Early-Onset HTLV-1-Associated Myelopathy/Tropical Spastic Paraparesis.

BACKGROUND: Vertical transmission of HTLV-1 could lead to the early development of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). This significantly affects quality of life and increases morbimortality. OBJECTIVE: To describe the epidemiological and clinical characteristics of patients with early-onset HAM/TSP, defined as disease onset before 20 years of age. METHODS: This is a retrospective study from an HTLV-1 clinical cohort between 1989 and 2019. We searched for medical records of patients with (1) diagnosis of HTLV-1 infection using two ELISA and/or one Western blot, (2) clinical diagnosis of HAM/TSP by neurological assessment, and (3) HAM/TSP symptom-onset before 20 years of age. RESULTS: A total of 38 cases were identified in the cohort; 25 were female (66%). The median age of onset was 14 years old. 31 (82%) cases had HTLV-1 testing done among family members; 22 out of 25 tested mothers (88%) were HTLV-1 positive. Most patients (27/34) were breastfed for more than one year. Disease progression measured through EDSS and IPEC-1 showed an upward trend towards worsening spasticity with 18 patients (47%) eventually requiring mobility aids. CONCLUSIONS: Cases of early-onset HAM/TSP are not of rare occurrence, which translates into many more years of dependency, the use of mobility aids, and increased overall morbidity

Impact of fractures and orthopedic surgeries in patients with HTLV-1 associated myelopathy/tropical spastic paraparesis.

INTRODUCTION: In patients with HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) gait disturbance is a predominant feature that leads to falls and fractures, which can further aggravate disability. We sought to evaluate the impact of fractures and orthopedic surgeries in patients with HAM/TSP. METHODS: We retrieved the medical records of HAM/TSP patients enrolled in our study center's HTLV-1 clinical cohort between 1989-2018. The selection criteria included: (1) diagnosis of HTLV-1 infection using two enzyme-linked immunosorbent assays and/or a confirmatory test, (2) clinical diagnosis of HAM/TSP by neurological assessment, and (3) fractures associated with HAM/TSP. RESULTS: We identified 24 cases of fractures, 70% of which were females. The median age at the time of fracture was 60 years (IQR=24). Six cases reported fractures in patients under 45 years old. Ten patients (42%) had hip/coccyx fractures, seven (29%) were in the lower extremities, and four (17%) in the upper extremities. Half of these patients reported the use of wheelchairs. Five patients who had previously used canes required the use of wheelchairs after the reported fracture. Eight patients underwent corrective orthopedic surgery as a result of the fracture. CONCLUSIONS: For HAM/TSP patients, fractures are a complication that can exacerbate their severe impairment

A review of the clinical and epidemiological aspects of Guillain-Barré syndrome in patients infected with SARS-CoV-2: An Extended Comprehensive Review

