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Journal of the Neurological Sciences Dec 2022We aimed to provide insights into transverse myelitis (TM) following COVID-19 by analyzing cases treated at tertiary care neurology centers and a systemic review of the...
INTRODUCTION
We aimed to provide insights into transverse myelitis (TM) following COVID-19 by analyzing cases treated at tertiary care neurology centers and a systemic review of the literature.
METHODS
The retrospective observational multi-center study was conducted at the four university neurology departments in Croatia, Slovenia, Serbia, and Austria. We searched for acute myelitis cases that occurred during or after COVID-19. A systemic review of the literature on COVID-19 and transverse myelitis was performed.
RESULTS
We identified 76 persons with TM associated with COVID-19, 13 from the multi-center study and 63 from the literature review. Most of the participants (55.6%) had an intermediate latency, 25.4% had short and 19% long latency from COVID-19 symptoms to TM. The clinical presentation consisted of the typical TM signs. More than half of the participants had inflammatory changes in the CSF, with rare patients having intrathecal OCB synthesis and positive serology for anti-MOG or anti-AQP4 antibodies. Persons with autonomic symptoms and CSF pleocytosis were significantly more common to have an intermediate latency of 8 to 21 days from COVID-19 to TM (p = 0.005 and p = 0.003; respectively). According to logistic regression analysis, only participants with lesions evident on spinal cord MRI compared to normal spinal cord MRI had reduced risks for poor recovery. >80% of participants were treated with a combination of corticosteroids and intravenous immunoglobulins or plasma exchange with 73% having incomplete recovery.
CONCLUSION
Our study further characterizes clinical, laboratory, and MRI features, as well as treatment of TM associated with COVID-19.
Topics: Humans; Myelitis, Transverse; Retrospective Studies; COVID-19; Magnetic Resonance Imaging; Multicenter Studies as Topic
PubMed: 36334503
DOI: 10.1016/j.jns.2022.120463 -
European Journal of Neurology Jun 2022Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics,... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND PURPOSE
Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics, treatment and prognosis of patients with myelitis attributable to neurosarcoidosis.
METHODS
We performed a retrospective cohort study and a systematic review and meta-analysis of neurosarcoidosis-associated myelitis.
RESULTS
Myelitis was identified in 41 of 153 (27%) neurosarcoidosis patients seen at our clinic from 2015 to 2020. Classification of neurosarcoidosis was definite in three (7%), probable in 29 (71%) and possible in nine patients (22%). The median (interquartile range) age at onset was 49 (41-53) years and 20 of the patients were female (49%). The presenting symptoms included muscle weakness in 31 of 41 patients (78%), sensory loss in 35 (88%) and micturition abnormalities in 30 (75%). Spinal magnetic resonance imaging showed longitudinally extensive myelitis in 27 of 36 patients (75%) and cerebrospinal fluid examination showed an elevated leukocyte count in 21 patients (81%). Initial treatment consisted of glucocorticoids in 38 of 41 patients (93%), with additional methotrexate or azathioprine in 21 of 41 patients (51%) and infliximab in 10 of 41 patients (24%). Treatment led to remission, improvement or stabilization of disease in 37 of 39 patients (95%). Despite treatment, 18 of 30 patients (60%) could not walk independently at the end of follow-up (median 36 months). A review of the literature published between 2000 and 2020 identified 215 patients with comparable clinical characteristics and results of ancillary investigations.
CONCLUSION
Sarcoidosis-associated myelitis is observed in 27% of neurosarcoidosis patients. Although treatment often led to a decrease in disease activity, residual neurological deficits leading to loss of ambulation occurred frequently.
Topics: Central Nervous System Diseases; Female; Humans; Magnetic Resonance Imaging; Male; Myelitis; Retrospective Studies; Sarcoidosis
PubMed: 35189010
DOI: 10.1111/ene.15295 -
Neurology(R) Neuroimmunology &... May 2021To define the characteristics and the outcome of myelitis associated with immune checkpoint inhibitors (ICIs).
OBJECTIVE
To define the characteristics and the outcome of myelitis associated with immune checkpoint inhibitors (ICIs).
