Characteristics of Spontaneous Spinal Cord Infarction and Proposed Diagnostic Criteria.

Importance: Spinal cord infarction (SCI) is often disabling, and the diagnosis can be challenging without an inciting event (eg, aortic surgery). Patients with a spontaneous SCI are often misdiagnosed as having transverse myelitis. Diagnostic criteria for SCI are lacking, hindering clinical care and research. Objective: To describe the characteristics of spontaneous SCI and propose diagnostic criteria. Design, Setting, and Participants: An institution-based search tool was used to identify patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1997 to December 2017 with a spontaneous SCI. Patients provided written consent to use their records for research. Participants were 18 years and older with a diagnosis of spontaneous SCI (n = 133), and controls were selected from a database of alternative myelopathy etiologies for validation of the proposed diagnostic criteria (n = 280). Main Outcomes and Measures: A descriptive analysis of SCI was performed and used to propose diagnostic criteria, and the criteria were validated. Results: Of 133 included patients with a spontaneous SCI, the median (interquartile range) age at presentation was 60 (52-69) years, and 101 (76%) had vascular risk factors. Rapid onset of severe deficits reaching nadir within 12 hours was typical (102 [77%]); some had a stuttering decline (31 [23%]). Sensory loss occurred in 126 patients (95%), selectively affecting pain/temperature in 49 (39%). Initial magnetic resonance imaging (MRI) spine results were normal in 30 patients (24%). Characteristic MRI T2-hyperintense patterns included owl eyes (82 [65%]) and pencil-like hyperintensity (50 [40%]); gadolinium enhancement (37 of 96 [39%]) was often linear and located in the anterior gray matter. Confirmatory MRI findings included diffusion-weighted imaging/apparent diffusion coefficient restriction (19 of 29 [67%]), adjacent dissection/occlusion (16 of 82 [20%]), and vertebral body infarction (11 [9%]). Cerebrospinal fluid showed mild inflammation in 7 of 89 patients (8%). Diagnostic criteria was proposed for definite, probable, and possible SCI of periprocedural and spontaneous onset. In the validation cohort (n = 280), 9 patients (3%) met criteria for possible SCI, and none met criteria for probable SCI. Conclusions and Relevance: This large series of spontaneous SCIs provides clinical, laboratory, and MRI clues to SCI diagnosis. The diagnostic criteria proposed here will aid clinicians in making the correct diagnosis and ideally improve future care for patients with SCI. The validation of these criteria supports their utility in the evaluation of acute myelopathy.

Diagnostic and therapeutic spinal interventions: Facet joint interventions.

Axial spine pain is a common condition that is due to facet joint disease in some patients. Local anesthetic blocks of the medial branches of the dorsal rami of the spinal nerves that innervate facet joints are used to identify painful facet joints. Subsequent radiofrequency neurotomy of the medial branches serving symptomatic facet joints may provide prolonged albeit impermanent pain relief. The diagnostic blocks and radiofrequency treatments are best validated in the cervical spine. Neurologists should be aware that patients with axial spine pain who are referred to a pain clinic or pain management facility are likely to be considered for diagnostic and therapeutic interventions directed at facet joints and their sensory nerve supply.

Diagnostic and therapeutic spinal interventions: Diskography.

Diskography (provocation diskography, disk stimulation) is an invasive diagnostic test performed to confirm or exclude internal disk disruption as the cause of axial spine pain. Diskography involves injecting fluid into the nucleus of the disk under manometric control; a positive response is reproduction of typical pain. Extensive but indirect literature validates diskography in the lumbar spine; it is less well-supported in the cervical or thoracic spine. Risks include rare instances of infection and neural injury; lumbar diskography may be associated with accelerated disk degeneration. Diskography has utility in establishing a diagnosis of diskogenic pain. When negative, it prevents inappropriate interventions directed at the disk; when positive, it prevents interventions directed at other axial pain generators. Its role in selecting patients for therapies directed at diskogenic pain is limited by the lack of available validated treatments.

Diagnostic and therapeutic spinal interventions: Epidural injections.

Epidural injections of local anesthetic or a corticosteroid are frequently given to diagnose and treat patients with radicular pain originating from any spinal level. The best-quality evidence supports a transforaminal approach in the lumbar spine. Many patients experience substantial benefit from a single therapeutic injection. Depending upon the benefit obtained, additional injections may be administered. Selective nerve blocks with local anesthetic alone can identify the spinal nerve mediating the patient's pain. Serious short-term risks are rare but occur; long-term risks are infrequent and can be due to systemic effects of multiple corticosteroid injections. Patients who have failed conservative therapy or are not candidates for surgical intervention can be considered for epidural steroid injections to relieve their radicular pain temporarily.

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is not always completely reversible: a case series.

