Transverse Myelopathy Research Articles

Ixekizumab-associated transverse myelopathy in a patient with psoriatic arthritis (P14-3.017)

<h3>Objective:</h3> We present the case of a woman with acute-onset myelopathy who presented with sensory symptoms and ataxia. We discuss her diagnosis of monoclonal antibody-induced short-segment transverse myelopathy and the potential mechanisms mediating this adverse reaction. <h3>Background:</h3> Monoclonal antibodies have provided novel therapeutic avenues for several disorders in oncology, rheumatology, and neurology. These treatments, however, have been noted to carry a rapidly growing host of adverse reactions, many of which involve the nervous system. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 57-year-old right-handed woman with psoriatic arthritis on monthly ixekizumab injections (a humanized monoclonal antibody directed against interleukin 17A) was admitted for a two-day history of progressive dysesthesias, which ascended from her feet to the lower abdomen. Her neurologic examination showed patellar and ankle hyperreflexia with large-fiber modality length-dependent sensory loss and preserved small-fiber modalities. The workup was notable for normal B12, homocysteine, copper, and zinc levels. No abnormal protein bands were identified on serum protein electrophoresis. A lumbar puncture yielded a bland CSF with two white cells, glucose of 65, protein of 43.5, and 0 oligoclonal bands. Serum ANA was positive with homogeneous with a 1:320 titer, reflecting to positive dsDNA and Scl70. A broad infectious workup was negative for HSV, VZV, CMV West Nile virus, Lyme, syphilis, HIV, and HTLV-2. MRI of the neuraxis was remarkable for a short-segment T2 hyperintense lesion of the dorsal column spanning T7–9. In the context of unrevealing etiological work-up, she was diagnosed with ixekizumab-induced transverse myelopathy and the biologic drug was stopped. She continued to improve during the following year without recurring CNS symptoms. <h3>Conclusions:</h3> We report the first case of ixekizumab-induced thoracic transverse myelopathy which we stipulate may result from dysregulation of the T-helper pathways given the antagonism of IL-17A. As shown by this clinical presentation, neurologists need to be aware of the adverse reactions of monoclonal antibodies. <b>Disclosure:</b> Dr. Rosso has nothing to disclose. Dr. Milano has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Jackson kelly. Dr. Gleichgerrcht has nothing to disclose.

Myeloneuropathy with Bilateral Foot Pain after Scrub Typhus Infection: An Antibody-Proven Case Report

Scrub typhus is an acute febrile illness caused by &lt;i&gt;Orientia tsutsugamushi&lt;/i&gt;. The most common neurological symptoms are headaches presenting with meningitis, meningoencephalitis, or encephalitis; however, transverse myelopathy and polyneuropathy are rare in typhoid infections. Herein, we report a laboratory and electrodiagnostically proven case of subacute myelopathy and polyneuropathy with slow progression after scrub typhus infection. A 64-year-old man complained of truncal numbness and a burning sensation in both feet. Magnetic resonance imaging did not reveal any definite changes in the spinal cord; however, serological tests showed immunoglobulin G antibodies against O. tsutsugamushi by enzyme-linked immunosorbent assay. An electrophysiological study showed myelopathy concurrent with polyneuropathy. To the best of our knowledge, this is the first immunochemical detection of antibodies in a patient with delayed neurological manifestations after scrub typhus infection.

Author Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction.

We are thankful for the interest and comments on our case report.1 Indeed, the longitudinally transverse myelopathy and the history of optic neuropathy in the present case might lead to a clinical suspicion of neuromyelitis optic spectrum disorders (NMOSDs) or other inflammatory demyelinating diseases. Although the negative serum AQP4-IgG and MOG-IgG antibodies could not deny the diagnosis of NMO/NMOSD, the large maternally inherited pedigree of genetically confirmed optic neuropathy highly suggested a multiorgan-involved mitochondrial disease rather than a coincidence of 2 separate diseases (LHON and NMOSD).2 Zalewski et al.3 reported that a significant MRI enhancement of the anterior gray matter was not uncommon and could even be observed in 39% of spinal cord infarctions.

