Steroid pulse therapy of radiological disease activity without clinical relapse in CLIPPERS
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LETTER TO THE EDITOR
Steroid pulse therapy of radiological disease activity without clinical relapse in CLIPPERS Takashi Hosaka 1 & Kiyotaka Nakamagoe 1
&
Naoki Tozaka 1 & Satoshi Aizawa 1 & Akira Tamaoka 1
Received: 17 May 2019 / Accepted: 29 August 2019 # Fondazione Società Italiana di Neurologia 2019
Dear Editor: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory central nervous system disease with abnormal post-gadolinium enhancement called “peppering” in the pons, cerebellum, and spinal cord in magnetic resonance imaging (MRI), pathological perivascular T cell lymphocytic infiltration, and clinical and radiological responses to corticosteroids [1]. Despite the good response to corticosteroids, the reduction of corticosteroids (≤ 20 mg/day) easily leads to relapse [2]. Furthermore, some patients required other immunosuppressive agents because of relapses and/or corticosteroid side effects, and some patients had symptom relapse even if they took these immunosuppressive agents [3]. Although diagnosis criteria for CLIPPERS have been developed and clinical and radiological criteria for CLIPPERS without a biopsy have been proposed [4], no guidelines for long-term therapeutic strategies have been established, and clinical trials for the effective treatment of steroid pulse therapy and/or amounts of oral prednisolone are desired. In this report, we described a CLIPPERS patient who had worsened abnormal post-gadolinium enhancement without clinical recrudescence, namely “radiological relapse.” This is the first case of a good long-term outcome with steroid pulse therapy for radiological disease activity without clinical relapse. We would like to propose a CLIPPERS therapeutic strategy in which we perform periodic MRI and treat the radiological relapse with steroid pulse therapy regardless of the presence/absence of clinical relapse.
* Kiyotaka Nakamagoe [email protected] 1
Department of Neurology, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki 305-8575, Japan
A 52-year-old man presented with dizziness and progressive gait disturbance. When he was admitted to our hospital for vertigo and general fatigue 18 years ago, his brain MRI showed abnormal post-gadolinium enhancements like “peppering” in the pons, and he was treated with steroid pulse therapy. Although he had not had clinical recrudescence after the first administration without treatment for 14 years, he had worsened general fatigue for 3 months and developed dizziness and gait disturbance, then was readmitted to our hospital. He presented with saccadic eye movement, ataxic speech, gaze-evoked nystagmus, and gait disturbance due to spastic and ataxic gait. His deep tendon reflexes were generally exaggerated, and pathological reflexes, including the Hoffman reflex, tremor reflex, and Babinski reflex, were observed. Laboratory blood examination revealed only mild kidney injury (BUN 14.8 mg/dl, Cre 1.10 mg/dl) and no increase in the
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