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At least 19 recordsLinked to original sources
PubMedDate not supplied

Progressive supranuclear palsy: report of two cases (author's transl).

Two cases of progressive supranuclear palsy (PSP) are reported in two men (49 and 75years old) who for one and four years respectively had sudden falling while walking. Rigidity of the neck was an carly feature that prgressed to involve the upper trunk while "subcortical dementia", dysarthria and dysphagia appeared. They had a complete paralysis of vertical eye movements and slow horizontal voluntary eye movements. Oculocephalic reflexes were intact. On caloric stimulation vestibulo-ocular responses were present but only slow saccadic eye movements were observed. With surface electrodes eye movements were studied during the REM phase of sleep. Our patients had both vertical and horizontal eye movements during paradoxal sleep. This findings is in keeping with a supranuclear ophtalmoplegia, and may help in antemorten diagnosis of PSP.

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PubMed1979

The fine structure of subcortical neurofibrillary tangles in progressive supranuclear palsy.

The fine structure of subcortical neurofibrillary tangles was investigated in pallidum, substantia nigra, periaqueductal gray, pontine reticular gray, and dentate nucleus of 5 autopsy cases of Progressive Supranuclear Palsy. Only tangles due to straight 150 A wide filaments have been detected. These findings, obtained from a large series of cases and areas examined, confirm previous observations on the fine structure of neurofibrillary tangles in Progressive Supranuclear Palsy and suggest that the association between tangles due to straight filaments and tangles due to twisted tubules, so far described in one case, is probably exceptional.

Aged↗
PubMed1979

The eye movement disorders of progressive supranuclear palsy.

In addition to the gross disturbances of vertical gaze, patients with progressive supranuclear palsy may show abnormalities in the saccadic and pursuit subsystems of horizontal gaze. Saccades are slower and smaller than normal. An attempt to elicit a large amplitude saccade will often result in a series of "fractionated" saccades. Pursuit eye movements become saccadic. The quick phases of vestibular and optokinetic nystagmus are of low amplitude. In the study presented, eye movements of 13 patients were recorded clinically and by electro-oculography, and data from the two methods, compared.

Adult↗
PubMed1977

The ocular motor defects in progressive supranuclear palsy.

The results of quantitative infrared horizontal eye movement recordings in 8 patients with progressive supranuclear palsy are presented. Some of the patients had total paralysis of vertical movements, but none had completely lost the ability to perform horizontal eye movements. All patients had a defect in ocular fixation previously undescribed in this condition: the universal presence of square-wave jerks. Analysis of refixation saccades demonstrated hypometria, slow velocity/amplitude relationships, and profound prolongation of duration. The pursuit abnormality, characterized clinically by "cogwheel" eye movements, represented the inability to match eye velocity to target velocity. The ratio of peak eye velocity to peak target velocity (pursuit gain) was 0.2 to 0.5. Defects in the vestibuloocular reflex included inability to increase the gain of the reflex (ratio of peak eye velocity to head velocity) during viewing of a visible, stationary target and failure to suppress the reflex when viewing a target rotating with the head.

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PubMed1979

Progressive supranuclear palsy and normal-pressure hydrocephalus.

In three patients who fulfilled the clinical criteria of progressive supranuclear palsy, radiologic investigations suggested normal-pressure hydrocephalus. Shunt procedures in all three resulted in temporary improvement of gait, mentation, and bladder control, but gaze paralysis and extrapyramidal findings did not change.

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PubMed1979

Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy.

The fine structure of neurofibrillary tangles in the hippocampal gyrus, substantia nigra, pontine nuclei and locus coeruleus of the brain was postmortem studied in a case of progressive supranuclear palsy. Straight tubules and twisted tubules were observed in both the cortical and subcortical neurofibrillary tangles. Most tubules appeared separately in each neuron but a few straight tubules were mixed with the twisted tubules in the cortical tangles. The implication and possible significance of this findings are discussed.

