In 1969 graham and oppenheimer suggested the term multiple system atrophy msa to describe and combine a set of different disorders. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Olivopontocerebellar atrophy information page what research is being done. Atrophy involved the cerebellum, pons, and middle cerebellar peduncles in all. Olivopontocerebellar atrophy opca is the degeneration of neurons in specific areas of the brain the cerebellum, pons, and inferior olivary nucleus. Multiple system atrophy msa is a severe neurodegenerative disease.
The umbrella term of opca includes common sporadic forms and uncommon genetic forms. Opca is present in several neurodegenerative syndromes, including inherited and noninherited forms of ataxia such as the hereditary spinocerebellar ataxia known as machadojoseph disease and multiple system atrophy msa, with which it. Olivopontocerebellar atrophy is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. This disease manifests as a part of other disorders, namely multiple system atrophy msa and spinocerebellar ataxia sca. Opca causes increasingly severe ataxia loss of coordination as well as other symptoms. Demographics prevalence of 24 per 100,000 population may be underestimated median age of onset is 55 years range 33 76 men. In case of sporadic opca, certain environmental conditions or exposure to chemicals due to an industrial setting can be risk. Msac used to be known as olivopontocerebellar atrophy opca. Olivopontocerebellar atrophy genetic and rare diseases. Olivopontocerebellar atrophy opca is a pathological label comprising a series of heterogeneous diseases whose only common factor is the loss of neurons in the ventral portion of the pons, inferior olives and cerebellar cortex. Msa what you need to know pdf multiple system atrophy. Olivopontocerebellar atrophy brain oxford academic.
The ninds supports and conducts a broad range of basic and clinical research on cerebellar degeneration, including work aimed at finding the causes of opca and ways to treat, cure, and, ultimately, prevent the disease. The olivopontocerebellar pathway consists of nerve fibers that connect the olivary nucleus to the pons and cerebellum. Those who study opca quickly learn that it is not a single entity, and that its nosology can be confusing. Ideal sources for wikipedia s health content are defined in the guideline wikipedia.
Olivopontocerebellar atrophy definition olivopontocerebellar atrophy opca is a group of disorders characterized by degeneration of three brain areas. Olivopontocerebellaratrophyinformationpage national. Specific binding in the striatum was significantly reduced in the multiple system atrophy patients as compared with the normal control group, with average reductions of 61% in the caudate nucleus p 0. It furthers the universitys objective of excellence in research, scholarship, and education by publishing worldwide.
Hereditary olivopontocerebellar atrophy opca is a specific health condition where nerves in certain part of the brain degenerate. This means that olivopontocerebellar atrophy, or a subtype of olivopontocerebellar atrophy, affects less than 200,000 people in the us population. Pontocerebellar fibers an overview sciencedirect topics. They have similar pathology and are characterized by various degrees of autonomic dysfunction, cerebellar.
All ofthe four patients described showed ct scan changes of cerebellar atrophy and dilatation of the 4th ventricle. Get a printable copy pdf file of the complete article 435k, or click on a page image below to browse page by page. Olivopontocerebellar degeneration opcd is a neurodegenerative disease, and one of the clinical manifestations of multiple systemic atrophy msa it is important to note that the current belief that olivopontocerebellar degeneration, shydrager syndrome and striatonigral degeneration are different manifestations of the same underlying disease, namely multiple systemic atrophy msa, is. Psychology definition of olivopontocerebellar atrophy. The topic olivopontocerebellar degeneration you are seeking is a synonym, or alternative name, or is closely related to the medical condition hereditary olivopontocerebellar atrophy. A group of genetic diseases in which there is progressive degeneration of the olivopontocerebellar pathway in the brain connecting the inferior olive, pons, and cerebellum. Source for information on olivopontocerebellar atrophy. The authors report 23 cases of opca, eight of which were associated with msa. Introduction olivopontocerebellar atrophy opca is a term created by ddjerine and thomas 1900 which comprises a series of heterogeneous diseases whose only common factor is the loss of neurons in the ventral portion of the pons, inferior olives and cerebellar cortex petito et al.
