[Primary angiitis of the central nervous system: an infrequent form of presentation]. / Angeítis primaria del sistema nervioso central: una forma de presentación poco habitual.

INTRODUCTION: Primary angiitis of the central nervous system (CNS) is a rare disease. Clinical signs and symptoms include headache and cognitive disorders associated to multifocal neurological deficits. A definitive diagnosis can only be achieved by means of a cerebromeningeal biopsy. CASE REPORT: We describe the case of a 15-year-old male who first reported lower back pain and progressive paresis of the right lower limb, later followed by laterocollis on the right side. Magnetic resonance (MR) scanning of the brain and spinal cord revealed a pseudotumoral lesion in the right cerebellum and two lesions in the spinal cord. Dexamethasone was administered and surgical resection of the cerebellar lesion was performed. Following surgery, the patient received corticoid treatment with progressive withdrawal. Full clinical recovery of the patient was achieved. A year later, the same patient was admitted to hospital again because of headaches and diplopia. A new MR brain scan showed a right frontotemporal lesion. Dexamethasone was administered and the patient recovered. Thirteen months later, he reported dysarthria and right-side hemiparesis. An MR brain scan revealed the presence of a number of bilateral lesions in the white matter. Results of a lumbar puncture showed lymphocytic pleocytosis and raised protein levels in cerebrospinal fluid. The laminae from the cerebromeningeal biopsy were reviewed and the results confirmed the hypothetical diagnosis of angiitis of the CNS. Treatment was established with intravenous cyclophosphamide in association with oral prednisone. CONCLUSIONS: Primary angiitis of the CNS is an infrequent disease and its pathogenesis remains unknown. The definitive diagnosis of these patients is histological. It courses spontaneously and generally has a fatal outcome. Treatment, which consists in an association of cyclophosphamide and prednisone, must be started as early as possible.

Angeítis primaria del sistema nervioso central: una forma de presentación poco habitual / Primary angiitis of the central nervous system: an infrequent form of presentation

Introducción. La angeítis primaria del sistema nervioso central (SNC) es una enfermedad rara. La clínica incluye cefaleas y alteraciones cognitivas asociadas a déficit neurológicos multifocales. El diagnóstico sólo es definitivo mediante biopsia cerebromeníngea. Caso clínico. Varón de 15 años de edad que inició un cuadro de dolores lumbares y paresia progresiva del miembro inferior derecho, seguido de laterocollis derecho. Se realizó una resonancia magnética (RM) cerebral y medular que reveló una lesión pseudotumoral cerebelosa derecha y dos lesiones medulares dorsales. Se le administró dexametasona y se le sometió a cirugía para la resección de la lesión cerebelosa. Tras la cirugía, se le mantuvo en tratamiento con corticoides con suspensión progresiva. Tuvo una recuperación clínica completa. Un año después volvió a ingresar por cefaleas y diplopía. Una nueva RM cerebral mostró una lesión frontotemporal derecha. Se le administró dexametasona y se recuperó. Trece meses después presentó disartria y hemiparesia derecha. La RM cerebral reveló múltiples lesiones bilaterales de la sustancia blanca. En la punción lumbar presentaba pleocitosis linfocitaria y proteinorraquia. Se revisaron las láminas de la biopsia cerebromeníngea, lo que confirmó la hipótesis del diagnóstico: angeítis del SNC. Se le medicó con ciclofosfamida endovenosa asociada a prednisolona oral. Conclusión. La angeítis primaria del SNC es una enfermedad muy poco frecuente y de patogenia desconocida. El diagnóstico de certeza es histológico. Su evolución es espontánea y generalmente fatal. El tratamiento, consistente en una asociación de ciclofosfamida y prednisolona, debe iniciarse lo más pronto posible

Introduction. Primary angiitis of the central nervous system (CNS) is a rare disease. Clinical signs and symptoms include headache and cognitive disorders associated to multifocal neurological deficits. A definitive diagnosis can only be achieved by means of a cerebromeningeal biopsy. Case report. We describe the case of a 15-year-old male who first reported lower back pain and progressive paresis of the right lower limb, later followed by laterocollis on the right side. Magnetic resonance (MR) scanning of the brain and spinal cord revealed a pseudotumoral lesion in the right cerebellum and two lesions in the spinal cord. Dexamethasone was administered and surgical resection of the cerebellar lesion was performed. Following surgery, the patient received corticoid treatment with progressive withdrawal. Full clinical recovery of the patient was achieved. A year later, the same patient was admitted to hospital again because of headaches and diplopia. A new MR brain scan showed a right frontotemporal lesion. Dexamethasone was administered and the patient recovered. Thirteen months later, he reported dysarthria and right-side hemiparesis. An MR brain scan revealed the presence of a number of bilateral lesions in the white matter. Results of a lumbar puncture showed lymphocytic pleocytosis and raised protein levels in cerebrospinal fluid. The laminae from the cerebromeningeal biopsy were reviewed and the results confirmed the hypothetical diagnosis of angiitis of the CNS. Treatment was established with intravenous cyclophosphamide in association with oral prednisone. Conclusions. Primary angiitis of the CNS is an infrequent disease and its pathogenesis remains unknown. The definitive diagnosis of these patients is histological. It courses spontaneously and generally has a fatal outcome. Treatment, which consists in an association of cyclophosphamide and prednisone, must be started as early as possible