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Advances in Pediatrics Aug 2023Guillain-Barre syndrome (GBS) is an immune-mediated disease of the peripheral nerves and cause of acute flaccid paralysis in children around the world. The most common... (Review)
Review
Guillain-Barre syndrome (GBS) is an immune-mediated disease of the peripheral nerves and cause of acute flaccid paralysis in children around the world. The most common type of GBS in North America targets myelin and leads to demyelinating neuropathy. Often there is a history of infection in the weeks preceding motor symptoms. GBS has been associated with different infections, including COVID. Children usually recover motor function, but autonomic instability and respiratory compromise can occur necessitating close observation and potentially intensive care unit admission.
Topics: Humans; Child; Adolescent; Guillain-Barre Syndrome; COVID-19; Myelitis
PubMed: 37422300
DOI: 10.1016/j.yapd.2023.04.001 -
Autoimmunity Reviews Dec 2023Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing neuroinflammatory autoimmune astrocytopathy, with a predilection for the optic nerves and spinal cord.... (Review)
Review
Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing neuroinflammatory autoimmune astrocytopathy, with a predilection for the optic nerves and spinal cord. Most cases are characterised by aquaporin-4-antibody positivity and have a relapsing disease course, which is associated with accrual of disability. Although the prognosis in NMOSD has improved markedly over the past few years owing to advances in diagnosis and therapeutics, it remains a severe disease. In this article, we review the evolution of our understanding of NMOSD, its pathogenesis, clinical features, disease course, treatment options and associated symptoms. We also address the gaps in knowledge and areas for future research focus.
Topics: Humans; Neuromyelitis Optica; Aquaporin 4; Prognosis; Autoantibodies
PubMed: 37852514
DOI: 10.1016/j.autrev.2023.103465 -
Brain : a Journal of Neurology Sep 2023The spectrum of MOG-IgG-associated disease (MOGAD) includes optic neuritis (ON), myelitis (MY), acute disseminated encephalomyelitis (ADEM), brainstem encephalitis,...
The spectrum of MOG-IgG-associated disease (MOGAD) includes optic neuritis (ON), myelitis (MY), acute disseminated encephalomyelitis (ADEM), brainstem encephalitis, cerebral cortical encephalitis (CE) and AQP4-IgG-negative neuromyelitis optica spectrum disorder (NMOSD). In MOGAD, MOG-IgG are usually detected in sera (MOG-IgGSERUM), but there have been some seronegative MOGAD cases with MOG-IgG in CSF (MOG-IgGCSF), and its diagnostic implications remains unclear. In this cross-sectional study, we identified patients with paired serum and CSF sent from all over Japan for testing MOG-IgG. Two investigators blinded to MOG-IgG status classified them into suspected MOGAD (ADEM, CE, NMOSD, ON, MY and Others) or not based on the current recommendations. The MOG-IgGSERUM and MOG-IgGCSF titres were assessed with serial 2-fold dilutions to determine end point titres [≥1:128 in serum and ≥1:1 (no dilution) in CSF were considered positive]. We analysed the relationship between MOG-IgGSERUM, MOG-IgGCSF and the phenotypes with multivariable regression. A total of 671 patients were tested [405 with suspected MOGAD, 99 with multiple sclerosis, 48 with AQP4-IgG-positive NMOSD and 119 with other neurological diseases (OND)] before treatment. In suspected MOGAD, 133 patients (33%) tested MOG-IgG-positive in serum and/or CSF; 94 (23%) double-positive (ADEM 36, CE 15, MY 8, NMOSD 9, ON 15 and Others 11); 17 (4.2%) serum-restricted-positive (ADEM 2, CE 0, MY 3, NMOSD 3, ON 5 and Others 4); and 22 (5.4%) CSF-restricted-positive (ADEM 3, CE 4, MY 6, NMOSD 2, ON 0 and Others 7). None of AQP4-IgG-positive NMOSD, multiple sclerosis or OND cases tested positive for MOG-IgGSERUM, but two with multiple sclerosis cases were MOG-IgGCSF-positive; the specificities of MOG-IgGSERUM and MOG-IgGCSF in suspected MOGAD were 100% [95% confidence interval (CI) 99-100%] and 99% (95% CI 97-100%), respectively. Unlike AQP4-IgG-positive NMOSD, the correlation between MOG-IgGSERUM and MOG-IgGCSF titres in MOGAD was weak. Multivariable regression analyses revealed MOG-IgGSERUM was associated with ON and ADEM, whereas MOG-IgGCSF was