METHODS
We performed a retrospective research in the databases of the French Pharmacovigilance Agency and the OncoNeuroTox network for patients who developed myelitis following treatment with ICIs (2011-2020). A systematic review of the literature was performed to identify similar cases.
RESULTS
We identified 7 patients who developed myelitis after treatment with ICIs (anti-PD1 [n = 6], anti-PD1 + anti-CTLA4 [n = 1]). Neurologic symptoms included paraparesis (100%), sphincter dysfunction (86%), tactile/thermic sensory disturbances (71%), and proprioceptive ataxia (43%). At the peak of symptom severity, all patients were nonambulatory. MRI typically showed longitudinally extensive lesions, with patchy contrast enhancement. CSF invariably showed inflammatory findings. Five patients (71%) had clinical and/or paraclinical evidence of concomitant cerebral, meningeal, caudal roots, and/or peripheral nerve involvement. Despite the prompt discontinuation of ICIs and administration of high-dose glucocorticoids (n = 7), most patients needed second-line immune therapies (n = 5) because of poor recovery or early relapses. At last follow-up, only 3 patients had regained an ambulatory status (43%). Literature review identified 13 previously reported cases, showing similar clinical and paraclinical features. All patients discontinued ICIs and received high-dose glucocorticoids, with the addition of other immune therapies in 8. Clinical improvement was reported for 10 patients.
CONCLUSION
Myelitis is a rare but severe complication of ICIs that shows limited response to glucocorticoids. Considering the poor functional outcome associated with longitudinally extensive myelitis, strong and protracted immune therapy combinations are probably needed upfront to improve patient outcome and prevent early relapses.
Topics: Adolescent; Adult; Aged; Female; Glucocorticoids; Humans; Immune Checkpoint Inhibitors; Immunotherapy; Magnetic Resonance Imaging; Male; Middle Aged; Myelitis; Neoplasms; Retrospective Studies
PubMed: 33637598
DOI: 10.1212/NXI.0000000000000967 -
European Journal of Neurology Nov 2022Aquaporin-4 IgG seropositive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD) might occur in association with cancer. According to diagnostic criteria, a probable... (Review)
Review
BACKGROUND
Aquaporin-4 IgG seropositive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD) might occur in association with cancer. According to diagnostic criteria, a probable paraneoplastic NMOSD can be diagnosed only in patients with isolated myelitis and adenocarcinoma or tumors expressing AQP4. The aim of this study was to explore the features of paraneoplastic NMOSD through a data-driven approach.
METHODS
A systematic literature review was performed. Patients with AQP4-IgG positivity in association with tumor in the absence of history of checkpoint inhibitors administration/central nervous system metastases were included. Demographic, clinical, and oncological data were collected. A hierarchical cluster analysis (HCA) was performed and data were compared between resulting clusters.
RESULTS
A total of 1333 records were screened; 46 studies (72 patients) fulfilled inclusion criteria. Median age was 54 (14-87) years; adenocarcinoma occurred in 41.7% of patients, and 44% of cases had multifocal index events. Cancer and NMOSD usually co-occurred. HCA classified patients in three clusters that differed in terms of isolated/multifocal attacks, optic neuritis, pediatric onset, and type of underlying tumor. Age, time from neoplasm to NMOSD onset, and tumor AQP4 staining did not differ between clusters.
CONCLUSIONS
Our data-driven approach reveals that paraneoplastic NMOSD does not present a homogeneous phenotype nor peculiar features. Accordingly, cancer screening may be useful in AQP4-IgG NMOSD regardless of age and clinical presentation.
Topics: Adenocarcinoma; Aquaporin 4; Autoantibodies; Humans; Immunoglobulin G; Neuromyelitis Optica
PubMed: 35767391
DOI: 10.1111/ene.15479 -
Child's Nervous System : ChNS :... Mar 2022Acute flaccid myelitis (AFM) is a rare disease that commonly affects young children. AFM's pathophysiology involves loss of lower motor neurons following a viral... (Review)
Review
BACKGROUND
Acute flaccid myelitis (AFM) is a rare disease that commonly affects young children. AFM's pathophysiology involves loss of lower motor neurons following a viral infection and induces acute asymmetric flaccid paralysis most commonly in the upper extremities. Nerve transfers have emerged as a treatment option for these patients with permanent motor deficits.
