-
AJNR. American Journal of Neuroradiology Mar 2023
Topics: Humans; Myelography; Tomography, X-Ray Computed
PubMed: 36822824
DOI: 10.3174/ajnr.A7726 -
Continuum (Minneapolis, Minn.) Aug 2015Spontaneous intracranial hypotension results from CSF volume depletion, nearly always from spontaneous CSF leaks. Spontaneous intracranial hypotension is increasingly... (Review)
Review
PURPOSE OF REVIEW
Spontaneous intracranial hypotension results from CSF volume depletion, nearly always from spontaneous CSF leaks. Spontaneous intracranial hypotension is increasingly diagnosed in practice; the number of atypical, unconfirmed, and doubtful cases is also increasing, as are treatment failures. These confront neurologists and create many challenges. This review provides neurologists with a guide to diagnosis, evaluation, and treatment of spontaneous intracranial hypotension.
RECENT FINDINGS
The clinical spectrum of spontaneous intracranial hypotension is expanding. Spontaneous CSF leak is considered a disorder with a variety of clinical manifestations and imaging features, sometimes quite different from what may be seen after dural puncture. The anatomy of the spontaneous CSF leak is frequently complex, with contributions from disorders of the connective tissue matrix and associated preexisting areas of dural weakness and meningeal diverticula. To locate the site of the leak, CT myelography is still the study of choice. For rapid-flow leaks, dynamic CT myelography has been very helpful, while slow-flow leaks can remain a lingering challenge. The fundamental question of whether a CSF leak is present in uncertain cases can be best answered by radioisotope cisternography. In most cases, epidural blood patch is the main treatment; however, bilevel or multilevel epidural injections are gaining some momentum as treatment for selected cases.
SUMMARY
This article outlines various clinical aspects of spontaneous intracranial hypotension, including headache characteristics, CSF changes, and imaging findings and their underlying mechanisms, as well as treatments and disease complications.
Topics: Cerebrospinal Fluid Leak; Headache; Humans; Intracranial Hypotension; Neuroimaging
PubMed: 26252593
DOI: 10.1212/CON.0000000000000193 -
Deutsches Arzteblatt International Jul 2020Spontaneous intracranial hypotension (SIH) is an underdiagnosed disease. Its incidence is estimated at 5 per 100 000 persons per year. (Review)
Review
BACKGROUND
Spontaneous intracranial hypotension (SIH) is an underdiagnosed disease. Its incidence is estimated at 5 per 100 000 persons per year.
METHODS
This review is based on a selective literature search in PubMed covering the years 2000-2019, as well as on the authors' personal experience.
RESULTS
The diagnostic and therapeutic methods discussed here are supported by level 4 evidence. SIH is caused by spinal leakage of cerebrospinal fluid (CSF) out of ventral dural tears or nerve root diverticula, or, in 2-5% of cases, through a fistula leading directly into the periradicular veins (CSF-venous fistula). In half of all patients, no CSF leak is demonstrable. A low CSF opening pressure on lumbar puncture is present in only one-third of patients; imaging studies are thus needed to confirm and localize a spinal CSF leak. Half of all patients in whom myelographic computed tomography (CT) reveals contrast medium reaching the epidural space have ventral dural tears, which tend to be located at upper thoracic spinal levels. Epidural blood patches applied under fluoroscopic or CT guidance can seal the CSF leak in 30-70% of patients, but 90% of patients with ventral dural tears will need operative closure. Some patients who have no visible epidural contrast medium on CT presumably do not have SIH, while others do, in fact, have a CSF leak from a diverticulum or a CSF-venous fistula and will need to have the site of the leak demonstrated with the aid of further studies, such as dynamic (subtraction) myelography in the lateral decubitus position.
CONCLUSION
The management of patients with SIH calls for complementary imaging studies to demonstrate the causative spinal CSF leak. Often, successful treatment requires surgical closure of the leak. In view of the sparse evidence available to date, controlled studies should be performed.
Topics: Cerebrospinal Fluid Leak; Contrast Media; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography; Tomography, X-Ray Computed
PubMed: 33050997
DOI: 10.3238/arztebl.2020.0480 -
The Veterinary Clinics of North... Aug 2022Cervical vertebral stenotic myelopathy is a common cause of ataxia in horses secondary to spinal cord compression. Early articles describing this problem indicate... (Review)
Review
Cervical vertebral stenotic myelopathy is a common cause of ataxia in horses secondary to spinal cord compression. Early articles describing this problem indicate genetic predisposition as a known risk factor. Further studies have shown the problem is a developmental abnormality which might have genetic predisposition and environmental influences.
