RESUMO
Tumefactive demyelinating lesions (TDL) are a rare occurrence among inflammatory demyelinating diseases of the central nervous system, distinguished by tumor-like lesions exceeding 2 cm in diameter. While various etiologies have been associated with TDL, only a limited number of case reports document the coexistence of acute disseminated encephalomyelitis (ADEM) and TDL. Here, we present the case of a female diagnosed with dengue fever two weeks prior, who subsequently developed left hemiparesis and encephalopathy. Both her brain magnetic resonance imaging (MRI) and clinical course align with the characteristics of tumefactive ADEM.
RESUMO
Background: Complementary and alternative medications (CAM) are common among patients with multiple sclerosis (MS) for physical and psychological support. However, there is insufficient data regarding the application of CAM in the different cultures and beliefs of each community as well as patient's status. Objective: To evaluate the prevalence and modalities of the use of CAM among patients with central nervous system idiopathic inflammatory demyelinating diseases (CNS-IIDD) in a tertiary care hospital. Methods: A cross-sectional study was conducted at Siriraj Hospital from June to December 2021 involving patients with MS, neuromyelitis optic spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), idiopathic transverse myelitis (iTM), and optic neuritis (ON) to examine the prevalence and mode of CAM use and its correlation with patient characteristics. Results: There were 107 patients. The diagnoses were MS (38), NMOSD (55), MOGAD (5), iTM (7), and ON (2). Most of the patients were female (89.7%), and 61.7% were diagnosed over 5 years. The mean Expanded Disability Status Scale was 2.63 (S.D., 2.38), and the median ambulation index was 0 (range 0-8.5). There were 68 patients (63.6%) with a history of CAM use for at least 3 months, while those with current use decreased to 62 (58.5%). Vitamins and minerals were the most commonly used, particularly vitamin D (97.1%) and calcium (47.7%). Both treatments were primarily prescribed (95.3%) rather than self-administered (24.3%). The main reasons for the use of CAM were to strengthen their health (48.6%) and relieve existing symptoms (28.0%). Conclusions: The use of CAM is common among patients with Thai CNS-IIDD. Further exploration of patient perspectives and preferences regarding CAM usage may contribute to a more comprehensive management approach for patients with CNS-IIDD.
RESUMO
Diagnosis of lymphomatosis cerebri (LC) is usually delayed because of its rarity and the need for pathological confirmation. The association of LC with humoral immunity has scarcely been reported. Herein, we present a woman with a 2-week history of dizziness and gait ataxia, followed by diplopia, altered mental status, and spasticity of all limbs. Magnetic resonance imaging (MRI) of the brain showed multifocal lesions involving bilateral subcortical white matter, deep gray structures, and brainstem. Oligoclonal bands and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies were present in cerebrospinal fluid (CSF) twice. She was initially treated with methylprednisolone but still worsening. A stereotactic brain biopsy confirmed the diagnosis of LC. This is a report on the distinctive coexistence of the rare CNS lymphoma variant and the anti-NMDAR antibody.
Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato , Neoplasias do Sistema Nervoso Central , Feminino , Humanos , Encefalite Antirreceptor de N-Metil-D-Aspartato/complicações , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/patologia , Encéfalo/patologia , Receptores de Aminoácido , Neoplasias do Sistema Nervoso Central/patologiaRESUMO
PURPOSE: To determine the aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) immunoglobulin G (IgG) serostatus and visual outcomes in patients with recurrent optic neuritis (rON) initially seeking treatment. DESIGN: Cross-sectional cohort study. PARTICIPANTS: The study identified patients by searching the Mayo Clinic computerized central diagnostic index (January 2000-March 2017). The 246 eligible patients fulfilled the following criteria: (1) initially seeking treatment for at least 2 consecutive episodes of optic neuritis (ON) and (2) serum available for testing. METHODS: Serum was tested for aquaporin-4 IgG and MOG IgG1 using an in-house validated flow cytometric assay using live HEK293 cells transfected with M1 aquaporin-4 or full-length MOG. MAIN OUTCOMES MEASURES: Aquaporin-4 IgG and MOG IgG1 serostatus, clinical characteristics, and visual outcomes. RESULTS: Among 246 patients with rON at presentation, glial autoantibodies were detected in 32% (aquaporin-4 IgG, 19%; MOG IgG1, 13%); 186 patients had rON only and 60 patients had rON with subsequent additional inflammatory demyelinating attacks (rON-plus group). The rON-only cohort comprised the following: double seronegative (idiopathic), 110 patients (59%); MOG IgG1 positive, 27 patients (15%; 4 with chronic relapsing inflammatory optic neuropathy); multiple sclerosis (MS), 25 patients (13%); and aquaporin-4 IgG positive, 24 patients (13%). The rON-plus cohort comprised the following: aquaporin-4 IgG positive, 23 patients (38%); MS, 22 patients (37%); double seronegative, 11 patients (18%); and MOG IgG1 positive, 4 patients (7%). The annualized relapse rate for the rON-only group was 1.2 for MOG IgG1-positive patients, 0.7 for double-seronegative patients, 0.6 for aquaporin-4 IgG-positive patients, and 0.4 for MS patients (P = 0.005). The median visual acuity (VA) of patients with the worst rON-only attack at nadir were hand movements in aquaporin-4 IgG-positive patients, between counting fingers and hand movements in MOG IgG1-positive patients, 20/800 in idiopathic patients, and 20/100 in MS patients (P = 0.02). The median VA at last follow-up for affected eyes of the rON-only cohort were counting fingers for aquaporin-4 IgG-positive patients, 20/40 for idiopathic patients, 20/25 for MS patients and MOG IgG1-positive patients (P = 0.006). At 5 years after ON onset, 59% of aquaporin-4 IgG-positive patients, 22% of idiopathic patients, 12% of MOG IgG1-positive patients, and 8% of MS patients were estimated to have severe visual loss. CONCLUSIONS: Glial autoantibodies (MOG IgG1 or aquaporin-4 IgG) are found in one third of all patients with rON. Aquaporin-4 IgG seropositivity predicts a worse visual outcome than MOG IgG1 seropositivity, double seronegativity, or MS diagnosis. Myelin oligodendrocyte glycoprotein IgG1 is associated with a greater relapse rate but better visual outcomes.
Assuntos
Aquaporina 4/imunologia , Autoanticorpos/sangue , Glicoproteína Mielina-Oligodendrócito/imunologia , Neurite Óptica/imunologia , Acuidade Visual , Doença Aguda , Adulto , Anticorpos Anti-Idiotípicos/sangue , Estudos Transversais , Feminino , Citometria de Fluxo , Seguimentos , Humanos , Imunoglobulina G/imunologia , Masculino , Pessoa de Meia-Idade , Neurite Óptica/sangue , Neurite Óptica/diagnóstico , Prognóstico , Recidiva , Estudos Retrospectivos , Tomografia de Coerência Óptica , Adulto JovemRESUMO
Vagoglossopharyngeal neuralgia (VGPN) is a very rare condition. VGPN with convulsive like attack is even rarer All of the cases had their head turned to the opposite side of facial pain. Hemifacial spasm occurring concurrently with VGPN has never been reported. Herein, we present the first case of VGPN that had ipsilateral hemifacial spasm and versive seizure-like movement to the same side of facial pain. We reported a 71-year-old man presenting with multiple episodes of intermittent sharp shooting pain arising on the right middle neck, followed by hemifacial spasm on right face. Then the patient became syncope while his head and gaze turned to the same side of the painful neck. Electrocardiography showed sinus arrest. Interictal Electroencephalography was normal. This patient initially responded to pregabalin for two weeks, then the symptoms became worse. Microvascular decompression and carbamazepine resulted in the complete remission of all symptoms after six months of follow-up. We could not explain the pathophysiology of unilateral versive seizure like movement.
