caa a22 4500 44580842X CHVBK 20180317145204.0 cr unu---uuuuu 170323e20111201xx s 000 0 eng 10.1007/s00415-011-6071-4 doi (NATIONALLICENCE)springer-10.1007/s00415-011-6071-4 Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study [Elektronische Daten] [O. Kastrup, J. van de Nes, T. Gasser, K. Keyvani] The aim of the study was to further determine the pathophysiology, clinical course, MRI-features and response to therapy of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which has recently been proposed as a rare chronic inflammatory central nervous system disorder responsive to immunosuppressive therapy. Three patients with this rare entity underwent serial clinical and bimonthly MRI follow-up over a period of up to 16months. Extensive laboratory work-up and brain biopsy were performed. Intravenous methylprednisolone or oral dexamethasone was administered as treatment, additionally cyclophosphamide in one patient. Clinically, diplopia, nystagmus, ataxia and facial paresthesia were the cardinal symptoms. Magnetic resonance imaging (MRI) disclosed patchy spot-like gadolinium enhancement in a "salt-and-pepper like appearance” in the pons, midbrain and cerebellum, in two cases with thalamic and in the other with spinal involvement. Brain biopsies demonstrated a predominantly angiocentric but also diffuse infiltration pattern by small mature lymphocytes. Treatment with steroids led to rapid clinical improvement and marked resolution of MRI lesions. As discontinuation of steroids led to clinical relapse, one patient was treated with a further course of steroids and the other with steroids and cyclophosphamide as immunosuppressive therapy. This led to stable remission with only mild clinical residue and normalization of MRI. Extensive laboratory and radiological work-up could not identify any other cause of the disease. Of note, in two cases a marked elevation of IgE in serum was found initially and throughout the course. CLIPPERS seems to be a distinct inflammatory central nervous system disorder. It shows characteristic MRI core features. Extrapontine involvement seems to be frequent. Histologically it is characterised by predominantly angiocentric infiltration by small mature lymphocytes. A pathogenetic relationship between the elevated IgE levels and the perivascular infiltrates can be presumed. It is responsive to immunosuppressive therapy and can require prolonged or maintenance treatment. Springer-Verlag, 2011 CLIPPERS nationallicence IgE nationallicence Lymphocytic nationallicence Infiltrate nationallicence Pontine nationallicence Kastrup O. Department of Neurology, University Duisburg-Essen, Hufelandstr. 55, 45122, Essen, Germany aut van de Nes J. Department of Neuropathology, University Duisburg-Essen, Essen, Germany aut Gasser T. Department of Neurosurgery, University Duisburg-Essen, Essen, Germany aut Keyvani K. Department of Neuropathology, University Duisburg-Essen, Essen, Germany aut Journal of Neurology Springer-Verlag 258/12(2011-12-01), 2140-2146 0340-5354 258:12<2140 2011 258 415 https://doi.org/10.1007/s00415-011-6071-4 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-6071-4 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kastrup O. Department of Neurology, University Duisburg-Essen, Hufelandstr. 55, 45122, Essen, Germany aut NATIONALLICENCE 700 1- van de Nes J. Department of Neuropathology, University Duisburg-Essen, Essen, Germany aut NATIONALLICENCE 700 1- Gasser T. Department of Neurosurgery, University Duisburg-Essen, Essen, Germany aut NATIONALLICENCE 700 1- Keyvani K. Department of Neuropathology, University Duisburg-Essen, Essen, Germany aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/12(2011-12-01), 2140-2146 0340-5354 258:12<2140 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer