Abstract Details

Title Long-Term Outcome of CLIPPERS in a Consecutive Series of 12 Patients

Topic Neurotoxicology

Presentation(s) S29 - (-)

Poster/Presentation
Number 005

Objective

Background (CLIPPERS) is a new CNS inflammatory disease recently defined .by 1) episodic brainstem symptoms, 2) characteristic punctate and curvilinear gadolinium-enhancement lesions peppering in the brainstem, mainly in the pons, on MR examination 3) and T-lymphocytic infiltrate with perivascular predominance on brain biopsy.

Design/Methods A nationwide study (inclusion period: February to July 2011) was implemented to collect clinical, MRI, CSF and brain biopsy characteristics of CLIPPERS, and evaluate the therapeutic management of this new CNS inflammatory disease.

Results Among the 12 included patients, 42 relapses were analysed. Relapses were characterized by 1) frequent cerebellar ataxia and diplopia 2) a mean duration of 2.5 months 3) a mean EDSS of 4. Besides typical findings of CLIPPERS, MR found brainstem mass effect (5 patients), extensive myelitis (3 patients), and closed ring enhancement (1 patient). Inconstant oligoclonal bands were found on CSF studies (4/ 12 patients) with increased CD4:CD8 T-cell ratio. Among 7 available brain biopsies, a perivascular CD4 T-lymphocytes staining was present in 5. 38/42 relapses were treated by pulse steroid therapy, leading to improvement with a mean residual EDSS of 1.9 (range 0-7). In untreated relapses, EDSS progressively increased until death (EDSS 10) in one case. In absence of long-term corticosteroid therapy (5 patients), the annualized relapse rate was 0.5 (0.25-3). For the 7 patients under oral steroids, no relapses occurred above 20 mg/d. No progressive course was observed. Final EDSS ? 4 (n=4), was correlated with previous severe relapses (EDSS ? 5), brainstem and spinal cord atrophy.

Conclusions CLIPPERS is a relapsing-remitting disorder without progressive forms. Long-term disability is correlated with severity of previous relapses. Prednisolone treatment above 20 mg/d prevents new relapses.

Authors/Disclosures
Pierre Labauge, MD PRESENTER	Dr. Labauge has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Jean Pelletier, MD, PhD (Dpt of Neurology, CHU Timone)	Dr. Pelletier has nothing to disclose.
	No disclosure on file
Christine Tranchant, MD (Hopital Civil)	No disclosure on file
	No disclosure on file
	No disclosure on file
Romain Lefaucheur (Hopital Charles Nicolle)	No disclosure on file
Richard S. Nicholas, FRCP (Imperial College Healthcare Trust)	Dr. Nicholas has nothing to disclose.
	No disclosure on file
	No disclosure on file
Michel G. Clanet, Prof	No disclosure on file
Patrice Desbordes	No disclosure on file
Eric Thouvenot, MD, PhD (CHU De Nimes - Hopital Caremeau)	Dr. Thouvenot has received personal compensation in the range of $0-$499 for serving as a Consultant for Biogen. Dr. Thouvenot has received personal compensation in the range of $0-$499 for serving as a Consultant for Merck. Dr. Thouvenot has received personal compensation in the range of $0-$499 for serving as a Consultant for Teva. Dr. Thouvenot has received personal compensation in the range of $0-$499 for serving as a Consultant for Novartis. Dr. Thouvenot has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Thouvenot has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. The institution of Dr. Thouvenot has received research support from Biogen. The institution of Dr. Thouvenot has received research support from Novartis. The institution of Dr. Thouvenot has received research support from Roche.
Laurent Magy, MD (Chru Dupeytren, Neurology Dept)	No disclosure on file
	No disclosure on file
	No disclosure on file
Giovanni Castelnovo	No disclosure on file
	No disclosure on file
	No disclosure on file

��ɫ��

��ɫ��