Multiphasic acute disseminated encephalomyelitis and differential with early onset multiple sclerosis

Area of Science:

• Neurology
• Pediatrics
• Neuroimmunology

Background:

• Multiple sclerosis is a common CNS demyelinating disorder in young adults, rarely seen before age 10.
• Acute disseminated encephalomyelitis (ADEM) is a monophasic CNS demyelinating disorder often following viral infections.
• Both conditions can mimic acute focal neurological syndromes, posing diagnostic difficulties.

Purpose of the Study:

• To present a pediatric case illustrating the diagnostic challenges in differentiating multiple sclerosis and ADEM.
• To emphasize the similarities in clinical presentation and neuroimaging between these two demyelinating diseases in childhood.

Main Methods:

• Case report of a nine-year-old girl with recurrent demyelinating episodes.
• Clinical assessment including neurological examination and brain MRI.
• Review of differential diagnostic criteria for pediatric demyelinating disorders.

Main Results:

• The patient presented with dysphonia, gait ataxia, eyelid myokymia, and brainstem disturbances at ages four and nine.
• Brain MRI revealed multiple demyelinating lesions in various CNS locations.
• The clinical and imaging findings were challenging to distinguish between MS and ADEM, particularly for the initial episode.

Conclusions:

• Pediatric demyelinating diseases like MS and ADEM can present with overlapping clinical and neuroimaging features.
• Diagnostic uncertainty is heightened during initial episodes, necessitating careful evaluation.
• This case underscores the importance of considering both entities in the differential diagnosis of acute neurological syndromes in children.