Paracentral Acute Middle Maculopathy Associated with Severe Anti-Mog (Myelin Oligodendrocyte Glycoprotein)-Positive Optic Neuritis.

Retinal complications in patients with inflammatory optic neuritis (ON) are generally related to post-infectious neuroretinitis and are considered uncommon in autoimmune/demyelinating ON, whether isolated or caused by multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD). More recently, however, cases with retinal complications have been reported in subjects positive for myelin oligodendrocyte glycoprotein (MOG) antibodies. We report a 53-year-old woman presenting with severe bilateral ON associated with a focal area of paracentral acute middle maculopathy (PAMM) in one eye. Visual loss recovered remarkably after high-dose intravenous corticosteroid treatment and plasmapheresis, but the PAMM lesion remained visible on both optical coherence tomography and angiography as an ischaemic lesion affecting the middle layers of the retina. The report emphasises the possible occurrence of retinal vascular complications in MOG-related optic neuritis, an important addition to the diagnosis of, and possible differentiation from, MS-related or NMOSD-related ON.

Comparison of Visual Evoked Potentials in Patients Affected by Optic Neuritis From Multiple Sclerosis or Neuromyelitis Optica Spectrum Disorder.

PURPOSE: To compare the visual evoked potentials (VEPs) of optic neuritis (ON) patients with multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and controls. To evaluate correlations between VEP and optical coherence tomography (OCT), contrast sensitivity (CS), and automated perimetry. METHODS: Fifty-five eyes with ON from 29 patients (MS = 14 and NMOSD = 15) and 57 eyes from 29 controls were evaluated using VEP, automated perimetry, CS, and optical coherence tomography. Three groups were analyzed: 1) MS eyes with history of ON (ON-MS), 2) NMOSD eyes with ON (ON-NMOSD), and 3) healthy controls. Groups were compared and associations between the parameters were tested. RESULTS: Compared to controls, ON-MS eyes showed significantly delayed N75 and P100 latencies when using a medium-sized stimulus (30'), and delayed P100 latency when using a large stimulus (1.5°), but similar amplitudes. Compared to controls, ON-NMOSD eyes showed significantly lower N75/P100 amplitudes (both stimulus sizes) and P100/N135 amplitudes (with the 30' stimulus), but latencies did not differ, except for a delayed P100 latency with the 30' stimulus. When comparing the 2 ON groups using the 1.5° stimulus, there was significant delay in P100 latency in ON-MS eyes and a reduction in N75/P100 amplitude in ON-NMOSD eyes. Peripapillary retinal nerve fiber layer, macular inner retinal layers, and CS measurements were significantly smaller in ON patients than in controls. A strong correlation was found between VEP parameters and inner retinal layer thickness in ON-NMOSD eyes. CONCLUSIONS: ON-MS eyes had normal amplitude and delayed VEP latency, whereas ON-NMOSD eyes displayed reduced amplitude and preserved latency when elicited by checkerboard stimulus with large 1.5° checks. Under such conditions, VEP may help distinguish resolved MS-related ON from resolved NMOSD-related ON.

Characterization of pain syndromes in patients with neuromyelitis optica.

