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Descriptor English: Myoclonic Cerebellar Dyssynergia
Descriptor Spanish: Disinergia Cerebelosa Mioclónica
Descriptor disinergia cerebelosa mioclónica
Entry term(s) atrofia cerebelosa dentada
disinergia cerebelosa
disinergia mioclónica cerebelosa
síndrome cerebeloso de Ramsay Hunt
Scope note: Afección caracterizada por ATAXIA CEREBELOSA progresiva combinada con MIOCLONÍA que generalmente se presenta en la tercera década de la vida o posteriormente. Las características clínicas adicionales pueden incluir CONVULSIONES focales y generalizadas, espasticidad, y DISCINESIAS. Se han descrito patrones de herencia autosómicos recesivos y autosómicos dominantes. En la anatomía patológica hay atrofia del núcleo dentado y conjuntivo braquial del CEREBELO, con afectación variable de la médula espinal, corteza cerebelosa y ganglios basales. (Joynt, Clinical Neurology, 1991, Ch37, pp60-1)
Descriptor Portuguese: Dissinergia Cerebelar Mioclônica
Descriptor French: Dyssynergie cérébelleuse myoclonique
Entry term(s): Ataxia, Dentate Cerebellar
Ataxias, Dentate Cerebellar
Atrophies, Dentate Cerebellar
Atrophy, Dentate Cerebellar
Cerebellar Ataxias, Dentate
Cerebellar Atrophy, Dentate
Cerebellar Dyssynergia
Cerebellar Dyssynergia, Myoclonic
Cerebellar Dyssynergias
Cerebellar Dyssynergias, Myoclonic
Cerebelloparenchymal Disorder V
Dentate Cerebellar Ataxia
Dentate Cerebellar Ataxias
Dentate Cerebellar Atrophies
Dentate Cerebellar Atrophy
Dentate Nucleus Syndrome, Ramsay Hunt
Dyssynergia Cerebellaris Myoclonica
Dyssynergia Cerebellaris Myoclonica Of Hunt
Dyssynergia Cerebellaris Progressiva
Dyssynergia, Cerebellar
Dyssynergia, Myoclonic Cerebellar
Dyssynergias, Cerebellar
Dyssynergias, Myoclonic Cerebellar
Myoclonic Cerebellar Dyssynergias
Ramsay Hunt Cerebellar Syndrome
Ramsay Hunt Dentate Syndrome
Spinodentate Atrophy
Tree number(s): C10.228.140.252.700.250
C10.228.854.787.500
C10.574.500.825.250
C16.320.400.780.500
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D002527
Scope note: A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal SEIZURES, spasticity, and DYSKINESIAS. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (From Joynt, Clinical Neurology, 1991, Ch37, pp60-1)
Annotation: do not confuse entry term RAMSAY HUNT CEREBELLAR SYNDROME with RAMSAY HUNT AURICULAR SYNDROME see HERPES ZOSTER OTICUS or RAMSAY HUNT PARALYSIS SYNDROME see PARKINSONIAN DISORDERS
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
Previous Indexing: Cerebellar Diseases (1966-1969)
Public MeSH Note: 2000; see CEREBELLAR DYSSYNERGIA 1991-1999; see CEREBELLAR ATAXIA 1970-1990
History Note: 2000(1970); use CEREBELLAR ATAXIA 1970-1990
DeCS ID: 22381
Unique ID: D002527
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1999/01/01
Revision Date: 2012/07/03
Myoclonic Cerebellar Dyssynergia - Preferred
Concept UI M0003859
Scope note A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal SEIZURES, spasticity, and DYSKINESIAS. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (From Joynt, Clinical Neurology, 1991, Ch37, pp60-1)
Preferred term Myoclonic Cerebellar Dyssynergia
Entry term(s) Ataxia, Dentate Cerebellar
Ataxias, Dentate Cerebellar
Atrophies, Dentate Cerebellar
Atrophy, Dentate Cerebellar
Cerebellar Ataxias, Dentate
Cerebellar Atrophy, Dentate
Cerebellar Dyssynergia
Cerebellar Dyssynergia, Myoclonic
Cerebellar Dyssynergias
Cerebellar Dyssynergias, Myoclonic
Dentate Cerebellar Ataxia
Dentate Cerebellar Ataxias
Dentate Cerebellar Atrophies
Dentate Cerebellar Atrophy
Dentate Nucleus Syndrome, Ramsay Hunt
Dyssynergia Cerebellaris Myoclonica
Dyssynergia Cerebellaris Progressiva
Dyssynergia, Cerebellar
Dyssynergia, Myoclonic Cerebellar
Dyssynergias, Cerebellar
Dyssynergias, Myoclonic Cerebellar
Myoclonic Cerebellar Dyssynergias
Ramsay Hunt Cerebellar Syndrome
Ramsay Hunt Dentate Syndrome
Cerebelloparenchymal Disorder V - Related but not broader or narrower
Concept UI M0574726
Preferred term Cerebelloparenchymal Disorder V
Entry term(s) Dyssynergia Cerebellaris Myoclonica Of Hunt
Spinodentate Atrophy



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