[Meningitis and myelitis by hypervirulent Klebsiella pneumoniae]. / Meningitis y mielitis por Klebsiella pneumoniae hipervirulenta.

Invasive liver abscess syndrome caused by hypervirulent Klebsiella. pneumoniae is a rare disease. This type of K. pneumoniae is aggressive and invasive, despite its sensitivity profile. We report the case of a 62-year-old man with diabetes mellitus, who was admitted to our hospital with meningeal syndrome. Within 24 hours of admission, Gram negative bacilli were isolated blood and cerebrospinal fluid cultures, which were later identified as K. pneumoniae. Liver abscess was detected by computed tomography. Despite early antibiotic treatment, the patient developed back pain that prevented him from moving and right hemiparesis. Increased signal from the central region of the spinal medulla compatible with myelitis was identified by magnetic resonance, for which he received methylprednisolone 1 g/day for 5 days. The patient evolved favorably. Infections caused by hypermucoviscous K. pneumoniae are aggressive and invasive, and more common in men with a history of diabetes mellitus, as in this case. These infections require early antibiotic treatment and the search of metastatic infections.

El síndrome de absceso hepático invasivo causado por cepas hipermucoviscosas de Klebsiella pneumoniae es una enfermedad poco frecuente. Esta serovariedad de Klebsiella se caracteriza por ser agresiva e invasiva pese a su perfil de sensibilidad. Se presenta el caso de un varón de 62 años con antecedentes de diabetes mellitus, que ingresó a nuestro centro con síndrome meníngeo. A las 24 horas del ingreso se aislaron en hemocultivos y en líquido cefalorraquídeo (LCR) bacilos Gram negativos que luego fueron tipificados como Klebsiella pneumoniae. Se identificó la presencia de un absceso hepático mediante tomografía computarizada. Pese al tratamiento antibiótico instaurado de manera temprana, el paciente evolucionó con dolor dorsal que le impedía movilizarse y hemiparesia derecha. En la resonancia magnética nuclear de columna se identificó aumento de la señal de la región central de la médula espinal compatible con mielitis por lo cual recibió tratamiento con metilprednisolona 1g/día por 5 días consecutivos. El paciente evolucionó de manera favorable. Las infecciones por K. pneumoniae hipermucoviscosas son agresivas e invasoras y más frecuentes en varones con antecedentes de diabetes mellitus, como en este caso. Su control requiere de un tratamiento antibiótico temprano y búsqueda de focos a distancia.

The real-world applicability of the 2023 international myelin oligodendrocyte glycoprotein antibody-associated disease criteria in a Latin American cohort.

BACKGROUND AND PURPOSE: The diagnostic criteria for myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) were published in 2023. We aimed to determine the performance of the new criteria in Latin American (LATAM) patients compared with the 2018 criteria and explore the significance of MOG-IgG titers in diagnosis. METHODS: We retrospectively reviewed the medical records of LATAM (Argentina, Chile, Brazil, Peru, Ecuador, and Colombia) adult patients with one clinical MOGAD event and MOG-IgG positivity confirmed by cell-based assay. Both 2018 and 2023 MOGAD criteria were applied, calculating diagnostic performance indicators. RESULTS: Among 171 patients (predominantly females, mean age at first attack = 34.1 years, mean disease duration = 4.5 years), 98.2% patients met the 2018 criteria, and of those who did not fulfill diagnostic criteria (n = 3), all tested positive for MOG-IgG (one low-positive and two without reported titer). Additionally, 144 (84.2%) patients met the 2023 criteria, of whom 57 (39.5%) had MOG-IgG+ titer information (19 clearly positive and 38 low-positive), whereas 87 (60.5%) patients had no MOG-IgG titer. All 144 patients met diagnostic supporting criteria. The remaining 27 patients did not meet the 2023 MOGAD criteria due to low MOG-IgG (n = 12) or lack of titer antibody access (n = 15), associated with the absence of supporting criteria. The 2023 MOGAD criteria showed a sensitivity of 86% (95% confidence interval = 0.80-0.91) and specificity of 100% compared to the 2018 criteria. CONCLUSIONS: These findings support the diagnostic utility of the 2023 MOGAD criteria in an LATAM cohort in real-world practice, despite limited access to MOG-IgG titration.

