Olivopontocerebellar atrophy (OPCA) is the degeneration of neurons in specific areas of the brain - the cerebellum, pons, and inferior olivary nucleus. (wikipedia.org)
OPCA is present in several neurodegenerative syndromes, including inherited and non-inherited forms of ataxia (such as the hereditary spinocerebellar ataxia known as Machado-Joseph disease) and multiple system atrophy (MSA), with which it is primarily associated. (wikipedia.org)
Olivopontocerebellar atrophy (OPCA) is a neurodegenerative syndrome characterized by prominent cerebellar and extrapyramidal signs, dysarthria, and dysphagia. (medscape.com)
MRI is the imaging study of choice in patients with olivopontocerebellar atrophy (OPCA) because CT scanning does not provide adequate resolution of the pons and cerebellum. (medscape.com)
The classification scheme for autosomal dominant OPCA overlaps with that of autosomal dominant spinocerebellar atrophies (SCAs) and autosomal dominant cerebellar atrophies (ADCAs). (medscape.com)
Care of olivopontocerebellar atrophy (OPCA) is directed to the treatment of symptoms. (medscape.com)
At times, olivopontocerebellar atrophy (OPCA) patients may require enteral feeding to decrease the risk of aspiration. (medscape.com)
As dysphagia progresses with olivopontocerebellar atrophy (OPCA), a pureed diet or enteral feeding may be required. (medscape.com)
We identified three cases of CBD with olivopontocerebellar atrophy (CBD-OPCA) that did not have a-synuclein-positive glial cytoplasmic inclusions of multiple system atrophy (MSA). (elsevierpure.com)
In addition, CBD-OPCA had TDP-43 immunoreactive neuronal and glial cytoplasmic inclusions and threads throughout the basal ganglia and in olivopontocerebellar system. (elsevierpure.com)
These results suggest that CBD-OPCA is a distinct clinicopathologic variant of CBD with olivopontocerebellar TDP-43 pathology. (elsevierpure.com)
Results: Patients with SCA3 exhibited reduced brain complexity within both the traditional olivopontocerebellar atrophy (OPCA) pattern and specific supratentorial regions. (tmu.edu.tw)
Dejerine and Thomas first used the term olivopontocerebellar atrophy (OPCA) in 1900 when they described 2 patients with a degenerative disorder leading to progressive cerebellar dysfunction and parkinsonism. (medscape.com)
Cerebellar atrophy4
Winkler, C. A case of olivo-pontine cerebellar atrophy and our conceptions of neo- and palaio-cerebellum. (wikipedia.org)
Infantile onset progressive cerebellar atrophy and anterior horn cell degeneration--a late onset variant of PCH-1? (nih.gov)
However, anterior horn cell degeneration has also been described in cases with later onset pontocerebellar atrophy and recently the spectrum has even been further extended to include the association of anterior horn cell degeneration and cerebellar atrophy without pontine involvement. (nih.gov)
Original descriptions of the main pathological subtypes, including Friedreich's ataxia, hereditary spastic paraplegia, olivopontocerebellar atrophy and cortical cerebellar atrophy, are revised. (bvsalud.org)
Associated with multiple system3
The most frequent autonomic symptoms associated with multiple system atrophy are a sudden drop in blood pressure upon standing ( orthostatic hypotension ), urinary difficulties, and erectile dysfunction in men. (medlineplus.gov)
Variations in the COQ2 gene have been associated with multiple system atrophy in people of Japanese descent, but this association has not been found in other populations. (medlineplus.gov)
Shy drager's syndrome causes shrinkage or atrophy of portions of your brain that are cerebellum, basal ganglia and brainstem. (naturalayurvedictreatment.com)
Researchers have described two major types of multiple system atrophy, which are distinguished by their major signs and symptoms at the time of diagnosis. (medlineplus.gov)
Hereditary1
Olivopontocerebellar atrophy is hereditary, but has an unknown genetic basis. (wikipedia.org)
Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia. (wikipedia.org)
Multiple system atrophy (MSA) is a progressive, sporadic and adult-onset neurodegenerative disease characterized by a combination of autonomic failure with parkinsonism, cerebellar ataxia and autonomic dysfunction ( 1 ). (frontiersin.org)
