1 - Infeciologia Pediátrica, Área de Pediatria, Hospital Dona Estefânia, Unidade Local de Saúde de São José
2 - Neurologia Pediátrica, Área de Pediatria, Hospital Dona Estefânia, Unidade Local de Saúde de São José
3 - Neurorradiologia, Hospital Dona Estefânia, Unidade Local de Saúde de São José
- Publicação em versão integral como caso de imagem
Resumo:
Introdução/Relato de caso: A male teenager from Guinea-Bissau, in Portugal for 15 days, presented with two months of progressive headache, decreased vision and left hemiparesis. On neurologic examination, the left eye was inwardly and downwardly deviated, with limited abduction and elevation, and predominantly distal left hemiparesis was noted. His gait was mildly ataxic with spastic circumduction of his left leg. Brain MRI showed multiple areas of FLAIR and T2 high signals involving both grey and white matter, particularly affecting the brainstem, thalami, and subcortical frontoparietal areas bilaterally. PML was suspected. HIV serology was positive, with HIV-1 viral load of 1,020,000 copies/mL and CD4+ of 126 cells/µL (3.6%). Cerebrospinal fluid revealed 18 mononuclear cells with positive John Cunningham Virus (JCV) PCR. Other opportunistic infections were excluded, including tuberculosis, toxoplasmosis, syphilis, and CMV. PML was diagnosed and triple antiretroviral therapy (TDF/FTC 1id + DTG 2id) was started. At 6-month follow-up, his ophthalmoparesis had resolved but the hemiparesis had persisted, despite intensive rehabilitation.
Conclusões: Progressive Multifocal Leukoencephalopathy (PML) is a severe, often fatal, disease caused by JCV (also known as human polyomavirus 2) associated with impaired immunity. Early HIV diagnosis and treatment is critical in treating PML, with estimates of median increase in survival from 0.4 to 1.8 years after highly active antiretroviral therapy introduction. Despite the declining incidence of HIV in developed countries, a high index of suspicion is necessary, especially in visitors from high-incidence countries.
Palavras Chave: HIV, Progressive Multifocal Leukoencephalopathy