What are the clinical subtypes of progressive supranuclear palsy (PSP)?

Answer

A study of 103 consecutive cases of pathologically proved PSP revealed 2 clinical subtypes.^[25]The first, called the Richardson syndrome, occurred in 54% of the cases and was associated with an early onset of postural instability, supranuclear gaze palsy, and cognitive dysfunction. The second, called PSP-Parkinsonism, occurred in 32% of cases and had features more typical of idiopathic PD, including a moderate response to levodopa. Fourteen cases (14%) could not be classified according to these criteria. At the molecular level, the 2 subtypes were characterized by distinct tau isoforms, suggesting that they are discrete nosologic entities.

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Media Gallery

Alpha-synuclein staining of the pons of an MSA case showing the positive glial inclusions (40x).
Rounded tau positive globose tangles in neurons of the subthalamic nucleus.
Neuronal loss in the substantia nigra with pigment-laden macrophages and neuromelanin pigment spilled into the neuropil background (pigment incontinence).
Tau-positive neuronal inclusions in neurons of the substantia nigra (no alpha synuclein-positive inclusions, as are found in Parkinson disease).
Subcortical white matter showing tau-positive perinuclear glial inclusions.
Subcortical white matter showing tau-positive perinuclear glial inclusions.
Cerebral cortex with ballooned neuron.
Cerebral cortex with tau-reactive cellular inclusions and neuropil threads.
Hematoxylin and eosin stained section of neocortex showing cortical Lewy body.
Neocortex stained alpha synuclein. The presence of cortical Lewy bodies is confirmed by the finding of alpha synuclein positive rounded cytoplasmic inclusion in neurons.
Neocortex stained with tau. Tau positive tangles in neurons of the cortex.

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Author

Stephen M Bloomfield, MD Associate Professor, Department of Neurosurgery, JFK Neuroscience Institute at JFK Medical Center, Hackensack Meridian School of Medicine at Seton Hall University

Stephen M Bloomfield, MD is a member of the following medical societies: American Academy of Pain Medicine, American Association for the Advancement of Science, American Association of Neurological Surgeons, American Epilepsy Society, American Medical Association, American Pain Society, Congress of Neurological Surgeons, International Association for the Study of Pain, Society for Neuroscience, West Virginia State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Philip A Hanna, MD Associate Professor of Neuroscience, JFK Neuroscience Institute at JFK Medical Center, Hackensack Meridian School of Medicine at Seton Hall University

Philip A Hanna, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Emad R Noor, MBChB Assistant Professor of Neurology and Clinical Neurophysiology, Hackensack Meridian School of Medicine at Seton Hall University; Attending Neurologist/Clinical Neurophysiologist, Neuroscience Institute at JFK Medical Center

Emad R Noor, MBChB is a member of the following medical societies: American Academy of Neurology, American Medical Association, Egyptian Society of Neurology, Psychiatry, and Neurosurgery

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alliance, Bioserenity, Ceribell, Eisai, Greenwich, LivaNova, Neurelis, Neuropace, Nexus, RSC, SK life science, Sunovion<br/>Serve(d) as a speaker or a member of a speakers bureau for: Alliance, Aquestive, Bioserenity, Eisai, Greenwich, LivaNova, Neurelis, SK life science, Sunovion<br/>Received research grant from: Cerevel, LivaNova, Greenwich, SK biopharmaceuticals, Takeda.

Additional Contributors

Arif I Dalvi, MD Director, Movement Disorders Center, NorthShore University Health System; Clinical Associate Professor of Neurology, University of Chicago Pritzker Medical School

Arif I Dalvi, MD is a member of the following medical societies: International Parkinson and Movement Disorder Society, European Neurological Society

Disclosure: Nothing to disclose.

Acknowledgements

Nestor Galvez-Jimenez, MD, MSc, MHA Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society

Disclosure: Nothing to disclose.

Christopher Luzzio, MD Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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