Frontostriatal deficit in Motor Neuron Disease/Amyotrophic Lateral Sclerosis (MND/ALS)

So far, cognitive derangements in MND/ALS have not been widely studied. Nevertheless, it seems that in subgroups of patients cognitive functions are impaired in different degree, so that often at least two sub-types of the syndrome are reported: Motor Neuron Disease/Amyotrophic Lateral Sclerosis/Dementia Syndrome (MND/ALS/DS) and Motor Neuron Disease/Amyotrophic Lateral Sclerosis/ Aphasia Syndrome (MND/ALS/AS. A third subtype showing both symptoms of cognitive impairment may be identified in subgroups of patients and denominated Motor Neuron Disease/Amyotrophic Lateral Sclerosis/Dementia-Aphasia Syndrome (MND/ALS/DAS). Frontostriatal system is reported as a network heavily damaged in MND/ALS/DS, MND/ALS/AS, MND/ALS/DAS. The system is plausibly responsible of motor skills and verbs production, hence to become aware of a possible frontostriatal deficit in subgroup of MND/ALS patients might consent us to link at the brain level (motor) action and verbs and possibly ideomotor praxia and verbs. We have used Goal-Oriented Perception Task (GOPT) and Action Fluency Task (AFT) in order to detect with some accuracy impairments related to gestaltic analysis directed toward a goal, and verb retrieval deficits possibly underlying executive system dysfunction that destabilizes the ability to mentally coordinate the information associated with a verb. These tests should consent to detect possible frontostriatal derangements. We have tested 10 MND/ALS patients and 10 healthy subjects matched fore age, sex and laterality. AFT showed that 3 out of 6 patients are heavily impaired in this test (6.3 (mean) verbs generated vs 13.3 of the control group). GOPT detected a remarkable impairment in all patients: p=0.0021 (grammatical side), p=0.0002 (perceptual side). Reported frontostriatal deficit in MND/ALS seems confirmed by this study, and probably it is more easily detected by GOPT than by AFT