Trans-spinal magnetic stimulation combined with kinesiotherapy as a new method for enhancing functional recovery in patients with spinal cord injury due to neuromyelitis optica: a case report.

BACKGROUND: Experimental studies have shown that repetitive trans-spinal magnetic stimulation (TsMS) decreases demyelination and enables recovery after spinal cord injury (SCI). However, the usefulness of TsMS in humans with SCI remains unclear. Therefore, the main objective of this study is to evaluate the effects of TsMS combined with kinesiotherapy on SCI symptoms. We describe a protocol treatment with TsMS and kinesiotherapy in a patient with SCI due to neuromyelitis optica (NMO)-associated transverse myelitis. CASE PRESENTATION: A 23-year-old white male with NMO spectrum disorders started symptoms in 2014 and included lumbar pain evolving into a mild loss of strength and sensitivity in both lower limbs. Five months later, the symptoms improved spontaneously, and there were no sensorimotor deficits. Two years later, in 2016, the symptoms recurred with a total loss of strength and sensitivity in both lower limbs. Initially, physiotherapy was provided in 15 sessions with goals of motor-sensory recovery and improving balance and functional mobility. Subsequently, TsMS (10 Hz, 600 pulses, 20-seconds inter-trains interval, at 90% of resting motor threshold of the paravertebral muscle) was applied at the 10th thoracic vertebral spinous process before physiotherapy in 12 sessions. Outcomes were assessed at three time points: prior to physiotherapy alone (T-1), before the first session of TsMS combined with kinesiotherapy (T0), and after 12 sessions of TsMS combined with kinesiotherapy (T1). The patient showed a 25% improvement in walking independence, a 125% improvement in balance, and an 18.8% improvement in functional mobility. The Patient Global Impression of Change Scale assessed the patient's global impression of change as 'much improved'. CONCLUSION: TsMS combined with kinesiotherapy may safely and effectively improve balance, walking independence, and functional mobility of patients with SCI due to NMO-associated transverse myelitis.

COVID-19 associated transverse myelitis: case report / Mielitis transversa asociada a COVID-19: reporte de un caso

Abstract Background: Transverse myelitis (TM) is a demyelinating inflammatory disease that presents with motor, sensory, and autonomic dysfunction, which may be acute or subacute. COVID-19-associated TM has been described in a scarce number of patients. Clinical case: A 15-year-old previously healthy male patient with respiratory disease before his neurological deterioration presented to the emergency room after developing a complete medullary syndrome located at the cervical-dorsal level, with ascending and symmetric paraparesis that rapidly progressed to paraplegia, with sensory dysfunction from the T3 level, sphincter dysfunction and sudden ventilatory deterioration that required mechanical ventilation. Magnetic resonance imaging was compatible with acute TM. Inflammatory and non-inflammatory etiologies were discarded. In addition, a positive severe acute respiratory syndrome coronavirus 2 test was obtained. Treatment included steroid pulses and plasmapheresis, with an insidious evolution. Conclusion: COVID-19 is an infrequent cause of TM and should be suspected when other etiologies have been ruled out.

Resumen Introducción: La mielitis transversa (MT) es una enfermedad inflamatoria desmielinizante que se presenta con disfunción motora, sensitiva y autonómica, de forma aguda o subaguda. La MT asociada al COVID-19 se ha escrito en un escaso número de pacientes. Caso clínico: Se presenta el caso de un masculino de 15 años previamente sano, quien cursaba con un cuadro respiratorio y que desarrollo un deterioro neurológico súbito que involucro un síndrome medular completo localizado en el nivel cérvico dorsal, con paraparesia simétrica que progreso a la paraplejia, con disfunción sensitiva desde el nivel medular de T3, disfunción de esfínteres y deterioro ventilatorio que requirió manejo avanzado de la vía aérea. Su resonancia magnética fue compatible con mielitis transversa aguda. Se descartaron causas inflamatorias y no inflamatorias de la patología. Además, se obtuvo un resultado positivo de SARS-COV-2. Se inició tratamiento con pulsos de metilprednisolona y plasmaféresis, con una evolución insidiosa. Conclusión: El COVID-19 es una causa infrecuente de MT y debe sospecharse cuando otras causas han sido descartadas.

COVID-19 associated transverse myelitis: case report.