Surgical management of facet cysts in the thoracic spine: Radiological manifestations and results of fenestration

BackgroundFacet cysts in the thoracic spine are a rare cause of thoracic myelopathy. We aimed to investigate the clinical/radiological features and surgical results of these lesions. MethodsNine thoracic facet cysts in eight patients (seven men, one woman) were diagnosed based on magnetic resonance imaging (MRI) and computed tomography (CT) with facet arthrography findings and surgically treated. The mean patient age was 71 (59–83) years. The cysts were distributed as follows: one each at T8–9 and T9–10, two each at T1–2 and T11–12, and three at T10–11. The mean follow-up period was 1.8 (1–5) years. Clinical and radiological features were retrospectively investigated, and surgical outcomes were evaluated according to modified Japanese Orthopaedic Association (JOA) scores for thoracic myelopathy (full score: 11). ResultsNeurological examination revealed progressive thoracic transverse myelopathy in all patients with a mean disease duration of 1.2 months (2 weeks–2 months). MRI revealed a total of nine cysts across the eight patients: four in the median region and five in the paramedian portion of the spinal canal. CT revealed degeneration in all involved facet joints. All the cysts were in communication with the neighboring facet joint confirmed by CT facet arthrography. All patients underwent bilateral fenestration, and the cysts were resected with the ligamentum flavum. The mean preoperative and postoperative modified JOA scores were 4.5 and 8.8, respectively. The mean recovery rate was 67.5%. Differences in the degree of local kyphosis were 2° or less between before and after surgery. On histopathology, synovial lining cells were not noted in any case. ConclusionsDecompression surgery is recommended for treating progressive myelopathy in patients with cystic lesions. Our study suggests that thoracic facet cyst resection with satisfactory surgical outcomes would be possible through fenestration.

Clinical features and outcomes from the Singapore Sjögren's syndrome study.

To study the clinical features, treatment and outcomes of primary Sjögren's Syndrome (pSS) in a Singapore cohort from an outpatient rheumatology clinic. Computerised Physician Order entry records of patients who fulfilled the 2016 ACR-EULAR classification criteria for pSS between 1993 and 2013 were retrospectively analysed. There were 102 patients, of which 96 (94.1%) were females, and 91 (89.2%) Chinese. Mean age at diagnosis was 49.3 ± 11.8 years, mean disease duration was 9.0 ± 4.6 years. The most common manifestations were keratoconjunctivitis sicca (99.0%), xerostomia (96.1%), arthralgia/arthritis (56.9%). Exocrine glandular enlargement comprised parotidomegaly (28, 27.5%), with concurrent submandibular and lacrimal gland enlargement in one. The nervous system (15.7%) was the most commonly affected internal organ, with peripheral nervous system (peripheral neuropathy, mononeuritis multiplex) involvement more common than central. Hydroxychloroquine was most frequently used (88.2%), followed by methotrexate (7.8%) and azathioprine (6.9%). Pulsed intravenous (IV) methylprednisolone 500 mg/day for 3 days was used in 5 patients followed by oral (4) or IV cyclophosphamide (1) for cardiomyopathy and interstitial lung disease (1), and neurological involvement (4). These comprised neuromyelitis optica, transverse myelopathy, cranial neuropathy, mononeuritis multiplex and/or peripheral neuropathy alone or in combination. Intravenous immunoglobulins (2.0%) was used for sensory neuropathy and mononeuritis multiplex; rituximab (1.0%) in 1 patient for treatment of non-Hodgkin's B-cell lymphoma. There were no deaths. Musculoskeletal manifestations were common, with the nervous system (peripheral more than central) the most common internal organ involved. Lymphoma was uncommon despite up to one-third of the cohort developing glandular enlargement.

