FIGURES LEGENDS
Figure 1: A ) Dropped head and proximal dominant limb weakness with kyphotic posture sustained before autologous stem cell transplant. B ) Notable amelioration of cervical muscular force, not adopting a kyphotic posture, after cyclophosphamide, bortezomib and dexamethasone.
Figure 2: Muscle biopsy with marked variation in fiber size and clusters of intracytoplasmic rods, often filling atrophic fibers, with no inflammatory cells, are the pathological hallmark. A : Gomori 100x; B : Gomori 400x; C : Myotilin Immunohistochemistry 200x; D : Electron dense rod in electron microscopy
Figure 3 . Bodyweight and muscle strength variation during clinical evolution and treatments.
(ASCT: autologous transplant; CyBorDex: cyclophosphamide, bortezomib, dexamethasone ; DTR: deep tendon reflexes ; Flex/ext: cervical flexion/extension; Hyper: hyperreflexia; IMNF: immunofixation in peripheral blood; Kg: kilograms; Len/dex: lenalidomide and dexamethasone; LL: lower limb; N: normal; Prox: proximal; Resp ins: respiratory insufficiency; Rot R / L: cervical rotation to the right/to the left ; UL: upper limb) Strength measured according to MRC ( Medical Research Council) scale; blue line: body weigh variation; orange shaded area: immunofixation negativity; green line: treatments)