[Tropical spastic paraparesis and HTLV-I associated myelopathy in infancy. A case report and review of the literature]. / Paraparesia espástica tropical/mielopatía asociada al HTLV-I en la infancia. Caso clínico y revisión bibliográfica.

INTRODUCTION: HTLV-I associated myelopathy is characterised by a clinical picture of slowly progressive spastic paraparesis that generally presents when the patient is at an age somewhere between his or her thirties and sixties; few cases have been reported involving children. It is a pathology that is prevalent in tropical regions that are endemic for HTLV-I (southern Japan, the Caribbean, Central and South America, and some areas of Africa). CASE REPORT: The authors report the case of a 9-year-old child from Guinea who was admitted to the Paediatric Neurology Unit in the Hospital de Santa Maria with a two-year-old clinical history of spastic paraparesis. Computerised tomography and magnetic resonance imaging did not show any alterations to the spinal cord. Somatosensory evoked potentials revealed a lesion in the posterior dorsolumbar spinal cord. The exclusion of other (infectious, metabolic and demyelinating) pathologies and the confirmation of infection by HTLV-I (by means of PCR) led to a diagnosis of myelopathy associated to this virus. Therapy was established with interferon alfa, but no appreciable significant improvement was observed. DISCUSSION: This case stands out because of the uncommonness of this pathology at the paediatric age. We review the most relevant aspects of this disorder at the earliest ages, above all with regard to its epidemiology, transmission, clinical symptoms and complementary diagnostic examinations. Known therapeutic options (corticoids, interferon alfa, antiretroviral agents, among others) and prognosis are also discussed.

Paraparesia espástica tropical/mielopatía asociada al HTLV-I en la infancia. Caso clínico y revisión bibliográfica / Tropical spastic paraparesis and HTLV-I associatd myelopathy in infancy. A case report and review of the literature

Introducción. La mielopatía asociada al HTLV-I se caracteriza por un cuadro de paraparesia espástica lentamente progresiva, que se inicia generalmente entre la tercera y la sexta década de la vida, y son raros los casos descritos en niños. Es una patología prevalente en regiones tropicales endémicas para el HTLV-I (sur del Japón, Caribe, América central y del sur y algunas regiones de África). Caso clínico. Se presenta un niño de 9 años, oriundo de Guinea, ingresado en la Unidad de Neurología Pediátrica del Hospital de Santa Maria con un cuadro de paraparesia espática de dos años de evolución. Ni en la tomografía computarizada ni en la resonancia magnética de la médula se detectaron alteraciones. Los potenciales evocados somatosensitivos revelaron una lesión cordonal posterior dorsolumbar. La exclusión de otras patologías, fundamentalmente infecciosas, metabólicas y desmielinizantes, y la confirmación de infección por el HTLV-I (mediante PCR) condujeron al diagnóstico de mielopatía asociada a este virus. Se trató con interferón α, sin apreciarse una mejora significativa. Discusión. Nuestro caso destaca por la rareza de esta patología en la edad pediátrica. Se revisan los aspectos más relevantes de esta patología en las edades más jóvenes, básicamente en lo relativo a su epidemiología, transmisión, manifestaciones clínicas y exámenes complementarios de diagnóstico. Se discuten, incluso, las opciones terapéuticas conocidas (corticoides, interferón α, antirretrovirales, entre otras) y el pronóstico (AU)

Introduction. HTLV-I associated myelopathy is characterised by a clinical picture of slowly progressive spastic paraparesis that generally presents when the patient is at an age somewhere between his or her thirties and sixties; few cases have been reported involving children. It is a pathology that is prevalent in tropical regions that are endemic for HTLV-I (southern Japan, the Caribbean, Central and South America, and some areas of Africa). Case report. The authors report the case of a 9-year-old child from Guinea who was admitted to the Paediatric Neurology Unit in the Hospital de Santa Maria with a twoyear-old clinical history of spastic paraparesis. Computerised tomography and magnetic resonance imaging did not show any alterations to the spinal cord. Somatosensory evoked potentials revealed a lesion in the posterior dorsolumbar spinal cord. The exclusion of other (infectious, metabolic and demyelinating) pathologies and the confirmation of infection by HTLV-I (by means of PCR) led to a diagnosis of myelopathy associated to this virus. Therapy was established with interferon alfa, but no appreciable significant improvement was observed. Discussion. This case stands out because of the uncommonness of this pathology at the paediatric age. We review the most relevant aspects of this disorder at the earliest ages, above all with regard to its epidemiology, transmission, clinical symptoms and complementary diagnostic examinations. Known therapeutic options (corticoids, interferon alfa, antiretroviral agents, among others) and prognosis are also discussed (AU)

[Urgent neurological consultation in children with systemic cancer]. / Consultas de urgencia en niños con cáncer sistémico.

Neurologic complications are common in children with cancer but the literature dealing with this subject is sparse. Utilizing a symptoms and signs approach we review the most common causes for requesting an urgent neurologic evaluation for this population. The spectrum of neurologic symptoms in children with cancer differs from adults and requires the consulting neurologist to have a thorough knowledge of childhood cancer and its effects on the nervous system.