Impacto de la pandemia COVID-19 en pacientes con enfermedades desmielinizantes / IMPACT OF THE COVID-19 PANDEMIC ON PATIENTS WITH DEMYELINIZING DISEASES

Introducción: A más de un año del inicio de la pandemia, el seguimiento y la atención presencial de pacientes con enfermedades desmielinizantes se ha visto modificado. Según la evidencia, pacientes con diagnóstico de esclerosis múltiple (EM), síndrome desmielinizante aislado (SDA), Síndrome Radiológico Aislado (SRA) o enfermedades del espectro de neuromielitis óptica (NMO) no parecen ser un grupo de riesgo para COVID19 por el hecho de tener la enfermedad. La presencia de ciertas condiciones puede hacerlos susceptibles de cursar infección severa. Se ha descripto una asociación de curso grave con drogas anti CD20, faltan datos sobre la respuesta a vacunas COVID19 en esta población. Objetivos: Establecer características clínico-epidemiológicas de pacientes con enfermedades desmielinizantes que han padecido COVID-19 y describir su evolución. Caracterizar población vacunada, evaluar acceso al seguimiento médico/ terapéutico durante la pandemia. Materiales y métodos: Estudio observacional descriptivo. Se revisaron las historias clínicas de 168 pacientes con EM, SDA y SRA y 33 pacientes con NMO correspondientes al Hospital de Clínicas José de San Martin. Mediante encuesta telefónica se evaluó adherencia al tratamiento, evolución clínica, infección COVID-19, vacunación y acceso durante la pandemia. Resultados: Se encontraron 49 pacientes que desarrollaron COVID-19 en el grupo de pacientes con EM, y 7 en el grupo de NMO. Del primer grupo ninguno requirió internación, mientras que en el segundo, 2 fueron hospitalizados y uno de ellos falleció. La complicación post-COVID más frecuente fue: astenia prolongada y 3 pacientes presentaron un brote de la enfermedad de base en los 3 meses posteriores. Cerca del 90% de nuestra población ya contaba con al menos 1 dosis de vacuna para SARS-CoV2. Se interrogó sobre el acceso a la consulta neurológica y casi el 70% de los pacientes otorgó máximo puntaje al acceso a consultas virtuales. Conclusión: Los pacientes con enfermedades desmielinizantes que cursaron COVID-19 no tuvieron complicaciones severas por la infección, con solamente 2 pacientes cursando un brote en los 3 meses posteriores. No observamos reacciones adversas severas post vaccinales, ni infección posterior, sólo 2 pacientes presentaron un brote en el período post aplicación. Gran cantidad de pacientes percibieron acceso fluido a sus neurólogos de manera virtual, lo que podría relacionarse con alta tasa de adherencia a sus tratamientos a pesar de la limitación a la consulta presencial.

Introduction: More than a year after the start of the pandemic, the follow-up and face-to-face care of patients with demyelinating diseases has been modified. According to the evidence, patients with a diagnosis of multiple sclerosis (MS), isolated demyelinating syndrome (ADS), Isolated Radiological Syndrome (RAS) or neuromyelitis optica (NMO) spectrum diseases do not seem to be a risk group for COVID19 due to the fact that they have the disease. The presence of certain conditions can make them susceptible to severe infection. A severe course association with anti-CD20 drugs has been described, data on the response to COVID19 vaccines in this population are lacking. Objectives: To establish clinical-epidemiological characteristics of patients with demyelinating diseases who have suffered from COVID-19 and describe their evolution. Characterize the vaccinated population, evaluate access to medical/therapeutic follow-up during the pandemic. Materials and methods: Descriptive observational study. The medical records of 168 patients with MS, ADS and ARS and 33 patients with NMO corresponding to the Hospital de Clínicas José de San Martin were reviewed. Through a telephone survey, adherence to treatment, clinical evolution, COVID-19 infection, vaccination, and access during the pandemic were evaluated. Results: 49 patients who developed COVID-19 were found in the MS patient group, and 7 in the NMO group. Of the first group, none required hospitalization, unlike in the second, 2 were hospitalized and one of them died. The most frequent post-COVID complication was: prolonged asthenia and 3 patients presented an outbreak of the underlying disease in the following 3 months. Close to 90% of our population already had at least 1 dose of SARS-CoV2 vaccine. Access to the neurological consultation was questioned and almost 70% of the patients gave the highest score to access to virtual consultations. Conclusion: Patients with demyelinating diseases who had COVID-19 did not have severe complications from the infection, with only 2 patients having an outbreak in the subsequent 3 months. We did not observe severe post-vaccinal adverse reactions, nor subsequent infection, only 2 patients presented an outbreak in the post-application period. A large number of patients perceived fluid access to their neurologists virtually, which could be related to a high rate of adherence to their treatments despite the limitation to face-to-face consultation

