[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)

[Schilder's disease: two new cases and a review of the literature]. / Enfermedad de Schilder: dos nuevos casos y revisión de la bibliografía.

INTRODUCTION: Schilder's disease, or diffuse myelinoclastic sclerosis, is an infrequent disease that presents clinically as a pseudotumoural demyelinating lesion, which makes its diagnosis more complicated as it can be mistaken for a tumour or an abscess. CASE REPORTS: We examine the case of a male who was healthy up to the age of 8 years, when symptoms of a left hemiparesis appeared with a subacute onset and which were associated to symptoms of intracranial hypertension. A brain CAT scan showed a hypodense lesion in the right temporoparietal region, and the hypothesis of a tumoural lesion (astrocyte) was suggested. Treatment was started with dexamethasone and furosemide, and a complete regression of the symptoms and a considerable decrease in the cerebral lesion were observed. The second case is that of a female adolescent who, at the age of 11, developed a clinical picture of subacute onset of left hemiplegia. A brain CAT scan revealed hypodense lesions with ring-shaped contrast enhancement. In view of the histological diagnosis of an astrocyte, radiotherapy and corticotherapy are started. After two months' treatment, a sharp involution of the lesions was observed, which led to the acceptance of the diagnostic hypothesis of Schilder's disease. Both children presented recurrence of the lesions three years and nine months, in the first and second case respectively, after the first episode. Treatment with corticoid therapy was started and gave good clinical and radiological responses. CONCLUSIONS: In the presence of a neurological deficit with a subacute onset, associated to a brain image showing a 'tumoural' lesion containing an important amount of oedema and little mass effect, diagnoses other than that of a brain tumour must be taken into account. It thus becomes possible to avoid invasive forms of treatment, such as surgical resection, which entail a number of sequelae.

Enfermedad de Schilder: dos nuevos casos y revisión de la bibliografía / Schilder's disease: two new cases and a review of the literature

Introducción. La enfermedad de Schilder o esclerosis mielinoclástica difusa es poco frecuente y su presentación clínica es la de una lesión desmielinizante pseudotumoral, lo cual ocasiona dificultades en el diagnóstico, ya que se puede confundir con un tumor o un absceso. Casos clínicos. El primer caso es un niño sano hasta la edad de 8 años, momento en que el se manifiesta un cuadro de hemiparesia izquierda de instalación subaguda asociado a síntomas de hipertensión intracraneal. La TAC cerebral mostró una lesión hipodensa en la región temporoparietal derecha, y se propuso la hipótesis de lesión tumoral (astrocitoma). Se inició tratamiento con dexametasona y furosemida y se verificó una regresión completa de la sintomatología y disminución considerable de la lesión cerebral. El segundo caso es el de una adolescente que, a los 11 años, desarrolla un cuadro de instalación subaguda de hemiplejía izquierda. La TAC craneoencefálica mostró seis lesiones hipodensas con captación de contraste en anillo. En vista del diagnóstico histológico de astrocitoma se inició radioterapia y corticoterapia. Tras dos meses de tratamiento se demostró una involución marcada de las lesiones, por lo que se aceptó la hipótesis diagnóstica de enfermedad de Schilder. Ambos niños presentaron recurrencia de las lesiones, en el primer caso 3 años después del primer episodio, y en el segundo, 9 meses después. Se realizó tratamiento con corticoterapia y se obtuvo una buena respuesta clínica y radiológica. Conclusión. En presencia de un déficit neurológico de instalación subaguda, asociado a una imagen cerebral con lesión `tumoral', que cuenta con un importante componente de edema y poco efecto de masa, deben considerarse diagnósticos alternativos al tumor cerebral. Así, se pueden evitar tratamientos invasivos, como la biopsia o, incluso, la resección quirúrgica, que acarrean secuelas (AU)

Introduction. Schilder’s disease, or diffuse myelinoclastic sclerosis, is an infrequent disease that presents clinically as a pseudotumoural demyelinating lesion, which makes its diagnosis more complicated as it can be mistaken for a tumour or an abscess. Case reports. We examine the case of a male who was healthy up to the age of 8 years, when symptoms of a left hemiparesis appeared with a subacute onset and which were associated to symptoms of intracranial hypertension. A brain CAT scan showed a hypodense lesion in the right temporoparietal region, and the hypothesis of a tumoural lesion (astrocytoma) was suggested. Treatment was started with dexamethasone and furosemide, and a complete regression of the symptoms and a considerable decrease in the cerebral lesion were observed. The second case is that of a female adolescent who, at the age of 11, developed a clinical picture of subacute onset of left hemiplegia. A brain CAT scan revealed hypodense lesions with ring-shaped contrast enhancement. In view of the histological diagnosis of an astrocytoma, radiotherapy and corticotherapy were started. After two months’ treatment, a sharp involution of the lesions was observed, which led to the acceptance of the diagnostic hypothesis of Schilder’s disease. Both children presented recurrence of the lesions three years and nine months, in the first and second case respectively, after the first episode. Treatment with corticoid therapy was started with good clinical and radiological responses. Conclusions. In the presence of a neurological deficit with a subacute onset, associated to a brain image showing a ‘tumoural’ lesion containing an important amount of oedema and little mass effect, diagnoses other than that of a brain tumour must be taken into account. It thus becomes possible to avoid invasive forms of treatment, such as surgical resection, which entail a number of sequelae (AU)