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Paroxysmal autonomic instability with dystonia after pneumococcal meningoencephalitis
Layal Safadieh1, Rana Sharara-Chami, Omar Dabbagh
1Division of Neurology, Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center, P.O. Box 11-0236, Riad El Solh, Beirut 1107 2020, Lebanon.
Insights
This case study details a rare instance of paroxysmal autonomic instability with dystonia in an infant following Streptococcus pneumoniae meningoencephalitis, highlighting a novel association and treatment response.
Area of Science:
- Neurology
- Infectious Diseases
- Pediatrics
Background:
- Streptococcus pneumoniae is a leading cause of bacterial meningitis with potential for severe neurological sequelae.
- Paroxysmal autonomic instability with dystonia (PAID) is a rare condition typically associated with traumatic brain injury or other CNS infections.
- Pneumococcal meningoencephalitis can lead to significant neurological impairment.
Purpose of the Study:
- To report the first known case of paroxysmal autonomic instability with dystonia (PAID) secondary to Streptococcus pneumoniae meningoencephalitis.
- To describe the clinical presentation and management of PAID in an infant.
- To highlight a novel association between pneumococcal infection and this autonomic disorder.
Main Methods:
- Case report of a seven-month-old infant.
- Clinical observation of neurological and autonomic symptoms including agitation, hypertension, tachycardia, diaphoresis, hyperthermia, and decerebrate posturing.
- Diagnosis of paroxysmal autonomic instability with dystonia (PAID).
- Treatment with clonidine, baclofen, and benzodiazepines.
Main Results:
- The infant developed right basal ganglia and hypothalamic infarctions following Streptococcus pneumoniae meningoencephalitis.
- Daily episodes of severe autonomic dysfunction and dystonic posturing were observed.
- The patient showed a positive response to pharmacotherapy including clonidine, baclofen, and benzodiazepines.
Conclusions:
- This is the first reported case of paroxysmal autonomic instability with dystonia (PAID) associated with pneumococcal meningoencephalitis.
- The findings suggest that pneumococcal meningoencephalitis is a potential cause of PAID.
- Pharmacological management with clonidine, baclofen, and benzodiazepines can be effective in treating PAID.
Abstract:
Streptococcus pneumoniae is a common cause of bacterial meningitis, frequently resulting in severe neurological impairment. A seven-month-old child presenting with Streptococcus pneumoniae meningoencephalitis developed right basal ganglia and hypothalamic infarctions. Daily episodes of agitation, hypertension, tachycardia, diaphoresis, hyperthermia, and decerebrate posturing were observed. The diagnosis of paroxysmal autonomic instability with dystonia was established. The patient responded to clonidine, baclofen, and benzodiazepines. Although this entity has been reported in association with traumatic brain injury, and as a sequel to some nervous system infections, this is the first case, to our knowledge, associated with pneumococcal meningoencephalitis.
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