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Motor neuron disease (MND) is increasingly regarded as encompassing multisystem disorders. Mild cognitive dysfunction suggestive of frontal lobe involvement occurs in around 33%1 of nondemented patients with ALS. Impaired performance has been identified among patients with ALS on tasks of executive function, such as planning, sequencing, initiating, and sustaining attention and, in particular, on tests of verbal fluency.2 There has also been some evidence to suggest abnormalities of language functioning and memory,3 although these have not been found consistently. Progressive muscular atrophy (PMA) affects only the lower motor neurons and is associated with longer survival than ALS. In this study, we sought to investigate the presence of cognitive, behavioral, or emotional dysfunction in this group.
Methods.
We recruited 12 patients with a diagnosis of PMA (eight men, mean age 52 year; SD = 9.0) from the MND Care & Research Centres at King’s College Hospital, London, and the Royal Victoria Infirmary, Newcastle. There was no significant …
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