= MYELINOCLASTIC DIFFUSE SCLEROSIS = SCHILDER DISEASE

= rare demyelinating disorder with episodic recurrence + remission

Age: children (5–14 years) >adults; M÷F = 1÷1

Histo: selective confluent demyelination with relative axonal sparing, perivascular inflammatory infiltrate, reactive astrocytosis (indistinguishable from multiple sclerosis)

• hemiplegia, aphasia, ataxia, blindness
• swallowing difficulties, progressive dementia
• increased intracranial pressure: headache, vomiting
• vision and speech impairment, deafness
• aphasia, seizures, personality changes, tremors, balance instability, incontinence, muscle weakness

Location: centrum semiovale in both hemispheres

• large well defined bilateral low-attenuation white matter lesions with mass effect
• enhancement with IV contrast material

Rx: usually responsive to corticosteroids

DDx:

1. Acute disseminated encephalomyelitis (history of recent viral illness, monophasic course, lesions less confluent, no mass effect / enhancement)
2. Adrenoleukodystrophy (bilaterally symmetric, confluent lesions, parietal location)
3. Tumor, abscess, infarct