Spontaneous intracranial hypotension presenting with progressive cognitive decline

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.

Hyperprolactinemia due to spontaneous intracranial hypotension

2015 ◽  
Vol 122 (5) ◽  
pp. 1020-1025 ◽  
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.

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.

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.

Novel hydrogel application in minimally invasive surgical approaches to spontaneous intracranial hypotension

2014 ◽  
Vol 121 (4) ◽  
pp. 976-982 ◽  
Author(s):  
Casey M. Chai ◽  
Matei A. Banu ◽  
William Cobb ◽  
Neel Mehta ◽  
Linda Heier ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Intracranial Hypotension ◽  
Fluid Collection ◽  
Spontaneous Intracranial Hypotension ◽  
Patch Repair ◽  
Surgical Approaches ◽  
Dural Defect ◽  
Dural Repair ◽  
Blood Patch ◽  
Csf Leaks

The authors report 2 cases of orthostatic headaches associated with spontaneous intracranial hypotension (SIH) secondary to CSF leaks that were successfully treated with an alternative dural repair technique in which a tubular retractor system and a hydrogel dural sealant were used. The 2 patients, a 63-year-old man and a 45-year-old woman, presented with orthostatic headache associated with SIH secondary to suspected lumbar and lower cervical CSF leaks, respectively, as indicated by bony defects or epidural fluid collection. Epidural blood patch repair failed in both cases, but both were successfully treated with the minimally invasive application of a hydrogel dural sealant as a novel adjunct to traditional dural repair techniques. Both patients tolerated the procedure well. Moreover, SIH symptoms and MRI signs were completely resolved at 1-month follow-up in both patients. The minimally invasive dural repair procedure with hydrogel dural sealant described here offers a viable alternative in patients in whom epidural blood patches have failed, with obscure recalcitrant CSF leaks at the cervical as well as lumbar spinal level. The authors demonstrate that the adjuvant use of sealant is a safe and efficient repair method regardless of dural defect location.

scholarly journals Spontaneous Intracranial Hypotension: Recommendations for Management

2013 ◽  
Vol 40 (2) ◽  
pp. 144-157 ◽  
Author(s):  
Farnaz Amoozegar ◽  
Darryl Guglielmin ◽  
William Hu ◽  
Denise Chan ◽  
Werner J. Becker
Keyword(s):  
Computed Tomography ◽  
Clinical Trials ◽  
Mr Imaging ◽  
Intracranial Hypotension ◽  
Literature Search ◽  
Spontaneous Intracranial Hypotension ◽  
Normal Brain ◽  
Non Invasive ◽  
Radionuclide Cisternography ◽  
Mr Myelography

A literature search found no clinical trials or guidelines addressing the management of spontaneous intracranial hypotension (SIH). Based on the available literature and expert opinion, we have developed recommendations for the diagnosis and management of SIH. For typical cases, we recommend brain magnetic resonance (MR) imaging with gadolinium to confirm the diagnosis, and conservative measures for up to two weeks. If the patient remains symptomatic, up to three non-directed lumbar epidural blood patches (EBPs) should be considered. If these are unsuccessful, non-invasive MR myelography, radionuclide cisternography, MR myelography with intrathecal gadolinium, or computed tomography with myelography should be used to localize the leak. If the leak is localized, directed EPBs should be considered, followed by fibrin sealant or neurosurgery if necessary. Clinically atypical cases with normal brain MR imaging should be investigated to localize the leak. Directed EBPs can be used if the leak is localized; non-directed EBPs should be used only if there are indirect signs of SIH.

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.

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