We retrospectively reviewed clinical and imaging findings in 11 patients with stroke-like migraine attacks after radiation therapy (SMART) syndrome to better understand this disorder previously thought to be reversible. Six men and 5 women had complex bouts of neurologic impairment beginning, on average, 20 years after cerebral irradiation. All had characteristic, unilateral gyriform enhancement on MR imaging that developed within 2-7 days and typically resolved in 2-5 weeks. Unlike prior reports, 45% had incomplete neurologic recovery manifesting as dysphasia, cognitive impairment, or hemiparesis. The remaining 55% recovered completely over an average of 2 months. Three of 11 patients developed cortical laminar necrosis. Brain biopsies in 4 of 11 did not demonstrate a specific pathologic substrate. These additional 11 patients contribute to the understanding of variability in stroke-like migraine attacks after radiation therapy syndrome, which often but not uniformly manifests with headaches and seizures, demonstrates a typical evolution of imaging findings, and may result in permanent neurologic and imaging sequelae.

Cervical spondylotic myelopathy.

The differential of cervical spondylotic myelopathy (CSM) is broad and includes multiple conditions that can cause and mimic myelopathy. In adults older than 55 years of age, CSM is the most common cause of myelopathy. This article summarizes the pathophysiology, clinical presentation, differential diagnosis, diagnostic evaluation, and natural history of CSM. Available treatment options and their complications are reviewed.

Cauda equina syndrome associated with longstanding instrumented spinal fusion.

BACKGROUND: This study sought to describe a patient with a slowly progressive cauda equina syndrome (CES) who had imaging findings of the CES that occurs as a rare complication of ankylosing spondylitis (AS), but whose spinal fusion was due to thoracolumbosacral instrumentation, not spondyloarthropathy. METHODS: A 53-year-old woman presented with a 5-year history of slowly progressive CES affecting sensory and sphincter more than motor function many years after sequential instrumented fusions of her thoracic and then lumbosacral spine. Imaging studies showed enlargement of the caudal sac and dorsal arachnoid diverticula eroding into posterior elements of the lumbar spine similar to findings reported in patients with CES in longstanding AS. Despite extensive evaluation, no other cause for her CES was found. We postulate that our patient's CES is on the same pathophysiologic basis as that proposed for patients with longstanding AS (fibrosis of peridural tissues with increase in transmitted cerebrospinal fluid pulse pressure). Because of reports that patients with CES due to longstanding AS are helped by placement of a lumboperitoneal shunt, our patient underwent this procedure. RESULTS: Seventeen months postoperatively, the patient's symptoms and neurologic examination are unchanged, and her previous slow progression has been halted. CONCLUSIONS: This case strongly suggests that extensive surgical thoracolumbosacral fusion can, rarely and after many years, be associated with lumbosacral nerve root damage and a slowly progressive CES that is similar to that seen in patients with spinal fusion due to longstanding AS.

Adult-onset opsoclonus-myoclonus syndrome.

BACKGROUND Little is known about adult-onset opsoclonus-myoclonus syndrome (OMS) outside of individual case reports. OBJECTIVE To describe adult-onset OMS. DESIGN Review of medical records (January 1, 1990, through December 31, 2011), prospective telephone surveillance, and literature review (January 1, 1967, through December 31, 2011). SETTING Department of Neurology, Mayo Clinic, Rochester, Minnesota. PATIENTS Twenty-one Mayo Clinic patients and 116 previously reported patients with adult-onset OMS. MAIN OUTCOME MEASURES Clinical course and longitudinal outcomes. RESULTS The median age at onset of the 21 OMS patients at the Mayo Clinic was 47 years (range, 27-78 years); 11 were women. Symptoms reported at the first visit included dizziness, 14 patients; balance difficulties, 14; nausea and/or vomiting, 10; vision abnormalities, 6; tremor/tremulousness, 4; and altered speech, 2. Myoclonus distribution was extremities, 15 patients; craniocervical, 8; and trunk, 4. Cancer was detected in 3 patients (breast adenocarcinoma, 2; and small cell lung carcinoma, 1); a parainfectious cause was assumed in the remainder of the patients. Follow-up of 1 month or more was available for 19 patients (median, 43 months; range, 1-187 months). Treatment (median, 6 weeks) consisted of immunotherapy and symptomatic therapy in 16 patients, immunotherapy alone for 2, and clonazepam alone for 1. Of these 19 patients, OMS remitted in 13 and improved in 3; 3 patients died (neurologic decline, 1; cancer, 1; and myocardial infarction, 1). The cause of death was of paraneoplastic origin in 60 of 116 literature review patients, with the most common carcinomas being lung (33 patients) and breast (7); the most common antibody was antineuronal nuclear antibody type 2 (anti-Ri, 15). Other causes were idiopathic in origin, 38 patients; parainfectious, 15 (human immunodeficiency virus, 7); toxic/metabolic, 2; and other autoimmune, 1. Both patients with N -methyl-D-aspartate receptor antibody had encephalopathy. Improvements were attributed to immunotherapy alone in 22 of 28 treated patients. CONCLUSIONS Adult-onset OMS is rare. Paraneoplastic and parainfectious causes (particularly human immunodeficiency virus) should be considered. Complete remission achieved with immunotherapy is the most common outcome.

