◽  
Todd D. Rozen
Keyword(s):  
Skull Base ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Csf Rhinorrhea ◽  
Spinal Imaging ◽  
Radiological Data ◽  
Single Instance ◽  
Csf Leakage ◽  
Csf Leaks

Object Spontaneous intracranial hypotension is an important cause of headaches and an underlying spinal CSF leak can be demonstrated in most patients. Whether CSF leaks at the level of the skull base can cause spontaneous intracranial hypotension remains a matter of controversy. The authors' aim was to examine the frequency of skull base CSF leaks as the cause of spontaneous intracranial hypotension. Methods Demographic, clinical, and radiological data were collected from a consecutive group of patients evaluated for spontaneous intracranial hypotension during a 9-year period. Results Among 273 patients who met the diagnostic criteria for spontaneous intracranial hypotension and 42 who did not, not a single instance of CSF leak at the skull base was encountered. Clear nasal drainage was reported by 41 patients, but a diagnosis of CSF rhinorrhea could not be established. Four patients underwent exploratory surgery for presumed CSF rhinorrhea. In addition, the authors treated 3 patients who had a postoperative CSF leak at the skull base following the resection of a cerebellopontine angle tumor and developed orthostatic headaches; spinal imaging, however, demonstrated the presence of a spinal source of CSF leakage in all 3 patients. Conclusions There is no evidence for an association between spontaneous intracranial hypotension and CSF leaks at the level of the skull base. Moreover, the authors' study suggests that a spinal source for CSF leakage should even be suspected in patients with orthostatic headaches who have a documented skull base CSF leak.

Cervical bone spur presenting with spontaneous intracranial hypotension

1998 ◽  
Vol 89 (3) ◽  
pp. 483-484 ◽  
Author(s):  
A. Giancarlo Vishteh ◽  
Wouter I. Schievink ◽  
Jonathan J. Baskin ◽  
Volker K. H. Sonntag
Keyword(s):  
Mr Imaging ◽  
Intracranial Hypotension ◽  
Cervical Spinal Cord ◽  
Contrast Material ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Presumptive Diagnosis ◽  
Content Type ◽  
Hindbrain Herniation

✓ Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is a rare but increasingly recognized cause of postural headaches. The exact cause of these CSF leaks often remains unknown. The authors treated a 32-year-old man with a unique cause of spontaneous intracranial hypotension. He suffered an excruciating headache that was exacerbated by his being in an upright position. The results of four-vessel cerebral angiography were negative; however, magnetic resonance (MR) imaging of the brain revealed pachymeningeal enhancement and hindbrain herniation. A presumptive diagnosis of spontaneous intracranial hypotension was made. Myelography revealed extrathecal contrast material ventral to the cervical spinal cord as well as an unusual midline bone spur at C5–6. The patient's symptoms did not resolve with the application of epidural blood patches, and he subsequently underwent an anterior approach to the C5–6 spur. After discectomy, a slender bone spur that had pierced the thecal sac was found. After its removal, the dural rent was closed using two interrupted prolene sutures. The patient was discharged home 2 days later. On follow up his symptoms had resolved, and on MR imaging the pachymeningeal enhancement had resolved and the cerebellar herniation had improved slightly.

Lateral decubitus digital subtraction myelography to identify spinal CSF–venous fistulas in spontaneous intracranial hypotension

2019 ◽  
Vol 31 (6) ◽  
pp. 902-905 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Franklin G. Moser ◽  
Ravi S. Prasad ◽  
Rachelle B. Cruz ◽  
...  
Keyword(s):  
Prone Position ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Lateral Decubitus Position ◽  
Csf Leak ◽  
Digital Subtraction ◽  
Spinal Imaging ◽  
Lateral Decubitus ◽  
Venous Fistula ◽  
Digital Subtraction Myelography

OBJECTIVESpontaneous spinal CSF–venous fistulas are a distinct type of spinal CSF leak recently described in patients with spontaneous intracranial hypotension (SIH). Using digital subtraction myelography (DSM) with the patient in the prone position, the authors have been able to demonstrate such fistulas in about one-fifth of patients with SIH in whom conventional spinal imaging (MRI or CT myelography) showed no evidence for a CSF leak (i.e., the presence of extradural CSF). The authors compared findings of DSM with patients in the lateral decubitus position versus the prone position and now report a significantly increased yield of identifying spinal CSF–venous fistulas with this modification of their imaging protocol.METHODSThe population consisted of 23 patients with SIH who underwent DSM in the lateral decubitus position and 26 patients with SIH who underwent DSM in the prone position. None of the patients had evidence of a CSF leak on conventional spinal imaging.RESULTSA CSF–venous fistula was demonstrated in 17 (74%) of the 23 patients who underwent DSM in the lateral decubitus position compared to 4 (15%) of the 26 patients who underwent DSM in the prone position (p < 0.0001). The mean age of these 16 women and 5 men was 52.5 years (range 36–66 years).CONCLUSIONSAmong SIH patients in whom conventional spinal imaging showed no evidence of a CSF leak, DSM in the lateral decubitus position demonstrated a CSF–venous fistula in about three-fourths of patients compared to only 15% of patients when the DSM was performed in the prone position, an approximately five-fold increase in the detection rate. Spinal CSF–venous fistulas are not rare among patients with SIH.

scholarly journals Spontaneous Intracranial Hypotension Treated with a Targeted CT-Guided Epidural Blood Patch