OBJECTIVE
To summarize the literature and report safety and efficacy outcomes following nerve transfers for recovery of shoulder abduction and external rotation, and elbow flexion and extension in pediatric patients with AFM. Recovery of at least antigravity function was defined as a successful outcome. This systematic review was performed according to the PRISMA guidelines. The PubMed, Embase and Cochrane databases were utilized.
RESULTS
Five studies comprising 44 patients (median age 2.95 years; 71% male), and 93 upper extremity nerve transfers were included. Thirty-eight patients received 65 nerve transfer procedures aiming for recovery of shoulder abduction and/or external rotation with a transfer to the axillary and/or suprascapular nerve. The recovery of shoulder abduction and external rotation was achieved in 40.7% (n = 11/27) and 60% (n = 6/10) of patients, respectively. Time from injury to surgery showed an inverse relationship with the odds for successful recovery (OR: 0.81; 95% CI: 0.64-1.02; p = 0.07); however, statistical significance was not reached. Successful recovery of elbow flexion with a transfer to the musculocutaneous was reported at a rate of 92.3% (n = 12/13). Successful re-innervation of the radial nerve with recovery of elbow extension was found in 75% (n = 6/8) of patients. No complications were reported.
CONCLUSIONS
Upper extremity nerve transfers appear to be promising and safe for AFM patients. Shoulder abduction is the most challenging upper extremity function to recover. Further studies are warranted to identify whether nerve transfers are associated with superior outcomes when performed earlier.
Topics: Brachial Plexus Neuropathies; Central Nervous System Viral Diseases; Child; Child, Preschool; Female; Humans; Male; Myelitis; Nerve Transfer; Neuromuscular Diseases; Range of Motion, Articular; Recovery of Function; Upper Extremity
PubMed: 34982205
DOI: 10.1007/s00381-021-05419-x -
PLoS Neglected Tropical Diseases Feb 2022A substantial amount of epidemiological data has been reported on Enterovirus D68 (EV-D68) infections after the 2014 outbreak. Our goal was to map the case fatality rate... (Meta-Analysis)
Meta-Analysis
A substantial amount of epidemiological data has been reported on Enterovirus D68 (EV-D68) infections after the 2014 outbreak. Our goal was to map the case fatality rate (CFR) and prevalence of current and past EV-D68 infections. We conducted a systematic review (PROSPERO, CRD42021229255) with published articles on EV-68 infections in PubMed, Embase, Web of Science and Global Index Medicus up to January 2021. We determined prevalences using a model random effect. Of the 4,329 articles retrieved from the databases, 89 studies that met the inclusion criteria were from 39 different countries with apparently healthy individuals and patients with acute respiratory infections, acute flaccid myelitis and asthma-related diseases. The CFR estimate revealed occasional deaths (7/1353) related to EV-D68 infections in patients with severe acute respiratory infections. Analyses showed that the combined prevalence of current and past EV-D68 infections was 4% (95% CI = 3.1-5.0) and 66.3% (95% CI = 40.0-88.2), respectively. The highest prevalences were in hospital outbreaks, developed countries, children under 5, after 2014, and in patients with acute flaccid myelitis and asthma-related diseases. The present study shows sporadic deaths linked to severe respiratory EV-D68 infections. The study also highlights a low prevalence of current EV-D68 infections as opposed to the existence of EV-D68 antibodies in almost all participants of the included studies. These findings therefore highlight the need to implement and/or strengthen continuous surveillance of EV-D68 infections in hospitals and in the community for the anticipation of the response to future epidemics.
Topics: Antibodies, Viral; Asthma; Central Nervous System Viral Diseases; Enterovirus D, Human; Enterovirus Infections; Humans; Myelitis; Neuromuscular Diseases; Prevalence; Respiratory Tract Infections
PubMed: 35134062
DOI: 10.1371/journal.pntd.0010073 -
Acute Flaccid Myelitis: Review of Clinical Features, Diagnosis, and Management with Nerve Transfers.Plastic and Reconstructive Surgery Jan 2023Acute flaccid myelitis (AFM) is a devastating neurologic condition in children, manifesting as acute limb weakness and/or paralysis. Despite increased awareness of AFM...
