Limb apraxia in corticobasal degeneration and progressive supranuclear palsy

Neurology. 2005 Feb 8;64(3):448-53. doi: 10.1212/01.WNL.0000150732.92567.BA.

Abstract

Objective: Corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) share pathologic features, and cortical and subcortical signs. Apraxia is frequently described in CBD and sometimes in PSP; however, it is difficult to distinguish ideomotor from limb-kinetic apraxia, and apraxia frequency is unclear. The authors set out to clarify the nature and frequency of apraxia in these diseases.

Methods: The authors compared probable CBD and PSP patients, matched for motor disability, to healthy age-matched controls on cognitive tests and the De Renzi ideomotor apraxia test.

Results: Cognitive impairment was similar, but more CBD (70.8%) than PSP (36%) patients had apraxia. CBD patients committed more apraxic errors of awkwardness and were more compromised on simple gestures; PSP patients committed more sequence errors.

Conclusions: While progressive supranuclear palsy (PSP) patients had ideomotor apraxia, the peculiar gesture compromise in corticobasal degeneration (CBD) suggests that limb-kinetic apraxia is dominant. In both illnesses, the movement production system of Roy and Square appears compromised: in CBD defective control of muscle activation seems likely, producing clumsy movements; in PSP, control of motor program activation appears defective, resulting in sequence errors and perseverations. The De Renzi test can reliably estimate apraxia frequency and may be used to distinguish limb-kinetic from ideomotor apraxia.

Publication types

  • Comparative Study

MeSH terms

  • Aged
  • Apraxia, Ideomotor / etiology*
  • Apraxia, Ideomotor / physiopathology
  • Apraxias / etiology*
  • Apraxias / physiopathology
  • Arm / physiopathology
  • Diagnosis, Differential
  • Dystonia / etiology
  • Dystonia / physiopathology
  • Female
  • Gestures
  • Humans
  • Male
  • Middle Aged
  • Movement Disorders / etiology
  • Movement Disorders / physiopathology
  • Neurodegenerative Diseases / complications*
  • Neurodegenerative Diseases / diagnosis
  • Neurodegenerative Diseases / physiopathology
  • Supranuclear Palsy, Progressive / complications*
  • Supranuclear Palsy, Progressive / diagnosis
  • Supranuclear Palsy, Progressive / physiopathology