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Figure 1: (Case 1: a-d) A man, aged 27 years, symptomatic for 7 years. (a and b) Oblique amyotrophy and wasting of intrinsic hand muscles. (c and d) Mild scoliosis of the spine. (Case 2: e-h) A man, aged 41 years, with upper limb involvement for 17 years and development of pyramidal signs in lower limb for 2 years. (f-h) Severe asymmetrical wasting of hand muscles and medial forearm (left > right). (Case 3: i-k) A 57-year old man, symptomatic from 15 years of age developed new-onset lower limb symptoms for 12 years. The illness progressed asymmetrically (left > right) and then attained a stationary phase

Figure 1: (Case 1: a-d) A man, aged 27 years, symptomatic for 7 years. (a and b) Oblique amyotrophy and wasting of intrinsic hand muscles. (c and d) Mild scoliosis of the spine. (Case 2: e-h) A man, aged 41 years, with upper limb involvement for 17 years and development of pyramidal signs in lower limb for 2 years. (f-h) Severe asymmetrical wasting of hand muscles and medial forearm (left > right). (Case 3: i-k) A 57-year old man, symptomatic from 15 years of age developed new-onset lower limb symptoms for 12 years. The illness progressed asymmetrically (left > right) and then attained a stationary phase