BACKGROUND: Transverse myelitis (TM) is a demyelinating inflammatory disease that presents with motor, sensory, and autonomic dysfunction, which may be acute or subacute. COVID-19-associated TM has been described in a scarce number of patients. CLINICAL CASE: A 15-year-old previously healthy male patient with respiratory disease before his neurological deterioration presented to the emergency room after developing a complete medullary syndrome located at the cervical-dorsal level, with ascending and symmetric paraparesis that rapidly progressed to paraplegia, with sensory dysfunction from the T3 level, sphincter dysfunction and sudden ventilatory deterioration that required mechanical ventilation. Magnetic resonance imaging was compatible with acute TM. Inflammatory and non-inflammatory etiologies were discarded. In addition, a positive severe acute respiratory syndrome coronavirus 2 test was obtained. Treatment included steroid pulses and plasmapheresis, with an insidious evolution. CONCLUSION: COVID-19 is an infrequent cause of TM and should be suspected when other etiologies have been ruled out.

INTRODUCCIÓN: La mielitis transversa (MT) es una enfermedad inflamatoria desmielinizante que se presenta con disfunción motora, sensitiva y autonómica, de forma aguda o subaguda. La MT asociada al COVID-19 se ha escrito en un escaso número de pacientes. CASO CLÍNICO: Se presenta el caso de un masculino de 15 años previamente sano, quien cursaba con un cuadro respiratorio y que desarrollo un deterioro neurológico súbito que involucro un síndrome medular completo localizado en el nivel cérvico dorsal, con paraparesia simétrica que progreso a la paraplejia, con disfunción sensitiva desde el nivel medular de T3, disfunción de esfínteres y deterioro ventilatorio que requirió manejo avanzado de la vía aérea. Su resonancia magnética fue compatible con mielitis transversa aguda. Se descartaron causas inflamatorias y no inflamatorias de la patología. Además, se obtuvo un resultado positivo de SARS-COV-2. Se inició tratamiento con pulsos de metilprednisolona y plasmaféresis, con una evolución insidiosa. CONCLUSIÓN: El COVID-19 es una causa infrecuente de MT y debe sospecharse cuando otras causas han sido descartadas.

Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática / Brown-Séquard syndrome as a presentation of idiopathic transverse myelitis

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

Acute disseminated encephalomyelitis following Saint Louis encephalitis virus infection.

Saint Louis encephalitis virus (SLEV) infection is an arbovirosis associated with a broad spectrum of neurological complications. We present a case of a 55-year-old man hailing from Manaus, a city situated in the heart of the Amazon Rainforest, who exhibited symptoms of vertigo, tremors, urinary and fecal retention, compromised gait, and encephalopathy 3 weeks following SLEV infection. Neuroaxis MRI revealed diffuse, asymmetric, and poorly defined margins hyperintense lesions with peripheral and ring enhancement in subcortical white matter, as well as severe spinal cord involvement. Serology for SLEV was positive both on serum and cerebrospinal fluid. To the best of our knowledge, the present report is the first to show brain lesions along with myelitis as a post-infectious complication of SLEV infection.

Brown-Séquard syndrome as a presentation of idiopathictransverse myelitis. / Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

"Clock dial pattern", a radiologic clue to neuro-chikungunya diagnosis: a case series / "Padrão em ponteiro de relógio", uma dica radiológica para o diagnóstico de neurochikungunya: série de casos

Abstract Background Chikungunya is a mosquito-borne disease caused by the chikungunya virus (CHIKV) and can lead to neurological complications in severe cases. Objective This study examined neuroimaging patterns in chikungunya cases during two outbreaks in Brazil to identify specific patterns for diagnosis and treatment of neuro-chikungunya. Methods Eight patients with confirmed chikungunya and neurological involvement were included. Clinical examinations and MRI scans were performed, and findings were analyzed by neuroradiologists. Data on age, sex, neurological symptoms, diagnostic tests, MRI findings, and clinical outcomes were recorded. Results Patients showed different neuroimaging patterns. Six patients exhibited a "clock dial pattern" with hyperintense dotted lesions in the spinal cord periphery. One patient had thickening and enhancement of anterior nerve roots. Brain MRI revealed multiple hyperintense lesions in the white matter, particularly in the medulla oblongata, in six patients. One patient had a normal brain MRI. Conclusion The "clock dial pattern" observed in spinal cord MRI may be indicative of chikungunya-related nervous system lesions. Isolated involvement of spinal cord white matter in chikungunya can help differentiate it from other viral infections. Additionally, distinct brainstem involvement in chikungunya-associated encephalitis, particularly in the rostral region, sets it apart from other arboviral infections. Recognizing these neuroimaging patterns can contribute to early diagnosis and appropriate management of neuro-chikungunya.

