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Spontaneous cerebrospinal fluid leakage causes orthostatic headache, and mental dullness, diplopia, and coma may occur. Most cases are due to a tear in the spinal CSF membranes. MRI shows enhancement of the dura, and special imaging, such as digital-subtraction myelography (shown in videos), may be required to show the leakage site.

Abstract BACKGROUND Spinal cerebrospinal fluid (CSF) leaks are the cause of spontaneous intracranial hypotension (SIH). OBJECTIVE To propose a surgical strategy, stratified according to anatomic location of the leak, for sealing all CSF leaks around the 360° circumference of the dura through a single tailored posterior approach. METHODS All consecutive SIH patients undergoing spinal surgery were included. The anatomic site of the leak was exactly localized. We used a tailored hemilaminotomy and intraoperative neurophysiological monitoring (IOM) for all cases. Neurological status was assessed before and up to 90 d after surgery. RESULTS Forty-seven SIH patients had an identified CSF leak between the levels C6 and L1. Leaks, anterior to the spinal cord, were approached by a transdural trajectory (n = 28). Leaks lateral to the spinal cord by a direct extradural trajectory (n = 17) and foraminal leaks by a foraminal microsurgical trajectory (n = 2). The transdural trajectory necessitated cutting the dentate ligament accompanied by elevation and rotation of the spinal cord under continuous neuromonitoring (spinal cord release maneuver, SCRM). Four patients had transient defiticts, none had permanent neurological deficits. We propose an anatomic classification of CSF leaks into I ventral (77%, anterior dural sac), II lateral (19%, including nerve root exit, lateral, and dorsal dural sac), and III foraminal (4%). CONCLUSION Safe sealing (with IOM) of all CSF leaks around the 360° surface of the dura is feasible through a single posterior approach. The exact surgical trajectory is selected according to the anatomic category of the leak.

When a tear occurs in one of the major arteries in the neck and allows blood to enter the wall of the artery and split its layers, the result is either stenosis or aneurysmal dilatation of the vessel. This process was long thought to be a rare cause of stroke, particularly in the absence of trauma, and the diagnosis was usually not made until the postmortem examination. 1 – 3 It was not until the late 1970s, when Fisher et al. 1 and Mokri et al. 2 described dissections of carotid and vertebral arteries as detected by modern diagnostic approaches, that dissections began to . . .

Background Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension. Methods Medical records and radiologic imaging of the two patients were reviewed. Results Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.  Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia. Conclusions Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.

Introduction Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lobes), as visualized on brain magnetic resonance imaging. However, the detection of these CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM). Methods We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years [range: 31–70 years]). Results Nine patients (42.8%) were found to have a CSF‐venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near‐complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain sagging, but in only three (25.0%) of the twelve patients in whom no CSF‐venous fistula could be detected (P = 0.0011), and who were treated with non‐targeted therapies. Discussion Concerns about a spinal CSF leak should not be dismissed in patients with frontotemporal brain sagging syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging the source of the loss of spinal CSF remains elusive in more than half of patients.

Frontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non-directed treatments is low. Some of these patients have a remote history of craniectomy/cranioplasty and we report a positive response to custom implant cranioplasty revision many years after their initial cranioplasty. We reviewed medical records and imaging studies of 61 consecutive patients with FTD sagging brain syndrome. A SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms before and after custom implant cranioplasty. Pre- and post-operative brain MRI was obtained to assess degree of brain sagging. Eight (13.1%) of the 61 patients had a history of craniectomy/cranioplasty 1.5-13.5 years prior to onset of symptoms of FTD sagging brain syndrome. The mean age of the one woman and seven men at the time of presentation to our medical center was 50 years (range, 26-68 years). None had sinking scalp flap syndrome. Prior treatments included epidural blood patching and dural reduction surgery. Custom cranial implant surgery was performed in four patients and resulted in prompt and remarkable improvement of symptoms in three patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in one patient. Brain MRI showed improvement of brain sagging. A disproportionate number of patients with FTD sagging brain syndrome have a remote history of supratentorial craniectomy/cranioplasty and revision cranioplasty should be considered.

We aimed to assess the frequency of connective tissue abnormalities among patients with cerebrospinal fluid (CSF) leaks in a prospective study using a large cohort of patients. We enrolled a consecutive group of 50 patients, referred for consultation because of CSF leak. All patients have been carefully examined for the presence of connective tissue abnormalities, and based on findings, patients underwent genetic testing. Ancillary diagnostic studies included echocardiography, eye exam, and histopathological examinations of skin and dura biopsies in selected patients. We identified nine patients with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome and other unclassified forms. In seven patients, spontaneous CSF leak was the first noted manifestation of the genetic disorder. We conclude that spontaneous CSF leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. We propose that there is a clinical basis for considering spontaneous CSF leak as a clinical manifestation of heritable connective tissue disorders, and we suggest that patients with CSF leaks should be screened for connective tissue and vascular abnormalities.

The patient then regained full consciousness for about 48 h, after which he deteriorated with a widely fluctuating level of consciousness, his Glasgow coma scale scores varying between 6 and 14 depending on his position. 2 mL fibrin glue (Tisseel, Baxter BioStience, Westlake Village, CA, USA) was administered percutaneously through an 18 gauge needle at the site of the CSF leak.

Intracranial hypotension is known to occur as a result of spinal cerebrospinal fluid (CSF) leaking, which may be iatrogenic, traumatic, or spontaneous. Headache is usually, but not always, orthostatic. Spontaneous cases are recognized more readily than in previous decades as a result of a greater awareness of clinical presentations and typical cranial magnetic resonance imaging findings. An underlying disorder of connective tissue that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. CT, MR, and digital subtraction myelography are the imaging modalities of choice to identify spinal CSF leakage. Spinal imaging protocols continue to evolve with improved diagnostic sensitivity. Epidural blood patching is the most common initial intervention for those seeking medical attention, and may be repeated several times. Surgery is reserved for cases that fail to respond or relapse after simpler measures. While the prognosis is generally good with intervention, serious complications do occur. More research is needed to better understand the genetics and pathophysiology of dural weakness as well as physiologic compensatory mechanisms, to continue to refine imaging modalities and treatment approaches, and to evaluate short- and long-term clinical outcomes.