Pharr et al. report the incidence and severity of ideomotor apraxia
(IMA) in patients with corticobasal degeneration (CBD) and progressive
supranuclear palsy (PSP).
Both patient groups were impaired, but at different degrees: PSP
patients impairment was limited only to transitive tasks, while CBD
patients were more severely affected, their performance being lower than
those of PSP patients in all conditions. In there paper no mention is
made about the presence of apraxia in daily living activity and its
consistency, the lack of automatic-voluntary dissociation and finally of
the scarcity of the most characteristic error type shown by focal (left
parietal) IMA patients, the use of hands or fingers to represent an
intended object (body-part-as-object). [1]
Apraxia is the neuropsychological hallmark of CBD. However, it must
be reminded that CBD apraxia is characterized by its presence in daily
living activities, its asymmetry, being limited, at least in the initial
phase, to one upper limb, its consistency. [2, 3]
Most typical is a disorder of motor control and specific activation
of simple movements, present in the execution of uni-manual and bi-manual
dexterity tasks. This pattern is reminiscent of a neglected form of
apraxia, the melokinetic or limb-kinetic apraxia (LKA), that, according to
Liepmann’s original description consists of “impaired, coarse, mutilate
execution of simple movements”, while more complex motion may not even be
possible, though the ideational plan is spared. [4] “Even the simplest
and more practiced movements, for which we have purely kinetic memory, are
coarse and rough, the skill deriving from daily experience being lost.”
The following points seem to characterize the apraxic deficit of CBD
patients: the selectivity of involvement of the limbs, in a centripetal
fashion, with sparing of oro-facial movements, severity of apraxic
deficit, which from the start affect daily life activities, leading in the
latest stages to a complete loss of the autonomy, thus in contrast to the
usual pattern of apraxia following focal lesions, which in most cases is
apparent only in testing conditions.
References:
1) Pharr V, Uttl B, Stark M, Litvan I, Fantie B, Grafman J. Comparison
of apraxia in corticobasal degeneration and progressive supranuclear
palsy. Neurology 2001;56:957-963.
2) Pillon B, Blin J, Vidailher M. The neuropsycholgocial pattern of
corticobasal degeneration. Neurology 1995;45:1477-1483.
3) Denes G, Mantovan MC, Gallana A, Cappelletti JY. Limb kinetic
apraxia. Movement Disorders 1998;13:468-477.
4) Liepmann H. Apraxia. (A translation from Ergebnisse der gesamten
Medizin, 1920;1:516-543) In Research Bulletin #506. Department of
Psychology, The University of Western Ontario, 1980.