ABSTRACT
RESUMEN Los medios de contraste iodados pueden presentar diferentes efectos adversos, siendo los más frecuentes daño renal y reacciones de hipersensibilidad. La ceguera cortical y amnesia global transitoria son complicaciones neurológicas raras descritas tras la administración de medios de contraste. Reportamos el caso de un paciente de 63 años, hipertenso que acude por cianosis, frialdad del segundo y tercer dedo de mano izquierda. Es sometido a arteriografía de miembro superior, usando 50 mL de iopamidol e inmediatamente desarrolla cefalea occipital asociada a visión borrosa bilateral que progresa rápidamente a ceguera; la tomografía cerebral reveló hiperdensidades subaracnoideas occipitales, sin efecto de masa. A las dieciocho horas del evento, el paciente presenta amnesia anterógrada con persistencia de amaurosis. Su tomografía cerebral de control no muestra trastornos focales. El paciente cursa con evolución favorable, la amaurosis bilateral y la cefalea ceden a las treinta horas del evento y es dado de alta sin mayores eventualidades a los diez días.
ABSTRACT Iodinated contrast substances may cause different adverse events, most frequently renal involvement and hypersensitivity reactions. Transient cortical blindness and global amnesia are rarely described neurological complications after the administration of contrast substances. We report the case of a 63-year old patient with high blood pressure who presented with cyanosis and cold sensation affecting the second and third digits of his left hand. The patient underwent an arteriography of the left arm, where 50-mL of iopamidol was used as a contrast substance, and he immediately developed occipital headache associated to bilateral blurred vision that rapidly progressed to blindness. A cerebral computed tomography (CT) scan showed occipital subarachnoid hypodense areas, with no mass effect. Eighteen hours after the event, the patient showed retrograde amnesia with persistent amaurosis. His control cerebral CT scan did now show any focal disorder. The patient had a good progression, both bilateral amaurosis and headache subsided thirty hours after the event, and the patient was uneventfully discharged after ten days.
ABSTRACT
Abstract X-linked adrenoleukodystrophy (X-ALD) represents a group of diseases characterized by the accumulation of very long chain fattyacids (VLCFAs) in the tissues. Its clinical manifestations are usually manifold. Visual changes may be present, but they often appear later in the disease. We describe here the case of a 9-year-old boy with X-ALD, whose first symptom was visual loss, which began at 8 years of age. His ophthalmologic evaluation revealed no alterations. Shortly thereafter, he suffered a head injury. The magnetic resonance imaging of brain revealed findings that led to the suspicion of X-ALD. The plasma VLCFA dosage confirmed this diagnosis. This report aims toshow that in cases of visual loss with a normal ophthalmic examination, a high index of suspicion should be given for conditions suchas X-ALD, since it affects the cortical routes related to vision. Fundoscopy findings appear late in X-ALD.
Resumo A adrenoleucodistrofia ligada ao X (X-ALD) representa um grupo de doenças caracterizadas pelo acúmulo de ácidos graxos de cadeia muito longa (VLCFAs) nos tecidos. Suas manifestações clínicas costumam ser múltiplas. Alterações visuais podem estar presentes, contudo costumam surgir mais tardiamente na doença. Descrevemos aqui o caso de um menino de 9 anos com X-ALD, cujo primeiro sintoma foi perda visual, iniciada aos 8 anos de idade. A sua avaliação oftalmológica não revelou alterações. Pouco tempo depois, ele sofreu um traumatismo craniano. A imagem de ressonância magnética de encéfalo revelou achados que levaram a suspeita de X-ALD. A dosagem dos VLCFAs no plasma confirmou este diagnóstico. Este relato tem como objetivo mostrar que em casos de perda visual com um exame oftalmológico normal, deve-se ter um alto índice de suspeita para condições como a X-ALD, pois a mesma afeta as vias corticais relacionadas à visão. Nessa doença, os achados da fundoscopia aparecem mais tardiamente.