Assuntos
Epilepsia Motora Parcial/complicações , Doenças do Nervo Glossofaríngeo/complicações , Espasmo Hemifacial/complicações , Neuralgia/complicações , Parada Sinusal Cardíaca/complicações , Síncope/complicações , Doenças do Nervo Vago/complicações , Idoso , Carbamazepina/uso terapêutico , Eletrocardiografia , Eletroencefalografia , Epilepsia Motora Parcial/diagnóstico , Epilepsia Motora Parcial/terapia , Doenças do Nervo Glossofaríngeo/diagnóstico , Doenças do Nervo Glossofaríngeo/terapia , Espasmo Hemifacial/diagnóstico , Espasmo Hemifacial/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Cirurgia de Descompressão Microvascular/métodos , Neuralgia/diagnóstico , Neuralgia/terapia , Parada Sinusal Cardíaca/diagnóstico , Parada Sinusal Cardíaca/terapia , Síncope/diagnóstico , Síncope/terapia , Doenças do Nervo Vago/diagnóstico , Doenças do Nervo Vago/terapiaAssuntos
Doenças Desmielinizantes/epidemiologia , Doenças Desmielinizantes/fisiopatologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Adolescente , Adulto , Idade de Início , Idoso , Aquaporina 4/sangue , Criança , Pré-Escolar , Doenças Desmielinizantes/imunologia , Etnicidade , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Fenótipo , Recidiva , Estudos Soroepidemiológicos , Sri Lanka/epidemiologia , Adulto JovemAssuntos
Autoanticorpos/imunologia , Proteína Glial Fibrilar Ácida/imunologia , Imunoglobulina G/imunologia , Mielite/fisiopatologia , Adolescente , Adulto , Idoso , Aquaporina 4/imunologia , Encéfalo/diagnóstico por imagem , Estudos de Casos e Controles , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mielite/diagnóstico por imagem , Mielite/imunologia , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/imunologia , Neuromielite Óptica/fisiopatologia , Medula Espinal/diagnóstico por imagem , Adulto JovemRESUMO
This study aimed to elucidate the incidence and characteristics of neurotoxicity in patients receiving methotrexate (MTX) treatment. A retrospective analysis was performed using data from the electronic cohort database spanning from January 1990 to December 2021. This review focused on patients who manifested neurotoxic symptoms post-MTX therapy, excluding patients with peripheral neuropathy. Of the 498 individuals who received MTX, 26 (5.22%) exhibited neurotoxicity. Pediatric patients (< 18 years) accounted for 18 cases (7.44%), whereas adults (> 18 years) comprised eight cases (3.13%). The median onset age was 11 years (range 4-15) in the pediatric cohort and 39.5 years (range 19-67) in the adult cohort. A predominant male predisposition was noted (21 patients, 80.77%). The majority of patients (21, 80.77%) experienced neurotoxic effects following multiple MTX administrations. Modes of MTX delivery included intrathecal (37.0%), intravenous (22.2%), and combined routes (40.7%). Clinical presentations were predominantly encephalopathy (69.2%), followed by encephalomyelopathy (15.4%), myelopathy (11.5%), and polyradiculopathy (3.8%). Fourteen patients recovered (53.85%). Risk factors were male sex, pediatric age (particularly above 10 years), and administration route (intrathecal in adults and intravenous in pediatrics). Although infrequent, MTX-related neurotoxicity has a substantial impact on patient prognosis, with potential development following even a single dose. Its radiological resemblance to diverse neuropathologies, such as cerebral infarction and subacute combined degeneration, necessitates vigilant diagnostic scrutiny.