What is the life expectancy of someone with olivopontocerebellar atrophy. The oliva olive is a rounded prominence on the surface of the medulla oblongata in the brain. Graham and oppenheimer first introduced the term multiple system atrophy msa in 1969 graham and oppenheimer 1969 to combine. Understanding the pathogenesis of multiple system atrophy. Pertaining to three particular parts of the brain namely, the oliva, the pons, and the cortex of the cerebellum. Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.
Multiple system atrophy msa is a sporadic adultonset progressive neurodegenerative disorder, characterized pathologically by degeneration of the basal ganglia and the olivopontocerebellar system and clinically known as parkinsonism, cerebellar ataxia, and autonomic dysfunction. Olivopontocerebellar atrophy an overview sciencedirect. The symptoms appear to be obvious at the age of 50 years old mostly. Differentiating multiple system atrophy from parkinsons disease. Olivopontocerebellar definition of olivopontocerebellar. Olivopontocerebellar atrophies is a descriptor in the national library of medicines controlled vocabulary thesaurus, mesh medical subject headings. Pd, olivopontocerebellar atrophy, and the shydrager syndrome.
Multiple system atrophy cerebellar subtype msac is a rare disease that causes areas deep in the brain, just above the spinal cord, to shrink atrophy. Olivopontocerebellar atrophy medical definition merriam. In the sporadic type of opca, at least some of the. Medical definition of olivopontocerebellar atrophy. Opca were mesencephalic atrophy, midbrain, frontal lobe and parietal atrophy.
Life expectancy of people with olivopontocerebellar atrophy and recent progresses and researches in olivopontocerebellar atrophy. Patients have classical parkinsons disease pd, multiple system atrophy. Atrophy is the result of a predominance of dissimilation over the processes of assimilation. Multiple system atrophy msa is a unifying term that brings together a group of rare, fatal neurodegenerative syndromes that used to be referred to as olivopontocerebellar atrophy, striatonigral degeneration, and shydrager syndrome. We describe three patients affected by olivopontocerebellar atrophy, of different types and at different stages of disease. The clinical manifestations reflect central autonomic and striatonigral degeneration as well as olivopontocerebellar atrophy. Olivopontocerebellar atrophy with dementia syndrome.
Olivopontocerebellar atrophy deafness genetic and rare. The basis pontis is atrophic, with loss of pontine neurones and transverse pontocerebellar fibers. Olivopontocerebellar atrophy opca is a term that describes the degeneration of neurons in specific areas of the brain the cerebellum, pons, and inferior olives. Olivopontocerebellar atrophy opca the brunt of pathology is in the olivopontocerebellar system, while the involvement of striatum and substantia nigra is less severe. Olivopontocerebellar atrophy opca is a group of neurodegenerative disorders that causes the cerebellum, pons, inferior olivae, and other parts of. Olivopontocerebellar atrophy opca is a term used for a progressive condition characterized by the degeneration of nerve cells neurons in specific areas of the brain. Olivopontocerebellar atrophy information mount sinai. An update on the cerebellar subtype of multiple system atrophy. What are the risk factors of hereditary olivopontocerebellar atrophy. Multiple system atrophy is a rare and fatal neurodegenerative disorder characterized by progressive autonomic failure, ataxia and parkinsonism in any combination. Between 1985 and 1987, 31 patients with sporadic olivopontocerebellar atrophy sopca and 3 patients with familial olivopontocerebellar atrophy fopca were examined in the neurologic clinic of. Bilateral vocal fold paresis and multiple system atrophy. This study is the first to combine pet and 3d mri based volumetric striatal and extrastriatal imaging in the same parkinsonian patients in order to differentiate. Urinary disturbance in olivopontocerebellar atrophy opca.
