Fact-sheet: CLIPPERS Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
Updated on 10/03/2018 at 2:39 PM
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Definition
CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a rare inflammatory disorder of the central nervous system.
Its etiology is unknown (immune system dysfunction).
This entity was first described in 2010.
The mean age at diagnosis is between 40 and 50 years, with a slight male predominance.
It preferentially involves the pons but may extend to the cerebellar peduncles or hemispheres, the midbrain, the basal ganglia, and the spinal cord.
It is a diagnosis of exclusion.
Clinical features
Neurologic signs are variable:
- cerebellar ataxia,
- dysarthria,
- diplopia.
The disease course is subacute.
CT
Brain CT is most often normal.
MRI
Brain MRI plays an essential role in the diagnosis:
- multiple, punctate and curvilinear T2 hyperintensities.
- the lesions enhance after gadolinium injection. Enhancement is curvilinear or punctate, "peppering the pons".
- no abnormality on T1, or late atrophy of the pons, cerebellum, or spinal cord.
Vascular intervention
Cerebral X-Ray Angiography, when performed (in case of diagnostic uncertainty), is normal.
Management
Brain MRI most often allows the diagnosis to be suggested.
Treatment is based on corticosteroid administration.
Corticosteroid dependence often develops, requiring the use of immunosuppressive therapy.
Spontaneous remissions may occasionally occur in the absence of treatment.
Differential diagnosis
Numerous differential diagnoses:
- Neurosarcoidosis
- Intravascular lymphoma
- Lymphomatoid granulomatosis
- Cerebral vasculitis
- Paraneoplastic syndromes
- Glioblastomas
- Chronic infections with perivascular involvement (tuberculosis, neurosyphilis, Whipple disease, etc.)
- Demyelinating disorders of the central nervous system
- Central nervous system histiocytosis