Migraine update. Diagnosis and treatment.

Migraine is a common intermittently debilitating neurovascular disorder that affects younger adults, especially women. The diagnosis is generally made based on clinical criteria, with neuroimaging used in some cases to exclude secondary causes of headache. This article reviews current understanding of the mechanisms underlying migraine and approaches to treating it.

Cervical spondylotic myelopathy.

BACKGROUND: Cervical spondylosis is part of the aging process and affects most people if they live long enough. Degenerative changes affecting the intervertebral disks, vertebrae, facet joints, and ligamentous structures encroach on the cervical spinal canal and damage the spinal cord, especially in patients with a congenitally small cervical canal. Cervical spondylotic myelopathy (CSM) is the most common cause of myelopathy in adults. REVIEW SUMMARY: The anatomy, pathophysiology, clinical presentation, differential diagnosis, diagnostic investigation, natural history, and treatment options for CSM are summarized. Patients present with signs and symptoms of cervical spinal cord dysfunction with or without cervical nerve root injury. The condition may or may not be accompanied by pain in the neck and/or upper limb. The differential diagnosis is broad. Imaging, typically with magnetic resonance imaging, is the most useful diagnostic tool. Electrophysiologic testing can help exclude alternative diagnoses. The effectiveness of conservative treatments is unproven. Surgical decompression improves neurologic function in some patients and prevents worsening in others, but is associated with risk. CONCLUSIONS: Neurologists should be familiar with this very common condition. Patients with mild signs and symptoms of CSM can be monitored. Surgical decompression from an anterior or posterior approach should be considered in patients with progressive and moderate to severe neurologic deficits.

When and how to investigate the patient with headache.

The common complaint of headache usually has a benign cause, most often a primary headache syndrome. The history and neurologic and general physical examinations usually permit a definitive diagnosis. When in doubt, diagnostic testing is indicated. Certain historical and examination findings increase the likelihood of a secondary headache disorder and the need for diagnostic testing. These include (1) recent head or neck injury; (2) a new, worse, worsening, or abrupt onset headache; (3) headache brought on by Valsalva maneuver or cough; (4) headache brought on by exertion; (5) headache associated with sexual activity; (6) pregnancy; (7) headache in the patient over the age of ~50; (8) neurologic findings and/or symptoms; (9) systemic signs and/or symptoms; (10) secondary risk factors, such as cancer or human immunodeficiency virus (HIV) infection. Less worrisome are headaches that wake the patient from sleep at night, always occur on the same side, or show a prominent effect of change in posture on the patient's pain. Diagnostic studies include neuroimaging, cerebrospinal fluid (CSF) examination, and blood tests, which are selected depending on the patient's history and findings. For most patients, the diagnostic test of choice is a magnetic resonance imaging (MRI) brain scan. Computed tomography (CT) of the brain is usually obtained in the setting of trauma or the abrupt onset of headache. CSF examination is useful in diagnosing subarachnoid bleeding, infection, and high and low CSF pressure syndromes.

Patient education in neurology.

Patient education is an important component of quality patient care and offers many potential benefits. Health care providers are responsible for giving patients the education they want and need to optimize their health and make health decisions. This review provides a definition of patient education; examines the potential benefits and effectiveness of patient education; builds a general framework for approaching patient education as it pertains to patient knowledge, behaviors, attitudes, and skills; and summarizes representative studies of patient education in selected neurologic conditions.

SMART: stroke-like migraine attacks after radiation therapy.

We describe two adults with stroke-like migraine attacks after radiation therapy (SMART syndrome), propose revised diagnostic criteria, and review the previously reported patients. 'SMART' is an acronym for a newly recognized syndrome which occurs as a delayed consequence of cerebral irradiation and consists of prolonged, unilateral, migrainous neurological symptoms with transient, dramatic cortical gadolinium enhancement of the affected cerebral hemisphere and is sometimes punctuated by generalized seizures and ipsilateral EEG slowing. Although the neurological symptoms can last for weeks, full recovery occurs. An appropriate evaluation should exclude alternative explanations.

Spine disorder case studies.

