Schilder's Disease: Case Study With Serial Neuroimaging

Abstract

Schilder's myelinoclastic diffuse sclerosis is a rare sporadic demyelinating disease that usually affects children between 5 and 14 years old. The disease often mimics intracranial neoplasm or abscess. We report a 9-year-old girl with Schilder's disease who presented with left hemiparesis. Cranial computed tomography and magnetic resonance imaging (MRI) showed large lesions in the subcortical white matter of the occipital and parietal lobes of both hemispheres that were indistinguishable from an abscess. A cerebrospinal fluid oligoclonal band test was found positive. A diagnosis of acute disseminated encephalomyelitis was then suspected. Serial MRI examinations revealed regression of parenchymal lesions, but there were new developing corpus callosum lesions. After 2 months, the patient presented with right-sided visual loss. MRI examination revealed a right optic nerve lesion. The patient had a dramatic clinical response to corticosteroid therapy. Subsequent control MRI examination revealed regression of both lesions. At 24 months of observation, the patient continued to do well without any complaints or neurologic sequelae.

Long-term Outcome of Schilder Disease Treated With Interferon-β

“…1 Because of its atypical presentations, Schilder disease often poses a diagnostic challenge. [2][3][4] Its course can be monophasic or relapsing, which is difficult to predict at the outset of the disease. Therefore, whether and when disease-modifying therapy (DMT) is helpful remains to be investigated.…”

An Autopsy Case of Schilder’s Variant of Multiple Sclerosis (Schilder’s Disease)

“…Mehler and Rabinowich [5] described a 12-year-old girl with bifrontal lesions involving the anterior corpus callosum. Kurul et al [8] reported a 9-year-old girl with lesions in bilateral parieto-occipital subcortical areas and genu and splenium of the corpus callosum. However, the pattern of extension to the corpus callosum seems to vary in each case.…”

Magnetic resonance imaging in the diagnosis of multiple sclerosis

“…Классическая болезнь Шильдера (периаксиальный диффузный лейкоэнцефалит) встречается крайне редко, в литературе описано 10 случаев заболевания, которые удовлетворяют диагностическим критериям [8]. Заболевание дебютирует в детском и молодом возрасте и клинически характеризуется острой психиатрической симптоматикой, внутричерепной гипертензией, интеллектуально-мнестическими нарушениями, двигательными нарушениями в виде геми-или тетрапарезов, дизартрией и дисфагией, уни-или билатеральной оптической нейропатией, гомонимной гемианопсией, прогрессивным ухудшением без повышения температуры [9].…”