BACKGROUND
Acute flaccid myelitis (AFM) is a devastating neurologic condition in children, manifesting as acute limb weakness and/or paralysis. Despite increased awareness of AFM following initiation of U.S. surveillance in 2014, no treatment consensus exists. The purpose of this systematic review was to summarize the most current knowledge regarding AFM epidemiology, cause, clinical features, diagnosis, and supportive and operative management, including nerve transfer.
METHODS
The authors systematically reviewed the literature based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases to search the keywords ("acute flaccid myelitis"), ('acute flaccid myelitis'/exp OR 'acute flaccid myelitis'), and (Acute AND flaccid AND myelitis). Included articles reported on (1) AFM diagnosis and (2) patient-specific data regarding epidemiology, cause, clinical features, diagnostic features, or management of AFM.
RESULTS
Ninety-nine articles were included in this review. The precise cause and pathophysiologic mechanism of AFM remain undetermined, but AFM is strongly associated with nonpolio enterovirus infections. Clinical presentation typically comprises preceding viral prodrome, pleocytosis, spinal cord lesions on T2-weighted magnetic resonance imaging, and acute onset of flaccid weakness/paralysis with hyporeflexia in at least one extremity. Supportive care includes medical therapy and rehabilitation. Early studies of nerve transfer for AFM have shown favorable outcomes for patients with persistent weakness.
CONCLUSIONS
Supportive care and physical therapy are the foundation of a multidisciplinary approach to managing AFM. For patients with persistent limb weakness, nerve transfer has shown promise for improving function in distal muscle groups. Surgeons must consider potential spontaneous recovery, patient selection, donor nerve availability, recipient nerve appropriateness, and procedure timing.
Topics: Child; Humans; Nerve Transfer; Neuromuscular Diseases; Myelitis; Paralysis; Muscle Hypotonia
PubMed: 36219869
DOI: 10.1097/PRS.0000000000009788 -
Journal of Clinical Neuroscience :... Oct 2023Lhermitte's phenomenon (LP) is a transient shock-like sensation that radiates down the spine into the extremities, usually with neck flexion. The potential efficacy and... (Review)
Review
INTRODUCTION
Lhermitte's phenomenon (LP) is a transient shock-like sensation that radiates down the spine into the extremities, usually with neck flexion. The potential efficacy and tolerability of various symptomatic therapies in the management of LP have not been systematically reviewed previously.
METHOD
A systematic review was conducted using PubMed, EMBASE, and the Cochrane Library from inception to August 2022 for peer-reviewed articles describing the treatment of patients with Lhermitte's phenomenon. The review adheres to the PRISMA guidelines and was registered on PROSPERO.
RESULTS
This systematic review included sixty-six articles, which included 450 patients with LP. Treatment of the underlying cause varied by aetiology. Whilst LP is most commonly considered in the context of structural pathology of the cervical cord, medication-induced LP was a common theme in the literature. The most common cause of medication-induced LP was platinum-based chemotherapy agents such as cisplatin and oxaliplatin. In medication-induced LP, symptoms typically resolved with cessation of the causative agent. Non-pharmacological treatment options were associated with mild-moderate symptomatic improvement. The most commonly used agents to treat patients with LP were carbamazepine and gabapentin, which resulted in variable degrees of symptomatic benefit.
CONCLUSIONS
No randomised studies currently exist to support the use of symptomatic therapies to treat LP. Observational data suggest that some therapies may yield a symptomatic benefit in the management of LP. However, this systematic review identified a significant paucity of evidence in the literature, which suggests that further controlled studies are needed to investigate the optimal management of this common neurologic phenomenon.