Resumo Antecedentes A chikungunya é uma doença transmitida por mosquitos causada pelo vírus chikungunya (CHIKV) e pode levar a complicações neurológicas em casos graves. Objetivo Este estudo examinou padrões de neuroimagem em casos de chikungunya durante dois surtos no Brasil para identificar padrões específicos para o diagnóstico e tratamento de neurochikungunya. Métodos Oito pacientes com chikungunya confirmada e envolvimento neurológico foram incluídos. Exames clínicos e ressonâncias magnéticas (RM) foram realizados, e os achados foram analisados por neurorradiologistas. Dados sobre idade, sexo, sintomas neurológicos, testes diagnósticos, achados de RM e desfechos clínicos foram registrados. Resultados Os pacientes apresentaram diferentes padrões de neuroimagem. Seis pacientes apresentaram um "padrão de mostrador de relógio" com lesões pontilhadas hiperintensas na periferia da medula espinhal. Um paciente apresentou espessamento e realce das raízes nervosas anteriores. A RM do cérebro revelou múltiplas lesões hiperintensas na substância branca, especialmente no bulbo em seis pacientes. Um paciente apresentou uma RM cerebral normal. Conclusão O "padrão de mostrador de relógio" observado na RM da medula espinhal pode ser indicativo de lesões do sistema nervoso relacionadas à chikungunya. O envolvimento isolado da substância branca da medula espinhal na chikungunya pode ajudar a diferenciá-la de outras infecções virais. Além disso, o envolvimento distinto do tronco cerebral na encefalite associada à chikungunya, especialmente na região rostral, a distingue de outras infecções por arbovírus. O reconhecimento desses padrões de neuroimagem pode contribuir para o diagnóstico precoce e manejo adequado da neurochikungunya.

Mielitis transversa aguda asociada a Covid-19. Reporte de caso

Desde el inicio de la pandemia por COVID-19 (coronavirus disease), en el 2019, se han descrito numerosas manifestaciones clínicas de la enfermedad causada por este virus, destacando el compromiso respiratorio, hematológico, cardiovascular y neurológico. Dentro de las manifestaciones y/o complicaciones neurológicas, se encuentra la mielitis aguda transversa por COVID-19(1), cuyo diagnóstico se ha realizado principalmente clínico-imagenológico y PCR (reacción de polimerasa en cadena), o serología (+) para COVID-19, con manejos y resultados no siempre afortunados. Presentamos el caso de un paciente con mielitis aguda transversa longitudinalmente extensa en relación a COVID-19, tratada con éxito clínico con rituximab.

Since the beginning of the COVID-19 pandemic in 2019, numerous clinical manifestations of the disease caused by this virus have been described, highlighting respiratory, hematological, cardiovascular, and neurological compromise. Among the neurological manifestations and/or complications, there is acute transverse myelitis due to COVID-19(1), whose diagnosis has been made mainly clinical-imaging and PCR or serology (+) for COVID-19, with management and results not always lucky. We present the case of a patient with longitudinally extensive acute transverse myelitis in relation to COVID-19, treated with clinical success with rituximab.

Enfermedad asociada a anticuerpos contra la glicoproteína de mielina de oligodendrocitos / Myelin oligodendrocyte glycoprotein antibody disease

Introducción: La enfermedad asociada a anticuerpos contra la glicoproteína de mielina del oligodendrocito (MOGAD, por sus siglas en inglés) es una entidad clínica recientemente identificada. La frecuencia de presentación del MOGAD es desconocida, pero se considera baja con respecto a otras enfermedades inflamatorias desmielinizantes. Materiales y métodos: Revisión narrativa de la literatura. Resultados: Las manifestaciones clínicas de esta condición son heterogéneas e incluyen neuritis óptica, mielitis, desmielinización multifocal del sistema nervioso central y encefalitis cortical. Se han descrito algunos hallazgos radiológicos que aumentan la sospecha diagnóstica, como el realce perineural del nervio óptico, el signo de la H en el cordón espinal y la resolución de lesiones T2 con el tiempo. El diagnóstico se basa en la detección de inmunoglobulinas G específicas contra MOG, en el contexto clínico adecuado. El tratamiento consiste en manejo de los ataques agudos con dosis altas de corticoides y en algunos casos se deberá considerar la inmunosupresión crónica, considerar la inmunosupresión crónica en pacientes con recurrencia o con discapacidad severa residual tras el primer evento. Conclusiones: En esta revisión narrativa se resumen los aspectos clave con respecto a la fisiopatología, las manifestaciones, el diagnóstico y el tratamiento de la MOGAD.

