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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
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Tremor in progressive supranuclear palsy.

Shinsuke Fujioka1, Avi A Algom2, Melissa E Murray3

  • 1Department of Neurology, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Department of Neuropathology, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Department of Neuroscience Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Department of Neurology, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, Fukuoka 814-0180, Japan.

Parkinsonism & Related Disorders
|April 4, 2016
PubMed
Summary

Tremor is more common in progressive supranuclear palsy (PSP) than previously thought, affecting 42% of patients. Carbidopa-levodopa may offer transient symptom relief for specific tremor types in PSP.

Keywords:
Carbidopa/levodopa therapyMAPT H1/H2 haplotypeNeuropathologyProgressive supranuclear palsyTremor

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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Progressive supranuclear palsy (PSP) is a neurodegenerative disease.
  • Tremor is considered a rare symptom of PSP.

Purpose of the Study:

  • To investigate the prevalence and characteristics of tremor in PSP patients.
  • To evaluate the response to carbidopa-levodopa therapy in PSP patients with tremor.

Main Methods:

  • Retrospective review of clinical data from 375 PSP patients.
  • Neuropathological assessment and immunohistochemical evaluation for tau and α-synuclein.
  • MAPT H1/H2 haplotype genotyping.

Main Results:

  • 42% (146/344) of PSP patients exhibited some form of tremor.
  • Postural/action and resting tremors were most common.
  • Tremor severity was predominantly mild; carbidopa-levodopa provided transient benefits for some tremor types.

Conclusions:

  • Tremor is a more frequent, though often inconspicuous, feature of PSP than previously recognized.
  • Carbidopa-levodopa therapy warrants consideration for managing specific tremor types in PSP, despite transient efficacy.