Acute Disseminated Encephalomyelitis
Conditions
Brief summary
Acute Disseminated Encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder of the central nervous system that predominantly affects children. It typically presents with an acute onset of multifocal neurological symptoms, often preceded by a viral infection or, less commonly, vaccination. ADEM is characterized radiologically by widespread, bilateral, asymmetric lesions in the brain and spinal cord, and is often monophasic in nature. Despite generally favorable outcomes, a subset of patients may experience significant neurological sequelae, prolonged recovery, or even conversion to chronic demyelinating disorders such as multiple sclerosis (MS) or multiphasic ADEM. The early identification of patients at risk for poor outcomes remains a clinical challenge, as the course of the disease is highly variable. Cerebrospinal fluid (CSF) analysis and magnetic resonance imaging (MRI) are essential components in the diagnostic workup of ADEM. Certain CSF features-such as pleocytosis, elevated protein levels, or the presence of oligoclonal bands-may reflect the underlying immunological activity and CNS inflammation. Similarly, specific MRI characteristics-such as lesion distribution, size, contrast enhancement, or involvement of deep gray matter-may correlate with disease severity and long-term prognosis. The clinical presentation of ADEM is heterogeneous. Common features include encephalopathy (ranging from irritability to coma), seizures, motor deficits, ataxia, visual disturbances, and brainstem symptoms. The severity and combination of these manifestations can vary widely between patients. Several studies suggest that certain clinical features may correlate with poorer prognosis, such as prolonged or deep coma, recurrent seizures, early need for intensive care, and delayed initiation of immunotherapy.
Detailed description
* To evaluate the prognostic value of cerebrospinal fluid (CSF) and magnetic resonanceimaging (MRI) findings at initial presentation in predicting six months neurological outcomes in pediatric patients diagnosed with ADEM. * Secondary aim : correlation between clinical presentation and prognosis.
Interventions
None listed
Sponsors
Study design
Eligibility
Inclusion criteria
1. \- First episode of acute disseminated encephalomyelitis( ADEM). 2. \- MRI and cerebrospinal fluid (CSF) performed within 7 days of symptom onset. 3. \- Age from 6 months up to 18 years
Exclusion criteria
1. \- History of prior demyelinating events 2. \- Alternative diagnoses (e.g., CNS infections, metabolic disorders).
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Neurological status assessed by the National Institutes of Health Stroke Scale (NIHSS) | At 3 months, and 6 months | The NIHSS quantifies neurological impairment. Scores range from 0 (no deficit) to 42 (severe stroke). Higher scores indicate worse neurological status. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Functional outcome assessed by the Modified Rankin Scale (mRS) | At 3 months and 6 months | The mRS measures the degree of disability or dependence in daily activities. Scores range from 0 (no symptoms) to 6 (death). Higher scores indicate worse functional outcomes. |