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

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Childhood demyelinating diseases with a prolonged remitting course and their relation to Schilder’s disease: report of two cases
  1. VINCENZO LEUZZI
  1. Dipartimento di Scienze Neurologiche e Psichiatriche dell’Età Evolutiva, Università La Sapienza, Roma, Italy
  2. Service de Neuropédiatrie, Université Catholique de Louvain, Bruxelles, Belgium
  3. Unité de Neurologie Infantile
  4. Unité de Neuroradiologie
  5. Unité de Neuropathologie, Centre Hospitalier Pellegrin, Bordeaux, France
  1. Dr Vincenzo Leuzzi, Istituto di Neuropsichiatria Infantile, Via dei Sabelli 108, 00185 Roma, Italy. Telephone 0039 6 44712221; fax 0039 6 4957857; emailleuzziv{at}shareware.it
  1. GILLES LYON,
  2. MARIA ROBERTA CILIO
  1. Dipartimento di Scienze Neurologiche e Psichiatriche dell’Età Evolutiva, Università La Sapienza, Roma, Italy
  2. Service de Neuropédiatrie, Université Catholique de Louvain, Bruxelles, Belgium
  3. Unité de Neurologie Infantile
  4. Unité de Neuroradiologie
  5. Unité de Neuropathologie, Centre Hospitalier Pellegrin, Bordeaux, France
  1. Dr Vincenzo Leuzzi, Istituto di Neuropsichiatria Infantile, Via dei Sabelli 108, 00185 Roma, Italy. Telephone 0039 6 44712221; fax 0039 6 4957857; emailleuzziv{at}shareware.it
  1. JEAN MICHEL PEDESPAN,
  2. DANIEL FONTAN
  1. Dipartimento di Scienze Neurologiche e Psichiatriche dell’Età Evolutiva, Università La Sapienza, Roma, Italy
  2. Service de Neuropédiatrie, Université Catholique de Louvain, Bruxelles, Belgium
  3. Unité de Neurologie Infantile
  4. Unité de Neuroradiologie
  5. Unité de Neuropathologie, Centre Hospitalier Pellegrin, Bordeaux, France
  1. Dr Vincenzo Leuzzi, Istituto di Neuropsichiatria Infantile, Via dei Sabelli 108, 00185 Roma, Italy. Telephone 0039 6 44712221; fax 0039 6 4957857; emailleuzziv{at}shareware.it
  1. JEAN-FRANÇOIS CHATEIL
  1. Dipartimento di Scienze Neurologiche e Psichiatriche dell’Età Evolutiva, Università La Sapienza, Roma, Italy
  2. Service de Neuropédiatrie, Université Catholique de Louvain, Bruxelles, Belgium
  3. Unité de Neurologie Infantile
  4. Unité de Neuroradiologie
  5. Unité de Neuropathologie, Centre Hospitalier Pellegrin, Bordeaux, France
  1. Dr Vincenzo Leuzzi, Istituto di Neuropsichiatria Infantile, Via dei Sabelli 108, 00185 Roma, Italy. Telephone 0039 6 44712221; fax 0039 6 4957857; emailleuzziv{at}shareware.it
  1. ANNE VITAL
  1. Dipartimento di Scienze Neurologiche e Psichiatriche dell’Età Evolutiva, Università La Sapienza, Roma, Italy
  2. Service de Neuropédiatrie, Université Catholique de Louvain, Bruxelles, Belgium
  3. Unité de Neurologie Infantile
  4. Unité de Neuroradiologie
  5. Unité de Neuropathologie, Centre Hospitalier Pellegrin, Bordeaux, France
  1. Dr Vincenzo Leuzzi, Istituto di Neuropsichiatria Infantile, Via dei Sabelli 108, 00185 Roma, Italy. Telephone 0039 6 44712221; fax 0039 6 4957857; emailleuzziv{at}shareware.it

https://doi.org/10.1136/jnnp.66.3.407

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    Schilder’s disease or myelinoclastic diffuse sclerosis is a rare acute or subacute demyelinating disorder which primarily affects children and young adults.1 2 We report the clinical and neuroradiological follow up of two boys affected by a demyelinating disease with a prolonged relapsing-remitting course, response to corticosteroids, and relatively good long term prognosis.

    Brain MRI of patient 1 at the age of 14.5 years. The T 2 weighted image (TR 2000/TE 50) shows a high intensive signal in the parieto-occipital white matter, involving the right centrum semiovale, with mass effect.

    The first patient presented at the age of 12 with a 2 month history of repeated episodes of headache and blurred vision followed by weakness in the left leg, lasting a few hours. Head CT and bilateral carotid angiography were normal. Two weeks later the left hemiparesis and headache recurred. T2 weighted images on brain MRI disclosed a hyperintense signal in the right parieto-occipital white matter of the centrum semiovale, without a mass effect. Flash visual evoked stimuli elicited a decreased potential on the left side. Motor and sensory nerve conduction were normal. Corticosteroid treatment (prednisone (1mg/kg/day)) reversed the clinical symptoms. At the age of 14, …

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