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Inês Correia ◽  
Inês Brás Marques ◽  
Rogério Ferreira ◽  
Miguel Cordeiro ◽  
Lívia Sousa
Keyword(s):  
Intracranial Hypotension ◽  
Epidural Blood Patch ◽  
Spontaneous Intracranial Hypotension ◽  
Complete Recovery ◽  
Csf Leak ◽  
Ct Guided ◽  
Blood Patch ◽  
History Of ◽  
Persistent Headache ◽  
Csf Leaks

Spontaneous intracranial hypotension (SIH) is an important cause of new daily persistent headache. It is thought to be due to spontaneous spinal cerebrospinal fluid (CSF) leaks, which probably have a multifactorial etiology. The classic manifestation of SIH is an orthostatic headache, but other neurological symptoms may be present. An epidural blood patch is thought to be the most effective treatment, but a blind infusion may be ineffective. We describe the case of a young man who developed an acute severe headache, with pain worsening when assuming an upright posture and relief gained with recumbency. No history of previous headache, recent cranial or cervical trauma, or invasive procedures was reported. Magnetic resonance imaging showed pachymeningeal enhancement and other features consistent with SIH and pointed towards a cervical CSF leak site. After failure of conservative treatment, a targeted computer tomography-guided EBP was performed, with complete recovery.

scholarly journals Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas

2016 ◽  
Vol 24 (6) ◽  
pp. 960-964 ◽  
Author(s):  
Wouter I. Schievink ◽  
Franklin G. Moser ◽  
M. Marcel Maya ◽  
Ravi S. Prasad
Keyword(s):  
Intracranial Hypotension ◽  
Patient Population ◽  
Treatment Options ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Digital Subtraction ◽  
Percutaneous Injection ◽  
Spinal Imaging ◽  
Venous Fistula ◽  
Digital Subtraction Myelography

OBJECTIVE In most patients with spontaneous intracranial hypotension, a spinal CSF leak can be found, but occasionally, no leak can be demonstrated despite extensive spinal imaging. Failure to localize a CSF leak limits treatment options. The authors recently reported the discovery of CSF-venous fistulas in patients with spontaneous intracranial hypotension and now report on the use of digital subtraction myelography in patients with spontaneous intracranial hypotension but no CSF leak identifiable on conventional spinal imaging (i.e., non–digital subtraction myelography). METHODS The patient population consisted of 53 consecutive patients with spontaneous intracranial hypotension who underwent digital subtraction myelography but in whom no spinal CSF leak (i.e., presence of extradural CSF) was identifiable on conventional spinal imaging. RESULTS The mean age of the 33 women and 20 men was 53.4 years (range 29–71 years). A CSF-venous fistula was demonstrated in 10 (19%) of the 53 patients. A CSF-venous fistula was found in 9 (27%) of the 33 women and in 1 (5%) of the 20 men (p = 0.0697). One patient was treated successfully with percutaneous injection of fibrin sealant. Nine patients underwent surgery for the fistula. Surgery resulted in complete resolution of symptoms in 8 patients (follow-up 7–25 months), and in 1 patient, symptoms recurred after 4 months. CONCLUSIONS In this study, the authors found a CSF-venous fistula in approximately one-fifth of the patients with recalcitrant spontaneous intracranial hypotension but no CSF leak identifiable on conventional spinal imaging. The authors suggest that digital subtraction myelography be considered in this patient population.

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.

Multiple Spinal CSF Leaks in Spontaneous Intracranial Hypotension. Do They Exist?

2021 ◽  
pp. 10.1212/CPJ.0000000000001084
Author(s):  
Wouter Ingmar Schievink ◽  
M. Marcel Maya ◽  
Franklin Moser ◽  
Ravi Prasad ◽  
Vikram Wadhwa ◽  
...  
Keyword(s):  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Spinal Imaging ◽  
Recent Group ◽  
Using Data ◽  
Digital Subtraction Myelography ◽  
Type 3 ◽  
Csf Leaks

AbstractObjective:To determine the frequency of multiple spinal CSF leaks in a recent group of patients with spontaneous intracranial hypotension (SIH) who were investigated with digital subtraction myelography (DSM).Methods:This observational study was conducted using data from a prospectively maintained data base of patients who meet the International Classification of Headache Disorders (ICHD)-III criteria for SIH. The patient population consisted of a consecutive group of 745 patients with SIH who underwent DSM between March 2009 and February 2020. Based on the results of DSM, participants were classified according to type and number of spinal CSF leaks.Results:Among 398 patients with SIH and extradural CSF on spinal imaging, multiplicity of CSF leaks was observed in none of 291 patients with type 1a ventral leaks and in four (6.2%) of 65 patients with type 1b (postero-) lateral leaks. Among 97 patients with SIH from spinal CSF-venous fistulas (type 3 leaks) who did not have extradural CSF on spinal imaging, nine patients (9.3%) had multiple fistulas (p<0.0001 for comparison between groups). Type 3 and type 1a or 1b CSF leaks coexisted in an additional five patients.Conclusions:Among patients with SIH, multiplicity of CSF leaks was observed radiographically in none of the patients with ventral leaks, in 6% of patients with lateral leaks, and in 9% of patients with CSF-venous fistulas. These results suggest that patients with SIH can be reassured that the occurrence of multiple CSF leaks is negligible to uncommon at most, depending on type of CSF leak.

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 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.

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