A 67 year old female presented with dizziness and vision changes. MRI showed enhancing lesions throughout the brain and spinal cord. Numerous punctate lesions were clustered in the pons, though enhancement was also noted in the supratentorial brain and cervical/thoracic cord (Figure 1). Workup for a source of malignancy and CSF studies were unrevealing. Brain biopsy demonstrated lymphocytic infiltrate with T-cell predominance in the perivascular and parenchymal spaces.
Figure 1.
Varied Sites of Enhancement in CLIPPERS. In (A) and (B), post-contrast coronal images demonstrate oval supratentorial enhancement in the right thalamus and frontal lobe. In (C), the typical punctate enhancement pattern is shown in the pons and cerebellum. In (D), post-contrast images of the cervical spine demonstrate disease involvement. There was also involvement of the thoracic cord (not shown).
The patient was diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Steroid initiation resulted in symptomatic improvement and resolution of the enhancing lesions on MR.
CLIPPERS is an uncommon, recently described cause of abnormal intracranial enhancement with a hallmark of clinical and radiologic corticosteroid responsiveness. 1 Presenting symptoms commonly include gait ataxia, diplopia, dysarthria, and altered sensorium in the extremities. Classic imaging findings include punctate pontine and cerebellar enhancement on MR; CNS lesions outside of the pons and cerebellum can be seen though are less common. 2
The pathogenesis of CLIPPERS remains uncertain. Histologic findings are notable for a perivascular T-cell predominant inflammatory infiltrate responsive to steroids, suggesting an immune mediated process. 3 We present a case of CLIPPERS with classic imaging findings in the pons, and with additional enhancement in the supratentorial brain and spinal cord.
Footnotes
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
ORCID iD
Michael Thomas https://orcid.org/0009-0004-9594-2128
References
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