M. Habib et al., CHARACTERIZING GESTURAL DISTURBANCES IN P RIMARY PROGRESSIVE APRAXIA - IMPLICATIONS FOR DIAGNOSIS AND NOSOGRAPHY, Revue neurologique, 151(10), 1995, pp. 541-551
Five cases are reported of patients with so-called primary progressive
apraxia, defined as a slowly worsening disturbance of gestural abilit
ies, without other major cognitive changes during a long period, in re
lation to degenerative cortical atrophy. All five cases, as other case
s in the litterature, share the following common features : 1) asymmet
rical onset of upper limb clumsiness, more often involving the left si
de, later involving the contralateral side and lower limbs; 2) after a
variable delay; the occurrence of symptoms suggesting subcortical inv
olvement (akinesia, limb stiffness, various kinds of movement disorder
s, dystonia, paresis of vertical gaze); 3) diffuse cortical atrophy ty
pically more pronounced in the superior parietal cortex opposite to th
e first side affected. The unusual nature of apraxia in all these case
s is pointed out and referred to as Luria's ''kinesthaesic apraxia'' a
scribed to a loss of ''selectivity'' of distal elementary movements. T
his pattern of symptoms and their specific outcome could represent a d
istinct entity.