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.

Case Reports in Medicine
|October 25, 2012
PubMed

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.

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