Hyperacute extensive spinal cord infarction and negative spine magnetic resonance imaging: a case report and review of the literature

Rationale:Spinal cord infarction (SCI) accounts for only 1% to 2% of all ischemic strokes and 5% to 8% of acute myelopathies. Magnetic resonance imaging (MRI) holds a role in ruling out non-ischemic etiologies, but the diagnostic accuracy of this procedure may be low in confirming the diagnosis, even when extensive cord lesions are present. Indeed, T2 changes on MRI can develop over hours to days, thus accounting for the low sensitivity in the hyperacute setting (ie, within 6 hours from symptom onset). For these reasons, SCI remains a clinical diagnosis. Despite extensive diagnostic work-up, up to 20% to 40% of SCI cases are classified as cryptogenic. Here, we describe a case of cryptogenic longitudinally extensive transverse myelopathy due to SCI, with negative MRI and diffusion-weighted imaging at 9 hours after symptom onset.Patient concerns:A 51-year-old woman presented to our Emergency Department with acute severe abdominal pain, nausea, vomiting, sudden-onset of bilateral leg weakness with diffuse sensory loss, and paresthesias on the trunk and legs.Diagnoses:On neurological examination, she showed severe paraparesis and a D6 sensory level. A 3T spinal cord MRI with gadolinium performed at 9 hours after symptom onset did not detect spinal cord alterations. Due to the persistence of a clinical picture suggestive of an acute myelopathy, a 3T MRI of the spine was repeated after 72 hours showing a hyperintense “pencil-like” signal mainly involving the grey matter from T1 to T6 on T2 sequence, mildly hypointense on T1 and with restricted diffusion.Interventions:The patient was given salicylic acid (100 mg/d), prophylactic low-molecular-weight heparin, and began neuromotor rehabilitation.Outcomes:Two months later, a follow-up neurological examination revealed a severe spastic paraparesis, no evident sensory level, and poor sphincteric control with distended bladder.Lessons:Regardless of its relatively low frequency in the general population, SCI should be suspected in every patient presenting with acute and progressive myelopathic symptoms, even in the absence of vascular risk factors. Thus, a clinical presentation consistent with a potential vascular syndrome involving the spinal cord overrides an initially negative MRI and should not delay timely and appropriate management.

CASE REPORT-NEUROMYELITIS OPTICA/ DEVIC'S DISEASE: A RARE DISEASE

Neuromyelitis Optica spectrum disorder or Devic’s disease are autoimmune diseases that manifest clinically with 6 core symptoms, which include acute optic neuritis, transverse longitudinal segment myelitis, and acute brainstem syndrome.{1}Neuromyelitis optica is characterized by attacks of optic neuritis and longitudinally extensive transverse myelitis. It is often confused with multiple sclerosis. Early discrimination between NMO and MS is important because the two diseases have different natural histories and treatment regimens. Despite the absence of a definitive therapeutic strategy for NMO syndrome, methylprednisolone pulse therapy is recommended in acute phase. Treatment strategies in relapse phases are aimed at preventing relapses, and increasing evidences show a better clinical response of immunosuppressive therapy than immunomodulating therapy. We report a case of NMSOD in a patient of age 55 , with transverse myelopathy as the initial manifestation. The patient presented with acute gait disturbance and hypoesthesia in right lower limb since 10 days . After thorough physical examination and various lab investigations and imaging, the patient received high dose methylprednisolone. The patient’s serum was positive for anti-AQP4 antibodies .During the course of treatment , patient again presented with complaint of blurred vision in the ER after 1 year. Diagnosis of NMSOD was established. After combining methylprednisolone and immunosuppressant therapy , the patient’s medical condition was stable and relapses were prevented.

