Abstract
A novel type of encephalomyelitis was first described as chronic lymphocytic inflammation
with pontine perivascular enhancement responsive to steroids (CLIPPERS) in 2010 and
few additional patients were reported since then. Partially due to its unknown aetiology
and a lack of pathognomonic features some have suggested that CLIPPERS may not represent
a distinct disease, but rather a syndrome with different underlying aetiologies. Here
we report a 49-year-old German female who presented with a number of clinical and
paraclinical features described as typical for CLIPPERS, while additionally showing
symptoms and findings compatible with primary angiitis of the CNS (PACNS). This case
may establish a previously unnoted link between two poorly understood autoimmune conditions
of the CNS.
Keywords
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References
- Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).Brain. 2010; 133: 2626-2634
- Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).J Neurol Neurosurg Psychiatry. 2012; 83: 15-22
- Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients.Arch Neurol. 2012; 69: 847-855
- The expanding phenotype of CLIPPERS: is it a disease or a syndrome?.J Neurol Neurosurg Psychiatry. 2012; 83: 2-3
- Primary angiitis of the CNS.Lancet Neurol. 2011; 10: 561-572
- Radiologically compatible CLIPPERS' may conceal a number of pathologies.Brain. 2011; 134: e187
- Brain biopsy is required in steroid-resistant patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).J Neurooncol. 2012; 107: 223-224
- Cutting-edge questions about CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids).Arch Neurol. 2012; 69: 819-820
Article info
Publication history
Published online: November 13, 2012
Accepted:
October 24,
2012
Received in revised form:
October 24,
2012
Received:
June 15,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
