Introduction: CLIPPERS syndrome (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory process of the central nervous system whose distinguishing features are the enhancing punctiform lesions in the brainstem that appear in the magnetic resonance images. Clinically, it is accompanied by dysarthria, ataxia and diplopia, and usually responds to treatment with corticoids. Pathologically, T lymphocytes appear infiltrated in the perivascular spaces of the brainstem.
Case report: We report the case of a 40-year-old woman with an initial subacute clinical picture of binocular diplopia, ataxia and dysarthria. The magnetic resonance brain scan revealed T2 hyperintense punctiform lesions in the stem, cerebellum, diencephalons and cortico-subcortical areas of both hemispheres, which were enhanced with contrast. An aetiological study was performed to rule out any underlying infectious, neoplastic or inflammatory origin, the results being negative. The patient was treated on two occasions with methylprednisolone, with a gradual lowering of the dosage, the response being favourable.
Conclusions: Diplopia and ataxia, as in our case, are practically always present. The MR findings consist of punctiform enhancing lesions located in the pons extending towards the cerebellum, basal ganglia and corpus callosum, the enhancement gradient becoming lower as the distance increases rostrally away from the cortex, and caudally towards the spinal cord. In the case of our patient, this gradient is not respected, and the density found was similar to that of lesions at the supratentorial level. The differential diagnosis is wide-ranging and justifies an extensive diagnostic study with, in certain cases, a biopsy study of brain tissue. The disease courses in a relapsing-remitting pattern and the earlier steroid therapy is established and the more prolonged it is, the better the prognosis will be.
Title: Sindrome CLIPPERS con distribucion atipica de las lesiones en la resonancia magnetica cerebral.
Introduccion. El sindrome CLIPPERS (chronic lymphocytic in?ammation with pontine perivascular enhancement responsive to steroids) es un proceso inflamatorio del sistema nervioso central cuyo rasgo distintivo son las lesiones puntiformes en el troncoencefalo captantes en los estudios de resonancia magnetica. Clinicamente, cursa con disartria, ataxia y diplopia, y suele responder a corticoides. Anatomopatologicamente, aparecen infiltrados de linfocitos T en los espacios perivasculares troncoencefalicos. Caso clinico. Mujer de 40 años con cuadro de instauracion subaguda de diplopia binocular, ataxia y disartria. En la resonancia magnetica cerebral presento lesiones puntiformes hipertintensas en secuencia T2 en el tronco, cerebelo, diencefalo y areas cortico-subcorticales bihemisfericas, que realzaron con contraste. Se realizo un estudio etiologico para descartar un origen infeccioso, neoplasico o inflamatorio subyacente, que resulto negativo. La paciente recibio tratamiento en dos ocasiones con metilprednisolona, con descenso progresivo de la dosis, con buena respuesta. Conclusiones. La diplopia y la ataxia, como en nuestro caso, estan presentes practicamente siempre. Los hallazgos en la RM consisten en lesiones captantes puntiformes localizadas en la protuberancia con extension hacia el cerebelo, ganglios basales y cuerpo calloso, con gradiente de captacion menor conforme se alejan rostralmente hacia la corteza, y caudalmente hacia la medula. En el caso de nuestra paciente, este gradiente no se respeta, encontrandose una densidad similar de las lesiones a nivel supratentorial. El diagnostico diferencial es amplio y justifica un estudio diagnostico extenso, y en casos seleccionados la biopsia cerebral. El curso de la enfermedad es remitente-recurrente, y el pronostico mejora cuanto mas precoz y prolongado es el tiempo de corticoterapia.