Challenges in multiple sclerosis diagnosis: Misunderstanding and misapplication of the McDonald criteria

Objective: To assess comprehension and application of the McDonald criteria. Background: Studies suggest that knowledge gaps for specific core elements of the McDonald criteria may contribute to multiple sclerosis (MS) misdiagnosis. Methods: Neurology residents (NR) and multiple sclerosis specialists (MSS) in North America completed a web-based survey. Results: A total of 160 participants were included: 72 NR and 88 MSS. Syndromes incorrectly identified as typical of MS included: complete transverse myelopathy (35% NR and 15% MSS), intractable vomiting/nausea/hiccoughs (20% NR and 5% MSS), and bilateral optic neuritis/unilateral optic neuritis with poor visual recovery (17% NR and 10% MSS). Periventricular magnetic resonance imaging (MRI) lesions were correctly identified by 39% NR and 52% MSS, and juxtacortical lesions were correctly identified by 28% NR and 53% MSS. The correct definition of “periventricular” was chosen by 38% NR and 61% MSS, and that of “juxtacortical” was chosen by 19% NR and 54% MSS. Regions incorrectly identified for MRI dissemination in space fulfillment included the optic nerve (31% NR and 26% MSS) and the subcortical white matter (11% NR and 18% MSS). The majority of participants assessed previous non-specific neurological symptoms without objective evidence of a central nervous system (CNS) lesion as sufficient for clinical dissemination in time. Conclusion: The McDonald criteria are often misunderstood and misapplied. Concerted educational efforts may prevent MS misdiagnosis.

Clinical Study of Thirteen Patients with Spinal Cord Infarction

BackgroundA concept of sensory tracts in the spinal cord has been established in relation to a dorsolateral pathway which is located in the posterior part of the lateral column and conveys the deep sense. MethodsThe clinical status at onset, neurological symptoms, and magnetic resonance imaging (MRI) findings in 13 patients of spinal cord infarction were studied. ResultsThe clinical status was acute in 11 patients and subacute in 2 patients. Palsy of the extremities was noted in 11 patients. Segmental sensory disturbance was shown in all patients. One patient showed disturbance of all senses and paraplegia, which indicated transverse myelopathy. In the other 12 patients, 11 patients showed impairment of pain sense although joint position sense was preserved, excluding 1 patient whose sensory disturbance showed dysesthesia alone. In these 11 patients, soft touch and vibration senses were impaired in 7 patients. Abnormality of spinal cord MRI was detected 7 patients. The lesions were located in the cervical cord in 3 patients, cervical to thoracic cord in 1 patient, and thoracic cord in 3 patients. ConclusionsIn the 11 patients in whom pain sense was impaired and joint position sense was preserved, involvement of the anterior spinal cord artery (ASCA) was the mainstay. Impairment of vibration sense was accompanied in 7 patients in patients of ASCA infarction. It was speculated that impairment of vibration sense can occur in patients with ASCA infarction whose ischemia spread to the dorsolateral pathway in the posterior part of the lateral column.

Myelopathy associated with mixed connective tissue disease: clinical manifestation, diagnosis, treatment, and prognosis.

Mixed connective tissue disease (MCTD) is a chronic autoimmune disease, which has a broad range of clinical manifestations shared by systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis. MCTD is featured with high serum titers of anti-ribonucleoprotein antibodies and multiple system involvement. Its spinal cord involvement mainly manifests as transverse myelopathy (TM) and longitudinal extensive transverse myelopathy (LETM). Myelopathy in MCTD is extremely rare, and is usually characterized by serious neurological complications, such as paralysis or muscular paresis, sensory impairment, and smooth muscle dysfunction. Progressive clinical manifestations combined with laboratory examinations and magnetic resonance imaging examinations play important roles in the diagnosis of this disease. In order to prevent permanent neurological damage to the spinal cord, plasmapheresis and intravenous immunoglobulin can be performed in patients at the early disease stage. Early high-dose corticosteroids combined with cyclophosphamide, followed by low doses of immunosuppressors, can improve the long-term prognosis of patients. There are only nine global cases reported on MCTD associated with myelopathy at present. The death rate and disability rate of myelopathy in MCTD are extremely high. In this review, the pathomechanisms, clinical manifestations, auxiliary examination, diagnosis, differential diagnosis, treatment, and prognosis of myelopathy in MCTD were systematically elucidated.