Introduction: The disease associated with antibodies against the myelin oligodendrocyte glycoprotein (MOGAD) is a recently identified clinical entity, with unknown frequency, but is considered low compared to other demyelinating inflammatory diseases. Materials And Methods: Narrative review. Results: The clinical manifestations are heterogeneous, ranging from optic neuritis or myelitis to multi-focal CNS demyelination or cortical encephalitis. There have been described characteristic MRI features that increase the diagnostic suspicion, such as perineural optic nerve enhancement, spinal cord H-sign or T2-lesion resolution over time. The diagnosis is based on the detection of specific G- immunoglobulins against MOG, in the suggestive clinical context. Acute treatment is based on high dose steroids and maintenance treatment is generally reserved for relapsing cases or patients with severe residual disability after the first attack. Conclusions: In this narrative review, fundamental aspects of pathophysiology, clinical and radiological manifestations, diagnosis and treatment of MOGAD are discussed.

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience / Espectro clínico de doença associada com anticorpos contra a glicoproteína de oligodendrócito de mielina no Brasil: uma experiencia de centro único

Abstract Background Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder. Objective To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center. Methods We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD. Results Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI. Conclusion Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

Resumo Antecedentes A doença associada ao anticorpo da glicoproteína da mielina de oligodendrócitos (anti-MOG; MOGAD) é uma doença neurológica imunomediada com um amplo espectro de apresentações clínicas que muitas vezes é difícil de distinguir de outras doenças desmielinizantes, como a esclerose múltipla e o distúrbio do espectro da neuromielite óptica. Objetivo Descrever as características clínicas e paraclínicas da MOGAD em um centro terciário brasileiro. Métodos Revisamos retrospectivamente os prontuários dos pacientes adultos e pediátricos que testaram positivos para anticorpos anti-MOG e apresentaram um quadro clínico e radiológico compatível com MOGAD. Resultados Quarenta e um pacientes (10 crianças) foram incluídos: 56% do sexo feminino, 58% caucasianos, idade média de início da doença foi 31 anos (intervalo de 6-64), com duração média da doença de 59,6 meses (intervalo de 1-264 meses). A apresentação inicial mais frequente foi neurite óptica (68%), seguida pela encefalomielite disseminada aguda (ADEM, 12%) e mielite (10%). Um curso monofásico da doença foi observado em 49%. EDSS foi de 2,1 na última visita. A maioria dos pacientes (83%) estava sob tratamento imunossupressor contínuo. Azatioprina foi o tratamento de primeira linha em 59%. Em todos os casos de ADEM, o envolvimento do cone medular e das raízes espinhais foi observado radiologicamente na ressonância magnética. Conclusão Os pacientes brasileiros com MOGAD apresentam um espectro clínico e radiológico semelhante aos fenótipos de MOGAD relatados anteriormente. O envolvimento do cone e das raízes espinhais parece estar frequentemente presente no MOGAD-ADEM e poderia servir como característica radiológica nesta entidade.

Prevalence of neuromyelitis optica spectrum disorder in Colombia: Analysis of the official Ministry of Health administrative registry.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a rare entity with severe inflammatory demyelinating events of the central nervous system with debilitating sequelae. Its global prevalence ranges between 0.5 and 4/100,000 individuals, with variations by region and ethnicity. Latin America lacks epidemiological data on the disease, and Colombian prevalence is unknown. OBJECTIVE: Prevalence of NMOSD in Colombia was estimated between 2017 and 2021 using the official Ministry of Health administrative database (SISPRO). METHODS: This is an observational, cross-sectional retrospective study, using data between January 2017 and December 2021 in the SISPRO database using the International Classification of Disease code for NMOSD G36.0. Prevalence by gender, age and geographic distribution was estimated using official government statistics for 2019. World Health Organization (WHO) standard population was used to adjust using the direct method. RESULTS: 2,650 patients were diagnosed with NMOSD; the average age was 44.9 years with an overall unadjusted prevalence of 5.3/100,000 individuals, higher for females (7.8) than for males (2.8). No significant changes (from 5.3 to 5.4) were seen after adjusting to the WHO standard. CONCLUSION: According to this study Colombia has one of the highest prevalence rates of NMOSD in Latin America, further studies are needed to elucidate the contributing factors.

