Acquired demyelinating syndromes encompass both acute and chronic immune-mediated disorders of the central nervous system (CNS) and include acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), transverse myelitis, multiple sclerosis (MS), and neuromyelitis optica (NMO). These disorders are all characterized by CNS inflammation. Although these syndromes overlap in their neurologic symptoms, the clinical severity, radiologic features, response to treatment, and prognosis for neurologic recovery vary between the disorders.
Given the complexity of acquired demyelinating syndromes, pediatric providers must have a high index of suspicion for these disorders. Subtle neurologic symptoms may be difficult to identify in young children. For example, sensory and visual symptoms are often difficult for young children to describe and may not be brought to the attention of a care provider or parent unless they are severe or impacting activities of daily living.
Patients with symptoms concerning for an acute or chronic demyelinating disorder require prompt evaluation so that a diagnosis can be confirmed and treatment initiated. This chapter reviews clinical features, differential diagnosis, and management of demyelinating disorders in the inpatient setting and then describes the prototypical acquired demyelinating syndromes in greater detail, highlighting the unique features of the disorders.
Acquired demyelinating syndromes cause various symptoms that localize to the CNS. Common symptoms include vision loss or visual disturbance (such as blurry or double vision), sensory abnormalities (including numbness or tingling), weakness, balance difficulties, gait abnormalities, and bowel and bladder dysfunction. Neurologic symptoms may localize to one particular area of the CNS (termed monofocal); however, symptoms and signs can affect various regions (polyfocal) and reflect the presence of diffuse inflammation throughout the CNS.
DIFFERENTIAL DIAGNOSIS OF DEMYELINATING DISORDERS IN CHILDREN
The differential diagnosis for an acute neurologic event in a previously well child is extensive and must be tailored to the individual’s clinical history and exam findings. Acquired demyelinating syndromes are diagnosed based on clinical criteria, with accompanying supportive laboratory and radiographic features. The prototypical demyelinating disorders are listed in Table 122-1 and discussed in greater detail later in the chapter. However, diagnosis of a demyelinating syndrome is often difficult at presentation, and other etiologies, such as infection, rheumatologic processes, and malignancy must be carefully considered prior to making the diagnosis. Table 122-2 provides a differential diagnosis that may be considered in the appropriate clinical context as well as supportive laboratory and imaging features to determine whether an alternative diagnosis exists.
TABLE 122-1Characteristic Features of Pediatric Acquired Demyelinating Syndromes |Favorite Table|Download (.pdf) TABLE 122-1 Characteristic Features of Pediatric Acquired Demyelinating Syndromes
|Diagnosis ||Clinical Features ||Radiographic Features ||CSF and other Labs ||Demographics |
|Acute disseminated encephalomyelitis (ADEM) || |
Acute encephalopathy with polyfocal neurologic symptoms
Seizures, fever, headache can occur
Lesions are asymmetric, large, and often have poorly defined borders
Visible lesions in the deep gray matter ...