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Journal of Neurology, Neurosurgery, and... Oct 2023We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial...
BACKGROUND
We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG).
METHODS
A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH.
RESULTS
SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided.
CONCLUSIONS
This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.
Topics: Humans; Intracranial Hypotension; Cerebrospinal Fluid Leak; Magnetic Resonance Imaging; Headache; Diagnosis, Differential
PubMed: 37147116
DOI: 10.1136/jnnp-2023-331166 -
Neurology India 2021Spontaneous intracranial hypotension (SIH) is a highly misdiagnosed and underdiagnosed disorder. (Review)
Review
BACKGROUND
Spontaneous intracranial hypotension (SIH) is a highly misdiagnosed and underdiagnosed disorder.
OBJECTIVE
Update evaluation and treatment of spontaneous intracranial hypotension.
METHODS AND MATERIAL
Narrative review.
RESULTS
Traditionally, SIH is diagnosed when a headache has developed spontaneously and in temporal relation to a CSF leak (evident on imaging) and/or CSF hypotension (lumbar puncture opening pressure <60 mm CSF). However, lumbar puncture is not mandatorily required to diagnose SIH. Besides headache, other symptoms such as nausea/vomiting in 50.6%, neck pain/stiffness in 33%, tinnitus in 19%, dizziness in 14%, hearing disturbances in 10.7%, followed by visual disturbances, vertigo, back pain, and cognitive symptoms may be present. In suspected cases of SIH, brain and spine should be evaluated with MRI. Dynamic computerized tomographic myelography is required to demonstrate the site of spinal CSF leak. Epidural blood patch (EBP) is a minimally invasive treatment for spontaneous intracranial hypotension (SIH) refractory to medical management and provides symptomatic relief in up to 90% of patients even in patients with bilateral subdural hematomas. The CSF-venous fistulas do not respond well to EBP, and the most definitive curative treatment is the surgical closure of the fistula.
CONCLUSIONS
The SIH is a distinct entity and requires a high index of suspicion for diagnosis. A post-contrast MRI should be included for evaluation of headaches. Spinal MRI should be done to demonstrate the site of leak. Epidural blood patch therapy is the most effective treatment of SIH. Most SDHs associated with SIH do not require treatment.
Topics: Blood Patch, Epidural; Brain; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Spine
PubMed: 35103002
DOI: 10.4103/0028-3886.332255 -
Lakartidningen Oct 2023Spontaneous intracranial hypotension (SIH) is a disease presenting mostly with orthostatic head and neck pain due to a spontaneous cerebrospinal fluid (CSF) leak or a...
Spontaneous intracranial hypotension (SIH) is a disease presenting mostly with orthostatic head and neck pain due to a spontaneous cerebrospinal fluid (CSF) leak or a CSF-venous fistula in the spinal region. It demonstrates typical MRI findings with sagging of the brain causing tension of the meninges and sometimes the cranial nerves. It shares some clinical similarities with post puncture headache but differs in its pathophysiological cause, diagnosis, and treatment. Many patients remain misdiagnosed or wait too long for the correct diagnosis. The diagnostic work-up includes an MRI of the head and spine in search of typical SIH signs. Myelography and CT scans are performed to identify the location of the CSF leak or CSF-venous fistula. Treatment options may involve (1) initial conservative treatment with bed rest, caffein and fluids, (2) interventions such as epidural blood patch, fibrin patch, and embolization, or (3) surgical closure of the leak.
Topics: Humans; Intracranial Hypotension; Blood Patch, Epidural; Brain; Headache; Fistula
PubMed: 37782313
DOI: No ID Found -
AJR. American Journal of Roentgenology Dec 2021CSF-venous fistulas (CVFs), first described in 2014, are an important cause of spontaneous intracranial hypotension. CVFs can be challenging to detect on conventional... (Review)
Review
CSF-venous fistulas (CVFs), first described in 2014, are an important cause of spontaneous intracranial hypotension. CVFs can be challenging to detect on conventional anatomic imaging because, unlike other types of spinal CSF leak, they do not typically result in pooling of fluid in the epidural space, and imaging signs of CVF may be subtle. Specialized myelographic techniques have been developed to help with CVF identification, but these techniques are not yet widely disseminated. This article reviews the current understanding of CVFs, emphasizing correlations between venous anatomy and imaging findings as well as potential mechanisms for pathogenesis, and describes current imaging techniques used for CVF diagnosis and localization. These techniques are broadly classified into fluoroscopy-based methods, including digital subtraction myelography and dynamic myelography, and cross-sectional methods, including decubitus CT myelography and MR myelography with intrathecal injection of gadolinium. Knowledge of these various options, including their relative advantages and disadvantages, is critical in the care of patients with spontaneous intracranial hypotension. Investigation is ongoing, and continued advances in knowledge about CVFs as well as in optimal imaging detection are anticipated.
Topics: Cerebrospinal Fluid Leak; Fluoroscopy; Humans; Magnetic Resonance Imaging; Myelography; Tomography, X-Ray Computed; Vascular Fistula
PubMed: 34191547
DOI: 10.2214/AJR.21.26182 -
Neuroimaging Clinics of North America Nov 2019Spinal MR imaging is excellent for identifying details of spinal anatomy, including intraspinal contents, neural foramina, joints, ligaments, intervertebral discs, and... (Review)
Review
Spinal MR imaging is excellent for identifying details of spinal anatomy, including intraspinal contents, neural foramina, joints, ligaments, intervertebral discs, and bone marrow. Cortical bony structures of the spine are better imaged using CT. Conventional and CT myelography is an alternative to MR imaging in those with contraindications to MR imaging or in evaluation of spinal cerebrospinal fluid leaks. Motion- and flow-related artifacts may occur during imaging and should not be mistaken for lesions. With advancements in MR imaging hardware and software, spinal MR imaging can expand its role in the delineation of normal and abnormal spinal anatomy.