Olivopontocerebellar atrophy is a neurodegenerative syndrome that arises spontaneously or through familial inheritance. Hie multimedia multiple system atrophy cerebellar subtype. Predisposing factors hereditary olivopontocerebellar atrophy is a genetic condition, and hence, a family history of the condition increases the risk. Multiple system atrophy msa is a neurodegenerative disorder that has the characteristics of 3 major diseases.
A group of diseases progressive degeneration occurs in a particular area of the brain olivopontocerebellar area which results in various neurological symptoms. Atrophy can be physiological and pathological, systemic and local. Olivopontocerebellar atrophy nord national organization. Full text full text is available as a scanned copy of the original print version. In sections stained for myelin, the intact descending corticospinal tracts stand out against the degenerate transverse fibers and the middle cerebellar peduncles. Request pdf current concepts in the treatment of multiple system atrophy msa is a progressive neurodegenerative disorder characterized by autonomic. The cerebellum is the part of the brain that plays a role in maintaining balance and posture as well as coordinating voluntary movement. The olivopontocerebellar atrophies report of 4 cases. The role of mri in the diagnosis of olivopontocerebellar. The trip database provides clinical publications about. Olivopontocerebellar atrophy opca is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the cns. Opca is present in several neurodegenerative syndromes, including inherited and noninherited forms of ataxia. Olivopontocerebellar atrophy belongs to a great category of neurodegenerative diseases, which characterized by cerebellar atrophy and mainly brain stem lesions. Mri in sporadic olivopontocerebellar atrophy and striatonigral.
Opca can be viewed as a finding of several diseases, and indicates a form of progressive ataxia abnormal or uncontrolled movements distinguished by characteristic findings in brain imaging studies and at. Reasons to combine these diverse categories under the heading of msa were the. The classification scheme for autosomal dominant opca overlaps with that of autosomal dominant spinocerebellar. Physiological atrophy is a function of the growth changes of an organism atrophy of the thymus during puberty, atrophy of the sex glands, skin, and bones in old people, and so on. Synonyms for olivopontocerebellar atrophy in free thesaurus. Oxford university press is a department of the university of oxford. Striatonigral degeneration an overview sciencedirect topics. Msac can be passed down through families inherited form. Olivopontocerebellar atrophydeafness syndrome is characterised by infancyonset olivopontocerebellar atrophy, sensorineural deafness and speech impairment. One patient also had pontine atrophy and another had atrophy of the vermis of the cerebellum. The term olivopontocerebellar atrophy opca has historically been used to describe a group of disorders that affect the central nervous system and are termed neurodegenerative diseases because they result in a progressive deterioration of nerve cells in certain parts of the brain. This produces a certain set of symptoms and needs appropriate treatment.
Article in japanese kaiho y1, namima t, uchi k, nakagawa h, aizawa m, nishimura y, orikasa s. Opca may be associated with degeneration of other systems in multisystem atrophy msa. Pdf olivopontocerebellar atrophy presenting with stridor. Olivopontocerebellar atrophy presenting with stridor article pdf available in the journal of the association of physicians of india 51. The diagnosis of olivopontocerebellar atrophy has been made, until now, by clinical criteria while typical anatomopathological changes are found at the autoptic studies. Parkinsonisms refer to a group of neurological conditions that cause movement features similar to those observed in parkinsons disease.
Know about hereditary olivopontocerebellar atrophy, its. Olivopontocerebellar atrophy information page national. Posts about sporadic olivopontocerebellar atrophy written by simon. Urinary disturbance in olivopontocerebellar atrophy opca changes during the progression of the disease. Your doctor will combine your health history and physical examination findings with imaging studies and laboratory tests to arrive at a diagnosis and to gauge your. Olivopontocerebellar atrophy opca is a neurodegenerative syndrome characterized by prominent cerebellar and extrapyramidal signs, dysarthria, and dysphagia. Links to pubmed are also available for selected references. Clinical diagnosis of olivopontocerebellar atrophy opca must be confirmed by radiologic demonstration of atrophy in an appropriate distribution.
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