Learning objectives
Identify the CLIPPERS syndrome Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids
Guide the diagnosis of CLIPPERS syndrome
Refresh the anatomical structures associated with CLIPPERS syndrome
Background
The CLIPPERS syndrome first describe for Pittock et al in 2010 as a brainstem encephalitis with a characteristic pattern of gadolinium enhancement on MRI, manifest by hyperintense punctiform lesions in T2 and FLAIR with contrast enhancement in T1 the bridge and midbrain has a particular punctiform pattern and may affect spinal cord and base ganglia.
Subacute pontocerebellar dysfunction, with or without CNS symptoms, cognitive dysfunction or myelopathy
Neurological symptoms that respond to corticosteroid therapy
There ir absence of peripheral nervous system disease, and lack of...
Findings and procedure details
Incidence an prevalence:
From 15 to 85 years , Both genders
Clinical presentation
Unstable gait, Dysarthria, Diplopia, Dizziness,, Nausea, Tinnitus, Tremor, Nystagmus, Paraparesis, Cognitive impairment
Subacute pontocerebellar dysfunction, with or without CNS symptoms, cognitive dysfunction or myelopathy
Neurological symptoms that respond to corticosteroid therapy
There ir absence of peripheral nervous system disease, and lack of a better alternative explanation for the clinical presentation
Tobin et al 2017 propose the Diagnostic criteria for the differentiation of CLIPPERS from its mimics
CLIPPERS defined: Patient that meets all...
Conclusion
Conclution
The radiological distribution of CLIPPERS is focused in the pons and adjacent rhombencephalic structures such as the cerebellar peduncles, medulla and the midbrain. The most important criteria is the clinical as well as radiologic responsiveness to glucocorticosteroid (GCS) based immunosuppression
MS, neurosarcoidosis, CNS lymphoma or CNS vasculitis are the main differential diagnosis.
Diagnosis is based on a combination of clinical, radiological and laboratory investigations
Personal information and conflict of interest
J. M. Hernandez Herrera; Sjo/CR - nothing to disclose
References
1.Pittock, SDebruyne, JKrecke, KNet al.(2010).Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).Brain133: 2626–2634, DOI:https://doi.org/10.1093/brain/awq164
2.Dudesek, ARimmele, FTesar, Set al.(2013).CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders.British society for immunology, Clinical and experimental immunology175: 385–396, DOI:https://doi.org/10.1111/cei.12204
3.Simon, NParratt, JDBarnett, MHet al.(2012).Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).J Neurol Neurosurg Psychiatry83: 15–22, DOI:https://doi.org/10.1136/jnnp-2011-301054
4.Wijntjes,...