Childhood demyelinating diseases with a prolonged remitting course and their relation to Schilder's disease: report of two cases

Leuzzi, Vincenzo; Lyon, G; Cilio, Maria Roberta; Pedespan, Jean Michel; Fontan, D; Chateil, Jean‐François; Vital, Anne

doi:10.1136/jnnp.66.3.407

Cited by 15 publications

(4 citation statements)

References 7 publications

(9 reference statements)

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“…These MRI findings differ from reported cases with MDS [1,3,7,16,21]. Histopathologic data from reported cases with MDS vary in the degree of detail but, in essence, they entail a well demarcated area of demyelination with reactive astrocytosis, macrophage infiltration and perivascular infiltration by lymphocytes consistent with a variant of MS [7,12,14,16,20]. Similar morphological findings were observed in the present case.…”

Section: Discussioncontrasting

confidence: 54%

See 1 more Smart Citation

Fulminant Course in a Case of Diffuse Myelinoclastic Encephalitis - A Case Report

Poppe¹,

Brück²,

Hahn³

et al. 2001

Neuropediatrics

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We report on a 10-year old previously healthy boy who exhibited a fulminant and nearly monophasic clinical course of demyelinating encephalitis with relapsing intracranial hypertension syndrome. Histologic examination of a diagnostic brain biopsy revealed an inflammatory demyelinating process with perivascular T lymphocytic infiltration and axonal damage reminiscent of multiple sclerosis-like lesions. In the brain, the DNA of human Herpes virus 6 (HHV6) was detectable. Eleven months after the initial symptoms and on maintainance with oral steroids, MRI showed demyelination of both hemispheres as well as demyelination of the brain stem and Wallerian degeneration. The boy exhibited a severe neurologic defect syndrome. The clinical and radiological course is unusual because of the asymmetric progression of the encephalitis and the extensive confluent lesions without demarcated border or enhancement of the rim after injection of gadolinium. The clinical course showed no definite steroid response. The pathogenetic relevance of HHV6 remains elusive. Although single patients with HHV6-associated encephalomyelitis have been reported, HHV6 DNA is occasionally detected in brains of healthy individuals.

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Section: Discussioncontrasting

confidence: 54%

“…The majority of authors describe good or at least partial clinical response to steroids in MDS and acute disseminated encephalomyelitis (ADEM) [1,3,6,7,9,12,14,16,19].…”

Section: Introductionmentioning

confidence: 99%

Fulminant Course in a Case of Diffuse Myelinoclastic Encephalitis - A Case Report

Poppe¹,

Brück²,

Hahn³

et al. 2001

Neuropediatrics

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“…These diseases consist of multiple sclerosis, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, and Schilder's disease. 1,3,4 In our case, a diagnosis of acute disseminated encephalomyelitis was suspected during the first attack because of the onset with impairment of consciousness, seizure, hemiparesis, and the appearance of oligoclonal bands in the cerebrospinal fluid. 5 But after the second attack, the possible diagnosis was multiple sclerosis or Schilder's disease.…”

Section: Discussionmentioning

confidence: 89%

Schilder's Disease: Case Study With Serial Neuroimaging

et al. 2003

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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.

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“…Additionally, it was noted that in SDs, CSF-restricted oligoclonal bands are generally absent, and that large bilateral areas of demyelination were mandatory for the diagnosis. 14 Some cases of SD represent adrenoleukodystrophy or “transitional” cases with MS. Adrenoleukodystrophy has been ruled out through clinical presentation, biochemical tests, and PAS.…”

Section: Discussionmentioning

confidence: 99%

Balo's concentric lesions with concurrent features of Schilder s disease in relapsing multiple sclerosis: neuropathological findings

Kurdi¹,

Ramsay²

2016

ACR

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Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo’s concentric sclerosis (BCS) and Schilder’s disease (SD) are two of these syndromes and can present as monophasic or in association with chronic MS. Both variants are difficult to distinguish when they present in acute stages. We describe an autopsy case of middle-aged female with a chronic history of MS newly relapsed with atypical demyelinating lesions, which showed concurrent features of BCS and SD. We also describe the neuropathological findings, and discuss the overlapping features between these two variants.

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Childhood demyelinating diseases with a prolonged remitting course and their relation to Schilder's disease: report of two cases

Cited by 15 publications

References 7 publications

Fulminant Course in a Case of Diffuse Myelinoclastic Encephalitis - A Case Report

Fulminant Course in a Case of Diffuse Myelinoclastic Encephalitis - A Case Report

Schilder's Disease: Case Study With Serial Neuroimaging

Balo's concentric lesions with concurrent features of Schilder s disease in relapsing multiple sclerosis: neuropathological findings

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