Assuntos
Metotrexato , Síndromes Neurotóxicas , Humanos , Metotrexato/efeitos adversos , Metotrexato/administração & dosagem , Síndromes Neurotóxicas/etiologia , Síndromes Neurotóxicas/epidemiologia , Masculino , Feminino , Adulto , Estudos Retrospectivos , Criança , Adolescente , Pré-Escolar , Idoso , Pessoa de Meia-Idade , Adulto Jovem , IncidênciaRESUMO
In Thailand, resource limitations lead many multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) patients to use off-label immunosuppressants. This study assesses the efficacy and safety of rituximab (RTX) with a CD19-based reinfusion regimen among Thai MS and NMOSD patients. A retrospective review of patients at the Faculty of Medicine Siriraj Hospital from January 1994 to April 2023 was conducted. The primary outcome assessed was the change in annualized relapse rate (ARR) for patients using RTX for over a year. Secondary outcomes included changes in the Expanded Disability Status Scale (EDSS) scores, time to the first relapse after RTX initiation for patients using RTX for over a year, and an evaluation of the safety of RTX. The study encompassed 36 MS and 39 NMOSD patients. A majority of patients (91.7% of MS and 79.5% of NMOSD) experienced no relapses during a median follow-up of 30 months (Interquartile range [IQR] 20-46) and 31 months (IQR 23-41), respectively. The median ARR significantly decreased in both MS (from 0.77 [IQR 0.42-1.83] to 0 [IQR 0-0], p < 0.001) and NMOSD (from 0.92 [IQR 0.68-1.78] to 0 [IQR 0-0.17], p < 0.001) patients after switching to RTX, with no difference between those following a fixed 6-month time point regimen and a CD19-based reinfusion regimen. Median EDSS scores improved significantly at the last follow-up visit in both groups. The mean time to the first subsequent relapse was 8.3 ± 3.0 months in MS and 6.8 ± 1.7 months in NMOSD. Mild adverse drug reactions occurred in 44% of patients. RTX effectively prevents relapses in Thai MS and NMOSD patients, with no observed serious adverse drug reactions.
Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Esclerose Múltipla , Neuromielite Óptica , Humanos , Rituximab/efeitos adversos , Neuromielite Óptica/tratamento farmacológico , Fatores Imunológicos/efeitos adversos , Esclerose Múltipla/tratamento farmacológico , Recidiva Local de Neoplasia , Estudos Retrospectivos , Antígenos CD19 , RecidivaRESUMO
OBJECTIVES: To evaluate the clinical outcomes and relapse rates in neurosarcoidosis patients administered infliximab. METHODS: A systematic review was conducted using the MEDLINE, EMBASE, SCOPUS, and Cochrane Library databases. The search included studies from their inception to March 2023. We included case-series studies with at least 10 neurosarcoidosis patients undergoing any treatment type. Studies were also required to report at least one of the following outcomes: response rate, overall survival rate, or relapse rate. This study adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A random-effects model facilitated the analysis of proportional treatment outcomes. Study quality was evaluated using the modified Newcastle-Ottawa quality assessment scale, while a funnel plot helped detect any publication bias. RESULTS: Seven studies comprising 237 patients with neurosarcoidosis were included in the analysis. Of these patients, 184 (77.6%) received treatment with infliximab. The pooled proportion of patients showing clinical improvement after infliximab treatment was 0.74 (95% CI 0.64-0.84, I2 = 49.73%). Relapse rates, derived from four studies, stood at 0.38 (95% CI 0.22-0.55, I2 = 56.92%). Most studies reported successful tapering or cessation of corticosteroid dosage in patients receiving infliximab. Adverse effects were reported in 52 (29.4%) patients, of which 39 out of 54 events (72.2%) were linked to infections. INTERPRETATION: Infliximab demonstrated potential improvement in clinical outcomes for patients with refractory neurosarcoidosis and showed potential for reducing the dosage of concurrent corticosteroids. However, a degree of relapse was observed, with infections being the primary concern for adverse events.
Assuntos
Corticosteroides , Doenças do Sistema Nervoso Central , Imunossupressores , Sarcoidose , Humanos , Infliximab/efeitos adversos , Imunossupressores/uso terapêutico , RecidivaRESUMO
OBJECTIVE: To evaluate the efficacy of rituximab (RTX) in stabilizing disability progression in secondary progressive multiple sclerosis (SPMS). METHODS: A systematic review was conducted, encompassing studies from inception to April 2023, utilizing the MEDLINE and EMBASE databases. Inclusion criteria comprised studies with a minimum of 3 SPMS patients receiving intravenous RTX in at least one infusion, with a follow-up duration of at least 6 months. Primary outcome measures included changes in Expanded Disability Status Scale (EDSS) scores. Mean differences in pre- and post-RTX EDSS scores were analyzed using a random-effects model. Meta-regression examined age at RTX initiation, pre-RTX EDSS scores, disease duration, and outcome reported time as variables. Secondary outcomes assessed changes in the annualized relapse rate (ARR). RESULTS: Thirteen studies, involving 604 SPMS patients, met the inclusion criteria. Following a mean follow-up of 2 years, the mean difference in EDSS scores (ΔEDSS = EDSSpre-RTX - EDSSpost-RTX) was -0.21 (95% CI -0.51 to 0.08, p = 0.16), indicating no significant variation. Multivariable meta-regression identified significant associations between EDSS score mean difference and pre-RTX EDSS scores, disease duration at RTX initiation, and outcome reported time. However, age at RTX initiation showed no significant association. Pre- and post-RTX ARR data were available for 245 out of 604 SPMS patients across seven studies, revealing a mean difference in ARR (ΔARR = ARRpre-RTX - ARRpost-RTX) of 0.74 (95% CI 0.19-1.29, p = 0.008). INTERPRETATION: RTX demonstrates efficacy in reducing relapse frequency and exhibits potential in stabilizing disability progression over a 2-year follow-up, particularly among individuals with shorter disease duration.
