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

Emily Owens, Keith Anthony Josephs, Rodolfo Savica, Anhar Hassan, Bryan Klassen, James Howard Bower, Demetrius Maraganore, Joseph Matsumoto, J. E. Ahlskog

Research output: Contribution to journalArticle

12 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1-5
Number of pages5
JournalJournal of Neurology
DOIs
StateAccepted/In press - Sep 13 2016

Fingerprint

Natural History
Gait
Freezing
Progressive Supranuclear Palsy
Hypokinesia
Parkinsonian Disorders
Tremor
Neuroimaging
Handwriting
Dysarthria
Paralysis
Parkinson Disease
Counseling
Extremities
Demography
Phenotype
Brain

Keywords

  • Falls
  • Gait freezing
  • PAGF
  • PSP

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

The clinical spectrum and natural history of pure akinesia with gait freezing. / Owens, Emily; Josephs, Keith Anthony; Savica, Rodolfo; Hassan, Anhar; Klassen, Bryan; Bower, James Howard; Maraganore, Demetrius; Matsumoto, Joseph; Ahlskog, J. E.

In: Journal of Neurology, 13.09.2016, p. 1-5.

Research output: Contribution to journalArticle

@article{90799dcecb0f41eb8bdf533fcf5bb59c,
title = "The clinical spectrum and natural history of pure akinesia with gait freezing",
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 = "Falls, Gait freezing, PAGF, PSP",
author = "Emily Owens and Josephs, {Keith Anthony} and Rodolfo Savica and Anhar Hassan and Bryan Klassen and Bower, {James Howard} and Demetrius Maraganore and Joseph Matsumoto and Ahlskog, {J. E.}",
year = "2016",
month = "9",
day = "13",
doi = "10.1007/s00415-016-8278-x",
language = "English (US)",
pages = "1--5",
journal = "Journal of Neurology",
issn = "0340-5354",
publisher = "D. Steinkopff-Verlag",

}

TY - JOUR

T1 - The clinical spectrum and natural history of pure akinesia with gait freezing

AU - Owens, Emily

AU - Josephs, Keith Anthony

AU - Savica, Rodolfo

AU - Hassan, Anhar

AU - Klassen, Bryan

AU - Bower, James Howard

AU - Maraganore, Demetrius

AU - Matsumoto, Joseph

AU - Ahlskog, J. E.

PY - 2016/9/13

Y1 - 2016/9/13

N2 - 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.

AB - 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.

KW - Falls

KW - Gait freezing

KW - PAGF

KW - PSP

UR - http://www.scopus.com/inward/record.url?scp=84987639442&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84987639442&partnerID=8YFLogxK

U2 - 10.1007/s00415-016-8278-x

DO - 10.1007/s00415-016-8278-x

M3 - Article

C2 - 27624121

AN - SCOPUS:84987639442

SP - 1

EP - 5

JO - Journal of Neurology

JF - Journal of Neurology

SN - 0340-5354

ER -