-
Journal of the American Board of Family... 2020Cervical spondylotic myelopathy (CSM) is a neurologic condition that develops insidiously over time as degenerative changes of the spine result in compression of the... (Review)
Review
Cervical spondylotic myelopathy (CSM) is a neurologic condition that develops insidiously over time as degenerative changes of the spine result in compression of the cord and nearby structures. It is the most common form of spinal cord injury in adults; yet, its diagnosis is often delayed. The purpose of this article is to review the pathophysiology, natural history, diagnosis, and management of CSM with a focus on the recommended timeline for physicians suspecting CSM to refer patients to a spine surgeon. Various processes underlie spondylotic changes of the canal and are separated into static and dynamic factors. Not all patients with evidence of cord compression will present with symptoms, and the progression of disease varies by patient. The hallmark symptoms of CSM include decreased hand dexterity and gait instability as well as sensory and motor dysfunction. magnetic resonance imaging is the imaging modality of choice in patients with suspected CSM, but computed tomography myelography may be used in patients with contraindications. Patients with mild CSM may be treated surgically or nonoperatively, whereas those with moderate-severe disease are treated operatively. Due to the long-term disability that may result from a delay in diagnosis and management, prompt referral to a spine surgeon is recommended for any patient suspected of having CSM. This review provides information and guidelines for practitioners to develop an actionable awareness of CSM.
Topics: Adult; Cervical Vertebrae; Humans; Magnetic Resonance Imaging; Spinal Cord Compression; Spinal Cord Diseases; Spondylosis
PubMed: 32179614
DOI: 10.3122/jabfm.2020.02.190195 -
Frontiers in Veterinary Science 2020Imaging is integral in the diagnosis of canine intervertebral disc disease (IVDD) and in differentiating subtypes of intervertebral disc herniation (IVDH). These include... (Review)
Review
Imaging is integral in the diagnosis of canine intervertebral disc disease (IVDD) and in differentiating subtypes of intervertebral disc herniation (IVDH). These include intervertebral disc extrusion (IVDE), intervertebral disc protrusion (IVDP) and more recently recognized forms such as acute non-compressive nucleus pulposus extrusion (ANNPE), hydrated nucleus pulposus extrusion (HNPE), and intradural/intramedullary intervertebral disc extrusion (IIVDE). Many imaging techniques have been described in dogs with roles for survey radiographs, myelography, computed tomography (CT), and magnetic resonance imaging (MRI). Given how common IVDH is in dogs, a thorough understanding of the indications and limitations for each imaging modality to aid in diagnosis, treatment planning and prognosis is essential to successful case management. While radiographs can provide useful information, especially for identifying intervertebral disc degeneration or calcification, there are notable limitations. Myelography addresses some of the constraints of survey radiographs but has largely been supplanted by cross-sectional imaging. Computed tomography with or without myelography and MRI is currently utilized most widely and have become the focus of most contemporary studies on this subject. Novel advanced imaging applications are being explored in dogs but are not yet routinely performed in clinical patients. The following review will provide a comprehensive overview on common imaging modalities reported to aid in the diagnosis of IVDH including IVDE, IVDP, ANNPE, HNPE, and IIVDE. The review focuses primarily on canine IVDH due to its frequency and vast literature as opposed to feline IVDH.
PubMed: 33195623
DOI: 10.3389/fvets.2020.588338 -
Neuroradiology Nov 2021Spontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with typical MRI findings among which engorgement of the venous sinuses, pachymeningeal... (Review)
Review
Spontaneous intracranial hypotension (SIH) is an orthostatic headache syndrome with typical MRI findings among which engorgement of the venous sinuses, pachymeningeal enhancement, and effacement of the suprasellar cistern have the highest diagnostic sensitivity. SIH is in almost all cases caused by spinal CSF leaks. Spinal MRI scans showing so-called spinal longitudinal extradural fluid (SLEC) are suggestive of ventral dural tears (type 1 leak) which are located with prone dynamic (digital subtraction) myelography. As around half of the ventral dural tears are located in the upper thoracic spine, additional prone dynamic CT myelography is often needed. Leaking nerve root sleeves typically associated with meningeal diverticulae (type 2 leaks) and CSF-venous fistulas (type 3 leaks) are proven via lateral decubitus dynamic digital subtraction or CT myelography: type 2 leaks are SLEC-positive if the tear is proximal and SLEC-negative if it is distal, and type 3 leaks are always SLEC-negative. Although 30-70% of SIH patients show marked improvement following epidural blood patches applied via various techniques definite cure mostly requires surgical closure of ventral dural tears and surgical ligations of leaking nerve root sleeves associated with meningeal diverticulae or CSF-venous fistulas. For the latter, transvenous embolization with liquid embolic agents via the azygos vein system is a novel and valuable therapeutic alternative.