BACKGROUND: Pain is common and refractory in spinal cord injury (SCI). Currently, most studies evaluated pain in male-predominant traumatic-SCI. Also, concomitant secondary pain syndromes and its temporal evolution were seldom reported. METHODS: We aimed to prospectively describe the main and secondary pain and its associated factors in inflammatory-SCI evaluating neuromyelitis optica (NMO) patients. In-remission NMO patients underwent neurological, imaging and autoantibody evaluations. Questionnaires detailing main and secondary pains, functional state, mood, catastrophizing, quality of life (QoL) and "non-motor symptoms" were used at two time points. RESULTS: Pain was present in 53 (73.6%) of the 72 patients included. At-level neuropathic pain was the most common main pain syndrome, affecting 32 subjects (60.4% of those with pain). Over 70% (n = 38) of this cohort reported two pain syndromes. Those without pain were significantly younger (26.1 ± 12.7 y.o. in those without pain and 40.1 ± 12.5, 37.2 ± 11.4 y.o. in those whose main pain was neuropathic and non-neuropathic, respectively, p = .001), and no differences in the inflammatory status were observed between groups. On follow-up, one-fifth (n = 11) had a different main pain syndrome from the first visit. Pain impacted QoL as much as disability and motor strength. CONCLUSION: Pain is a prevalent and disabling non-motor symptom in NMO-SCI. Most patients experience more than one pain syndrome which can change in time even in the absence of clinical relapse. Age of the inflammatory-SCI was a major determinant of pain. Acknowledging temporal changes and multiplicity of pain syndromes in NMO-SCI may give insights into more precise designs of clinical trials and general management of pain in SCI. SIGNIFICANCE: In this longitudinal study with NMO-related SCI, pain affected almost three-quarters of patients with NMO. Over 70% have more than one pain syndrome and at-level neuropathic pain is the most common type of pain syndrome. Patients without pain were significantly younger but had the same burden of inflammatory lesions than those with pain. During follow-up, up to one fifth of patients presented with changes in the main pain syndromes, which can occur even in the absence of clinical activity of the inflammatory disease. In this cohort, Pain affected quality of life as much as disability or motor strength.

Migraine in 746 patients with multiple sclerosis.

Migraine adds to the burden of patients suffering from multiple sclerosis (MS). The ID-migraine is a useful tool for screening migraine, and the Migraine Disability Assessment questionnaire can evaluate disease burden. The aim of the present study was to assess the presence and burden of migraine in patients with MS. METHODS Patients diagnosed with MS attending specialized MS units were invited to answer an online survey if they also experienced headache. RESULTS The study included 746 complete responses from patients with MS and headache. There were 625 women and 121 men, and 69% of all the patients were aged between 20 and 40 years. Migraine was identified in 404 patients (54.1%) and a moderate-to-high burden of disease was observed in 68.3% of the patients. CONCLUSION Migraine is a frequent and disabling type of primary headache reported by patients with MS.

Outer Retinal Dysfunction on Multifocal Electroretinography May Help Differentiating Multiple Sclerosis From Neuromyelitis Optica Spectrum Disorder.

Purpose: To evaluate the intermediate and outer retina of patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) using OCT and multifocal electroretinography (mf-ERG). Methods: Patients with MS (n = 30), NMOSD (n = 30), and healthy controls (n = 29) underwent visual field (VF), OCT, and mf-ERG testing. The eyes were distributed into 5 groups: MS with or without history of ON (MS+ON, MS-ON), NMOSD with or without ON (NMOSD+ON, NMOSD-ON), and controls. The thickness of the macular retinal nerve fiber layer (mRNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer, outer plexiform layer, outer nuclear layer, and photoreceptor layer was measured. mf-ERG P1 and N1 responses were registered and grouped in 3 sets of rings. The groups were compared using GEE models, and effect size (ES) calculated. Results: Compared to controls, GCL and IPL thickness was significantly smaller in MS+ON (both p < 0.01), MS-ON (p < 0.01 and p = 0.015, respectively), NMOSD+ON (both p < 0.01) and NMOSD-ON (p = 0.03 and p = 0.018, respectively). ES was >0.80. mRNFL was smaller in three of the above groups (p < 0.01, p < 0.001, and p = 0.028; ES > 0.80) but not in MS-ON eyes (p = 0.18). No significant difference was observed for the remaining layers. Compared to controls, P1 and N1 peak times were shorter in MS (p-values in the range 0.049-0.002, ES < 0.50; and 0.049-0.010; ES < 0.50, respectively) but not in NMOSD. These abnormalities were strongly correlated with intermediate and outer retinal layer thickness. Conclusions: mf-ERG data suggest outer retinal abnormalities in MS, but not in NMOSD. Our results may help understand how the two conditions differ regarding retinal damage.