P3.01-98 The Onset and Clinical Course of Transverse Myelopathy with Lung Cancer

P3.01-98 The Onset and Clinical Course of Transverse Myelopathy with Lung Cancer

Myelopathy in West Nile virus encephalitis: Report of a case and review of literature

Context: In West Nile virus (WNV) encephalitis, polio-like illness has been reported but there is no report on acute transverse myelopathy. Design, Setting and Participants: We report a patient with WNV myelopathy admitted in a tertiary care teaching hospital, India along with review of the literature. Findings: A 34 year-old lady presented with fever, headache, diarrhea, seizure, bulbar weakness and quadriplegia for 20 days. Her encephalopathy, bulbar and upper limb weakness improved within few days but flaccid areflexic paraplegia persisted till 6 months with a horizontal sensory level at D3. Electromyography was suggestive of anterior horn cell involvement and somatosensory evoked potential was unrecordable. MRI revealed middle cerebellar peduncle, pons and whole of spinal cord involvement. We could get 11 articles with spinal cord involvement in WNV infection in the medical literature through PubMed search. Their clinical, MRI and electro-diagnostic findings and outcome have been discussed. Conclusion/Clinical Relevance: Acute transverse myelitis may occur in WNV encephalitis and EMG may be helpful in confirming anterior horn cell involvement and predicting outcome.

Severe neurologic complications of immune checkpoint inhibitors: a single-center review.

Immune checkpoint inhibitors (ICIs) are a promising class of anticancer drugs associated with immune-related adverse events (IRAEs). In registration studies of selected cancer populations, neurologic IRAEs were rare. Post-marketing experience describing their prevalence in clinical practice continues to be reported. A retrospective cohort of patients treated at our institution with ICIs from 2005 to 2017 was identified. Patients with new neurologic ICD codes documented during or after ICI treatment were enrolled. Comprehensive medical record review identified patients with neurologic IRAEs causally linked to ICIs. This study focused on CTCAE grade 2-4 IRAEs. 526 patients were screened; 55 candidate patients were identified; 5 cases met criteria for neurologic IRAEs, an incidence of 0.95% (n = 5/526). IRAEs identified were transverse myelopathy, demyelinating sensorimotor polyneuropathy, oculomotor nerve palsy, sensory neuropathy, and posterior reversible encephalopathy syndrome. ICIs were held in three patients, rechallenged in one, and dose-reduced in one. Corticosteroids were given in three patients, and response varied from complete symptom resolution to minimal response and ultimately death. Other treatments were based on IRAE presentation, including gabapentin, antihypertensives, and IV immunoglobulin. Patients with combination therapy appeared to suffer more severe IRAEs producing more substantial long-term morbidity and mortality. In this clinical practice study, the incidence of neurologic IRAEs from ICIs was 0.95%. Although rare, neurologic IRAEs can be highly variable and severe, and patients with combination immunotherapy appeared to suffer more severe IRAEs. Neurologists play an important role in the early identification and management of IRAEs to reduce long-term morbidity and mortality.

Intrathecal Methotrexate-Induced Necrotizing Myelopathy: A Case Report and Review of Histologic Features.

