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Diffuse Cerebral Sclerosis of Schilder
Definition: A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73)

Synonym(s): Alpers Syndrome / Balo Concentric Sclerosis / Cerebral Sclerosis, Diffuse / Encephalitis Periaxialis / Myelinoclastic Diffuse Sclerosis / Poliodystrophia Cerebri / Schilder Disease / Alpers Progressive Infantile Poliodystrophy / Alpers' Disease / Alpers' Syndrome / Alpers-Huttenlocher Syndrome / Balo's Concentric Sclerosis / Encephalitis Periaxialis Concentrica / Encephalitis Periaxialis Diffusa / Schilder's Disease / Alper Disease / Alper Syndrome / Alper's Disease / Alper's Syndrome / Alpers Huttenlocher Syndrome / Concentric Sclerosis, Balo / Concentric Sclerosis, Balo's / Diffuse Cerebral Scleroses / Diffuse Cerebral Sclerosis / Diffuse Scleroses, Myelinoclastic / Diffuse Sclerosis, Myelinoclastic / Disease, Alpers' / Disease, Schilder / Disease, Schilder's / Myelinoclastic Diffuse Scleroses / Schilders Disease / Scleroses, Balo's Concentric / Scleroses, Myelinoclastic Diffuse / Sclerosis, Diffuse Cerebral / Sclerosis, Myelinoclastic Diffuse / Syndrome, Alpers / Syndrome, Alpers-Huttenlocher
MeSH 2010 © U.S. National Library of Medicine®.
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  Web resources for "Diffuse Cerebral Sclerosis of Schilder"
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view the robot description of the site Alpers Syndrome Symptoms, Diagnosis, Treatments and Causes - RightDiagnosis.comwww.wrongdiagnosis.com 3
view the robot description of the site Whonamedit - Schilder's diseasewww.whonamedit.com 6
view the HON description of the site HONcode - Orphanet: Alpers syndromewww.orpha.net 3
view the robot description of the site Schilder's Disease Information Page: National Institute of Neurological Disorders and Stroke (NINDS)www.ninds.nih.gov 6
view the robot description of the site rare-diseases — National Organization for Rare Disorderswww.rarediseases.org  
 
 

Broader term(s): - Autoimmune Diseases of the Nervous System - Nervous System Diseases - Brain Diseases - Central Nervous System Diseases

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Broader term(s): - Nervous System Diseases
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Broader term(s): - Autoimmune Diseases of the Nervous System - Nervous System Diseases

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Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy


  Broader term(s):
      Diseases
          Immune System Diseases
              Autoimmune Diseases
                  Autoimmune Diseases of the Nervous System
                      Demyelinating Autoimmune Diseases, CNS 
      Diseases
          Nervous System Diseases
              Demyelinating Diseases
                  Demyelinating Autoimmune Diseases, CNS 
              Central Nervous System Diseases
                  Brain Diseases 
              Autoimmune Diseases of the Nervous System
                  Demyelinating Autoimmune Diseases, CNS 

MeSH 2010 © U.S. National Library of Medicine®.

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