Central nervous system in motor neuron disease

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Brownell B, Oppenheimer DR, Trevor Hughes J. The central nervous system in motor neuron disease. J Neurol Neurosurg Psychiatry 1970;33:338–57.

Neurodegenerative syndromes, including Amyotrophic lateral sclerosis  (ALS), are complex polygenic disorders resulting in multisystem impairment of neocortical network connectivity. Specific to ALS is dysfunction of the unique human corticomotoneuronal system, which is the basis of many early features of ALS, a singularly human disorder. Early deficits include loss of vocalisation requiring complex respiration, impaired fractionation of digits and thumb opposability, difficulty with upright walking with ability to navigate uneven and tricky surfaces while erect, and as part of Frontotemporal demention (FTD), impaired language and associated socialisation.1 The hallmark TAR DNA-binding protein 43  (TDP-43) pathology, seen in >95% of patients with ALS, is largely restricted to corticofugal projecting neurons. This when taken together with sophisticated MRI imaging and threshold tracking using transcranial magnetic stimulation convincingly points to a cortical origin of...

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