Topics: Animals; Cervical Vertebrae; Genetic Predisposition to Disease; Horse Diseases; Horses; Spinal Cord Diseases; Spinal Stenosis
PubMed: 35953144
DOI: 10.1016/j.cveq.2022.05.002 -
Acta Neurologica Belgica Feb 2020Spontaneous intracranial hypotension (SIH) results from spinal cerebrospinal fluid (CSF) leaking. An underlying connective tissue disorder that predisposes to weakness... (Review)
Review
Spontaneous intracranial hypotension (SIH) results from spinal cerebrospinal fluid (CSF) leaking. An underlying connective tissue disorder that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. During the last decades, a much larger number of spontaneous cases are identified and a far broader clinical SIH spectrum is recognized. Orthostatic headache is the main presentation symptom of SIH; some patients also have other manifestations, mainly cochlear-vestibular signs and symptoms. Differential diagnosis with other syndromes presenting with orthostatic headache is crucial. Brain CT, brain MR, spine MRI, and MRI myelography are the imaging modalities of first choice for SIH diagnosis. Invasive imaging techniques, such as myelography, CT myelography, and radioisotopic cisternography, are progressively being abandoned. No randomized clinical trials have assessed the treatment of SIH. In a minority of cases, SIH resolved spontaneously or with only conservative treatment. If orthostatic headache persists after conservative treatment, a lumbar epidural blood patch (EBP) without previous leak identification (so-called "blind" EBP) is a widely used initial intervention and may be repeated several times. If EBPs fail, after the CSF leak sites identification using invasive imaging techniques, other therapeutic approaches include: a targeted epidural patch, surgical reduction of dural sac volume, or direct surgical closure. The prognosis is generally good after intervention, but serious complications may occur. More research is needed to better understand SIH pathophysiology to refine imaging modalities and treatment approaches and to evaluate clinical outcomes.
Topics: Headache; Humans; Intracranial Hypotension
PubMed: 31215003
DOI: 10.1007/s13760-019-01166-8 -
Annals of Emergency Medicine Jan 2022
Topics: Adult; Cerebrospinal Fluid Leak; Emergency Service, Hospital; Headache; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Male; Manipulation, Spinal; Myelography; Tomography, X-Ray Computed
PubMed: 34949418
DOI: 10.1016/j.annemergmed.2021.07.111 -
The American Journal of Medicine Mar 2019Atraumatic spinal emergencies often present a diagnostic and management dilemma for health care practitioners. Spinal epidural abscess, cauda equina syndrome, and spinal... (Review)
Review
Atraumatic spinal emergencies often present a diagnostic and management dilemma for health care practitioners. Spinal epidural abscess, cauda equina syndrome, and spinal epidural hematoma are conditions that can insidiously present to outpatient medical offices, urgent care centers, and emergency departments. Unless a high level of clinical suspicion is maintained, these clinical entities may be initially misdiagnosed and mismanaged. Permanent neurologic sequela and even death can result if delays in appropriate treatment occur. A focused, critical review of 34 peer-reviewed articles was performed to identify current data about accurate diagnosis of spinal emergencies. This review highlights the key features of these 3 pathological entities with an emphasis on appropriate diagnostic strategy to intervene efficiently and minimize morbidity.
Topics: Anti-Bacterial Agents; Blood Coagulation Disorders; Cauda Equina Syndrome; Debridement; Decompression, Surgical; Delayed Diagnosis; Diagnosis, Differential; Diagnostic Errors; Emergencies; Epidural Abscess; Hematoma, Epidural, Spinal; Humans; Magnetic Resonance Imaging; Myelography; Primary Health Care; Sciatica; Tomography, X-Ray Computed
PubMed: 30291829
DOI: 10.1016/j.amjmed.2018.09.022 -
AJNR. American Journal of Neuroradiology Jan 2020Digital subtraction myelography is a valuable diagnostic technique to detect the exact location of CSF leaks in the spine to facilitate appropriate diagnosis and... (Review)
Review
Digital subtraction myelography is a valuable diagnostic technique to detect the exact location of CSF leaks in the spine to facilitate appropriate diagnosis and treatment of spontaneous spinal CSF leaks. Digital subtraction myelography is an excellent diagnostic tool for assessment of various types of CSF leaks, and lateral decubitus digital subtraction myelography is increasingly being used to diagnose CSF-venous fistulas. Lateral decubitus digital subtraction myelography differs from typical CT and fluoroscopy-guided myelograms in many ways, including equipment, supplies, and injection and image-acquisition techniques. Operators should be familiar with techniques, common pitfalls, and artifacts to improve diagnostic yield and prevent nondiagnostic examinations.