Topics: Cerebrospinal Fluid Leak; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography; Spine
PubMed: 34297176
DOI: 10.1007/s00234-021-02766-z -
JAMA Neurology Mar 2021Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain. (Meta-Analysis)
Meta-Analysis
IMPORTANCE
Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain.
OBJECTIVE
To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment outcomes for SIH.
DATA SOURCES
Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline-compliant systematic review and meta-analysis of the literature on SIH. Three databases were searched from inception to April 30, 2020: PubMed/MEDLINE, Embase, and Cochrane. The following search terms were used in each database: spontaneous intracranial hypotension, low CSF syndrome, low CSF pressure syndrome, low CSF volume syndrome, intracranial hypotension, low CSF pressure, low CSF volume, CSF hypovolemia, CSF hypovolaemia, spontaneous spinal CSF leak, spinal CSF leak, and CSF leak syndrome.
STUDY SELECTION
Original studies in English language reporting 10 or more patients with SIH were selected by consensus.
DATA EXTRACTION AND SYNTHESIS
Data on clinical presentation, investigations findings, and treatment outcomes were collected and summarized by multiple observers. Random-effect meta-analyses were used to calculate pooled estimates of means and proportions.
MAIN OUTCOMES AND MEASURES
The predetermined main outcomes were the pooled estimate proportions of symptoms of SIH, imaging findings (brain and spinal imaging), and treatment outcomes (conservative, epidural blood patches, and surgical).
RESULTS
Of 6878 articles, 144 met the selection criteria and reported on average 53 patients with SIH each (range, 10-568 patients). The most common symptoms were orthostatic headache (92% [95% CI, 87%-96%]), nausea (54% [95% CI, 46%-62%]), and neck pain/stiffness (43% [95% CI, 32%-53%]). Brain magnetic resonance imaging was the most sensitive investigation, with diffuse pachymeningeal enhancement identified in 73% (95% CI, 67%-80%) of patients. Brain magnetic resonance imaging findings were normal in 19% (95% CI, 13%-24%) of patients. Spinal neuroimaging identified extradural cerebrospinal fluid in 48% to 76% of patients. Digital subtraction myelography and magnetic resonance myelography with intrathecal gadolinium had high sensitivity in identifying the exact leak site. Lumbar puncture opening pressures were low, normal (60-200 mm H2O), and high in 67% (95% CI, 54%-80%), 32% (95% CI, 20%-44%), and 3% (95% CI, 1%-6%), respectively. Conservative treatment was effective in 28% (95% CI, 18%-37%) of patients and a single epidural blood patch was successful in 64% (95% CI, 56%-72%). Large epidural blood patches (>20 mL) had better success rates than small epidural blood patches (77% [95% CI, 63%-91%] and 66% [95% CI, 55%-77%], respectively).
CONCLUSIONS AND RELEVANCE
Spontaneous intracranial hypotension should not be excluded on the basis of a nonorthostatic headache, normal neuroimaging findings, or normal lumbar puncture opening pressure. Despite the heterogeneous nature of the studies available in the literature and the lack of controlled interventional studies, this systematic review offers a comprehensive and objective summary of the evidence on SIH that could be useful in guiding clinical practice and future research.
Topics: Blood Patch, Epidural; Conservative Treatment; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Treatment Outcome
PubMed: 33393980
DOI: 10.1001/jamaneurol.2020.4799 -
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 -
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 -
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 -
AJNR. American Journal of Neuroradiology Mar 2023
Topics: Humans; Myelography; Tomography, X-Ray Computed
PubMed: 36822824
DOI: 10.3174/ajnr.A7726