Absence of cognitive, behavioral, or emotional dysfunction in progressive muscular atrophy.

摘要

Motor neuron disease (MND) is increasingly regarded as encompassing multisystem disorders. Mild cognitive dysfunction suggestive of frontal lobe involvement occurs in around 33% 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. 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 family history or childhood motor disorder suggestive of spinal muscular atrophy in any patients with PMA. Twenty-five healthy control participants (16 men, mean age 50 years; SD = 10.4) were recruited matched for gender, age, years of education, and full-scale IQ. PMA patients had slightly higher levels (U = 74.5, p = 0.029) of depression according to the Hospital Anxiety and Depression Scale,4 but these were well below that which would indicate clinical levels of an affective disorder. Participants had no history of eerebrovascular disease (including ischemia), hyperten-sion or diabetes, and no patients had symptoms of respiratory weakness (forced vital capacity <70%). All participants provided informed written consent, and the study was approved by the London Multicenter Research Ethics Committee. Participants underwent an extensive neuropsychological battery comprising tests of cognitive, behavioral, and emotional change.