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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 -
The New England Journal of Medicine Dec 2021
Review
Topics: Brain; Cerebrospinal Fluid Leak; Headache; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography; Optic Nerve; Subarachnoid Space
PubMed: 34874632
DOI: 10.1056/NEJMra2101561 -
The American Journal of Medicine Dec 2021Cauda equina syndrome is a potentially devastating spinal condition. The diagnosis of cauda equina syndrome lacks sensitivity and specificity, sometimes occurring after... (Review)
Review
Cauda equina syndrome is a potentially devastating spinal condition. The diagnosis of cauda equina syndrome lacks sensitivity and specificity, sometimes occurring after irreparable neurological damage has happened. Timely diagnosis and treatment is imperative for optimal outcomes and for avoiding medicolegal ramifications. Cauda equina syndrome results from conditions that compress the nerves in the lumbosacral spinal canal. Although no consensus definition exists, it generally presents with varying degrees of sensory loss, motor weakness, and bowel and bladder dysfunction (the latter of which is required to definitively establish the diagnosis). A thorough history and physical exam is imperative, followed by magnetic resonance or computed tomography imaging myelogram to aid in diagnosis and treatment. Once suspected, emergent spinal surgery referral is indicated, along with urgent decompression. Even with expeditious surgery, improvements remain inconsistent. However, early intervention has been shown to portend greater chance of neurologic recovery. All providers in clinical practice must understand the severity of this condition. Providers can optimize long-term patient outcomes and minimize the risk of litigation by open communication, good clinical practice, thorough documentation, and expeditious care.
Topics: Cauda Equina Syndrome; Decompression, Surgical; Disease Management; Epidural Abscess; Hematoma, Epidural, Spinal; Humans; Intervertebral Disc Displacement; Lumbar Vertebrae; Magnetic Resonance Imaging; Myelography; Neurologic Examination; Spinal Injuries; Spinal Neoplasms; Spinal Stenosis; Spondylolisthesis; Tomography, X-Ray Computed
PubMed: 34473966
DOI: 10.1016/j.amjmed.2021.07.021 -
Neuroimaging Clinics of North America Nov 2019Spontaneous intracranial hypotension (SIH) is a clinical syndrome that is increasingly recognized as an important and treatable secondary cause of headaches. Insight... (Review)
Review
Spontaneous intracranial hypotension (SIH) is a clinical syndrome that is increasingly recognized as an important and treatable secondary cause of headaches. Insight into the condition has evolved significantly over the past decade, resulting in a greater understanding of the underlying pathophysiology, development of new diagnostic imaging tools, and a broadening array of targeted treatment options. This article reviews the clinical presentation and pathogenesis of SIH, discusses the important role of imaging in diagnosis, and describes how imaging guides treatment.
Topics: Bed Rest; Blood Patch, Epidural; Caffeine; Fluid Therapy; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography; Tomography, X-Ray Computed
PubMed: 31677732
DOI: 10.1016/j.nic.2019.07.006 -
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 -
Continuum (Minneapolis, Minn.) Jun 2021Spontaneous intracranial hypotension is a disorder caused by spinal CSF leakage. This article reviews the clinical presentation, diagnosis, and treatment of spontaneous... (Review)
Review
PURPOSE OF REVIEW
Spontaneous intracranial hypotension is a disorder caused by spinal CSF leakage. This article reviews the clinical presentation, diagnosis, and treatment of spontaneous intracranial hypotension.
RECENT FINDINGS
The hallmark symptom of spontaneous intracranial hypotension is acute orthostatic headache; however, clinical presentations can be heterogeneous. New evidence shows that lumbar puncture is not always necessary or sufficient to establish the diagnosis. Some patients may have normal opening pressure, which suggests that insufficiency of CSF volume (hypovolemia) rather than CSF pressure might be the underlying mechanism. Several neuroimaging modalities can aid in diagnosis and localization of the CSF leakage, including brain MRI, spinal MRI, CT myelography, digital subtraction myelography, and radionuclide cisternography. Complications, such as subdural hematoma, can lead to a change in the headache pattern and potentially life-threatening consequences. Conservative treatments, such as fluid supplementation, can provide temporary relief; however, epidural blood patches, especially targeted ones, are more effective and definitive. For patients with refractory spontaneous intracranial hypotension, surgical repair of spinal CSF leakages should be considered.
SUMMARY
Brain and spinal MRIs are important for the diagnosis and treatment of patients with spontaneous intracranial hypotension. Early treatment with epidural blood patches may be considered to shorten the disease duration and minimize the potential risk of complications.
Topics: Cerebrospinal Fluid Leak; Headache; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography
PubMed: 34048402
DOI: 10.1212/CON.0000000000000979 -
Current Pain and Headache Reports Jan 2021To provide an update on recent developments in the understanding, diagnosis, and treatment of CSF-venous fistula (CVF). (Review)
Review
PURPOSE OF REVIEW
To provide an update on recent developments in the understanding, diagnosis, and treatment of CSF-venous fistula (CVF).
RECENT FINDING
CVF is a recently recognized cause of spontaneous intracranial hypotension (SIH), an important secondary headache, in which an aberrant connection is formed between the spinal subarachnoid space and an adjacent spinal epidural vein permitting unregulated loss of CSF into the circulatory system. CVFs often occur without a concurrent epidural fluid collection; therefore, CVF should be considered as a potential etiology for patients with SIH symptomatology but without an identifiable CSF leak. Imaging plays a critical role in the detection and localization of CVFs, with a number of imaging techniques and provocative maneuvers described in the literature to facilitate their localization for targeted and definitive treatment. Increasing awareness and improving the localization of CVFs can allow for improved outcomes in the SIH patient population. Future prospective studies are needed to determine the diagnostic performance of currently available imaging techniques as well as their ability to inform workup and guide treatment decisions.
Topics: Cerebrospinal Fluid Leak; Fistula; Humans; Intracranial Hypotension; Magnetic Resonance Imaging; Myelography; Subarachnoid Space; Veins
PubMed: 33475890
DOI: 10.1007/s11916-020-00921-4