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Brainstem multiple sclerosis in an 11-year-old child presenting as acute disseminated encephalomyelitis
S P Mostafapour1, D Enzmann, W North
1Department of Diagnostic Radiology, Stanford University School of Medicine, CA 94305-5235, USA.
Insights
Distinguishing acute disseminated encephalomyelitis from multiple sclerosis can be challenging. This case highlights diagnostic difficulties in acute focal demyelinating syndromes, emphasizing the need for careful evaluation.
Area of Science:
- Neurology
- Neuroimmunology
Background:
- Multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) are demyelinating central nervous system disorders.
- Both can initially manifest as acute focal demyelinating syndromes, complicating diagnosis.
Observation:
- An 11-year-old girl presented with vomiting, hypertension, and focal brainstem signs.
- MRI revealed a solitary brainstem demyelinating lesion consistent with ADEM.
Findings:
- Empirical treatment with corticosteroids led to near-full recovery.
- A subsequent episode with new lesions 6 months later prompted an MS diagnosis due to temporal separation and CNS location.
Implications:
- This case illustrates the diagnostic challenges in differentiating ADEM from MS in initial presentations of acute focal demyelinating syndromes.
- It underscores the importance of longitudinal monitoring for accurate diagnosis and management of demyelinating disorders.
Abstract:
Multiple sclerosis and acute disseminated encephalomyelitis are demyelinating disorders of the central nervous system that can present initially as an acute focal demyelinating syndrome. We report an 11-year-old girl who initially presented with intractable vomiting and hypertension and later developed a subacute onset of focal neurologic (brainstem) signs. Magnetic resonance imaging (MRI) demonstrated a large solitary demyelinating lesion of the brain stem consistent with acute disseminated encephalomyelitis. Because of the morbidity associated with biopsy and its questionable value in the course of management of this patient, she was treated empirically with aggressive supportive measures and high-dose corticosteriod therapy. She had near full recovery, with only minimal neurologic sequelae. Six months later, she presented with similar focal neurologic signs, and a new lesion was found on MRI. Because of the separation of her two episodes in time and central nervous system location, a diagnosis of multiple sclerosis was made. Herein, we used this patient to illustrate the difficulty in distinguishing acute disseminated encephalomyelitis from multiple sclerosis in patients who present initially with an acute focal demyelinating syndrome.