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BACKGROUND: Magnetic resonance imaging is essential to diagnose progressive multifocal leukoencephalopathy. The broad radiological spectrum may partially be explained by genetic viral mutations and their differential neurotropism. Recent pharmacovigilance-magnetic resonance imaging studies have provided new insight into pathophysiology and radiological markers of early stages. However, how lesions evolve and why certain anatomical locations are more frequently affected remains unknown. We aim to describe a new sign - T2/fluid-attenutated inversion recovery mismatch - as a complementary marker of cavitated lesions and propose a link with the milky-way appearance, a key early sign. Furthermore, we hypothesise viral dissemination routes. METHODS: We conducted a retrospective longitudinal study from January 2010 to January 2020, to analyse clinical and magnetic resonance imaging features of 13 progressive multifocal leukoencephalopathy individuals at the symptomatic stage (mean age 58.3 years (SD ± 16.8) - 61.5% were women). RESULTS: The most prevalent pathology was HIV (61.5%) and motor deficit prevailed regarding other symptoms (76.9%). Frontal lobes (76.9%), middle cerebellar peduncle (61.5%), cerebellum (61.5%), and pons (53.8%) were most commonly affected, and the cortico-ponto-cerebellar pathway seemed involved in these patients. Five patients had a pure radiological pattern. Milky-way appearance was the most frequent radiological sign (58.3%). Five patients with milky-way appearance had concomitantly T2/fluid-attenuated inversion recovery mismatch (P = 0.02). This sign showed high sensitivity and specificity (100-71%, P = 0.02) to assess evolved lesions besides diffusion. CONCLUSION: The possible tract-dependent spread, as well as clinical and genetic, have implications on the MRI variability of progressive multifocal leukoencephalopathy. The milky-way appearance could reflect a transitional phase towards evolved lesions, the latter demonstrated by T2/fluid-attenuated inversion recovery mismatch. Both could be key magnetic resonance imaging signs to diagnose progressive multifocal leukoencephalopathy at the symptomatic stage.

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AIM: To analyse the clinical findings, complementary examinations and prognosis of patients with progressive multifocal leukoencephalopathy (PML) treated in our institution, comparing populations with and without associated human immunodeficiency virus (HIV). PATIENTS AND METHODS: A retrospective study of the medical records of patients with probable or definite PML was carried out. Clinical variables, complementary studies (cerebrospinal fluid, magnetic resonance imaging of the brain) and prognostic variables were analysed. Non-parametric statistical tests were used to compare HIV-positive and HIV non-positive populations. RESULTS: Fourteen patients with definite and one probable diagnosis of PML were included. Nine patients had PML associated with HIV; five had other immunosuppressive conditions (two, chronic lymphatic leukaemia; one, multiple sclerosis; one, neuromyelitis optica; and one, neurosarcoidosis); and one, no obvious immunosuppressive condition. The population with HIV presented heterogeneous dirty-appearing white matter lesions more frequently (77.7% versus 16.67%; p = 0.0247) in the cerebral MRI. No other significant differences were identified in the remaining variables analysed. CONCLUSION: HIV/AIDS is the pathology most frequently associated with PML. With the use of immunomodulator drugs its appearance is reported in a variety of other diseases. Heterogeneous dirty-appearing white matter lesions were significantly more common in HIV patients.

TITLE: Espectro clinico de la leucoencefalopatia multifocal progresiva: diferencias y similitudes en pacientes con y sin virus de la inmunodeficiencia humana.Objetivo. Analizar los hallazgos clinicos, examenes complementarios y pronostico de los pacientes con leucoencefalopatia multifocal progresiva (LMP) atendidos en nuestra institucion, comparando las poblaciones con y sin virus de la inmunodeficiencia humana (VIH) asociado. Pacientes y metodos. Estudio retrospectivo de historias clinicas de pacientes con LMP probable o definitiva. Se analizaron variables clinicas, estudios complementarios (liquido cefalorraquideo, resonancia magnetica cerebral) y variables pronosticas. Mediante pruebas estadisticas no parametricas se realizo la comparacion entre las poblaciones con y sin VIH. Resultados. Se incluyo a 14 pacientes con diagnostico de LMP definitiva y uno probable. Nueve pacientes presentaron LMP asociada a VIH; cinco, otras condiciones de inmunoafectacion (dos, leucemia linfatica cronica; uno, esclerosis multiple; uno, neuromielitis optica; y uno, neurosarcoidosis); y uno, sin condicion inmunosupresora evidente. La poblacion con VIH presento con mayor frecuencia lesiones de la sustancia blanca heterogeneas de aspecto «sucio¼ (77,7% frente a 16,67%; p = 0,0247) en la resonancia magnetica cerebral. No se identificaron otras diferencias significativas en las restantes variables analizadas. Conclusion. El VIH/sida es la patologia mas frecuente asociada a LMP. Con el uso de farmacos inmunomoduladores se describe su aparicion en una variedad de otras enfermedades. Las lesiones de la sustancia blanca heterogeneas de aspecto «sucio¼ fueron significativamente mas frecuentes en pacientes con VIH.

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The polyomavirus are a family of opportunistic virus, which belongs to the JC virus whose primary manifestation is the Progressive Multifocal Leukoencephalopathy and neuronopathy of granulosa cells. Recently given the use of PCR for BK virus in transplant patients have also been described CNS pathologies in relation to this, and most frequent encephalitis and leukoencephalopathy. Each entity has a compatible clinical and genetic testing have allowed a diagnosis of high specificity, however the RNM is the great diagnostic pillar in these pathologies, allowing differentiate the different entities, the progression of lesions and response to treatment, especially in cases where it is indicated antiretroviral therapy, as gadolinium enhancement and mass effect may suggest Syndrome Immune and worse prognosis.

Los Poliomavirus son una familia de virus oportunistas, al cual pertenece el virus JC cuya principal manifestación es la Leucoencefalopatía Multifocal Progresiva y la Neuronopatía de Células Granulosas. Recientemente, dada la utilización de PCR para virus BK en LCR en pacientes trasplantados, también se han descrito patologías de SNC en relación a éste, siendo lo más frecuente la Encefalitis y la Leucoencefalopatía. Cada entidad tiene un contexto clínico compatible y las pruebas genéticas han permitido un diagnóstico de alta especificidad, sin embargo, la RNM es el gran pilar diagnóstico en estas patologías, permitiendo diferenciar las distintas entidades, la progresión de las lesiones y la respuesta a tratamiento, especialmente en los casos en que está indicado la terapia antirretroviral, pues la captación de gadolinio y el efecto de masa pueden sugerir un Síndrome de Reconstitución Inmune y peor pronóstico.

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A 55-year-old woman with known relapsing-remitting multiple sclerosis (RRMS) on natalizumab (Tysabri®) for 3 years was admitted to the hospital with worsening word-finding difficulties and gait instability. Neurologic examination revealed right hemianopia, right arm hemiplegia, right-sided sensory loss, and global aphasia. The patient underwent MRI and PET imaging with concurrent electroencephalogram. She was subsequently diagnosed with natalizumab-induced progressive multifocal leukoencephalopathy (PML) and treated with plasmapheresis, intravenous immunoglobulin, and high-dose intravenous steroids. Steroids were continued over a 3-month hospital course and tapered upon discharge. Speech, arm strength, and ambulation have since improved.

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