Migraine in 746 patients with multiple sclerosis / Enxaqueca em 746 pacientes com esclerose múltipla

ABSTRACT Migraine adds to the burden of patients suffering from multiple sclerosis (MS). The ID-migraine is a useful tool for screening migraine, and the Migraine Disability Assessment questionnaire can evaluate disease burden. The aim of the present study was to assess the presence and burden of migraine in patients with MS. Methods: Patients diagnosed with MS attending specialized MS units were invited to answer an online survey if they also experienced headache. Results: The study included 746 complete responses from patients with MS and headache. There were 625 women and 121 men, and 69% of all the patients were aged between 20 and 40 years. Migraine was identified in 404 patients (54.1%) and a moderate-to-high burden of disease was observed in 68.3% of the patients. Conclusion: Migraine is a frequent and disabling type of primary headache reported by patients with MS.

RESUMO Enxaqueca piora o sofrimento do paciente que tem esclerose múltipla (EM). ID-migraine é uma ferramenta útil para seleção de pacientes com enxaqueca e Migraine Disability Assessment (MIDAS) é um questionário que avalia o impacto da doença. O objetivo do presente estudo foi avaliar a presença e impacto de enxaqueca em pacientes com EM. Métodos: Pacientes diagnosticados com EM e tratados em clínicas especializadas foram convidados a responder um questionário online se também apresentassem cefaleia. Resultados: O estudo incluiu 746 participantes com cefaleia e EM que preencheram completamente as respostas. Foram 625 mulheres e 121 homens, sendo 69% dos pacientes com idade entre 20 e 40 anos. Enxaqueca foi identificada em 404 pacientes (54,1%) e moderado a grave impacto da doença foi observado em 68,3% dos casos. Conclusão: Enxaqueca é uma cefaleia primária frequente e incapacitante relatada por pacientes com EM.

No correlation was observed between vitamin D levels and disability of patients with multiple sclerosis between latitudes 18° and 30° South.

Objective: Vitamin D has taken center stage in research and treatment of multiple sclerosis (MS). The objective of the present study was to assess the serum vitamin D levels of a large population of patients with MS and controls living in a restricted tropical area. Methods: Data from 535 patients with MS and 350 control subjects were obtained from 14 cities around the Tropic of Capricorn. Results: The mean serum 25-OH vitamin D level was 26.07 ± 10.27 ng/mL for the control subjects, and 28.03 ± 12.19 ng/mL for patients with MS. No correlation was observed between vitamin D levels and the disability of patients over the disease duration. Conclusion: At least for the region around the Tropic of Capricorn, serum levels of vitamin D typically are within the range of 20 to 30 ng/mL for controls and patients with MS.

No correlation was observed between vitamin D levels and disability of patients with multiple sclerosis between latitudes 18° and 30° South / Não foi observada correlação entre os níveis séricos de vitamina D e a incapacidade em pacientes com esclerose múltipla entre as latitudes Sul 18° e 30°

ABSTRACT Objective: Vitamin D has taken center stage in research and treatment of multiple sclerosis (MS). The objective of the present study was to assess the serum vitamin D levels of a large population of patients with MS and controls living in a restricted tropical area. Methods: Data from 535 patients with MS and 350 control subjects were obtained from 14 cities around the Tropic of Capricorn. Results: The mean serum 25-OH vitamin D level was 26.07 ± 10.27 ng/mL for the control subjects, and 28.03 ± 12.19 ng/mL for patients with MS. No correlation was observed between vitamin D levels and the disability of patients over the disease duration. Conclusion: At least for the region around the Tropic of Capricorn, serum levels of vitamin D typically are within the range of 20 to 30 ng/mL for controls and patients with MS.