[Neuromyelitis optica spectrum disorder in a patient with human immunodeficiency virus]. / Neuromielitis óptica en paciente con virus de inmunodeficiencia humana.

Background: Neuromyelitis optica spectrum (NMO) is an autoimmune condition with preferential target at the optic nerves and spinal cord. Although HIV infection can also cause neuritis and myelitis, the entity of HIV related to NMO has recently been elucidated, however, little is known about the context of this disease. Objective: To describe the clinical characteristics, imaging, treatment, and functional prognosis in an HIV-positive patient who developed an episode of longitudinally extensive transverse myelitis (LETM) with positive anti-AQP4 antibodies. Clinic case: 36-year-old man with a history of HIV diagnosed in 2017, on antiretroviral treatment. On March 2021 he was admitted for study due to complete spinal cord syndrome, corroborating in MRI a longitudinally extensive lesion from T8-L1, with CSF with and AQP4 seropositivity, a diagnosis of NMO was integrated by Wingerchuk criteria and rituximab is started with symptomatic improvement, objectifying it with the Expanded Disability Status Scale (EDSS) from 4 to 1. Conclusion: NMO entity related to HIV is rare, this phenomenon being classically found at the time of diagnosis or after the start of treatment when the immune system is still capable of developing an exaggerated immune response, however in the case we report the debut of NMO occurred 3 years after diagnosis, contrasting with previously reported cases, so we suggest that some other mechanisms could be involved, such as altered regulation of B cells and a direct viral effect.

Introducción: la neuromielitis óptica (NMO) es una condición autoinmune con blanco preferencial a nivel de nervios ópticos y médula espinal. Recientemente se ha elucidado la entidad de VIH relacionada a NMO; no obstante, aún se conoce poco sobre el contexto de esta enfermedad. El objetivo de este trabajo es describir las características clínicas, de imagen, tratamiento y el pronóstico funcional en un paciente VIH positivo, quien desarrolló un episodio de mielitis longitudinalmente extensa (LETM) con anticuerpos anti-AQP4 positivos. Caso clínico: hombre de 36 años con antecedente de VIH diagnosticado en 2017, en tratamiento antirretroviral y antecedente de sarcoma de Kaposi. Inicia su padecimiento en marzo de 2021 con dolor en región lumbar, acompañado de debilidad y anestesia en miembros pélvicos, agregándose incontinencia vesical y distensión abdominal. A la exploración se integra síndrome medular completo con nivel en T8-T9, corroborándose en RM lesión longitudinalmente extensa desde T8-L1, con LCR con proteinorraquia y seropositividad a AQP4. Se integra diagnóstico de NMO por criterios de Wingerchuk y se inicia tratamiento con rituximab con mejoría sintomática, objetivándola con una escala expandida del estado (EDSS) de 4 a 1. Conclusión: la entidad de NMO relacionada al VIH es poco frecuente, siendo clásicamente encontrado este fenómeno al momento del diagnóstico o posterior al inicio del tratamiento, cuando el sistema inmune aún resulta capaz de desarrollar una respuesta inmune exagerada. Sin embargo, en el caso que reportamos el debut de la NMO fue posterior a tres años del diagnóstico, contrastando con los casos previamente reportados, por lo que sugerimos que podrían intervenir algunos otros mecanismos, como la alteración en la regulación de las células B y un efecto viral directo.

Epidemiology of demyelinating diseases in Mexico: A registry-based study.

