Email updates

Keep up to date with the latest news and content from BMC Neurology and BioMed Central.

Open Access Case report

Need for prolonged immunosupressive therapy in CLIPPERS-a case report

Juerd Wijntjes1*, Ernest J Wouda1, Carl EH Siegert2, Giorgos B Karas3 and Annemarie MM Vlaar1

Author Affiliations

1 Department of Neurology, Sint Lucas Andreas Hospital, Jan Tooropstraat 164, 1061 AE, Amsterdam, Netherlands

2 Department of Internal Medicine, Sint Lucas Andreas Hospital, Amsterdam, Netherlands

3 Department of Radiology, Sint Lucas Andreas Hospital, Amsterdam, Netherlands

For all author emails, please log on.

BMC Neurology 2013, 13:49  doi:10.1186/1471-2377-13-49

Published: 24 May 2013

Abstract

Background

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) was first described in 2010 by Pittock and colleagues. All reported patients presented with diplopia and gait ataxia and had similar typical MRI findings with punctuate gadolinium enhancement of the pons. Alternative diagnoses were excluded by means of laboratory, radiological and histological tests. All patients were successfully treated with steroids. We present a case in which the steroid therapy was switched to long term immunosuppressive therapy, leading to several severe side-effects, but sustained clinical improvement.

Case presentation

A 63-year-old male presented with sub-acute diplopia and progressive gait ataxia. During admission his neurological condition worsened and he developed multiple cranial nerve deficits, paraparesis and urine retention. MRI-findings were remarkable with punctuate enhancement with gadolinium of the pons. Cerebrospinal fluid only showed elevated protein levels and all other additional investigations were normal. The probable diagnosis of CLIPPERS was made and intravenous corticosteroids were administered. This led to rapid clinical recovery and decreased enhancement on the MRI-scan. Long-term oral immunosuppressive therapy was started. One-and-a-half year later our patient has no recurrence of neurological symptoms, however due to the side effects of the immunosuppressive therapy he was readmitted several times.

Conclusion

CLIPPERS presents with distinctive clinical and MRI-findings and may be diagnosed after excluding other differential diagnoses. Patients are treated with corticosteroids with good clinical results. Since short term glucocorticoid treatment results into relapse of the disease, longer term immunosuppressive therapy appears to be mandatory for sustained improvement, although accompanied by severe side effects.

Keywords:
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; CLIPPERS; Ataxia; Diplopia