Central nervous system (CNS) relapse of acute lymphoblastic leukemia (ALL) is associated with a poor prognosis. However, prophylactic measures, including intrathecal (IT) methotrexate, reduce the incidence of CNS relapse in these patients considerably. Unfortunately, IT methotrexate can cause several neurologic complications, including transverse myelopathy; ie, the development of isolated spinal cord dysfunction over hours or days following the IT infusion of methotrexate, but in the absence of a compressive lesion. Transverse myelopathy following IT methotrexate is a well-established clinical phenomenon, but the histologic features have been described only very rarely. We report the autopsy findings from a 31-year-old man with a history of T-cell ALL who received prophylactic IT methotrexate in anticipation of a bone marrow transplant. Microscopic examination showed transverse necrosis of the thoracic cord, with massive infiltration by macrophages and lymphocytes, and perivascular lymphocytic infiltrates. There was cavitary necrosis of cervical and lumbar spinal cord involving the entire gray matter and focal white matter, as well as extensive subpial vacuolar degeneration of the dorsal and lateral columns.

Etiologic spectrum and prognosis in noncompressive acute transverse myelopathies: An experience of 80 patients at a tertiary care facility.

We evaluated the spectrum of acquired demyelinating and inflammatory disorders in patients presenting with an acute transverse myelopathy. We also studied differences between an acute idiopathic transverse myelitis and myelitis resulting from other etiologies. Eighty consecutive patients with acute transverse myelopathy were included. At inclusion, clinical profile, serum and cerebrospinal fluid parameters, brain and spinal cord magnetic resonance imaging, and visual evoked potentials were obtained. All patients were given methylprednisolone therapy. Patients were followed up for 6 months. Outcome was assessed using modified Barthel index. A modified Barthel index score of ≤12 indicated a poor prognosis. Majority (n = 49; 61.25%) of patients had idiopathic acute transverse myelitis. Eleven cases had neuromyelitis optica spectrum disorders (8 had anti-aquaporin antibody positivity). Multiple sclerosis was diagnosed in 7 cases. Eight cases had infectious or parainfectious myelitis. Longitudinally extensive transverse myelitis was noted in 66 (82.5%) patients. Seventeen patients had abnormalities in the brain. Majority of patients improved following methylprednisolone therapy. On univariate analysis, delay in administering methylprednisolone therapy, poor modified Barthel index at discharge, and extensive cord involvement were associated with severe residual disability. On multivariate analysis, delayed initiation of methylprednisolone was identified as a poor prognostic factor. A variety of inflammatory, infective, demyelinating, and autoimmune disorders present with acute transverse myelopathy. Early institution of methylprednisolone reduces the disability in these patients.

A Systematic Review of Non-Traumatic Spinal Cord Injuries in Sub-Saharan Africa and a Proposed Diagnostic Algorithm for Resource-Limited Settings.

Non-traumatic myelopathy is common in Africa and there are geographic differences in etiology. Clinical management is challenging due to the broad differential diagnosis and the lack of diagnostics. The objective of this systematic review is to determine the most common etiologies of non-traumatic myelopathy in sub-Saharan Africa to inform a regionally appropriate diagnostic algorithm. We conducted a systemic review searching Medline and Embase databases using the following search terms: "Non traumatic spinal cord injury" or "myelopathy" with limitations to epidemiology or etiologies and Sub-Saharan Africa. We described the frequencies of the different etiologies and proposed a diagnostic algorithm based on the most common diagnoses. We identified 19 studies all performed at tertiary institutions; 15 were retrospective and 13 were published in the era of the HIV epidemic. Compressive bone lesions accounted for more than 48% of the cases; a majority were Pott's disease and metastatic disease. No diagnosis was identified in up to 30% of cases in most studies; in particular, definitive diagnoses of non-compressive lesions were rare and a majority were clinical diagnoses of transverse myelitis and HIV myelopathy. Age and HIV were major determinants of etiology. Compressive myelopathies represent a majority of non-traumatic myelopathies in sub-Saharan Africa, and most were due to Pott's disease. Non-compressive myelopathies have not been well defined and need further research in Africa. We recommend a standardized approach to management of non-traumatic myelopathy focused on identifying treatable conditions with tests widely available in low-resource settings.

