Disease Information for Olivopontocerebellar degeneration

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Clinical Manifestations
Signs & Symptoms
Akinetic rigid frozen Posture
Ataxia
Athetosis
Basal ganglion signs
Cerebellar movement disorder/signs
Chorea signs
Choreoathetoid movement
Choreoathetosis
Dysdiadochokinesia/Alternating failure
Gait disturbance/abnormality
Limb Ataxia
Nystagmus
Staggering Gait
Tremor
Tremor in Elderly
Tremor,coarse
Truncal ataxia
Unsteadiness movement/Gait in a child
True Vertigo Sign Confirmed
Disease Progression
Course/Chronic disorder
Course/Chronic only
Parkinsonism plus Other CNS issues
Demographics & Risk Factors
Population Group
Aged Adult
Sex & Age Groups
Population/Adult Aged Only
Population/Adult/all
Diagnostic Test Results
CT Scan
CT Scan/Head Brain/cerebellar atrophy
MRI/Head Cerebellar atrophy
Associated Diseases & Rule outs
Rule Outs
Parkinsons disease
Associated Disease & Complications
Cerebellar ataxia
Cerebellar lesion
Congenital Nystagmus
Olivopontocerebellar degeneration
Parkinsonism, secondary
Parkinsons disease
REM Behavior disorder/RBD
Akinetic Rigid Syndrome
Ataxia Disorder
Demyelinating Disease
Disease Mechanism & Classification
Class
CLASS/Cerebellar disorder (ex)
CLASS/Neurologic (category)
Pathophysiology
Pathophysiology/Cerebral demyelination
Pathophysiology/CNS degeneration
Pathophysiology/Demyelination
Pathophysiology/Neurologic degenerative disorder (ex)
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Use/Age/atrophic disorder (category)
PROCESS/Congenital CNS disorder (ex)
PROCESS/Developmental degenerative neurological disorder (ex)
PROCESS/Hereditary SCA (spinocerebellar ataxias)
Synonyms
Synonym
Ataxia Presenile, Ataxias Presenile, atrophy Dejerine Thomas, atrophy Déjerine Thomas, Atrophy Olivo Ponto Cerebellar, Atrophy Olivopontocerebellar, Atrophy Pontoolivocerebellar, Degeneration Olivo Ponto Cerebellar, Degeneration Olivopontocerebellar, Degenerations Olivo Ponto Cerebellar, Degenerations Olivopontocerebellar, Dejerine Thomas atrophy, Déjerine Thomas atrophy, Dejerine Thomas Syndrome, Déjérine Thomas syndrome, Dejerine Thomas syndrome (disorder), Olivo Ponto Cerebellar Atrophy, Olivo Ponto Cerebellar Degeneration, Olivo Ponto Cerebellar Degenerations, Olivocerebellar atrophy, Olivopontocerebellar Atrophies, olivopontocerebellar atrophy, olivopontocerebellar degeneration, Olivopontocerebellar degeneration (disorder), Olivopontocerebellar Degenerations, OPCA, OPCA Olivopontocerebel atrop, OPCA Olivopontocerebellar atrophy, Pontoolivocerebellar Atrophies, Pontoolivocerebellar Atrophy, Presenile Ataxia, PRESENILE ATAXIA SYNDROME, Presenile Ataxias, Syndrome Dejerine Thomas, Thomas' syndrome, Synonym/Dejerine-Thomas atrophy, Synonym/Hereditary OPCA, Synonym/OPCA (Types 1,2,3,4,5), Synonym/OPCA Fickler-Winkler type, Synonym/OPCA Holguin type (Var), Synonym/OPCA Menzel type (Var), Synonym/OPCA Schut-Haymaker type (Var), Synonym/OPCA with dementia and extrapyramidal sx, Synonym/OPCA with retinal degeneration (var), Synonym/Presenile ataxia syndrome, Synonym/SCA Cuban type (Var), Synonym/Spinocerebellar ataxia (type 1,2,7)
Definition

A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei; Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA; The familial form has an earlier onset (second decade) and may feature spinal cord atrophy; The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY; (From Adams et al, Principles of Neurology, 6th ed, )----------------------------------------------Multiple System Atrophy; MSA; Progressive Autonomic Failure with Multiple System Atrophy; Shy-Drager Syndrome; (SDS); Sporadic OPCA; Sporadic Olivopontocerebellar Atrophy; Striatonigral Degeneration (SND); Multiple system atrophy (MSA) is a rare progressive neurological disorder characterized by a varying combination of symptoms; Affected individuals may experience symptoms similar to those found in Parkinson’s disease (parkinsonism); cerebellar signs such as progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia); and impaired functioning of the portion of the nervous system (autonomic nervous system) that regulates certain involuntary body functions (autonomic failure) such as heart rate, blood pressure, sweating, and bowel and bladder control; The exact cause of multiple system atrophy is unknown; The term multiple system atrophy has generated significant controversy and confusion in the medical literature; The term now encompasses three conditions once thought to be separate disorders, specifically Shy-Drager syndrome, striatonigral degeneration, and sporadic olivopontocerebellar atrophy; Additionally, there is a hereditary form of olivopontocerebellar atrophy that is not part of the multiple system atrophy spectrum; [NORD]-----------------------------------------Olivopontocerebellar Atrophy, Hereditary; Hereditary OPCA; Subdivisions: OPCA with dementia and extrapyramidal signs: OPCA with retinal degeneration; OPCA, Fickler-Winkler type; OPCA, Holguin type; OPCA, Menzel type; OPCA, Schut-Haymaker type

OPCA1; OPCA2; OPCA3; OPCA4; OPCA5;[ DDX: SCA, Cuban type; SCA1,SCA2, SCA7, Spinocerebellar Ataxia 1, Spinocerebellar Ataxia 2, Spinocerebellar Ataxia 7}; Hereditary olivopontocerebellar atrophy (OPCA) is a rare group of disorders characterized by progressive balance problems (disequilibrium), progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia), and difficulty speaking or slurred speech (dysarthria); [NORD2005]

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NEJM (The New England Journal of Medicine)
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