Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with lesions distributed predominantly in spinal cord.
Brain(2015)
Abstract
Sir,
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a newly described immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010). The authors concluded that the characterized features of CLIPPERS are punctate and curvilinear pattern ‘peppering’ lesions within the pons on post-contrast T1-weighted images, together with an initial responsiveness to steroids therapy. Three of eight clinic patients in the original article were described foci of punctate gadolinium enhancement lesions extending down to spinal cord. To the best of our knowledge, the enhancing lesions distributed in pontine and peripontine regions, and some cases may additionally extend to the spinal cord, thalamus, basal ganglia, capsula interna and the cerebral white matter (Pittock et al. , 2010). However, cases with lesions predominantly distributed in spinal cord have not been reported, now we present a patient with lesions distributed mainly in the spinal cord and with lesions extending up to the medulla oblongata.
A previously healthy 53-year-old man noticed general weakness and abnormal fatigue for 2 weeks before admission. He then developed numbness of bilateral lower limbs, walking difficulties and incontinence for urine. There was no evidence of diplopia, gait ataxia or dysarthria. The past medical history and family history were negative. Neurological examination on admission revealed muscle strength score of bilateral lower limbs was 2 of 5 (Medical Research Council scale), tendon reflexes were decreased in the knees and ankles, hypoaesthesia of bilateral lower limbs, bilateral pathological reflexes were positive. The rest of the neurological examinations were normal.
Laboratory investigations revealed complete blood …
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Key words
pontine perivascular enhancement,chronic lymphocytic inflammation,spinal cord,steroids
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