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Expanding the Clinical, Radiological and Neuropathological Phenotype of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)
JNNP 83:15-22, Simon, N.G.,et al, 2012
See this aricle in Pubmed

Article Abstract
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the CNS with a predilection for the hindbrain and responsive to immunotherapy. Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. We propose that the title of the syndrome be amended to chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids to more accurately reflect the distribution of the radiological findings.
 
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axonal degeneration
axonal injury
brachium pontis
brain biopsy
cerebellar lesion
cerebellum
cerebral cortical atrophy
chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
cognition
dementia
gadolinium
MRI,abnormal
MRI,contrast enhanced
MRI,nodular enhancement
neuroaxonal degeneration
neuropathology
perivascular enhancement
pons,atrophy
pons,lesion of
pontocerebellar atrophy
prognosis
steroid therapy,CNS treatment and complications with
treatment of neurologic disorder

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