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Descriptor English: Diffuse Cerebral Sclerosis of Schilder
Descriptor Spanish: Esclerosis Cerebral Difusa de Schilder
Descriptor Portuguese: Esclerose Cerebral Difusa de Schilder
Descriptor French: Sclérose cérébrale diffuse de Schilder
Entry term(s): Alper Disease
Alper Syndrome
Alper's Disease
Alper's Syndrome
Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
Alpers Disease
Alpers Huttenlocher Syndrome
Alpers Progressive Infantile Poliodystrophy
Alpers Syndrome
Alpers' Disease
Alpers' Syndrome
Alpers-Huttenlocher Syndrome
Balo Concentric Sclerosis
Balo's Concentric Sclerosis
Cerebral Sclerosis, Diffuse
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
Encephalitis Periaxialis
Encephalitis Periaxialis Concentrica
Encephalitis Periaxialis Diffusa
Myelinoclastic Diffuse Scleroses
Myelinoclastic Diffuse Sclerosis
Neuronal Degeneration Of Childhood With Liver Disease, Progressive
Poliodystrophia Cerebri
Progressive Neuronal Degeneration of Childhood with Liver Disease
Progressive Sclerosing Poliodystrophies
Progressive Sclerosing Poliodystrophy
Schilder Disease
Schilder's Disease
Schilders Disease
Scleroses, Balo's Concentric
Scleroses, Myelinoclastic Diffuse
Sclerosis, Diffuse Cerebral
Sclerosis, Myelinoclastic Diffuse
Sudanophilic Cerebral Sclerosis
Syndrome, Alpers
Syndrome, Alpers-Huttenlocher
Tree number(s): C10.114.375.112
C10.228.140.400
C10.228.140.695.562.112
C10.314.350.112
C20.111.258.250.175
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D002549
Scope note: 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)
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CN congenital
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 2000; see CEREBRAL SCLEROSIS, DIFFUSE 1963-1999; for ENCEPHALITIS PERIAXIALIS see ENCEPHALITIS PERIAXIALIS 1963-1999; for BALO'S DISEASE, ENCEPHALITIS PERIAXIALIS DIFFUSA, MYELINOCLASTIC DIFFUSE SCLEROSIS, & SCHILDER'S DISEASE see ENCEPHALITIS PERIAXIALIS 1990-1999
History Note: 2000(1963)
DeCS ID: 2589
Unique ID: D002549
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2000/01/01
Date of Entry: 1999/01/01
Revision Date: 2013/07/08
Diffuse Cerebral Sclerosis of Schilder - Preferred
Concept UI M0003893
Scope note 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)
Preferred term Diffuse Cerebral Sclerosis of Schilder
Entry term(s) Cerebral Sclerosis, Diffuse
Diffuse Cerebral Scleroses
Diffuse Cerebral Sclerosis
Diffuse Scleroses, Myelinoclastic
Diffuse Sclerosis, Myelinoclastic
Disease, Schilder
Disease, Schilder's
Encephalitis Periaxialis
Encephalitis Periaxialis Concentrica
Encephalitis Periaxialis Diffusa
Myelinoclastic Diffuse Scleroses
Myelinoclastic Diffuse Sclerosis
Poliodystrophia Cerebri
Schilder Disease
Schilder's Disease
Schilders Disease
Scleroses, Myelinoclastic Diffuse
Sclerosis, Diffuse Cerebral
Sclerosis, Myelinoclastic Diffuse
Sudanophilic Cerebral Sclerosis
Balo Concentric Sclerosis - Related but not broader or narrower
Concept UI M0007338
Preferred term Balo Concentric Sclerosis
Entry term(s) Balo's Concentric Sclerosis
Concentric Sclerosis, Balo
Concentric Sclerosis, Balo's
Scleroses, Balo's Concentric
Alpers Syndrome - Related but not broader or narrower
Concept UI M0003894
Preferred term Alpers Syndrome
Entry term(s) Alper Disease
Alper Syndrome
Alper's Disease
Alper's Syndrome
Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
Alpers Disease
Alpers Huttenlocher Syndrome
Alpers Progressive Infantile Poliodystrophy
Alpers' Disease
Alpers' Syndrome
Alpers-Huttenlocher Syndrome
Disease, Alpers'
Neuronal Degeneration Of Childhood With Liver Disease, Progressive
Progressive Neuronal Degeneration of Childhood with Liver Disease
Progressive Sclerosing Poliodystrophies
Progressive Sclerosing Poliodystrophy
Syndrome, Alpers
Syndrome, Alpers-Huttenlocher



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