Spinal cord involvement in primary CNS lymphoma

LETM is a common syndrome and the diagnosis of CNS lymphoma is not usually considered in the list of differentials. Primary CNS lymphoma can present as longitudinally extensive transverse myelopathy. Failure to suspect and evaluate leads to delay in diagnosis and treatment. PCNSL may be non contrast enhancing on gadolinium enhanced MRI. CSF analysis should be done preferably before starting corticosteroids as it is usual practice in treatment of transverse myelitis, as steroids may lead to transient improvement and mask the correct diagnosis. Repeated CSF examinations may be needed to clinch the diagnosis.

What is the cause of transverse myelopathy that developed suddenly? Needs accumulation of cases

What is the cause of transverse myelopathy that developed suddenly? Needs accumulation of cases

Magnetic Resonance Imaging and Clinical Features in Acute and Subacute Myelopathies.

Differential diagnosis of acute and subacute transverse myelopathy includes inflammatory, infectious, vascular, metabolic and paraneoplastic etiologies. Information on the diagnostic approach to transverse myelopathy with regard to daily clinical practice is provided. The differentiation between five lesion patterns on magnetic resonance imaging (MRI) in myelitis may be helpful: (1)longitudinal extensive transverse myelitis, (2)short segment ovoid or peripherally located, (3)"polio-like", (4)granulomatous and (5)segmental with rash. A correlation with these imaging features is supported if the clinical course and neurological symptoms are known. Although the mean interval from onset to nadir of symptoms in spinal cord infarction is 1 h, an overlap with a fulminant course of myelitis is possible, and impaired diffusion may also occur in acute inflammatory processes. As a result, laboratory testing, including aquaporin-4 antibodies and cerebrospinal fluid analysis, is crucial for the correct interpretation of imaging findings. Moreover, the discrimination of acute complete and acute partial transverse myelitis is advantageous in order to identify diverse entities, the latter often being a precursor to multiple sclerosis. Additional brain imaging is mandatory in suspected demyelinating, infectious, neoplastic and systemic autoimmune disease. Asymmetrical lesion pattern restricted to individual tracts or dorsal columns indicates subacute combined degeneration of the spinal cord and, in addition to deficiency syndromes, a paraneoplastic etiology should be considered.

Etiological, clinical, and radiological features of longitudinally extensive myelopathy in Chinese patients

Longitudinally extensive myelopathy (LEM) is a rare spinal syndrome, and was mostly assessed in western populations. In order to investigate the etiological, clinical, and radiological features of LEM in Chinese patients, we retrospectively analyzed eighty-nine (40 men and 49 women, median age 45.9±15.7years) patients with LEM hospitalized in China-Japan Friendship Hospital. LEM comprised autoimmune inflammatory myelitis (n=53), metabolic and compressive disorders (n=13), vascular diseases (n=10), neoplastic diseases (n=7), infectious diseases (n=4), and syringomyelia (n=2). Neuromyelitis optica spectrum disorders (NMOSD) was the most common cause of transverse myelopathy identified in LEM (38/89 [42.7%]) characterized by intractable vomiting and hiccups and painful tonic spasms. Subacute combined degeneration and anterior spinal artery syndrome accounted for the largest non-transverse LEM, which selectively affected the spinal dorsal and/or lateral columns and the spinal anterior region, respectively. Radicular pain was common in anterior spinal artery syndrome. Postrema (n=15, 39.5%) and cervical (n=31, 81.6%) lesions were significantly increased in NMOSD versus non-NMOSD (n=7, 13.7% and n=34, 66.7%, respectively, p<0.05]. Axial T2-weighted MRI indicated that 46 (51.7%) patients exhibited complete lesions; 43 (48.3%) patients exhibited non-transverse lesions, mainly unilateral or symmetrical tract lesions. Twenty-four (51.1%) LEM patients exhibited distinct gadolinium contrast enhancement. In this Chinese cohort, LEM was primarily attributed to NMOSD. While the etiological distribution in the non-NMOSD group was different from western populations, clinical and imaging features may facilitate a differential diagnosis.