[A case of prolonged paroxysmal sympathetic hyperactivity]
Insights
Paroxysmal sympathetic hyperactivity (PSH) in a child, a rare condition, was managed with various medications. Some drugs effectively reduced attacks, while others did not, highlighting varied treatment responses.
Area of Science:
- Pediatric Neurology
- Critical Care Medicine
- Neuropharmacology
Background:
- Paroxysmal sympathetic hyperactivity (PSH) is a rare but severe condition, often following hypoxic-ischemic encephalopathy.
- Prompt diagnosis and effective management are crucial for improving patient outcomes.
Observation:
- A 4-year-old girl with PSH post-cardiopulmonary arrest exhibited significant sympathetic overactivity and dystonia.
- Treatment involved multiple medications, with morphine, bromocriptine, propranolol, and clonidine showing efficacy in reducing attack frequency.
Findings:
- Gabapentin, baclofen, dantrolene, and benzodiazepines were ineffective in managing the patient's PSH symptoms.
- Plasma catecholamine levels correlated with PSH episodes, rising during attacks and normalizing between them.
- Complete control of PSH paroxysms was not achieved, with symptoms persisting beyond 500 days.
Implications:
- This case highlights the challenges in pediatric PSH management and the differential drug responses observed.
- Understanding plasma catecholamine fluctuations provides insights into PSH pathophysiology.
- Further case studies are needed to refine treatment strategies and elucidate the underlying mechanisms of PSH.
Abstract:
We report the case of a 4-year-old girl who presented with paroxysmal sympathetic hyperactivity (PSH), after developing severe hypoxic-ischemic-encephalopathy because of cardiopulmonary arrest. She showed dramatic paroxysmal sympathetic activity with dystonia. She was treated with wide variety of medications against PSH, which were found to be effective in previous studies. Among them, morphine, bromocriptine, propranolol, and clonidine were effective in reducing the frequency of her attacks while gabapentin, baclofen, dantrolene, and benzodiazepine were ineffective. Though the paroxysms decreased markedly after the treatment, they could not be completely controlled beyond 500 days. Following the treatment, levels of plasma catecholamines and their urinary metabolites decreased to normal during inter- paroxysms. However, once a paroxysm had recurred, these levels were again very high. This case study is considered significant for two rea- sons. One is that PSH among children have been rarely reported, and the other is that this case of prolonged PSH delineated the transition of plasma catecholamines during the treatment. The excitatory: inhibitory ratio (EIR) model proposed by Baguley was considered while dis- cussing drug sensitivity in this case. Accumulation of similar case studies will help establish more effective treatment strategies and elucidate the pathophysiology of PSH.
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