Spine and limb pain related to degenerative, wear-and-tear changes of the disks, joints, and soft tissues of the spine (spondylosis) is exceedingly common. Identical spine and limb pain can be caused by fracture, tumor, or infection. Patients who have a history of significant trauma, signs or symptoms of systemic disease, neurologic impairment, and profound pain require further evaluation, and some may require surgical intervention to decompress the spinal nerves, spinal cord, or cauda equina or to stabilize adjacent spinal segments. The discovery of red flags in the history or on examination can identify patients who should have imaging and possibly surgical intervention. Neurologic assessment is required to determine which patients could benefit from surgery. The natural history for most acute spondylotic conditions is favorable. Treatment for acute and chronic spine-related pain syndromes is available.

When and how to investigate the patient with headache.

The very common symptom of headache most often has a benign cause and can usually be diagnosed following a thorough history and neurologic and focused general physical examinations. Because the potential etiologies can threaten life and neurological function, headaches provoke understandable concern on the part of the patient and health care provider. Thoughtful testing can exclude the worrisome underlying causes. This article reviews the neurological and general symptoms and signs that should prompt diagnostic testing and the specific diagnostic studies that are recommended. The worrisome factors include recent head or neck injury; a new, worse, worsening, or abrupt-onset headache; headache brought on by exertion or Valsalva maneuver/cough; new headache in the patient over the age of 50; neurological symptoms and/or abnormal signs; systemic symptoms and/or signs; and secondary risk factors such as a history of cancer or human immunodeficiency virus infection. Less worrisome are headaches that wake the patient from sleep at night, side-locked headaches, and prominent effect of change in posture on the pain. The diagnostic investigations include neuroimaging, cerebrospinal fluid examination, and blood testing in specific situations.

Are the headache guidelines useful in therapeutic decisions for primary care physicians, general neurologists, and headache specialists?

The International Headache Society Classification of Headache Disorders has been widely accepted as the gold standard for classification of headache. Initially a research tool, this classification is now increasingly used in the daily practice of headache medicine. Accurate diagnosis is a prerequisite to planning a therapeutic approach. The three commentaries here discuss the use of this tool in the setting of primary care, general neurology, and subspecialty headache medicine. As the Section Editor, I hope these perspectives are helpful to the reader.

Migraine practice patterns among neurologists.

OBJECTIVE: To assess the attitudes, knowledge, and practice patterns of US neurologists regarding migraine management relative to the US Headache Consortium Guidelines (the Guidelines). METHODS: Two samples of 600 neurologists each were selected from the American Academy of Neurology membership database. The first group received a Migraine Attitudes, Knowledge, and Practice Patterns (MKAPP) Survey. The second group received a Clinical Vignette (CV) Survey, presenting two patient histories and correspondent questions. RESULTS: The MKAPP Survey showed that most neurologists felt that migraine was primarily a disease of the brain with a well-established neurobiological basis (69%) and an important part of their practice (60%). Most (53%) indicated that they routinely used neuroimaging in evaluating severe headache, an approach not recommended by the Guidelines. Most favored acute treatment limits, but 36% did not agree with the Guidelines that acute treatment should be limited to 2 or 3 days/week. In the CV Survey, for vignette 1, most (91%) correctly diagnosed migraine, 31% requested neuroimaging in the absence of indications, 64% appropriately recommended a triptan, and 45% recommended a preventive medication in the absence of indications. For vignette 2, 78% diagnosed migraine, 71% appropriately ordered neuroimaging, 80% appropriately recommended a preventive medication, and 38% prescribed a triptan in face of clear contraindication. CONCLUSIONS: Educational initiatives aiming to increase the awareness of the Guidelines among neurologists should highlight the full range of migraine symptoms that support the diagnosis, appropriate use of neuroimaging, indications for preventive treatments, issues of triptan cardiovascular safety, and preventing rebound headaches.

Reversible, strokelike migraine attacks in patients with previous radiation therapy.

We report 2 adults with a past history of radiation therapy to the head for malignancy (one with primary B-cell lymphoma confined to the skull and the other with multiple hemangioendotheliomas) who developed episodes consistent with migraine with and without aura. In addition to more typical migraine attacks and beginning many years after their radiation therapy, both patients have experienced infrequent, stereotyped, prolonged, reversible neurologic deficits associated with headache, occasional seizures, and striking, transient, cortical gadolinium enhancement of the posterior cerebral gyri on MRI. Interictal MRI brain scans show stable abnormalities consistent with the patients' previous radiation therapy. The neurologic deficits often progressed over a few days, sometimes lasted weeks, and completely resolved. Electroencephalograms did not show epileptiform activity. Thorough investigation showed no residual or recurrent tumor and no recognized cause for the patients' attacks. We postulate a causal relationship between the patients' remote radiation therapy and their prolonged, strokelike migraine attacks. Radiation-induced vascular changes could provoke the episodes, with or without an underlying migraine diathesis. Recognition of this syndrome can help avoid invasive testing.