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Brain Slump Caused by Jugular Venous Stenoses Treated by Stenting: A Hypothesis to Link Spontaneous Intracranial Hypotension with Idiopathic Intracranial Hypertension.

ABSTRACT: Spontaneous intracranial hypotension, of which brain slump is an extreme expression, is caused by a cerebrospinal fluid leak. The reason the leak develops in the first place, however, is unknown, and some cases can be very difficult to manage. We describe a patient with severe symptoms of spontaneous intracranial hypotension and brain slump documented by magnetic resonance imaging whose clinical syndrome and structural brain anomaly resolved completely after treatment directed exclusively at improving cranial venous outflow. Diagnostics included computed tomography (CT) venography, catheter venography, and jugular venoplasty. CT venography showed narrowing of both internal jugular veins below the skull base. Catheter venography confirmed that these were associated with pressure gradients. Jugular venoplasty performed on two separate occasions as a clinical test gave temporary respite. Lasting remission (2 years of follow-up) was achieved by stenting the dominant internal jugular vein. These findings and this outcome suggest a mechanism for the development of spontaneous intracranial hypotension that would link it to idiopathic intracranial hypertension and have cranial venous outflow obstruction as the underlying cause.

SUBMITTER: Higgins N

PROVIDER: S-EPMC4520985 | biostudies-other | 2015 Jul

REPOSITORIES: biostudies-other

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Project description:Background and purposeAtypical clinical presentations of spontaneous intracranial hypotension include obtundation, memory deficits, dementia with frontotemporal features, parkinsonism, and ataxia. The purpose of this study was to compare clinical and imaging features of spontaneous intracranial hypotension with typical-versus-atypical presentations.Materials and methodsClinical records and neuroimaging of patients with spontaneous intracranial hypotension from September 2005 to August 2014 were retrospectively evaluated. Patients with classic spontaneous intracranial hypotension (n = 33; mean age, 41.7 ± 14.3 years) were compared with those with intracranial hypotension with atypical clinical presentation (n = 8; mean age, 55.9 ± 14.1 years) and 36 controls (mean age, 41.4 ± 11.2 years).ResultsPatients with atypical spontaneous intracranial hypotension were older than those with classic spontaneous intracranial hypotension (55.9 ± 14.1 years versus 41.7 ± 14.3 years; P = .018). Symptom duration was shorter in classic compared with atypical spontaneous intracranial hypotension (3.78 ± 7.18 months versus 21.93 ± 18.43 months; P = .015). There was no significant difference in dural enhancement, subdural hematomas, or cerebellar tonsil herniation. Patients with atypical spontaneous intracranial hypotension had significantly more elongated anteroposterior midbrain diameter compared with those with classic spontaneous intracranial hypotension (33.6 ± 2.9 mm versus 27.3 ± 2.9 mm; P < .001) and shortened pontomammillary distance (2.8 ± 1 mm versus 5.15 ± 1.5 mm; P < .001). Patients with atypical spontaneous intracranial hypotension were less likely to become symptom-free, regardless of treatment, compared with those with classic spontaneous intracranial hypotension (χ(2) = 13.99, P < .001).ConclusionsIn this sample of 8 patients, atypical spontaneous intracranial hypotension was a more chronic syndrome compared with classic spontaneous intracranial hypotension, with more severe brain sagging, lower rates of clinical response, and frequent relapses. Awareness of atypical presentations of spontaneous intracranial hypotension is paramount.

