Isolated brain stem lesion in children: is it acute disseminated encephalomyelitis or not?

G Alper1, G Sreedher, G Zuccoli

  • 1Division of Child Neurology, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. gulay.alper@chp.edu

Insights

Acute demyelinating lesions in the brain stem can be challenging to diagnose in children. This study suggests that some isolated brain stem lesions may represent a rare, localized form of acute disseminated encephalomyelitis (ADEM).

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Demyelinating Diseases

Background:

  • Isolated brain stem lesions with acute neurological symptoms pose a diagnostic challenge in pediatric patients.
  • While acute disseminated encephalomyelitis (ADEM) often involves the brain stem, solitary lesions are uncommon.

Purpose of the Study:

  • To investigate the characteristics and outcomes of children presenting with inflammatory lesions confined to the brain stem.
  • To determine if these cases represent a distinct clinical entity or a variant of ADEM.

Main Methods:

  • Retrospective review of 6 pediatric patients with brain stem-confined inflammatory lesions.
  • Clinical assessment, neuroimaging (MRI), and diagnostic workup for underlying etiologies.
  • Evaluation of treatment response, particularly to high-dose corticosteroids.

Main Results:

  • Two patients were diagnosed with connective tissue disorders (CNS lupus, localized scleroderma).
  • One case had an undetermined etiology, and three cases showed features of monophasic demyelination.
  • The three monophasic cases exhibited vasogenic edema, responded dramatically to corticosteroids, and achieved full recovery with no relapses.
  • Follow-up MRI in these three cases showed complete lesion resolution.

Conclusions:

  • Monofocal demyelination confined to the brain stem can occur in children.
  • These cases, particularly those with a monophasic course and good response to steroids, are best classified as a localized form of ADEM.
  • ADEM, typically multifocal, can rarely present as a solitary brain stem lesion.

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