Related Experiment Videos
[Acute disseminated encephalomyelitis in a 3-month-old infant]
T Taketani1, M Kimura, K Kishi
1Department of Pediatrics, Shimane Medical University, Izumo, Shimane.
Insights
Acute disseminated encephalomyelitis (ADEM) is rare in infants. This case highlights successful treatment of a 3-month-old with ADEM using high-dose gamma-globulin therapy, showing significant symptom improvement.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute disseminated encephalomyelitis (ADEM) is an autoimmune-mediated demyelinating disease affecting the central nervous system.
- Early-onset ADEM, particularly before one year of age, is exceptionally rare.
Observation:
- A 3-month-old infant presented with somnolence, poor feeding, vomiting, and neurological deficits including impaired visual and auditory responses and decreased muscle tone.
- Cerebrospinal fluid analysis revealed pleocytosis, elevated protein, and positive myelin basic protein. EEG showed diffuse slow wave activity, and auditory brainstem response indicated significant dysfunction.
- MRI demonstrated multifocal demyelinating lesions in the internal capsule, cerebellum, and brainstem.
Findings:
- The infant was diagnosed with ADEM and treated with high-dose gamma-globulin therapy due to contraindications for corticosteroids (hypertension).
- Clinical symptoms showed continuous improvement post-treatment.
- Two-year follow-up revealed normal growth and development with no recurrence of ADEM.
Implications:
- High-dose gamma-globulin therapy is a viable alternative treatment for ADEM in very young infants or those with contraindications to corticosteroids.
- This case expands the understanding of rare early-onset ADEM and its management in infants.
- Early diagnosis and appropriate treatment are crucial for favorable outcomes in pediatric ADEM.
Abstract:
Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease showing multifocal central nervous system lesions due to an autoimmune disorder. We reported a 3-month-old girl with ADEM. One week after having a cold, she presented with somnolence, poor feeding and vomiting. When she was admitted three days after the onset, she could neither fix or follow objects with her eyes nor respond to sound. Her muscle tone was decreased. Cerebrospinal fluid examination revealed pleocytosis, elevated protein concentration and positive myelin basic protein. No oligoclonal band was detected. Diffuse monomorphic slow wave activity was noted on the electroencephalogram. Only wave I was present bilaterally on the auditory brainstem response. T2 weighted images of magnetic resonance imaging revealed multiple areas of high signal in the right posterior limb of the internal capsule, white matter of the cerebellum and brainstem. She was diagnosed as having ADEM, and underwent high dose gamma-globulin therapy. Corticosteroids were not given because of her high blood pressure. The clinical symptoms improved continuously before and after the administration. Two years after the onset, she showed normal growth and development without reoccurrence. The age at onset of childhood ADEM is usually 3 or 4 years. ADEM before one year of age is very rare. The demyelinating lesions of this case corresponded to the regions which normally become myelinated by 3 months. Although ADEM is usually treated with corticosteroids, high dose gamma 1-globulin therapy can be considered if patients are very young or have a high risk for corticosteroid, or respond poorly to corticosteroids.