Morphometric quantification of the cervical limb motor cells in controls and in amyotrophic lateral sclerosis

The cortico-diaphragmatic pathway involvement in amyotrophic lateral sclerosis: Neurophysiological, respiratory and clinical considerations

“…This finding is in line with previous electrophysiological reports claiming that in ALS, ventilatory failure from diaphragmatic weakness may be due to a substantial degeneration of phrenic motor neurones [43,44]. In fact, phrenic motor neurones are located in regions very likely to be affected by the disease [45], and major morphometric abnormalities have been reported in the phrenic nerve [46].…”

Dissociated small hand muscle involvement in amyotrophic lateral sclerosis detected by motor unit number estimates

“…7 The pattern of degeneration of anterior horn cells in the spinal cord is random, and there is no predilection for certain motor neuron cell columns, 13 although the C-8 segments tend to be preferentially involved in ALS. 12,14 The anterior horn cells to both the APB and ADM muscles are segmentally close and equal involvement of their respective motor neuron pools would be anticipated. However, the APB corticomotor neurons far outnumber those of the ADM, and the connections of the former are much more extensive than those of the latter.…”

Measures and markers in Amyotrophic Lateral Sclerosis

“…The demonstration of denervation atrophy of muscle fibers is usually associated with increased connective tissue; unlike other denervating diseases such as neuropathy, muscle fiber type grouping is less uniformly observed. 67,68 Muscle biopsy is useful in patients for whom the involvement of lower motor neurons is in doubt either clinically or physiologically. It is not part of the routine evaluation at most centers, but remains a useful ancillary test in atypical patients.…”