-
Annals of Neurology Dec 1995
Topics: Humans; Olivopontocerebellar Atrophies
PubMed: 8526474
DOI: 10.1002/ana.410380621 -
Neurology Mar 1956
Topics: Atrophy; Cerebellopontine Angle; Disease; Olivopontocerebellar Atrophies
PubMed: 13297122
DOI: 10.1212/wnl.6.3.218 -
Yonsei Medical Journal 1988
Topics: Adolescent; Adult; Aged; Diagnosis, Differential; Female; Humans; Korea; Male; Middle Aged; Olivopontocerebellar Atrophies; Spinocerebellar Degenerations
PubMed: 3195154
DOI: 10.3349/ymj.1988.29.3.233 -
Clinical Genetics Jun 1989We present a kindred with a relatively pure cerebellar degeneration that demonstrates X-linked recessive inheritance. The unique clinical picture of affected patients in... (Review)
Review
We present a kindred with a relatively pure cerebellar degeneration that demonstrates X-linked recessive inheritance. The unique clinical picture of affected patients in our kindred is characterized by an infantile onset of ataxia; very slow rate of progression; normal strength, reflexes, and sensation; and cerebellar degeneration with involvement of the olive and pons demonstrated by neuroimaging techniques. The distinction between this and other reported olivopontocerebellar degenerations is made on the basis of the clinical features and mode of inheritance. It is not clear if the distinct clinical pattern in this kindred represents variable expression of a previously reported condition, allelic variance of previously reported kindreds, or a separate clinical entity. Molecular analysis, currently underway, may help settle the issue.
Topics: Adult; Brain; Child, Preschool; Genetic Linkage; Humans; Magnetic Resonance Imaging; Male; Olivopontocerebellar Atrophies; Pedigree; Spinocerebellar Degenerations; Tomography, X-Ray Computed; X Chromosome
PubMed: 2661059
DOI: 10.1111/j.1399-0004.1989.tb02966.x -
Movement Disorders : Official Journal... Oct 2006Olivopontocerebellar atrophy (OPCA) is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the... (Comparative Study)
Comparative Study Review
Olivopontocerebellar atrophy (OPCA) is a pathological label implying not only olivopontocerebellar changes, but also cases with more widespread lesions involving the CNS. This polytopic pathological background accounts for clinical complexity, essentially defined as cerebellar-plus syndrome. The term "OPCA" is applicable to an increasing number of neurodegenerative syndromes, including autosomal dominant ataxia, complicated spastic paraplegia, multiple-system atrophy (MSA), and many cases of idiopathic late-onset cerebellar ataxia (ILOCA), some of whom also turn out to have MSA. OPCA may also be part of the pathological hallmark of other disorders, such as prion disorders, mitochondrial encephalomyopathies, and hereditary metabolic diseases. Sporadic OPCA and ILOCA with cerebellar-plus presentation and neuroimaging evidence of brainstem and cerebellar atrophy may represent interchangeable eponyms. Just a quarter of such cases evolve to MSA within 5 years of the onset of symptoms. Therefore, the assumption that MSA and sporadic OPCA necessarily are one and the same disease is no longer tenable. Our review suggests that the label "OPCA" is useful to designate a clinicopathological syndrome that has a variety of etiologies carrying a poor prognosis, particularly if associated with autonomic failure as occurs in MSA.
Topics: Brain Stem; Cerebellum; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Multiple System Atrophy; Neurologic Examination; Olivopontocerebellar Atrophies; Prognosis; Spinocerebellar Ataxias; Tomography, X-Ray Computed
PubMed: 16874757
DOI: 10.1002/mds.21052 -
Ryoikibetsu Shokogun Shirizu 2001
Review
Topics: Female; Humans; Male; Olivopontocerebellar Atrophies
PubMed: 11528786
DOI: No ID Found -
International Review of Neurobiology 1997This chapter deals with neuropsychological disturbances in patients with bilateral cerebellar damage (BCD), i.e., epileptic patients chronically receiving phenytoin,... (Review)
Review
This chapter deals with neuropsychological disturbances in patients with bilateral cerebellar damage (BCD), i.e., epileptic patients chronically receiving phenytoin, patients with olivopontocerebellar atrophy (OPCA), and Friedreich's ataxia (FA) versus those with unilateral cerebellar damage (UCD), i.e., patients with cerebellar strokes. BCD patients showed: (i) impaired executive functions in planning and programming of daily activities, elaborating and using structures, and difficulty in abstract thinking, functions that are related to cerebello-frontal loops; (ii) deficits in visuospatial organization for a concrete task and deficient visual-spatial working memory, functions related to cerebello-parietal loops; (iii) lower general intellectual abilities than controls (especially those with OPCA); (iv) difficulties with memory retrieval, diminished global memory quotient, and reduced spatial working memory ability; and (v) slower speed of information processing, as measured by simple and multiple choice reaction time (RT). In UCD patients, neuropsychological and neurobehavioral abilities were deficient for 2-5 months; after this time period, their performances returned to normal. In both BCD and UCD patients, single photon emission computed tomography (SPECT) studies showed different degrees of "reverse" cerebellar-->basal ganglia-->frontoparietal diaschisis which may underlie permanent or transitory neuropsychological deficits. The relationships among neuropsychological findings, SPECT studies, and chemical neuroanatomy are discussed.
Topics: Cerebellar Diseases; Friedreich Ataxia; Functional Laterality; Humans; Movement; Neuropsychological Tests; Olivopontocerebellar Atrophies; Reaction Time; Tomography, Emission-Computed, Single-Photon
PubMed: 9378599
DOI: 10.1016/s0074-7742(08)60361-x -
Ryoikibetsu Shokogun Shirizu 1999
Review
Topics: Diagnosis, Differential; Humans; Olivopontocerebellar Atrophies; Prognosis; Quality of Life
PubMed: 10434644
DOI: No ID Found -
British Journal of Hospital MedicineMost doctors have vague and imprecise notions of what is meant by the terms olivopontocerebellar atrophy, Shy-Drager syndrome, striatonigral degeneration, multiple... (Review)
Review
Most doctors have vague and imprecise notions of what is meant by the terms olivopontocerebellar atrophy, Shy-Drager syndrome, striatonigral degeneration, multiple system atrophy and multisystem degenerations. This article attempts to tame this nosological jungle.
Topics: Atrophy; Autonomic Nervous System Diseases; Cerebellar Diseases; Corpus Striatum; Humans; Olivopontocerebellar Atrophies; Shy-Drager Syndrome; Substantia Nigra
PubMed: 7921508
DOI: No ID Found -
Annals of Neurology May 1995
Topics: Atrophy; Humans; Nervous System Diseases; Olivopontocerebellar Atrophies
PubMed: 7755347
DOI: 10.1002/ana.410370502