Topics: Humans; Antineoplastic Agents, Alkylating; Benzodiazepines; Carbamazepine; Cervical Cord; Cisplatin
PubMed: 37603922
DOI: 10.1016/j.jocn.2023.08.017 -
Transverse myelitis in systemic lupus erythematosus: A case report and systematic literature review.Autoimmunity Reviews Jun 2022Acute transverse myelitis (TM) is a rare complication secondary to systemic lupus erythematosus (SLE) that can cause patients' extensive and severe neuropsychiatric... (Review)
Review
OBJECTIVE
Acute transverse myelitis (TM) is a rare complication secondary to systemic lupus erythematosus (SLE) that can cause patients' extensive and severe neuropsychiatric disorders. Due to the rarity of the onset of acute TM, there is still no standard treatment protocol. This study was to summarize the clinical features of SLE-TM through a case report and systematic review.
METHODS
We report a case of acute TM with the initial symptoms of headache and fever on admission to hospital, with lesions in medulla oblongata, cervical medulla, and thoracic medulla. Furthermore, all cases of SLE combined with acute TM from January 1975 to February 2022 were concluded and reviewed to compare the disease's current treatment strategies and prognosis.
RESULTS
Patients with SLE-TM are mainly female (97.65%), with an average age of 36.89, a TM incidence of 24.51% and a longitudinal myelitis (LM) incidence of 67.76%. In addition, 68.63% of patients present an increased albumin, and only 16.50% of patients could recover. 32.35% of patients showed positive anti-cardiolipin antibody. Moreover, the patients who could recover are generally younger than those in the improved and paraparesis groups. After classifying the statistical results twice according to magnetic resonance imaging results and prognosis respectively, the erythrocyte sedimentation rate (ESR) in LM group was significantly higher than that in the other two groups. The positive rate of anti-DNA and anti-cardiolipin antibody (ANCL) in TM group was significantly higher than that of the other groups. According to the prognostic grouping, ESR in the recovery group was significantly higher than those in the other two groups. The positive ANCL in the poor prognosis group was slightly higher than that in the other two groups.
CONCLUSION
We offer a novel insight for this rare disease and hope to bring some inspiration the basic research for SLE-TM.
Topics: Adult; Antibodies, Anticardiolipin; Female; Humans; Lupus Erythematosus, Systemic; Magnetic Resonance Imaging; Myelitis, Transverse; Prognosis
PubMed: 35452852
DOI: 10.1016/j.autrev.2022.103103 -
Critical Care (London, England) Jul 2020Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is responsible for the global spread of coronavirus disease (COVID-19). Our understanding of the impact this...
INTRODUCTION
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is responsible for the global spread of coronavirus disease (COVID-19). Our understanding of the impact this virus has on the nervous system is limited. Our review aims to inform and improve decision-making among the physicians treating COVID-19 by presenting a systematic analysis of the neurological manifestations experienced within these patients.
METHODS
Any study, released prior to May 20, 2020, that reported neurological manifestations in patients infected by SARS-CoV-2 was systematically reviewed using the PRISMA (Preferred Reporting Items for Systemic review and Meta-Analysis) statement.
RESULTS
Our systematic review included data from 37 articles: twelve retrospective studies, two prospective studies, and the rest case reports/series. The most commonly reported neurological manifestations of COVID-19 were myalgia, headache, altered sensorium, hyposmia, and hypogeusia. Uncommonly, COVID-19 can also present with central nervous system manifestations such as ischemic stroke, intracerebral hemorrhage, encephalo-myelitis, and acute myelitis, peripheral nervous manifestations such as Guillain-Barré syndrome and Bell's palsy, and skeletal muscle manifestations such as rhabdomyolysis.
CONCLUSION
While COVID-19 typically presents as a self-limiting respiratory disease, it has been reported in up to 20% of patients to progress to severe illness with multi-organ involvement. The neurological manifestations of COVID-19 are not uncommon, but our study found most resolve with treatment of the underlying infection. Although the timeliness of this review engages current challenges posed by the COVID-19 pandemic, readers must not ignore the limitations and biases intrinsic to an early investigation.
Topics: COVID-19; Coronavirus Infections; Humans; Nervous System Diseases; Pandemics; Pneumonia, Viral
PubMed: 32660520
DOI: 10.1186/s13054-020-03121-z