XXXX . A clinical study of 36 cases.

@entity1 @entity9718 @entity3520 @entity2045 @entity2845 @entity7463 @entity1742 @entity528 @entity4653
The presenting features and their subsequent evolution in 36 @entity1 with pathologically proven or clinically probable @entity528 are described. The most common initial complaint was of a unilateral 'clumsy, stiff or jerky arm' (n = 20). Typically the arm was @entity2845 , rigid and apraxic. In about a third of these the arm was held in a striking and characteristic fixed @entity3520 . Jerking of the arm, due to action and stimulus-sensitive @entity2045 accompanied these symptoms in about a third of the cases. The next most common presentation (n = 10) was difficulty walking due to clumsiness and loss of fine motor control of one leg due to @entity1742 or dysequilibrium, or a combination of both. Sensory symptoms in the affected arm heralded the onset of illness in three and accompanied a motor disturbance in two cases. Presentation with @entity4653 or a behavioural syndrome were rare. The symptoms progressed slowly, usually involving first the ipsilateral arm and leg, but gradually spread to affect all four limbs. After a mean follow-up of 5.2 years (range 2-8 years) gait difficulties and a @entity7463 had emerged in most @entity1 and @entity4653 and @entity9718 were common. Higher mental function was relatively preserved in most and a cortical sensory loss was evident in a quarter of cases.