RESUMO Objetivo: Vitamina D assumiu um papel central na pesquisa e tratamento da esclerose múltipla (EM). O objetivo deste estudo foi avaliar os níveis séricos de vitamina D de pacientes com EM e controles que residem em uma área tropical. Métodos: Foram analisados dados de 535 pacientes com EM e 350 indivíduos controle em 14 cidades próximas ao Trópico de Capricórnio. Resultados: O valor médio da determinação de 25-OH vitamina D foi 26,07 ± 10,27 ng/mL para controles e 28,03 ± 12,19 ng/mL para pacientes com EM. Não houve correlação entre os níveis de vitamina D e o grau de incapacidade ao longo da duração da doença. Conclusão: Pelo menos na região que cerca o Trópico de Capricórnio, os níveis séricos de vitamina D estão entre valores de 20 a 30 ng/mL tanto para controles quanto para pacientes com EM.

Voiding dysfunction in patients with neuromyelitis optica spectrum disorders.

AIMS: We assessed the lower urinary tract symptoms (LUTS) and urodynamic findings in patients with neuromyelitis optica spectrum disorders (NMO-SD), a recently defined neurological disease. METHODS: We prospectively evaluated seven men and 23 women (mean age 41.1 ± 13.5 years) with an established diagnosis of NMO-SD who were invited to participate irrespective of the presence of LUTS. Neurological evaluation was assessed with the Expanded Disability Status Scale (EDSS) and LUTS were evaluated with the Overactive Bladder questionnaire (OAB-V8) and the International Prostate Symptom Score (I-PSS). All patients underwent videourodynamics, transabdominal urinary tract sonography, urine culture, and serum creatinine levels. RESULTS: The mean time of disease duration was 33.8 ± 30.8 months. Neurological evaluation showed a mean EDSS score of 5.3 ± 1.8. The most frequent videourodynamic findings were detrusor-sphincter dyssynergia (DSD) and detrusor overactivity (DO) in 11 (36.6%) patients, DSD without DO in seven (23.3%) and DO without DSD in six (20.0%) patients. Voiding dysfunction assessed by I-PSS and OAB-V8 increased with the degree of neurological impairment (P = 0.018; r = 0.42 and P = 0.006; r = 0.48 respectively). Patients with DSD had higher I-PSS (18.5 ± 11.4 vs 7.0 ± 9.2; P = 0.029) and OAB-V8 scores (22.8 ± 15.8vs 9.1 ± 7.8; P = 0.008), and worse neurological impairment (mean EDSS 5.9 ± 1.8 vs 4.5 ± 1.5; P = 0.027). CONCLUSIONS: Most patients with NMO-SD have LUTS and voiding dysfunction, with DSD and DO as the main urodynamic findings. The severity of the neurological disease is a predictive factor for the occurrence of voiding dysfunction and detrusor-sphincter dyssynergia.

Quantification of retinal neural loss in patients with neuromyelitis optica and multiple sclerosis with or without optic neuritis using Fourier-domain optical coherence tomography.

PURPOSE: We compared retinal nerve fiber layer (RNFL) and macular thickness measurements in patients with multiple sclerosis (MS) and neuromyelitis optica (NMO) with or without a history of optic neuritis, and in controls using Fourier-domain (FD) optical coherence tomography (OCT). METHODS: Patients with MS (n = 60), NMO (n = 33), longitudinal extensive transverse myelitis (LETM, n = 28) and healthy controls (n = 41) underwent ophthalmic examination, including automated perimetry, and FD-OCT RNFL and macular thickness measurements. Five groups of eyes were compared: MS with or without previous optic neuritis, NMO, LETM, and controls. Correlation between OCT and visual field (VF) findings was investigated. RESULTS: With regard to most parameters, RNFL and macular thickness measurements were significantly smaller in eyes of each group of patients compared to controls. MS eyes with optic neuritis did not differ significantly from MS eyes without optic neuritis, but measurements were smaller in NMO eyes than in all other groups. RNFL (but not macular thickness) measurements were significantly smaller in LETM eyes than in controls. While OCT abnormalities were correlated significantly with VF loss in NMO/LETM and MS, the correlation was much stronger in the former. CONCLUSIONS: Although FD-OCT RNFL and macular thickness measurements can reveal subclinical or optic neuritis-related abnormalities in NMO-spectrum and MS patients, abnormalities are predominant in the macula of MS patients and in RFNL measurements in NMO patients. The correlation between OCT and VF abnormalities was stronger in NMO than in MS, suggesting the two conditions differ regarding structural and functional damage. (ClinicalTrials.gov number, NCT01024985.).