Topics: Cerebrospinal Fluid Leak; Female; Humans; Male; Myelography
PubMed: 31857327
DOI: 10.3174/ajnr.A6368 -
Neurology May 2022Brain sagging dementia (BSD), caused by spontaneous intracranial hypotension (SIH), is a rare syndrome that is only recently recognized, mimicking the clinical findings... (Review)
Review
Brain sagging dementia (BSD), caused by spontaneous intracranial hypotension (SIH), is a rare syndrome that is only recently recognized, mimicking the clinical findings of behavioral variant frontotemporal dementia (bvFTD). Being aware of its signs and symptoms is essential for early diagnosis and treatment in this potentially reversible form of dementia. Our objective was to identify cases of BSD in the literature and present its clinical characteristics, diagnostic workup, treatment options, and outcome. The review was reported according to PRISMA guidelines and registered with the PROSPERO database (CRD42020150709). MEDLINE, EMBASE, PsychINFO, and Cochrane Library were searched. There was no date restriction. The search was updated in April 2021. A total of 983 articles were screened and assessed for eligibility. Twenty-nine articles (25 case reports and 4 series) and 70 patients were selected for inclusion. No cranial leak cases were identified. BSD diagnosis should be made based on clinical signs and symptoms and radiologic findings. There is a male predominance (F:M ratio 1:4) and a peak incidence in the 6th decade of life. The main clinical manifestation is insidious onset, gradually progressive cognitive and behavioral changes characteristic for bvFTD. Headache is present in the majority of patients (89%). The presence of brain sagging and absence of frontotemporal atrophy is an absolute criterion for the diagnosis. CSF leak is identified with myelography and digital subtraction myelography. The treatment and repair depend on the etiology and extent of the dural defect, although an epidural blood patch is the first-line treatment in most cases. With treatment, 81% experienced partial and 67% complete resolution of their symptoms. This review highlights the most important clinical aspects of BSD. Due to the sparse evidence and lack of BSD awareness, many patients are likely left undiagnosed. Recognizing this condition is essential to provide early treatment to reverse the cognitive and behavioral changes that may otherwise progress and fully impair the patient. Moreover, patients with longstanding SIH must be assessed carefully for cognitive and behavioral changes.
Topics: Brain; Female; Frontotemporal Dementia; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Male; Myelography
PubMed: 35338080
DOI: 10.1212/WNL.0000000000200511 -
Chinese Neurosurgical Journal Aug 2022Spinal extradural meningeal cysts (SEMCs) are rare lesions of the spinal canal. Although closure of the dural defect can achieve satisfactory therapeutic effects,... (Review)
Review
Spinal extradural meningeal cysts (SEMCs) are rare lesions of the spinal canal. Although closure of the dural defect can achieve satisfactory therapeutic effects, locating the fistula is difficult. This review summarizes the methods for locating the fistula of SEMCs and the distribution and features of fistula sites.This was a non-systematic literature review of studies on SEMCs. We searched PubMed for English-language articles to summarize the methods of locating the defect. The search words were "epidural arachnoid cyst," "dural cyst," "epidural cyst," and "epidural meningeal cyst." For the defect location component of the study, case reports, studies with a sample size less than four, controversial ventral dural dissection(s), and undocumented fistula location reports were excluded.Our review showed that radiography and computed tomography (CT) may show changes in the bony structure of the spine, with the largest segment of change indicating the fistula site. Occasionally, magnetic resonance imaging (MRI) can show a cerebrospinal fluid (CSF) flow void at the fistula site. The middle segment of the cyst on sagittal MRI, the largest cyst area, and cyst laterality in the axial view indicate the fistula location. Myelography can show the fistula location in the area of the enhanced cyst and subarachnoid stenosis. Digital subtraction or delayed CT can be used to observe the location of the initial cyst filling. Cine MRI and time-spatial labeling inversion pulse techniques can be used to observe CSF flow. Steady-state image construction interference sequence MRI has a high spatial resolution. Neuroendoscopy, MRI myelography, and ultrasound fistula detection can be performed intraoperatively. Moreover, the fistula was located most often in the T12-L1 segment.Identifying the fistula location is difficult and requires a combination of multiple examinations and experience for comprehensive judgment.
PubMed: 36045421
DOI: 10.1186/s41016-022-00291-3