Topics: Humans; Magnetic Resonance Imaging; Myelography; Spine; Tomography, X-Ray Computed
PubMed: 31677724
DOI: 10.1016/j.nic.2019.08.001 -
Clinical Radiology Oct 2020Idiopathic spinal cord herniation is a rare but important and increasingly recognised cause of myelopathy seen in the thoracic spine. The factors that contribute to the... (Review)
Review
Idiopathic spinal cord herniation is a rare but important and increasingly recognised cause of myelopathy seen in the thoracic spine. The factors that contribute to the aetiology of the condition and of the dural defect through which the cord herniates remain under debate. We discuss the clinical features and proposed pathophysiology of the condition, and illustrate key imaging findings on MRI, fluoroscopy and computed tomography (CT) myelography to establish the diagnosis, and discuss relevant differential diagnoses. Awareness and recognition of the condition is key as surgical intervention can improve outcomes for patients.
Topics: Diagnosis, Differential; Disease Progression; Hernia; Herniorrhaphy; Humans; Spinal Cord Diseases; Thoracic Vertebrae
PubMed: 32499121
DOI: 10.1016/j.crad.2020.04.013 -
Radiographics : a Review Publication of... 2020CT myelography is an important imaging modality that combines the advantages of myelography and the high resolution of CT. It provides a detailed delineation of... (Review)
Review
CT myelography is an important imaging modality that combines the advantages of myelography and the high resolution of CT. It provides a detailed delineation of pathologic spine conditions, especially those involving the thecal sac and its contents. However, the role of CT myelography has dramatically and appropriately decreased with the advent of MRI, which provides a noninvasive method to demonstrate pathologic spine conditions with high signal intensity in soft tissues. At the present time, CT myelography is often performed in patients who require evaluation of the thecal sac but have a contraindication to undergoing MRI. However, there remain many situations in which CT myelography is indicated and plays a critical role in patient treatment. The authors review common and uncommon indications for CT myelography and demonstrate various pathologic conditions in which CT myelography plays a vital role in patient treatment in this modern era of MRI.RSNA, 2020.
Topics: Humans; Myelography; Spinal Diseases; Tomography, X-Ray Computed
PubMed: 32058837
DOI: 10.1148/rg.2020190135 -
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 -
Cureus Dec 2023Degenerative cervical myelopathy (DCM) is a spinal condition of growing importance due to its increasing prevalence within the ageing population. DCM involves the... (Review)
Review
Degenerative cervical myelopathy (DCM) is a spinal condition of growing importance due to its increasing prevalence within the ageing population. DCM involves the degeneration of the cervical spine due to various processes such as disc ageing, osteophyte formation, ligament hypertrophy or ossification, as well as coexisting congenital anomalies. This article provides an overview of the literature on DCM and considers areas of focus for future research. A patient with DCM can present with a variety of symptoms ranging from mild hand paraesthesia and loss of dexterity to a more severe presentation of gait disturbance and loss of bowel/bladder control. Hoffman's sign and the inverted brachioradialis reflex are also important signs of this disease. The gold standard imaging modality is MRI which can identify signs of degeneration of the cervical spine. Other modalities include dynamic MRI, myelography, and diffusion tensor imaging. One important scoring system to aid with the diagnosis and categorisation of the severity of DCM is the modified Japanese Orthopaedic Association score. This considers motor, sensory, and bowel/bladder dysfunction, and categorises patients into mild, moderate, or severe DCM. DCM is primarily treated with surgery as this can halt disease progression and may even allow for neurological recovery. The surgical approach will depend on the location of degeneration, the number of cervical levels involved and the pathophysiological process. Surgical approach options include anterior cervical discectomy and fusion, corpectomy, or posterior approach (laminectomy ± fusion). Conservative management is also considered for some patients with mild or non-progressive DCM or for patients where surgery is not an option. Conservative treatment may include physical therapy, traction, or neck immobilisation. Future recommendations include research into the prevalence rate of DCM and if there is a difference between populations. Further research on the benefit of conservative management for patients with mild or non-progressive DCM would be recommended.
PubMed: 38213348
DOI: 10.7759/cureus.50387 -
Neurosurgery Clinics of North America Jul 2024Cerebrospinal fluid-venous fistulas (CSFVFs) were first described in 2014 and have since become an increasingly diagnosed cause of spontaneous intracranial hypotension... (Review)
Review
Cerebrospinal fluid-venous fistulas (CSFVFs) were first described in 2014 and have since become an increasingly diagnosed cause of spontaneous intracranial hypotension due to increased clinical recognition and advancements in diagnostic modalities. In this review, the authors discuss CSFVF epidemiology, the variety of clinical presentations, the authors' preferred diagnostic approach, recent advancements in diagnostic methods, treatment options, current challenges, and directions of future research.
Topics: Humans; Intracranial Hypotension; Cerebral Veins; Vascular Fistula; Cerebrospinal Fluid
PubMed: 38782524
DOI: 10.1016/j.nec.2024.02.003