Desde 2019, se han venido publicando casos de pacientes con COVID-19 que desarrollaron el Síndrome de Guillain-Barré (GBS). Esta revisión explora mecanismos que expliquen fisiopatología, características clínicas, hallazgos de laboratorio y características imagenológicas en estos pacientes. Metodología: Búsqueda bibliográfica de estudios publicados en NCBI y Scielo, entre diciembre de 2019 y abril de 2022. Resultados: Se encontraron noventa artículos, 53 de los cuales se incluyen esta síntesis. No se encontraron estudios que expliquen una asociación entre GBS y COVID-19. Clínicamente, se encontró arreflexia (56.95%), hiporreflexia (19.44%), debilidad muscular (65.28%), alteración de la marcha (12.5%), hipoestesia (26.39%), parestesia (30.55%) y alteración de la micción (6.94%). Los hallazgos en el líquido cefalorraquídeo incluyeron disociación albumino-citológica (66.67%) y un nivel promedio de proteínas de 140.23 mg/dL (DE: 106.71). Algunos casos mostraron realce de las leptomeninges cervicales, tronco encefálico y nervios craneales en tests de resonancia magnética. La variante predominante de GBS fue polineuropatía desmielinizante inflamatoria aguda (56.94%). Los hallazgos en los estudios de conducción nerviosa incluyeron ausencia de ondas F (61.54%), aumento de la latencia motora distal (80%), disminución de la amplitud motora (93.1%) y disminución de la velocidad de conducción motora (75%). Los nervios principalmente involucrados fueron el tibial (20.21%), peroneal (24.47%), mediano (20.21%) y cubital (18.09%). La alteración más frecuente de los nervios craneales fue parálisis facial bilateral (25%) y unilateral (13.89%).Conclusión: La variante primaria del Síndrome de Guillain-Barré (GBS) fue la Polineuropatía Dismielinizante Inflamatoria Aguda. El análisis del líquido cefalorraquídeo reveló una disociación albumino-citológica como el hallazgo más común, y las imágenes en tests de resonancia magnética mostraron incremento de los nervios craneales. Otro hallazgo diferencial fue el menor compromiso del sistema autónomo.Since 2019, cases of patients with COVID-19 who developed Guillain-Barré Syndrome (GBS) have been reported. This review explores mechanisms that explain pathophysiology, clinical features, laboratory findings, and imaging characteristics in these patients.Methodology: A bibliographic search was made of studies on the topic published in NCBI and Scielo, between December 2019 and April 2022.Results: Ninety articles were found, 53 of which were included in this article. No studies were found that explain an association between GBS and COVID19. Specific clinical manifestations found were areflexia (56.95%), hyporeflexia (19.44%), muscle weakness (65.28%), gait disturbance (12.5%), hypoesthesia (26.39%), paresthesia (30.55%), and micturition disturbance (6.94%). The CSF findings included albumin-cytological dissociation (66.67%), and an average protein level of 140.23 mg/dL (SD: 106.71). Some cases reported enhancement of the cervical leptomeningeal, brainstem and cranial nerves on magnetic resonance imaging tests. The predominant variant of GBS was acute inflammatory demyelinating polyneuropathy (56.94%). The findings in the nerve conduction studies were the absence of F waves (61.54%), increased distal motor latency (80%), decreased motor amplitude (93.1%), and decreased motor conduction velocity (75%). In addition, the nerves mainly involved were the tibial (20.21%), peroneal (24.47%), median (20.21%), and ulnar (18.09%). The most frequent alteration of cranial nerves was bilateral (25%) and unilateral (13.89%) facial palsy.Conclusion: The primary GBS variant was Acute Inflammatory Demyelinating Polyneuropathy. Cerebrospinal fluid analysis revealed albumin-cytological dissociation as the most common finding, and MRI tests showed cranial nerves enhancements. An additional differential feature was the lower commitment of the autonomous system

Frecuencia de las señales de alarma en pacientes con cefalea, atendidos en la emergencia de un hospital de Lima.

Las cefaleas en emergencia suelen ser un reto diagnóstico para el evaluador cuando se trata de diferenciarlas entre primaria o secundaria a un trastorno neurológico subyacente. Objetivo: Describir la frecuencia de las señales de alarma en cefaleas secundarias y primarias en pacientes que acuden a emergencia. Materiales y Métodos: Estudio prospectivo de series de casos, que acuden a la Emergencia de Medicina de Adultos del Hospital Nacional Cayetano Heredia por cefalea, en un mes. Se excluyeron a los pacientes con cefalea traumática. Se usó un formato para la obtención de datos. Se clasificó a los pacientes en cefalea primaria o secundaria y se determinó la presencia de señales de alarma. Resultados: Se registraron 32 pacientes. Veintiséis (81%) tuvieron cefalea secundaria. De éstas, 9 se debieron a causas infecciosas y 9 a trastornos vasculares intracraneales. La cantidad de señales de alarma presentes en las cefaleas secundarias vario de 3 a 8, con una moda de 5; y en las primarias de 0 a 2, con una moda de 2. El empeoramiento progresivo y el examen neurológico anormal se presentaron en 96% de los casos de cefalea secundaria. Conclusiones: Las señales de alarma, empeoramiento progresivo y examen neurológico anormal fueron los más frecuentes. Las cefaleas secundarias fueron más comunes que las cefaleas primarias. Se hace énfasis en la utilidad de usar esta metodología en emergencia