This commentary is on the case series by Sa et al. Biopsy of an affected brain structure will primarily help to rule out important differential diagnoses. Clinical and Experimental Immunology 2014, 175: 336–48.Diagnosis, pathogenesis and treatment of myositis: recent advances. S.S. Korsakova,
4 years (range 16–86 years) Initial symptoms usually evolve subacutely over several weeks. Adult patients with atypical features may have a similarly unfavorable outcome.
on pages First described in 2010, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a predominantly brainstem‐based condition of unknown etiology.These cases present a diagnostic and therapeutic challenge.
Prompt pulse GCS treatment of relapses may limit clinical worsening during relapses and permanent neurological sequelae CLIPPERS is a newly described pontine‐centric inflammatory disorder with distinct clinical and radiological features.
SLE, rheumatoid arthritis), infections (e.g.
viral, bacterial, fungal, parasitic), malignancies (e.g. methylprednisolone 1 g over 5 days), followed by oral GCS. Although at least partial benefits were reported, their GCS‐independent efficacy could not been proved so far:
A case reportA new defined entity among the spectrum of central nervous system inflammation: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)CLIPPERS‐like MRI findings in a patient with multiple sclerosisCutting‐edge questions about CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)Serial stereotactic biopsy of brainstem lesions in adults improves diagnostic accuracy compared with MRI onlyA mysterious case: chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS) syndrome (Meeting abstract 364, American Federation for Medical Research and Participating Societies Regional Meetings: Southern Regional Program Abstracts, 2013)Requirement for safety monitoring for approved multiple sclerosis therapies: an overviewMonoclonal antibodies in treatment of multiple sclerosisDisease‐modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategiesThe expanding phenotype of CLIPPERS: is it a disease or a syndrome?Vasogenic edema in Bickerstaff's brainstem encephalitis: a serial MRI studyAn elderly patient with Bickerstaff brainstem encephalitis and transient episodes of brainstem dysfunctionCase of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids showing features common to multiple sclerosisChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): no evidence for antibodies to neuronal surface antigens (Meeting abstract 0918, Annual Meeting of the Association of British Neurologists, 2011)CLIPPERS: rare disease or tip of the iceberg? This patient was also subsequently found to have caudal root enhancement. Clinical and Experimental Immunology 2014, 175: 425–38. Due to the varied clinical presentation and the potential for diagnostic confusion, Simon Numerous punctate or nodular enhancing lesions bilaterally within at least two of the three following anatomical locations: pons, brachium pontis, cerebellumIndividual radiological lesions are small but may coalesce to form larger lesions (mass effect may suggest an alternative diagnosis)Lesions may occur in the spinal cord, basal ganglia or cerebral white matter but should be decreasing density with increasing distance from the pons/hindbrainAbsence of the following radiological features: Some key features that were previously found helpful in discriminating CLIPPERS from non‐CLIPPERS etiology are contrast‐enhancing lesions (<3mm and nonconfluent), symmetric post‐gadolinium‐enhancing lesions, and homogenous T2‐signal abnormality not significantly exceeding the area of post‐gadolinium T1 enhancement.
2017;19(5):17.
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