Recurrent myelinoclastic diffuse sclerosis: a case report of a child with Schilder's variant of multiple sclerosis

Area of Science:

• Neurology
• Neuroimmunology
• Pediatric Neurology

Background:

• Myelinoclastic diffuse sclerosis (MDS), also known as Schilder's disease, is a rare, severe, and often fatal demyelinating disease of the central nervous system (CNS).
• MDS primarily affects children and is characterized by extensive demyelination, typically presenting as large, unilateral or bilateral, symmetrical white matter lesions in the brain, often mimicking an intracranial mass lesion.

Observation:

• This report details the first documented case of recurrent intracranial MDS.
• The patient experienced a third episode of demyelination, which uniquely involved the cervical spinal cord in addition to the brain.

Findings:

• The recurrent demyelination extending to the cervical spinal cord in this case of MDS is a novel observation.
• This presentation suggests a potential variant of MDS, possibly a childhood form of multiple sclerosis (MS) or a distinct subset of the disease.

Implications:

• This finding expands the known clinical spectrum of myelinoclastic diffuse sclerosis.
• Recognizing this potential variant is crucial for accurate diagnosis and management of pediatric demyelinating disorders.
• Further research is warranted to delineate this potential Schilder's variant of multiple sclerosis and its unique characteristics.