Subcortical neurofibrillary degeneration presenting as steele‐richardson‐olszewski and other related syndromes: A review of 90 pathologically verified cases

@article{deBruin1994SubcorticalND,
  title={Subcortical neurofibrillary degeneration presenting as steele‐richardson‐olszewski and other related syndromes: A review of 90 pathologically verified cases},
  author={Veralice Meireles Sales de Bruin and A. J. Lees},
  journal={Movement Disorders},
  year={1994},
  volume={9},
  url={https://api.semanticscholar.org/CorpusID:44405908}
}
Only 62 patients (69%) had clinically definite SROS based on the criteria of Maher and Lees, and neurofibillary degeneration of subcortical structures with involvement of the internal pallidum, the subthalamic nucleus, and substantia nigra was considered essential for the pathological diagnosis.

Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study.

Progressive onset of early postural instability with falls or supranuclear vertical palsy in the fifth decade, should suggest the diagnosis of PSP and appropriate treatment of the dysphagia needs to be explored.

Clinical heterogeneity in progressive supranuclear palsy: Problems of clinical diagnostic criteria of NINDS‐SPSP in a retrospective study of seven Japanese autopsy cases

It is demonstrated that the clinical features of PSP may change dramatically according to the disease stage and target symptoms should be selected based on time and stage to optimize patient quality of life.

Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy.

Clinical and pathological findings suggest that PSP-PNLA should be considered a variant of PSP, with earlier gait abnormalities and difficulty with handwriting, but later falls, rigidity and dysphagia than PSP.

Progressive supranuclear palsy: a clinicopathological study of 21 cases

The presence, at the time of death, of symptoms and signs that are used in published clinical criteria for the diagnosis of progressive supranuclear palsy was searched for in typical PSP cases.

Accuracy of clinical diagnosis of progressive supranuclear palsy

Although application of National Institute of Neurological Disorders and the Society for PSP possible category marginally improved the accuracy of initial clinical diagnosis, none of the existing operational criteria could significantly improve accuracy of the final clinical diagnosis.

Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

Criteria that support the diagnosis of progressive supranuclear palsy, and that exclude diseases often confused with PSP, are presented.

Clinical and Genetic Aspects of Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is, after Parkinson's disease, the most common form of degenerative parkinsonism. Several clinical features are used in the recognition of this disorder as well

Structural basis of dementia in neurodegenerative disorders

While in Alzheimer’s disease (AD) mental decline is mainly related to synaptic and neuritic pathologies, other degenerative disorders show variable substrates of dementia involving different cortical and/or subcortical circuits which may or may not be superimposed by cortical Alzheimer lesions.

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Sixteen autopsy‐verified progressive supranuclear palsy cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit with minimal benefit and in general the benefit was minimal and occurred only early in the course of illness.

[Steele-Richardson-Olszewski disease without ophthalmoplegia. 6 clinico-anatomic cases].

Six clinico-pathological cases (4 males and 2 females) with a Parkinson-like syndrome which lasted an average of 5 1/2 years are reported, which could be considered as a particular form of Steele-Richardson-Olszewski disease without ophthalmoplegia.

A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome.

A patient presenting with the characteristic clinical features of Steele-Richardson-Olszewski syndrome is described, in whom neuropathological examination revealed atypical features, including

[Senile subcortical neurofibrillary degeneration with the presence of twisted tubules and straight filaments. Atypical form of progressive supranuclear paralysis].

This case with wide-spread subcortical neurofibrillary changes, cell loss and gliosis in a 81 year old man is described, interpreted as a variety of progressive supranuclear palsy despite the lack of the typical suPRanuclear ophtalmoplegia and of superior colliculi involvement.

Motor nuclear involvement in progressive supranuclear palsy.

The second case of a woman studied pathologically as well as clinically, and striking ophthalmoplegia and tonic rigidity as the outstanding clinical features are presented.

Severe cerebral atrophy in progressive supranuclear palsy: a case report.

A 60-year-old woman with a history of hypertension and chronic headache initially presented with irritative personality change and mild but steadily progressive dementia and oral tendency, left-sided

Heterogeneous system degeneration of the central nervous system associated with peripheral neuropathy

The purpose of this paper is to describe the clinical and pathological findings of a case which falls into this category and which presents additional features not hitherto described.

Diffuse lewy body disease presenting with supranuclear gaze palsy, parkinsonism, and dementia: A case report

A 67‐year‐old man with a family history of parkinsonism had visual complaints due to difficulty in convergence, which was followed 2 years later by development of bradykinesia and rigidity, which revealed diffuse Lewy body disease with no evidence of neurofibrillary tangles involving either subcortical or brain stem structures.

NEUROFIBRILLARY TANGLES IN PROGRESSIVE SUPRANUCLEAR PALSY: ELECTRON MICROSCOPIC OBSERVATIONS

The most severely affected regions of the brain in PSP included the subthalamic, vestibular, and dentate nuclei in addition to those usually involved in postencephalitic Parkinsonism.

Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy?

It is suggested that the late onset dementia with argyrophilic grains syndrome is also characterized by the presence of tangles and threads with the topographical distribution of progressive supranuclear palsy.
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