Shrinkage1
MSA causes deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that affect internal body functions and motor control. (middlesexhealth.org)
Neurodegenerative disorder4
Multiple system atrophy (MSA) is a rare and progressive neurodegenerative disorder. (frontiersin.org)
Multiple system atrophy is a relentlessly progressive neurodegenerative disorder causing pyramidal, cerebellar, and autonomic dysfunction. (msdmanuals.com)
Multiple system atrophy (MSA) is a horrible and unrelenting neurodegenerative disorder with an uncertain etiology and pathophysiology. (biomedcentral.com)
Multiple system atrophy (MSA) is a fatal neurodegenerative disorder that presents clinically with varying combinations of autonomic, parkinsonian, cerebellar, and pyramidal dysfunction [ 15 ]. (biomedcentral.com)
Cerebellum1
Neurological diseases that feature cerebellar degeneration include ischemic or hemorrhagic stroke (when there is lack of blood flow or oxygen to the cerebellum), cerebellar cortical atrophy, multisystem atrophy, and olivopontocerebellar degeneration. (neurological.org.nz)
Reduced cerebral cortical but elevated striatal concentration of somatostatin-like immunoreactivity in dominantly inherited olivopontocerebellar atrophy. (mcmaster.ca)
Multiple29
Multiple system atrophy is a progressive brain disorder that affects movement and balance and disrupts the function of the autonomic nervous system. (medlineplus.gov)
Multiple system atrophy has a prevalence of 2 to 5 per 100,000 people. (medlineplus.gov)
Multiple system atrophy is a complex condition that is likely caused by the interaction of multiple genetic, environmental, and lifestyle factors. (medlineplus.gov)
Studies suggest that several common variations in the SNCA gene are associated with an increased risk of multiple system atrophy in people of European descent. (medlineplus.gov)
Multiple system atrophy can be explained as cell loss and gliosis or a proliferation of astrocytes in damaged areas of the central nervous system. (wikipedia.org)
Clinical features and natural history of multiple system atrophy (MSA) have been established in four recent series. (bmj.com)
1- 4 Multiple system atrophy is usually defined by the predominance of parkinsonian (MSA-P type) or cerebellar (MSA-C type) features. (bmj.com)
They include multiple system atrophy (MSA), progressive supranuclear palsy (PSP ), corticobasal degeneration (CBD) and Dementia with Lewy bodies (DLB). (parkinson.ca)
Multiple System Atrophy (MSA) is a progressive brain disorder caused by loss of nerve cells in specific areas of the brain. (parkinson.ca)
Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body's involuntary (autonomic) functions, including blood pressure, and motor control. (middlesexhealth.org)
The clinical definition of multiple system atrophy (MSA) is a progressive, idiopathic, degenerative process beginning in adulthood. (medscape.com)
Neurons2
The pathology of AD is complex but characterized by loss of neurons, brain atrophy, extra-cellular deposition of amyloid Beta (Aβ) plaques, and intracellular accumulation of neurofibrillary tangles composed of phosphorylated tau protein. (aao.org)
The term olivopontocerebellar atrophy is used when the disorder starts later in life and the process is a primary degeneration of cerebellar neurons. (nih.gov)
Disorders1
Available at https://www.ninds.nih.gov/health-information/disorders/olivopontocerebellar-atrophy . (medscape.com)
Complications1
The prognosis of Olivopontocerebellar atrophy type 3 may include the duration of Olivopontocerebellar atrophy type 3, chances of complications of Olivopontocerebellar atrophy type 3, probable outcomes, prospects for recovery, recovery period for Olivopontocerebellar atrophy type 3, survival rates, death rates, and other outcome possibilities in the overall prognosis of Olivopontocerebellar atrophy type 3. (checkorphan.org)
Dejerine1
Dejerine-Thomas atrophy" J. J. Dejerine, A. Thomas. (wikipedia.org)
Spinal1
Unlike the similarly named spinal muscular atrophy , PMA does not occur in children. (healthline.com)
Onset1
Progressive muscular atrophy (PMA) is a rare adult-onset motor neuron disease. (healthline.com)
Typically shows1
brain imaging typically shows cerebellar and brain stem atrophy. (beds.ac.uk)