Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia

Topic: Brain proteins, microglia and lab models

We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis.

Key findings

• These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis.

Why it matters

This single autopsy case showed that when tau pathology was prominent in the motor cortex but minimal in the basal ganglia and brain stem, a person with progressive supranuclear palsy can lack all its classic clinical features and show only a slowly progressive upper motor syndrome. It is one case report and offers no treatment, cure or prevention for Alzheimer's disease.

New to this subject? Start with our plain-English guide: Digital health →

Study details

• Evidence strengthLow

Read the original

• DOI: 10.1016/j.jns.2012.09.005
• PubMed: 23026537
• Original source →

May need a subscription OpenAlex found no free copy of the full text (checked 6 Feb 1020), so the publisher may charge for it. The PubMed abstract is free.

@article{nagao2012-23026537,
  author  = {Nagao, S. and Yokota, O. and Nanba, R. and Takata, H. and Haraguchi, T. and Ishizu, H. and Ikeda, C. and Takeda, N. and others},
  title   = {{Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia}},
  journal = {Journal of the neurological sciences},
  year    = {2012},
  doi     = {10.1016/j.jns.2012.09.005},
  pmid    = {23026537},
  url     = {https://doi.org/10.1016/j.jns.2012.09.005}
}

Studies this one cites

Referenced by this study and also in the alzhub library, so you can read them here.

• Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism Brain : a journal of neurology · 2005-03-23 · usefulness score 55/100 · Getting a diagnosis
• Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges The Lancet. Neurology · 2009-03-01 · usefulness score 54/100 · Getting a diagnosis
• Neuropathology of variants of progressive supranuclear palsy Current opinion in neurology · 2010-08-01 · usefulness score 51/100 · Digital health tools
• Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy Current opinion in neurology · 2008-12-01 · usefulness score 46/100 · Digital health tools
• Clinicopathological characterization of Pick's disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions Acta neuropathologica · 2009-02-05 · usefulness score 44/100 · Digital health tools

Later studies that cite this one

1 study in the alzhub library lists this one in its references.

• Clinical Spectrum of Tauopathies Frontiers in neurology · 2022-07-14 · usefulness score 59/100 · Digital health tools

Related studies in Digital health tools

• Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia Neurocase · 2011-12-20 · usefulness score 40/100
• Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology European neurology · 2003-01-01 · usefulness score 40/100
• Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy Movement disorders : official journal of the Movement Disorder Society · 2007-11-01 · usefulness score 52/100
• Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration Journal of neurology · 1999-09-01 · usefulness score 48/100
• Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients Parkinsonism & related disorders · 2013-08-19 · usefulness score 43/100
• The syndrome of 'pure akinesia' and its relationship to progressive supranuclear palsy Neurology · 1994-06-01 · usefulness score 40/100

Similar studies

• Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria 2017 · Getting a diagnosis
• Progressive Supranuclear Palsy—A Global Review 2025 · Risk factors
• What features improve the accuracy of the clinical diagnosis of progressive supranuclear palsy-parkinsonism (PSP-P)? 2010 · Disease mechanisms
• Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study 1997 · Clinical safety
• Diagnosis Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Syndrome 2020 · Biomarkers
• Atypical progressive supranuclear palsy with corticospinal tract degeneration 2006 · Disease mechanisms

More on Digital health tools

• A review that covers this ground Tools and talk: an evolutionary perspective on the functional deficits associated with amyotrophic lateral sclerosis Muscle & nerve · 2013-12-18 · usefulness score 39/100
• Newest in this subject Speaker-Wise Dementia Screening fromNaturalistic Speech with Lexical Entropy, Semantic Drift, and Multimodal Fusion Research Square · 2026-10-21 · Preprint — not yet peer-reviewed · usefulness score 16/100
• Newest in this subject Integrating Multisource Target Evidence with Machine-Learning Potency Prediction for Alzheimer’s Disease Drug Discovery bioRxiv · 2026-10-20 · Preprint — not yet peer-reviewed · usefulness score 16/100

See all studies in Digital health tools →