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Published on: July 4, 2007
Adult Onset Acute Disseminated Encephalomyelitis: A Case Report
João R Corrêa1, Ana P Silva1, Joana Coelho1
1Internal Medicine, Centro Hospitalar Universitário Cova da Beira, Covilhã, PRT.
Abstract:
Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune demyelinating disorder of the central nervous system that can mimic other neurological diseases, such as multiple sclerosis. ADEM is thought to manifest in the presence of environmental triggers, namely viral or bacterial infections, with multiple simultaneous neurological deficits, frequently accompanied by encephalopathy. Here, we report the case of a 49-year-old female patient who presented in the emergency department with encephalopathy, right-side muscle weakness, dizziness, vertigo, ataxia, and postural imbalance, preceded by symptoms suggesting recent pharyngitis/sialadenitis three weeks prior. Cerebrospinal fluid analysis revealed lymphocytic pleocytosis, elevated protein levels, normal glucose levels, no oligoclonal bands, and culture and viral studies were negative. After a normal cerebral computed tomography, brain and cervical spine magnetic resonance imaging (MRI) revealed multiple, T2-weighted hyperintense supratentorial and infratentorial white matter lesions, including the right cerebellar peduncle and posterior limb of the left internal capsule. The diagnosis of ADEM was made, and the patient was treated with high-dose intravenous glucocorticoids followed by oral tapering with clinical improvement. During follow-up, the control MRI was compatible with the diagnosis. This case illustrates the diagnostic approach of a patient presenting with subacute neurological deficits and the importance of contemplating possible differential diagnoses and swiftly initiating treatment.
Insights
Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune brain disorder. Prompt diagnosis and high-dose glucocorticoid treatment led to clinical improvement in a patient with ADEM.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Acute disseminated encephalomyelitis (ADEM) is a rare, autoimmune demyelinating disorder of the central nervous system.
- ADEM often mimics other neurological conditions like multiple sclerosis and is triggered by infections.
Observation:
- A 49-year-old female presented with encephalopathy, motor deficits, dizziness, vertigo, and ataxia.
- Symptoms followed a recent pharyngitis/sialadenitis.
- Cerebrospinal fluid showed lymphocytic pleocytosis and elevated protein, with negative cultures and viral studies.
Findings:
- Brain and cervical spine MRI revealed multiple T2-weighted hyperintense white matter lesions.
- Diagnostic imaging confirmed lesions in the cerebellar peduncle and internal capsule.
- The patient was diagnosed with ADEM.
Implications:
- This case highlights the diagnostic challenges and differential diagnoses in patients with subacute neurological deficits.
- Early recognition and treatment with high-dose intravenous glucocorticoids are crucial for favorable outcomes in ADEM.
- Follow-up MRI confirmed the diagnosis and treatment efficacy.
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