title: Orofacial apraxia in motor neuron disease.
abstract: BACKGROUND
  Cognitive and behavioral impairments are considered to occur frequently in amyotrophic lateral sclerosis/motor neuron disease (MND). Rarely, apraxia has been reported in MND. Orofacial, or buccofacial, apraxia is characterized by a loss of voluntary control of facial, lingual, pharyngeal and masticatory muscles in the presence of preserved reflexive and automatic functions of the same muscles.
  
  
  METHODS
  We report a patient with MND who presented with spastic dysarthria and asymmetric orofacial apraxia. She progressed to frontotemporal dementia (FTD).
  
  
  RESULTS
  Clinical and neurophysiological examinations were suggestive of bulbar-onset MND-FTD. Tractography showed a reduction of fractional anisotropy in the centrum semiovale, corona radiata, corticomedullary pathway and inferior aspect of the medulla; the changes were more severe on the left side. To our knowledge, this is the first report of an asymmetric presentation of an apraxic syndrome in MND-FTD.
authors.affiliation: Department of Neurosciences, Centro Hospitalar Lisboa Norte-Hospital de Santa Maria, University of Lisbon, Lisbon, Portugal.
authors.forename: Patrícia Pita
authors.initials: PP
authors.lastname: Lobo
authors.forename: Susana
authors.initials: S
authors.lastname: Pinto
authors.forename: Luz
authors.initials: L
authors.lastname: Rocha
authors.forename: Sofia
authors.initials: S
authors.lastname: Reimão
authors.forename: Mamede
authors.initials: M
authors.lastname: de Carvalho
keywords: Amyotrophic lateral sclerosis; Motor neuron disease; Orofacial apraxia; Primary lateral sclerosis; Tractography