Optical coherence tomography evaluation of retinal nerve fiber layer in longitudinally extensive transverse myelitis.

OBJECTIVE: To compare optical coherence tomography (OCT) measurements on the retinal nerve fiber layer (RNFL) of healthy controls and patients with longitudinally extensive transverse myelitis (LETM) without previous optic neuritis. METHOD: Twenty-six eyes from 26 patients with LETM and 26 control eyes were subjected to automated perimetry and OCT for comparison of RNFL measurements. RESULTS: The mean deviation values from perimetry were significantly lower in patients with LETM than in controls (p<0.0001). RNFL measurements in the nasal quadrant and in the 3-o'clock segment were significantly smaller in LETM eyes than in controls. (p=0.04 and p=0.006, respectively). No significantly differences in other RNFL measurements were found. CONCLUSION: Patients with LETM may present localized RNFL loss, particularly on the nasal side of the optic disc, associated with slight visual field defects, even in the absence of previous episodes of optic neuritis. These findings emphasize the fact that patients with LETM may experience attacks of subclinical optic nerve damage.

Optical coherence tomography evaluation of retinal nerve fiber layer in longitudinally extensive transverse myelitis / Avaliação da camada de fibras nervosas da retina na mielite transversal longitudinalmente extensa usando tomografia de coerência óptica

OBJECTIVE: To compare optical coherence tomography (OCT) measurements on the retinal nerve fiber layer (RNFL) of healthy controls and patients with longitudinally extensive transverse myelitis (LETM) without previous optic neuritis. METHOD: Twenty-six eyes from 26 patients with LETM and 26 control eyes were subjected to automated perimetry and OCT for comparison of RNFL measurements. RESULTS: The mean deviation values from perimetry were significantly lower in patients with LETM than in controls (p<0.0001). RNFL measurements in the nasal quadrant and in the 3-o'clock segment were significantly smaller in LETM eyes than in controls. (p=0.04 and p=0.006, respectively). No significantly differences in other RNFL measurements were found. CONCLUSION: Patients with LETM may present localized RNFL loss, particularly on the nasal side of the optic disc, associated with slight visual field defects, even in the absence of previous episodes of optic neuritis. These findings emphasize the fact that patients with LETM may experience attacks of subclinical optic nerve damage.

OBJETIVO: Comparar as medidas da camada de fibras nervosas da retina (CFNR) usando a tomografia de coerência óptica (TCO) em indivíduos normais e pacientes com mielite transversal longitudinalmente extensa (MTLE) sem episódio prévio de neurite óptica. MÉTODO: Vinte e seis olhos de 26 pacientes com MTLE e 26 olhos normais foram submetidos à campimetria computadorizada e TCO para comparação das medidas da CFNR. RESULTADOS: Valores do parâmetro desvio médio da campimetria computadorizada foram significativamente menores nos pacientes com MTLE do que nos controles (p<0,001). Medidas da CFNR no quadrante nasal e no segmento 3 horas foram significativamente menores nos olhos dos pacientes com MTLE do que nos olhos normais (p=0,04 e p=0,006, respectivamente). Não foi encontrada diferença significante nas outras medidas da CFNR avaliadas. CONCLUSÃO: Pacientes com MTLE podem apresentar perda localizada da CFNR, particularmente na região nasal do disco óptico, associada a defeitos discretos de campo visual, mesmo na ausência de episódio prévio de neurite óptica. Estes achados sugerem que pacientes com MTLE podem apresentar acometimento subclínico do nervo óptico.