RESUMO
Tumefactive demyelinating lesions (TDL), characterized by large (≥ 2 cm) demyelinating lesions mimicking tumors, are a rare manifestation of the central nervous system inflammatory demyelinating diseases (CNS-IDD). Distinguishing TDL from other brain lesions can be challenging, often necessitating biopsy or advanced diagnostics. The natural history of TDL varies among races. This study aimed to assess demographics, clinical and radiological features, laboratory findings, management, and outcomes of Thai patients with TDL. We retrospectively reviewed records of twenty-six patients with TDL from the Multiple Sclerosis and Related Disorders registry from two tertiary medical centers. Among 1102 CNS-IDD patients, 26 (2.4%) had TDL. The median age at TDLs onset was 34.5 years (range 17-75); 69.2% were female. Over 70% manifested TDL as their initial CNS-IDD presentation. Common presenting symptoms included motor deficits, sensory disturbances, and cognitive problems. About two-fifths exhibited multiple lesions, most frequently in the frontoparietal region (46.2%). Half of the patients showed an incomplete ring on post-contrast T1-weighted imaging, with peripheral diffusion-weighted imaging restriction in twenty-one patients. T2-hypointense rims were present in thirteen (56.5%) patients. Brain biopsy was performed in 12 cases (46.1%). Serum aquaporin-4 immunoglobulin was positive in 16.7% of tested (4/24) cases. Serum myelin oligodendrocyte glycoprotein immunoglobulin was negative in all thirteen patients tested. Twenty patients (76.9%) received intravenous corticosteroids for TDL attacks. After the median follow-up period of 48 months (range 6-300), 23.1% experienced CNS-IDD relapses. Median Expanded Disability Status Scale at TDL diagnosis was 4.3 (range 0.0-9.5), and improved to 3.0 (range 0.0-10.0) at the last follow-up. This study suggested that TDL were rare among Thai CNS-IDD patients, frequently presenting as a monophasic condition with a favorable outcome.
Assuntos
Doenças Desmielinizantes , Esclerose Múltipla , Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Doenças Desmielinizantes/diagnóstico , Doenças Desmielinizantes/patologia , Imunoglobulinas , Imageamento por Ressonância Magnética , Esclerose Múltipla/diagnóstico por imagem , Estudos Retrospectivos , Tailândia/epidemiologiaRESUMO
BACKGROUND: Central nervous system inflammatory demyelinating diseases (CNSIDDs) have notable interracial heterogeneity. The epidemiology of CNSIDDs in Thailand, a mainland Southeast Asian country, is unknown. OBJECTIVES: To determine the cumulative incidence, point prevalence, and disease burden of neuromyelitis optica spectrum disorder (NMOSD) and other CNSIDDs in Thailand using population-based data of Chumphon. METHODS: Searching for CNSIDD patients at a public secondary care hospital in Chumphon, the only neurology center in the province, from January 2016 to December 2021 was implemented using relevant ICD-10-CM codes. All diagnoses were individually ascertained by a retrospective chart review. Cumulative incidence, point prevalence, attack rate, mortality rate, and disability-adjusted life years (DALYs) were calculated. RESULTS: Aquaporin 4-IgG-positive NMOSD was the most prevalent CNSIDD in the Thai population at 3.08 (1.76-5.38) per 100,000 persons. The prevalence of multiple sclerosis (MS) followed at 0.77 (0.26-2.26) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) at 0.51(0.14-1.87) per 100,000 adults. In the pediatric population, the incidence of acute disseminated encephalomyelitis was 0.28 (0.08-1.02) per 100,000 persons/year. Among other idiopathic demyelinating diseases, idiopathic optic neuritis had the highest incidence at 0.58 (0.24-0.92) per 100,000 persons/year, followed by acute transverse myelitis at 0.44 (0.14-0.74). Idiopathic demyelinating brainstem syndrome was also observed at 0.04 (0.01-0.25) per 100,000 persons/year. Although most had a fair recovery, disability was worst among NMOSD patients with DALYs of 3.61 (3.00-4.36) years per 100,000 persons. Mortality rate was the highest in NMOSD as well. CONCLUSION: CNSIDDs are rare diseases in Thailand. The prevalence is comparable to that of East Asian populations. A nationwide CNSIDDs registry would better elaborate the epidemiology of these diseases.