BACKGROUND: Demyelinating diseases (DD) are a group of chronic neurological diseases associated with loss and injury of brain or spinal cord regions. These conditions could trigger impairment of neurological functions and disability from earlier stages of life. Epidemiological data on DD remains insufficient for decision-making in the Mexican healthcare system. This study aims to describe the epidemiology of DD based on data from Mexico's National Registry of Demyelinating Diseases. METHODS: A cross-sectional, registry-based, observational study was performed. We analyzed 408 reports of multiple sclerosis (331, 81%), neuromyelitis optica spectrum disorder (67, 16%), chronic recurrent inflammatory optic neuropathy (5, 1%), clinically isolated syndrome (4, 0.9%), and autoimmune encephalitis (1, 0.2%) reported across 2021. RESULTS: The time from first symptoms to diagnosis of any DD was about 3 years. A treatment failure history was detected in 40% of patients. It was estimated that NMOSD accounts for 20% of all disorders. There was evidence that the use of brand-name and generic IFN drug products lead to increased therapeutic failures. CONCLUSION: Our research team suggests reinforcing educational programs and activities based on diagnosis and clinical management improvement to first-contact physicians and specialty doctors and promoting awareness in the whole population.

Clinical presentation of the first demyelinating event in pediatrics / [Presentación clínica de primer evento desmielinizante en pediatría.]

Introduction: Isolated Clinical Syndrome (ACS) refers to the first clinical event with characteristics suggestive of multiple sclerosis (MS). Clinical case: We report the case of a previously healthy 8-year-old male patient hospitalized for altered gait with suspicion of transverse myelitis. Spinal MRI was performed showing evidence of hyperintense D3-D5 lesion in T2. He receives treatment with intravenous corticosteroid therapy and with the result of oligoclonal bands in serum and CSF, a diagnosis of ACS is made. Conclusion: The objective is to describe a rare form of manifestation of demyelinating disease in pediatric age and to assess the importance of timely diagnosis and treatment.

Introducción: El síndrome Clínico Aislado (SCA) hace referencia al primer evento clínico con características sugestivas de esclerosis múltiple (EM). Caso clínico: Se comunica el caso de un paciente masculino de 8 años previamente sano internado por alteración de la marcha con sospecha de mielitis transversa. Se realiza RMN medular donde se evidencia lesión en D3-D5 hiperintensa en T2. Recibe tratamiento con corticoterapia endovenosa y con resultado de bandas oligoclonales en suero y LCR se realiza diagnóstico de SCA. Conclusión: El objetivo es describir una forma poco frecuente de manifestación de enfermedad desmielinizante en la edad pediátrica y valorar la importancia del diagnóstico y tratamiento oportuno.

Disautonomía gastrointestinal como manifestación de mielitis transversa: Reporte de caso

La mielitis transversa, de origen inflamatorio, es una afectación rara de la médula espinal que afecta a uno o varios niveles. La etiología incluye esclerosis múltiple, causas infecciosas o trastornos del espectro de la neuromielitis óptica. Se presenta de forma aguda, con síntomas motores, sensoriales y/o disautonómicos como los gastrointestinales y urinarios. El diagnóstico se basa en la sintomatología, evolución y se confirma por punción lumbar, resonancia magnética nuclear y analítica sanguínea completa. Se presenta el caso clínico de una paciente con mielitis transversa, que debutó con sintomatología gastrointestinal, síntomas motores y confirmación diagnóstica con resonancia magnética nuclear.

Inflammatory transverse myelitis is a rare condition that affects one or more levels of the spinal cord. Its etiology includes multiple sclerosis, infectious causes, or disorders within the spectrum of neuromyelitis optica. It presents acutely with motor, sensory, and/or dysautonomic symptoms, such as those related to the gastrointestinal and urinary systems. Diagnosis is based on symptomatology, evolution, and is confirmed by lumbar puncture, magnetic resonance imaging, and complete blood analysis. We present a clinical case of a patient with transverse myelitis who presented with gastrointestinal symptoms, motor symptoms, and was diagnosed with magnetic resonance imaging.

Encephalomyelitis in a patient with monkeypox: an unusual complication.

A new outbreak of monkeypox has been reported worldwide with CNS complications like encephalitis or myelitis being extremely rare. We present a case of a 30-year-old man with PCR-confirmed diagnosis of monkeypox who developed rapid neurological deterioration with extensive inflammatory involvement of the brain and spinal cord on MRI. Because of the clinical and radiological resemblance to acute disseminated encephalomyelitis (ADEM), it was decided to indicate treatment with high-dose corticosteroids for 5 days (without concomitant antiviral management due to lack of availability in our country). Given the poor clinical and radiological response, 5 days of immunoglobulin G were administered. During follow-up the patient's clinical condition improved, physiotherapy was started and all associated medical complications were controlled. To our knowledge, this is the first reported monkeypox case with severe CNS complications treated with steroids and immunoglobulin in the absence of specific antiviral treatment.