associated with ADEM and CE. The number needed to test for MOG-IgGCSF to diagnose one additional MOGAD case was 13.3 (14.3 for ADEM, 2 for CE, 19.5 for NMOSD, infinite for ON, 18.5 for MY and 6.1 for Others). In terms of MOG-IgGSERUM/CSF status, most cases were double-positive while including either serum-restricted (13%) or CSF-restricted (17%) cases. These statuses were independently associated with clinical phenotypes, especially in those with ON in serum and CE in CSF, suggesting pathophysiologic implications and the utility of preferential diagnostic testing. Further studies are warranted to deduce the clinical and pathological significance of compartmentalized MOG-IgG.
Topics: Humans; Aquaporin 4; Autoantibodies; Cross-Sectional Studies; Encephalitis; Immunoglobulin G; Multiple Sclerosis; Myelin-Oligodendrocyte Glycoprotein; Myelitis; Neuromyelitis Optica; Optic Neuritis
PubMed: 37061817
DOI: 10.1093/brain/awad122 -
The Medical Clinics of North America Jan 2024Acquired demyelinating syndromes (ADS) are a heterogenous group of inflammatory demyelinating conditions that include presentations of optic neuritis, transverse... (Review)
Review
Acquired demyelinating syndromes (ADS) are a heterogenous group of inflammatory demyelinating conditions that include presentations of optic neuritis, transverse myelitis, and acute demyelinating encephalomyelitis. They can be monophasic or can develop into relapsing episodes of the initial demyelinating event or evolve to include other types of demyelination. Significant progress has been made in differentiating subtypes of ADS that differ in their tendency to relapse and in which anti-inflammatory therapies are effective. Differentiating between these subtypes is important for the optimal management of these patients. Clinical features, labs (especially autoantibodies), and MRI findings can help to differentiate between the different ADS.
Topics: Humans; Magnetic Resonance Imaging; Demyelinating Diseases; Diagnosis, Differential
PubMed: 37951658
DOI: 10.1016/j.mcna.2023.05.017 -
Seminars in Ultrasound, CT, and MR Oct 2023Myelitis is an extensive group of pathologies, including inflammatory, demyelinating, and infectious disorders, sometimes mimicking tumors. This article will discuss... (Review)
Review
Myelitis is an extensive group of pathologies, including inflammatory, demyelinating, and infectious disorders, sometimes mimicking tumors. This article will discuss infectious myelitis, mainly the patterns of spinal cord involvement caused by each infectious agent and the contribution of magnetic resonance imaging as a major tool to establish the specific diagnosis.
Topics: Humans; Myelitis; Diagnosis, Differential; Magnetic Resonance Imaging; Myelitis, Transverse; Spinal Cord
PubMed: 37555684
DOI: 10.1053/j.sult.2023.03.015 -
Handbook of Clinical Neurology 2024Paraneoplastic myelopathies are a rare but important category of myelopathy. They usually present with an insidious or subacute progressive neurologic syndrome. Risk... (Review)
Review
Paraneoplastic myelopathies are a rare but important category of myelopathy. They usually present with an insidious or subacute progressive neurologic syndrome. Risk factors include tobacco use and family history of cancer. Cerebrospinal fluid analysis usually shows lymphocytic pleocytosis with elevated protein. MRI findings suggest that paraneoplastic myelopathies include longitudinally extensive T2 hyperintensities that are tract-specific and accompanied by enhancement, but spinal MRIs can also be normal. The most commonly associated neural antibodies include amphiphysin and collapsin-response-mediator-protein-5 (CRMP5/anti-CV2) antibodies with lung and breast cancers being the most frequent oncologic accompaniments. The differential diagnosis of paraneoplastic myelopathies includes nutritional deficiency myelopathy (B12, copper) as well as autoimmune/inflammatory conditions such as primary progressive multiple sclerosis or spinal cord sarcoidosis. Patients treated with immune checkpoint inhibitors for cancer may develop myelitis, that can be considered along the spectrum of paraneoplastic myelopathies. Management of paraneoplastic myelopathy includes oncologic treatment and immunotherapy. Despite these treatments, the prognosis is poor and the majority of patients eventually become wheelchair-dependent.
