Schilder type multiple sclerosis
Updates to Article Attributes
Schilder's disease, considered a variant of multiple sclerosis (MS), is an extremely rare progressive demyelinating process that begins in childhood1.
Epidemiology
The disease is extremely rare with less than 10 cases reported in the literature that fulfill the original Posner diagnostic criteria1.
Clinical Presentation
Symptoms include aphasia, balance instability, dementia, headache, incontinence, personality changes, seizures, tremors, visual disturbances and weakness1.
Diagnostic Criteria
Proposed by Posner in 1985, all six criteria must be met 2.
- 1
- 2-2 large plaques-: should be in each brain hemisphere predominantly in centrum semiovale. Lesions are greater than2cm2 cm in 2/3 dimensions - no additional lesions identified
- no peripheral nervous system abnormality
- normal adrenal function
- normal serum long chain fatty acids
- pathologic/histologic changes consistent with subacute/chronic myelinoclastic diffuse sclerosis
Radiographic features
MRI
Often has 1 - 2-2 large demyelinating plaques, which are hyperintense on T2 weighted images, in each brain hemisphere predominantly in the centrum semiovale. Lesions should be greater than 2 cm in 2/3 dimensions 3.
Note that the diagnosis cannot be made on imaging findings alone.
Treatment
Patients have a poor prognosis. Current recommendations are high dose corticosteroids and supportive care3.
-<p><strong>Schilder's disease,</strong> considered a variant of <a href="/articles/multiple-sclerosis">multiple sclerosis (MS)</a>, is an extremely rare progressive demyelinating process that begins in childhood<sup>1</sup>.</p><h4>Epidemiology </h4><p>The disease is extremely rare with less than 10 cases reported in the literature that fulfill the original Posner diagnostic criteria<sup>1</sup>.</p><h4>Clinical Presentation</h4><p>Symptoms include aphasia, balance instability, dementia, headache, incontinence, personality changes, seizures, tremors, visual disturbances and weakness<sup>1</sup>.</p><h5>Diagnostic Criteria </h5><p>Proposed by Posner in 1985, all six criteria must be met <sup>2</sup>. </p><ul>-<li>1 - 2 large plaques - should be in each brain hemisphere predominantly in centrum semiovale. Lesions are greater than 2cm in 2/3 dimensions</li>- +<p><strong>Schilder's disease,</strong> considered a variant of <a href="/articles/multiple-sclerosis">multiple sclerosis (MS)</a>, is an extremely rare progressive demyelinating process that begins in childhood<sup> 1</sup>.</p><h4>Epidemiology </h4><p>The disease is extremely rare with less than 10 cases reported in the literature that fulfill the original Posner diagnostic criteria<sup> 1</sup>.</p><h4>Clinical Presentation</h4><p>Symptoms include aphasia, balance instability, dementia, headache, incontinence, personality changes, seizures, tremors, visual disturbances and weakness<sup> 1</sup>.</p><h5>Diagnostic Criteria </h5><p>Proposed by Posner in 1985, all six criteria must be met <sup>2</sup>. </p><ul>
- +<li>1-2 large plaques: should be in each brain hemisphere predominantly in centrum semiovale. Lesions are greater than 2 cm in 2/3 dimensions</li>
-</ul><h4>Radiographic features</h4><h5>MRI</h5><p>Often has 1 - 2 large demyelinating plaques, which are hyperintense on T2 weighted images, in each brain hemisphere predominantly in the centrum semiovale. Lesions should be greater than 2 cm in 2/3 dimensions <sup>3</sup>.</p><p>Note that the diagnosis cannot be made on imaging findings alone.</p><h4>Treatment</h4><p>Patients have a poor prognosis. Current recommendations are high dose corticosteroids and supportive care<sup>3</sup>.</p>- +</ul><h4>Radiographic features</h4><h5>MRI</h5><p>Often has 1-2 large demyelinating plaques, which are hyperintense on T2 weighted images, in each brain hemisphere predominantly in the centrum semiovale. Lesions should be greater than 2 cm in 2/3 dimensions <sup>3</sup>.</p><p>Note that the diagnosis cannot be made on imaging findings alone.</p><h4>Treatment</h4><p>Patients have a poor prognosis. Current recommendations are high dose corticosteroids and supportive care <sup>3</sup>.</p>