A case of tumor-like inflammatory demyelinating disease with progressive brain and spinal cord involvement / Um caso de doença desmielinizante inflamatória semelhante a tumor com envolvimento progressivo de cérebro e medula espinhal

CONTEXT: Tumor-like inflammatory demyelinating disease (TIDD) usually occurs in the brain and rarely occurs in the spinal cord. TIDD appears to be very similar to tumors such as gliomas on imaging, which may lead to incorrect or delayed diagnosis and treatment. CASE REPORT: Because of headache and incoherent speech, a 24-year-old Chinese male presented to our hospital with a two-week history of respiratory infections. After dexamethasone treatment, his symptoms still got worse and surgery was performed for diagnostic purposes. Histological examination revealed that the lesion was inflammatory. Further lesions appeared in the spine (T3 and T4 levels) after two months and in the right occipital lobe after three months. After intravenous immunoglobulin (IVIG) and methylprednisolone treatment, his symptoms improved. CONCLUSION: Progressive lesions may damage the brain and spinal cord, and long-term prednisolone and IVIG therapy are beneficial in TIDD patients.

CONTEXTO: A doença desmielinizante inflamatória tumoral (DDIT) geralmente ocorre no cérebro e raramente na medula espinhal. A DDIT é muito semelhante a tumores tais como gliomas em exames de imagem, o que pode conduzir a diagnóstico e tratamento tardios e incorretos. RELATO DO CASO: Por causa de dor de cabeça e discurso incoerente, um homem chinês de 24 anos de idade foi ao hospital com história de duas semanas de infecções respiratórias. Após o tratamento com dexametasona, seus sintomas ficaram ainda piores e a cirurgia foi realizada para fins de diagnóstico. O exame histológico revelou que a lesão era inflamatória. Mais lesões apareceram na coluna vertebral (níveis T3 e T4) após dois meses, e no lobo occipital direito depois de três meses. Depois de tratamento com imunoglobulina intravenosa (IGIV) e metilprednisolona, seus sintomas melhoraram. CONCLUSÃO: Lesões progressivas podem danificar o cérebro e a medula espinhal, e prednisolona a longo prazo e terapia de IGIV são benéficas em pacientes DDIT.

Síndrome de Miller Fisher: presentación de un caso clínico / Miller Fisher syndrome: review of a clinic case

Presentamos el caso una paciente con síndrome de Miller Fisher, una entidad neurológica infrecuente considerada como una variante del síndrome de Guillain-Barre. El pronóstico aún sin terapéutica parece ser favorable. Se realiza una revisión bibliográfica haciendo particular énfasis en la ubicación de la lesión, métodos paraclínicos y tratamiento actual

Insuficiencia supra-renal na infancia associada a severas alteracoes do sistema nervoso central: relato de um caso de adrenoleucodistrofia / Supra-renal failure in infancy associated with severe alterations of central nervous system: a adrenoleukodystrophy case report

E apresentado um caso de adrenoleucodistrofia (ALD), doenca metabolica hereditaria ligada ao sexo e caracterizada por alteracoes no metabolismo de acidos graxos de cadeia muito longa (Very Long-Chain Fatty Acids - VLCFA), manifestando-se clinicamente por insuficiencia adrenal e anormalidades neurologicas progressivas. Sao discutidas a forma de apresentacao da doenca e o seu diagnostico, sendo enfatizada a importancia de ter-se presente a possibilidade de ALD em criancas portadoras de doenca de Addison, porque trata-se de molestia sujeita a intervencao terapeutica, com prevencao e ate regressao dos disturbios neurologicos