Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A pediatric case report with six year follow-up

Is chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in children the same condition as in adults?

“…All patients experienced a recurrence of both symptoms and radiological progression soon after steroid tapering. This was consistent with previous reports both in adult and paediatric patients . Brain biopsies revealed neuro‐axonal injury in addition to extensive lymphohistiocytic involvement, which may explain the disability and cerebral atrophy.…”

“…All patients fulfilled the recently published diagnostic criteria of CLIPPERS . In these three patients, disease progression was more severe than in previously reported adult and paediatric cases (Table ). Two of the patients described died after multiple relapses on immunotherapies and the third developed CNS B‐cell lymphoma.…”

“…This was consistent with previous reports both in adult 1 and paediatric patients. 6,8 Brain biopsies revealed neuroaxonal injury in addition to extensive lymphohistiocytic involvement, which may explain the disability and cerebral atrophy. The progressive loss of tissue integrity, which is likely to result in secondary neuroaxonal injury, may explain the reduced response to immunotherapies over time.…”

“…2,3 However, in a series of 15 adult patients, four died a median of 27 months from symptom onset. 4 Only a few cases of paediatric CLIPPERS have been reported [5][6][7][8] and only one case with a pathological description. 8 Disease progression and outcome in paediatric cases were thought to be similar to adult patients with a non-progressive course and sequelae correlated with severe relapses.…”

“…4 Only a few cases of paediatric CLIPPERS have been reported [5][6][7][8] and only one case with a pathological description. 8 Disease progression and outcome in paediatric cases were thought to be similar to adult patients with a non-progressive course and sequelae correlated with severe relapses. 2 We report three cases with a clinical, radiological, and pathological phenotype consistent with CLIPPERS.…”