The width of the third ventricle associates with cognition and behaviour in motor neuron disease

Autores de IIS La Fe

• Juan Francisco Vázquez Costa

  Autor

• Sara Carratalà Boscà

  Autor

• José Ignacio Tembl Ferrairo

  Autor

• Victoria Fornés Ferrer

  Autor

• Jordi Perez Tur

  Autor

• 

  Luis Martí Bonmatí

  Autor

• 

  Teresa Sevilla Mantecón

  Autor

Grupos

• Hemostasia, Trombosis, Arteriosclerosis y Biología vascular

• Investigacion Biomédica en Imagen (GIBI 2-30)

• Investigación en Neuroinmunología y Esclerosis Múltiple

• Patología Neuromuscular y Ataxias

Abstract

Objectives An enlarged width of the third ventricle (WTV) has been described in amyotrophic lateral sclerosis (ALS) patients, although its clinical meaning is unknown. The aims of this study were to evaluate the contribution of demographical, clinical and genetic factors to the WTV in different motor neuron disease (MND) phenotypes and to assess its brain structural correlates. Materials and methods The WTV was measured by transcranial ultrasound in 107 MND patients (82 diagnosed with classical ALS, 16 with progressive muscular atrophy and 9 with primary lateral sclerosis) and 25 controls. Genetic analysis, and neurological and neuropsychological examinations were performed in patients. Brain volumetric analysis of MR images was obtained in 85 patients. The association of WTV with demographical, clinical, genetic and neuropsychological variables as well as with brain volumes was assessed by multivariable models. Results Eighteen patients were diagnosed with genetic MND and 42.3% of patients showed executive or behavioural impairment (EBI). MND patients showed larger WTV than controls. The WTV was significantly associated with age, spinal onset and the presence of EBI, but not with the genetic background, the phenotype or disability. Greater WTV was also associated with reduced subcortical grey matter volume, but not with the cortical or the white matter volume. Conclusions The enlargement of the WTV found in the different MND phenotypes is attributable to the subcortical grey matter atrophy and is associated with cognitive and behavioural impairment. Larger longitudinal studies are needed to determine its role as biomarker in MND patients with frontotemporal dementia.

Keywords

• amyotrophic lateral sclerosis; cognitive impairment; motor neuron disease; primary lateral sclerosis; progressive muscular atrophy; third ventricle; ultrasound