Assuntos
Esclerose Múltipla , Neuromielite Óptica , Criança , Humanos , Neuromielite Óptica/epidemiologia , Estudos Retrospectivos , Tailândia , Glicoproteína Mielina-Oligodendrócito , Autoanticorpos , Aquaporina 4RESUMO
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) is considered a complex multifactorial disorder. Most cases are sporadic, and familial NMOSD is assumed as a rare occurrence. However, few studies reported familial aggregation of the disorder. OBJECTIVES: To report familial NMOSD cases in Thailand and conduct a systematic review of familial NMOSD. METHODS: A retrospective chart review of familial NMOSD patients at the university hospital was performed. Articles related to "genetic" and "NMOSD" were systematically searched and reviewed. We included NMOSD patients whose one or more relatives were diagnosed with the same disease or multiple sclerosis (MS). Data regarding demographics, clinical features, disease outcomes, and genetic testing were collected and analyzed using descriptive statistics. RESULTS: We identified 6 familial cases from 165 NMOSD cases (3.6%) at our hospital and gathered 77 cases from a systematic review, totaling 83 cases from 40 families. The mean (SD) age at onset was 37.2 (18.0) years. Familial NMOSD involved 1-2 generations with mainly 2 affected individuals. The most common kinship pattern was siblingship in 21 families (52.5%). Initial syndromes were mostly optic neuritis and transverse myelitis. Serum aquaporin-4 IgG was positive in 79.7% of cases. Median number of relapses was 3 (range 1-26). Median expanded disability status scale in the last visit was 2 (range 0-8). Reported human leukocyte antigens (HLA) alleles shared between familial cases were HLA-A*01 and HLA-DRB1*03. CONCLUSION: Familial clustering of NMOSD is more common than would be expected in the general population. The demographic, clinical, and outcome profiles of familial cases were not different from sporadic cases. Certain specific HLA haplotypes were shared among familial cases. Our systematic review highlighted complex genetic predisposition to NMOSD.
