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Olivopontocerebellar Atrophy (OA)/Multiple System Atrophy
Published in Charles Theisler, Adjuvant Medical Care, 2023
Olivopontocerebellar atrophy is progressive degeneration of the neurons deep in the brain just above the spinal cord (olivopontocerebellar pathway that connects the inferior olive, pons, and cerebellum). OA is characterized by progressive balance problems (disequilibrium), impairment of the ability to coordinate voluntary movements (cerebellar ataxia), and difficulty speaking or slurred speech (dysarthria).1 No specific treatment exists for individuals with OA. Treatment is symptomatic and supportive.1
Olivopontocerebellar Atrophy
Published in W. R. Wayne Martin, Functional Imaging in Movement Disorders, 2019
Olivopontocerebellar atrophy is a chronic progressive neurological disease of undetermined cause characterized pathologically by degeneration of neurons in the inferior olives, pons, and cerebellum. The disease occurs both sporadically and with hereditary transmission.
Autonomic dysfunction
Published in Jeremy Playfer, John Hindle, Andrew Lees, Parkinson's Disease in the Older Patient, 2018
A syndrome of chronic autonomic failure associated with the parkinsonian features of rigidity, tremor and akinesia was first described by Shy and Drager in 1960.21 There are three major clinical groups within the definition of multiple system atrophy (MSA): Parkinsonian form – 20% of cases. Extrapyramidal features with autonomic failure. May indicate striatonigral degeneration.Cerebellar form – 20% of cases. Cerebellar and/or pyramidal features. Pathology indicates olivopontocerebellar atrophy/degeneration.Multiple form – 60% of cases. Combination of parkinsonian and cerebellar or pyramidal features. Multiple neuronal system degeneration.
Revisiting the pathoanatomy of pseudobulbar affect: mechanisms beyond corticobulbar dysfunction
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2018
The earliest description of PCL is often attributed to Charles Darwin, but the clinicopathological characterization of the condition was not published until 1886 by Oppenheim and Siemerling (5,6) and the first accounts of “emotional lability” in ALS were reported by Pierre Marie in 1892 (7). Since the earliest descriptions of PCL, there has been no consensus about the key anatomical substrate of the syndrome. Oppenheim and Siemerling have linked PCL to subcortical white matter pathology in the frontal lobes, Bechterew (8) advocated primary thalamic involvement, Brissaud (9,10) argued that the key pathology is the anterior limb of the internal capsules and Wilson suggested that the constellation of opercular involvement and corticopontine tract degeneration is required for PCL pathogenesis (11). While historical reports of PCL have explored pathological patterns based on post mortem examination, lesion studies and neurosurgical case series provided further anatomical insights. PCL has been observed after resection of vermian tumours in children (12) and in patients with cerebellar-type multiple system atrophy (13) indicating that the cerebellum plays a fundamental role in the aetiology of PCL. Post-stroke studies highlighted the importance of bilateral lesions in PCL pathogenesis (14), but it is not until the advent of quantitative neuroimaging techniques that PCL has been comprehensively studied in vivo (15). PCL is most commonly observed in ALS (15), traumatic brain injury (16,17), multiple sclerosis (18), post-stroke (19), Alzheimer's disease (20) and movement disorders (3,21,22). It has also been associated with a range of other neurological conditions including ADEM (23), PML (24), Wilson's disease, central pontine myelinolysis (25), and olivopontocerebellar atrophy. Interestingly, transient forms of PCL have been reported in cerebellar paraneoplastic syndromes (26), Bickerstaff encephalitis (27), and deep-brain stimulation (28) providing further anatomical cues on the aetiology of PCL.