Topics: Humans; Spinal Cord Diseases; Autoantibodies; Autoimmune Diseases; Neoplasms
PubMed: 38494277
DOI: 10.1016/B978-0-12-823912-4.00017-7 -
Neuro-ophthalmology (Aeolus Press) 2024Tuberculosis (TB) is a global health concern and central nervous system (CNS) TB leads to high mortality and morbidity. CNS TB can manifest as tubercular meningitis,... (Review)
Review
Tuberculosis (TB) is a global health concern and central nervous system (CNS) TB leads to high mortality and morbidity. CNS TB can manifest as tubercular meningitis, tuberculoma, myelitis, and arachnoiditis. Neuro-ophthalmological involvement by TB can lead to permanent blindness, ocular nerve palsies and gaze restriction. Visual impairment is a dreaded complication of tubercular meningitis (TBM), which can result from visual pathway involvement at different levels with varying pathogenesis. Efferent pathway involvement includes cranial nerve palsies and disorders of gaze. The purpose of this review is to outline the various neuro-ophthalmological manifestations of TB along with a description of their unique pathogenesis and management. Optochiasmatic arachnoiditis and tuberculomas are the most common causes of vision loss followed by chronic papilloedema. Abducens nerve palsy is the most commonly seen ocular nerve palsy in TBM. Gaze palsies with deficits in saccades and pursuits can occur due to brainstem tuberculomas. Corticosteroids are the cornerstone in the management of paradoxical reactions, but other immunomodulators such as thalidomide and infliximab are being explored. Toxic optic neuropathy caused by ethambutol necessitates careful monitoring and immediate drug discontinuation. Cerebrospinal fluid diversion through ventriculo-peritoneal shunting may be required in patients with hydrocephalus in stage I and II of TBM to prevent visual impairment. Early diagnosis and prompt management are crucial to prevent permanent disability. Prevention strategies, public health initiatives, regular follow-up and timely intervention are essential in reducing the burden of CNS TB and its neuro-ophthalmological complications.
PubMed: 38487360
DOI: 10.1080/01658107.2023.2281435 -
MBio Aug 2023Survivors of Powassan encephalitis often have persistent neurological disease. A new mouse model replicates some elements of the human disease and demonstrates the...
Survivors of Powassan encephalitis often have persistent neurological disease. A new mouse model replicates some elements of the human disease and demonstrates the presence of viral RNA in the brain as well as myelitis more than 2 mo after the acute infection. The related tick-borne encephalitis and West Nile Neuroinvasive Disease (WNND) also have common neurological sequelae, and models for these better-studied diseases provide evidence for prolonged virus, RNA, and inflammation in some cases, in addition to damage from the acute encephalitic disease. A better understanding of the biological basis for persistent signs and symptoms after Powassan encephalitis, currently a rare disease, could benefit from further studies of the more prevalent flaviviral encephalitides.
Topics: Animals; Mice; Humans; Encephalitis Viruses, Tick-Borne; Encephalitis, Tick-Borne; Mice, Inbred C57BL; Flavivirus Infections; Nervous System Diseases
PubMed: 37338444
DOI: 10.1128/mbio.00712-23