Neurologic and cutaneous infection by Clostridium piliforme in a kitten with systemic Tyzzer disease.

Tyzzer disease (TD) is a highly fatal condition of animals caused by Clostridium piliforme and characterized pathologically by enteritis, hepatitis, myocarditis, and occasionally encephalitis. Cutaneous lesions have been reported only rarely in animals with TD, and infection of the nervous system has not been described in cats, to our knowledge. We describe here neurologic and cutaneous infection by C. piliforme in a shelter kitten with systemic manifestations of TD and coinfection with feline panleukopenia virus. Systemic lesions included necrotizing typhlocolitis, hepatitis, myocarditis, and myeloencephalitis. The cutaneous lesions consisted of intraepidermal pustular dermatitis and folliculitis, with necrosis of keratinocytes and ulceration. Clostridial bacilli were identified within the cytoplasm of keratinocytes by fluorescence in situ hybridization, and a PCR assay was positive for C. piliforme. C. piliforme can infect keratinocytes leading to cutaneous lesions in cats with the location suggesting direct contact with contaminated feces as a route of infection.

Mielitis transversa aguda asociada con infección por SARS-CoV-2 / Acute Transverse Myelitis Associated to SARS-CoV-2 Infection

Resumen Además de provocar problemas respiratorios, la infección con el SARS-CoV-2 puede causar un espectro amplio de complicaciones neurológicas como son cefalea, ageusia, anosmia, encefalopatía, enfermedad cerebrovascular, síndrome de Guillain-Barré y otras polineuropatías, meningoencefalitis, encefalopatía hemorrágica necrotizante aguda y síndromes del sistema nervioso central asociados a inflamación. En esta revisión presentamos 39 casos de mielitis transversa aguda (MTA) asociados a infección por el SARS-CoV-2, confirmado por una prueba de reacción en cadena de la polimerasa (PCR) por hisopado nasofaríngeo y/o de antígeno en plasma. Los análisis bioquímicos de los pacientes confirmaron la ausencia de otros patógenos y de autoanticuerpos involucrados en inflamación del sistema nervioso, excepto por 2 casos, uno con autoanticuerpos contra la glicoproteína de la mielina de los oligodendrocitos (anti-MOG IgG) y otro con anti-MOG IgG y antidescarboxilasa del ácido glutámico (anti-GAD65), indicativos de una enfermedad autoinmune del sistema nervioso central. Los estudios de imagenología de resonancia magnética (RMN) confirmaron el diagnóstico de MTA. Aunque no se puede inferir causalidad, es muy probable que los casos aislados de MTA sean consecuencia de un proceso para o postinfeccioso en la COVID-19. La comprensión de los mecanismos que desencadenan estos trastornos neurológicos durante o al final de la infección viral, ayudarán a optimizar las estrategias terapéuticas para el manejo del paciente.

Abstract In addition to causing respiratory problems, SARS-CoV-2 can cause a wide spectrum of neurological complications such as headaches, ageusia, anosmia, encephalopathy, cerebrovascular disease, Guillain-Barré syndrome and other polyneuropathies, meningoencephalitis, acute necrotizing hemorrhagic encephalopathy, and central nervous system syndromes associated with inflammation. In this review we present 39 cases of acute transverse myelitis (ATM) associated to SARS-CoV-2 infection. All cases had a positive polymerase chain reaction (PCR) nasopharyngeal swab and/or antigen test. Biochemical analyses confirmed the absence of other pathogens and autoantibody-mediated neuroinflammatory disease, except for two cases, one with autoantibodies against the oligodendrocyte myelin glycoprotein (anti-MOG IgG) and another with anti-MOG IgG and anti-glutamic acid decarboxylase (anti-GAD65). Magnetic Resonance Imaging (MRI) studies confirmed the diagnosis of ATM. Although causality cannot be inferred, it is likely that isolated cases of ATM are the consequence of a para or post-infectious process in SARS-CoV-2. In this work, the probable causes of ATM associated with SARS-CoV-2 are discussed. The understanding of the mechanisms behind these neurological disorders triggered by a viral infection will help to optimize the therapeutic strategies for patient management.