title: [Amyotrophic lateral sclerosis manifesting as cognitive disorders. Value of brain perfusion scintigraphic tomography in intensive care].
abstract: BACKGROUND
  Cognitive disorders have been described in amyotrophic lateral sclerosis, but usually after the diagnosis has ben established.
  
  
  METHODS
  A 57-year-old man was intubated for acute respiratory distress subsequent to pneumonia and diaphragm palsy. He had a 2-year history of drug-resistant depression and deterioration of cognitive functions. A pyramidal syndrome associated with biopsy-proven chronic neurogenic atrophy led to the diagnosis of amyotrophic lateral sclerosis. The electromyogram did not contribute to diagnosis. Brain MRI only evidenced moderate bilateral frontal-temporal atrophy. Brain SPECT demonstrated major perfusion defects in the frontal lobes.
  
  
  CONCLUSIONS
  This patient had amyotrophic lateral sclerosis and frontal-temporal dementia with an unusually late onset clinical presentation: cognitive disorder was the inaugural sign. Brain SPECT and muscle biopsy enabled us to identify the cortical and peripheral motor neurone involvement in this uncooperative intensive care patient totally dependent on mechanical ventilation.
authors.affiliation: Services de Pneumologie et Réanimation médicale, CHU Bretonneau, Tours.
authors.forename: E
authors.initials: E
authors.lastname: Hazouard
authors.forename: A M
authors.initials: AM
authors.lastname: Bergemer-Fouquet
authors.forename: C
authors.initials: C
authors.lastname: Hommet
authors.forename: P
authors.initials: P
authors.lastname: Corcia
authors.forename: J P
authors.initials: JP
authors.lastname: Cottier
authors.forename: D
authors.initials: D
authors.lastname: Beauchamp
authors.forename: J L
authors.initials: JL
authors.lastname: Baulieu
authors.forename: B
authors.initials: B
authors.lastname: de Toffol
mesh_terms: D001706:Biopsy; D003422:Critical Care; D003704:Dementia / Q000000981:diagnostic imaging* / Q000473:pathology; D004292:Dominance, Cerebral / Q000502:physiology; D005625:Frontal Lobe / Q000098:blood supply / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000000981:diagnostic imaging* / Q000473:pathology; D018482:Muscle, Skeletal / Q000473:pathology; D009483:Neuropsychological Tests; D012039:Regional Blood Flow / Q000502:physiology; D012151:Resuscitation; D013702:Temporal Lobe / Q000098:blood supply / Q000000981:diagnostic imaging; D015899:Tomography, Emission-Computed, Single-Photon