CLIPPERS the brain biter: An adolescent’s experience of a rare CNS disorder
Introduction: CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a rare inflammatory Central Nervous System (CNS) disorder prominently affecting the brainstem. We present an adolescent’s reflection on this condition further complicated with E...
| Main Authors: | , , , |
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| Format: | Conference or Workshop Item |
| Language: | English English |
| Published: |
2019
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| Subjects: | |
| Online Access: | http://irep.iium.edu.my/75193/ http://irep.iium.edu.my/75193/7/PP269%20-%20Muhamad%20Azamin%20Anuar.pdf http://irep.iium.edu.my/75193/13/CLIPPERS%20-%2075193.pdf |
| Summary: | Introduction:
CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a rare inflammatory Central Nervous System (CNS) disorder prominently affecting the brainstem. We present an adolescent’s reflection on this condition further complicated with Epstein-Barr virus (EBV) induced CNS Lymphoma.
Case summary:
A 16-year-old boy presented about 5 years ago with a balance problem. He was diagnosed by magnetic resonance imaging after ongoing debate whether this is juvenile multiple sclerosis. He initially responded well to methylprednisolone but developed acute deterioration requiring 8 weekly cycle of Infliximab and Methylprednisolone for 8 cycles. He then confirmed cerebellar lymphoma 2 years later hence commenced on chemotherapy and cranial radiotherapy after posterior fossa decompression.
He gradually losses his motor skills, dense left hemiparesis and spasticity. He ended up with tracheostomy and gastrostomy due to poor bulbar function. Now, he is on full dependency care as he cannot sit unaided and needing regular pressure sore monitoring. He also requiring regular chest physiotherapy and suctioning and cystostomy for urinary incontinence. He has multiple PICU admissions due to recurrent aspiration, acute cerebellar oedema and post posterior fossa decompression.
His main worry at present is family breakdown as mum is his sole carer and having regular nightmare about death. He changed to new school to meet his care demands and unsure how to adapt to new environment. Social experience makes him anxious due to lack of understanding about his condition. He is evidently having low self-esteem and confidence that reflects his physical condition and social interaction.
Children palliative care team has been involved since diagnosis to support him and family. He is understandably depressed and clinical psychologist input bear minimal impact. He is fully aware of the current situation and his only wish at present is to rap Eminem’s songs like he used to do it before.
Conclusion:
Early aggressive treatment in CLIPPERS aim to prevent neuroaxonal loss. However, due to bellicose nature of the condition, the prognosis is relatively poor. Managing adolescent expectation after gradual deterioration is challenging especially, he is aware that achieving ‘normality’ is impossible as the treatment advancement still in limbo. |
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