Project description:BackgroundIdiopathic intracranial hypertension (IIH) is a syndrome of elevated intracranial pressure of unknown etiology. Unilateral or bilateral transverse sinus (TS) or transverse-sigmoid junction stenosis is present in about 30%-93% of these patients. There is an ongoing debate on whether venous sinus stenosis is the cause of IIH or a result of it. The subset of IIH patients who continue to have clinical deterioration despite maximum medical therapy is termed as "refractory IIH." Traditionally, cerebrospinal fluid diversion surgeries (ventriculoperitoneal shunt and lumboperitoneal shunt) and optic nerve sheath fenestration (ONSF) were the mainstays of treatment for refractory IIH. In the last decade, venous sinus stenting (VSS) has emerged as a safe and effective option for treating refractory IIH patients with venous sinus stenosis. Through this study, we want to share our experience with venous stenting in refractory IIH patients with venous sinus stenosis associated with a significant pressure gradient (≥10 mm Hg).MethodsRetrospective chart review of all the patients diagnosed with refractory IIH who underwent VSS or angioplasty at our comprehensive stroke center from November 2016 to March 2019.ResultsA total of seven refractory IIH patients underwent VSS or angioplasty within the specified period. The mean age was 39 years. Eighty-five percent of the patients were women (n = 6). The mean body mass index (BMI) was 37 kg/m2. Headache was the most common symptom (85%, n = 6) followed by transient visual obscurations (71%, n = 5) and pulsatile tinnitus (57%; n = 4). All patients had papilledema. Fifty-seven percent of patients (n = 4) had impaired visual field. Mean lumbar opening pressure was 40.6 cm H2O (SD = 9.66; 95% CI = 33.5-47.7). All patients were on maximum doses of acetazolamide ± furosemide. Six patients (85%) had dominant right transverse-sigmoid sinus. Fifty-seven percent of the patients had severe right transverse ± sigmoid sinus stenosis (n = 4) and the rest (43%) had bilateral TS stenosis (n = 3). Prestenting mean trans-stenosis pressure gradient was 18 mm Hg (SD = 6.16; 95% CI = 13.43-22.57). Six patients (85%) were treated with TS stenting and one (15%) with only angioplasty. Poststenting mean trans-stenosis pressure gradient was 4.8 mm Hg (SD = 6.6; 95% CI = -0.1-9.7). All patients were able to come off their medications with significant improvement in neurological and ophthalmological signs and symptoms. No procedure-related complications occurred.ConclusionTS stenting ± angioplasty is a safe and effective means of treating refractory IIH with venous sinus stenosis associated with a significant pressure gradient (≥10 mm Hg).

Project description:Background and purposeIn medically refractory idiopathic intracranial hypertension, optic nerve sheath fenestration or CSF shunting is considered the next line of management. Venous sinus stenosis has been increasingly recognized as a treatable cause of elevated intracranial pressure in a subset of patients. In this article, we present the results of the largest meta-analysis of optic nerve sheath fenestration, CSF shunting, and dural venous sinus stenting. This is the only article that compares these procedures, to our knowledge.Materials and methodsWe performed a PubMed search of all peer-reviewed articles from 1988 to 2014 for patients who underwent a procedure for medically refractory idiopathic intracranial hypertension.ResultsOptic nerve sheath fenestration analysis included 712 patients. Postprocedure, there was improvement of vision in 59%, headache in 44%, and papilledema in 80%; 14.8% of patients required a repeat procedure with major and minor complication rates of 1.5% and 16.4%, respectively. The CSF diversion procedure analysis included 435 patients. Postprocedure, there was improvement of vision in 54%, headache in 80%, and papilledema in 70%; 43% of patients required at least 1 additional surgery. The major and minor complication rates were 7.6% and 32.9%, respectively. The dural venous sinus stenting analysis included 136 patients. After intervention, there was improvement of vision in 78%, headache in 83%, and papilledema in 97% of patients. The major and minor complication rates were 2.9% and 4.4%, respectively. Fourteen additional procedures were performed with a repeat procedure rate of 10.3%. Three patients had contralateral stent placement, while 8 had ipsilateral stent placement within or adjacent to the original stent. Only 3 patients required conversion to CSF diversion or 2.2% of patients with stents.ConclusionsPatients with medically refractory idiopathic intracranial hypertension have traditionally undergone a CSF diversion procedure as the first intervention. This paradigm may need to be re-examined, given the high technical and clinical success and low complication rates with dural venous sinus stenting.

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Brain Slump Caused by Jugular Venous Stenoses Treated by Stenting: A Hypothesis to Link Spontaneous Intracranial Hypotension with Idiopathic Intracranial Hypertension.

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