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PubMed1979

[Polygraphic study of nocturnal sleep in three degenerative diseases: ALS, oligo-ponto-cerebellar atrophy, and progressive supranuclear palsy].

A polygraphic study of nocturnal sleep was carried out on 12 patients suffering from amyotrophic lateral sclerosis (ALS), 6 patients suffering from olivio-ponto-cerebellare-atrophia (OPCA) and 9 patients suffering from the Steele-Richardson disease (SR). A disturbance of sleep--if it exists--always runs parallel with the course of the disease. No disturbances were registered in the group of patients suffering from ALS, in the OPCA group a specific disease of paradoxal sleep is observed. The sleep disturbances are more global in the group of patients suffering from the SR disease. A particular electrooculogram was taken from the patients from the awakening and from the paradoxal sleep. Respiratory difficulties were registered on certain patients of each of the three groups. These sleep disturbances, registered by the polygraph, were compared with those observed on patients presenting a cortical disease.

Adult↗
PubMed2026

Apolipoprotein E Alleles Across the Spectrum of Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis.

We conducted a systematic review and meta-analysis of associations between apolipoprotein E (APOE) alleles and frontotemporal lobar degeneration (FTLD)-spectrum disorders. MEDLINE, Embase, CENTRAL, and Google Scholar were searched. APOE2 and APOE4 carrier status were compared between FTLD-spectrum disorders and healthy controls (HCs) or individuals with Alzheimer's disease (AD). Forty studies were included. APOE4 carriage was more frequent in frontotemporal dementia (FTD) compared with HC (OR = 1.72; 95% CI = 1.45-2.04) and less common than in AD (OR = 0.35; 95% CI = 0.29-0.42). In contrast, APOE2 carriage was less prevalent in FTD relative to HC (OR = 0.83; 95% CI = 0.70-0.98) but more frequent compared with AD (OR = 1.80; 95% CI = 1.29-2.52). APOE4 effects were most pronounced in behavioral variant FTD. In clinically confirmed progressive supranuclear palsy (PSP), APOE4 carriage was not associated with PSP. Analysis restricted to pathologically confirmed PSP cases, however, showed lower APOE4 carriage in PSP than in healthy controls (OR = 0.78, 95% CI = 0.65-0.94), although this association failed to reach the multiplicity-adjusted significance threshold. APOE2 carriage was not associated with PSP in either clinically established or pathologically confirmed samples. Evidence was insufficient to establish or exclude associations for other FTLD-spectrum disorders because of the limited available data. In conclusion, APOE alleles show distinct associations across the FTLD spectrum.

Humans↗
PubMed1975

Disorders of movement: a mercifully short primer.

The best known biochemical pathway in the brain is probably the nigrostriatal system. Even so, its relationship to Parkinsonism is incompletely understood. Its relationship to Huntington's chorea and the dyskinetic responses is even less well understood. The significance of this system in other related disorders such as Heterogeneous system degeneration, progressive supranuclear palsy, and striatonigral degeneration is still more speculative. In an effort to elucidate underlying neuropharmacology, trials with levodopa, amantadine, haloperidol, and lithium have been undertaken. This increasing concentration in biochemical mediators reflects changing patterns in neurology where new emphasis on biochemical pathways is replacing in current interest the emphasis previously placed on more traditional neuro-anatomical pathways such as the classical ascending sensory and descending motor tracts.

Bulbar Palsy, Progressive↗
PubMedDate not supplied

[Anatomo-clinical study of the Steele-Richardson-Olszewski presenile syndrome (Description of a case)].

A case of Progressive Supranuclear Palsy (The Steele-Richardson-Olszewski Syndrome) is presented. The chief clinical features are described. The neuropathological investigation confirmed the clinical diagnosis showing neuronal depopulation, gliosis and neurofibrillary degeneration with the characteristic topography in the brain stem, cerebellum and the subthalamic nuclei. They conclude with the hypothesis that sometimes a pregress cranial trauma can have an important role in the onset of this disease.

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