Assuntos
Neuromielite Óptica , Humanos , Adulto , Neuromielite Óptica/epidemiologia , Neuromielite Óptica/genética , Estudos Retrospectivos , Autoanticorpos , Recidiva Local de Neoplasia , Aquaporina 4Assuntos
Hemorragia dos Gânglios da Base/complicações , Espasmo/etiologia , Adulto , Anticonvulsivantes/uso terapêutico , Hemorragia dos Gânglios da Base/diagnóstico por imagem , Hemorragia dos Gânglios da Base/patologia , Carbamazepina/uso terapêutico , Humanos , Imageamento por Ressonância Magnética , Masculino , Espasmo/diagnóstico por imagem , Espasmo/tratamento farmacológico , Resultado do TratamentoRESUMO
BACKGROUND: Spasticity is a common and disabling problem in multiple sclerosis (MS), but its effect in other CNS inflammatory demyelinating diseases (CNSIDDs), such as neuromyelitis optica spectrum disorder (NMOSD) is not widely studied. This study aims to compare subjective and objective measurements of spasticity in NMOSD patients and determine associated factors. METHODS: A prospective cross-sectional study was performed on CNSIDD patients attending the Multiple Sclerosis and Related Disorders Clinic at Siriraj Hospital, a tertiary hospital in Thailand, from June to November 2020 was performed. MS, NMOSD, and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) patients were included. Patients' self-rated Numeric Rating Scale (NRS) for spasticity and clinician-evaluated Modified Ashworth Scale (MAS) scores on the same visit were compared and assessed for correlations. Data on characteristics of patients including demographics, number of transverse myelitis (TM) attacks, disease duration, and Expanded Disability Status Scale (EDSS) score were collected. RESULTS: Seventy-nine CNSIDD patients were included with 25 MS, 53 NMOSD, and 1 MOGAD. There was a statistically significant correlation between NRS and MAS scores (r = 0.934, p < 0.001). Spasticity was more commonly observed in NMOSD patients compared to MS (34% vs 8%, p = 0.016). Clinical characteristics strongly associated with spasticity were higher number of TM attacks (p < 0.001), severe TM attacks (p < 0.001), longitudinally extensive transverse myelitis attacks (p < 0.001), longer disease duration (p = 0.025), higher EDSS (p < 0.001), and pyramidal Functional System Scale scores (p = 0.001). CONCLUSIONS: Patients' self-reported NRS score had a good correlation with clinician-evaluated MAS score for spasticity assessment in NMOSD and CNSIDD patients overall. Number and severity of TM attacks were associated with spasticity. Spastic patients had more disability measured by EDSS.
Assuntos
Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Estudos Transversais , Humanos , Espasticidade Muscular/diagnóstico , Espasticidade Muscular/etiologia , Glicoproteína Mielina-Oligodendrócito , Neuromielite Óptica/complicações , Neuromielite Óptica/diagnóstico , Estudos ProspectivosRESUMO
BACKGROUND: An increasing number of reports on associations between neoplasms and neuromyelitis optica spectrum disorder (NMOSD) have been published over the past decade. However, types of neoplasms and temporal relationships have not been widely studied. OBJECTIVE: To report cases and determine the associations between neoplasms and NMOSD. METHOD: A retrospective chart review of possible paraneoplastic NMOSD patients at a university hospital was performed. Articles related to "neoplasm" and "NMOSD" were systematically searched and reviewed. We included aquaporin-4 (AQP4)-IgG-seropositive NMOSD patients whose onset of NMOSD and cancer diagnosis or recurrence were within 24 months of one another. Temporal relationship, types of neoplasms involved, treatments, and outcomes of both NMOSD and neoplasms were determined. The subgroup analysis was based on the AQP4 expression of neoplasm histology. RESULTS: We identified 3 cases (1.3%) from a cohort of 224 AQP4-IgG-seropositive NMOSD at our hospital and retrieved 68 cases from a systematic review, totaling 71 cases of possible paraneoplastic NMOSD. The median age at onset of NMOSD was 55 (IQR 41-64) years. Eighty percent were female. The most frequently identified types of neoplasms were lung and breast, accounting for 21.1% and 18.3%, respectively. The other tumor types were ovarian tumors and hematologic malignancy, both at 12.7%. The most commonly identified tissue histology was adenocarcinoma (52.1%). We also reported the first case of melanoma in an NMOSD patient. Twenty-eight patients (39.4%) were diagnosed with cancer before the onset of NMOSD with a median duration of 9.5 (range 1-24) months. Of those, eight patients had NMOSD after surgical removal of neoplasms, and one patient had NMOSD after radiotherapy of prostate adenocarcinoma. Twenty-three patients (32.4%) had NMOSD before cancer diagnosis by a median of 3 (range 1-24) months, and the rest were diagnosed concurrently during the same admission. Three cases were diagnosed with NMOSD around the time of tumor recurrence. Tumor tissue expressed AQP4 in 82.4%. CONCLUSION: A small proportion of AQP4-IgG-positive NMOSD is associated with malignancy. In newly diagnosed NMOSD patients without symptoms of neoplasms, screening for age- and risk-appropriate cancer should be recommended, similar to the general population. The occurrence of NMOSD in cancer patients might suggest tumor recurrence.