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Journal of Neurology

, Volume 263, Issue 12, pp 2419–2423 | Cite as

The clinical spectrum and natural history of pure akinesia with gait freezing

  • Emily OwensEmail author
  • Keith A. Josephs
  • Rodolfo Savica
  • Anhar Hassan
  • Bryan Klassen
  • James Bower
  • Demetrius Maraganore
  • Joseph Matsumoto
  • J. E. Ahlskog
Original Communication

Abstract

Gait freezing as a presenting and relatively restricted condition is uncommon but a distinctive disorder. This entity was initially defined as “pure akinesia with gait freezing”, and later a neuropathological substrate of progressive supranuclear palsy has been recognized. Limited studies have reported the clinical evolution after presentation, which is important for patient counseling. The objective of this study was to assess the demographic and clinical features, treatment-response, neuroimaging, and evolution of pure akinesia with gait freezing. A retrospective review of patients with this phenotype as previously defined was performed. Patients included had no or minimal limb rigidity and/or bradykinesia and no resting tremor, and all underwent neuroimaging of the brain after onset. Inclusion criteria were met by 30 patients, who were followed up to 21 years after symptom onset. During their course, 28 patients had falls (93 %), 12 patients had dysarthria (40 %), and 13 had handwriting changes (43 %). All patients had progression of their gait disorder over time, but with a variable interval until falls occurred. None of the patients developed vertical gaze palsy or met diagnostic criteria for an alternative parkinsonian disorder. Pure akinesia with gait freezing is a distinctive disorder that can be recognized in the clinic. Despite the previously reported progressive supranuclear palsy-like neuropathology, the clinical course is much less aggressive and disabling than classic Richardson syndrome, although fall risk eventually develops in nearly all patients. Bradykinesia, tremor, and rigidity do not develop, distinguishing pure akinesia with gait freezing from Parkinson’s disease and other parkinsonian disorders.

Keywords

Gait freezing PSP Falls PAGF 

Notes

Acknowledgments

Funding was provided by the Mayo Clinic Rochester Department of Neurology.

Compliance with ethical standards

Conflicts of interest

There are no relevant conflicts of interest for all authors. Dr. Josephs receives support from the National Institute of Health. Dr. Klassen receives research support from Medtronic Inc. Dr. Bower receives research support from the Parkinson’s Disease Foundation.

Ethical standards

The study was performed in accordance with the 1964 Declaration of Helsinki and complies with the institutional ethical standards. Consent was obtained from all participants.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Emily Owens
    • 1
    Email author
  • Keith A. Josephs
    • 1
  • Rodolfo Savica
    • 1
  • Anhar Hassan
    • 1
  • Bryan Klassen
    • 1
  • James Bower
    • 1
  • Demetrius Maraganore
    • 2
  • Joseph Matsumoto
    • 1
  • J. E. Ahlskog
    • 1
  1. 1.Department of NeurologyMayo ClinicRochesterUSA
  2. 2.Neurological Institute NorthShore University HealthSystemEvanstonUSA

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