Teaching Neuro Images : Punctuate and curvilinear enhancement peppering the pons responsive to steroids

Abstract

A 48-year-old man developed progressive vertigo, ataxia, and dysarthria. MRI demonstrated gadolinium-enhancing abnormalities in the pons and cerebellar peduncles (figure, A). Further investigations excluded neurosarcoidosis, CNS lymphoma, granulomatosis, and paraneoplastic or

CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders

“…Clinical manifestations may be heterogeneous, multi‐faceted and variable in individual cases, but comprise essentially the following. Commonly prominent symptoms related to multi‐locular brainstem including cranial nerve and cerebellar involvement, which may present in various combinations or rarely in isolation (e.g. ataxia, dysarthria, oculomotor abnormalities, tingling of the face, vertigo) ; and Possible additional features such as: (i) symptoms referable to long tract affections and/or a spinal cord syndrome (e.g. pyramidal tract signs, spasticity, para‐/tetraparesis, altered limb superficial and deep sensation, sphincteric dysfunction) ; (ii) pseudobulbar affect (pathological crying and laugther) ; (iii) cognitive dysfunctions (e.g.…”

Síndrome CLIPPERS con distribución atípica de las lesiones en la resonancia magnética cerebral

“…Desde que Pittock et al [1] describiesen la entidad, se han publicado una veintena de casos más, ampliando el espectro clínico de este síndrome [2][3][4][5][6][7][8][9]. En ellos, las alteraciones oculomotoras y la ataxia están presentes en el diagnóstico prácticamente siempre.…”

CLIPPERS syndrome

“…Предположение о наличии этого синдрома должно возникать при выявлении характерной нейровизуализационной картины. Для ранних стадий заболевания типичны выраженный клинический и нейровизуализационый ответ на терапию ГК и ухудшение клинической и радиологической картины при их отмене [1,5,8]. Именно такая динамика болезни была у нашего пациента.…”