title: ALS patients with ability to communicate after long-term mechanical ventilation have confined degeneration to the motor neuron system.
abstract: OBJECTIVE
  To clarify the position in the amyotrophic lateral sclerosis (ALS) spectrum, of a subgroup of patients who maintained the ability to communicate after long-term mechanical ventilation (LTMV) by tracheostomy.
  
  
  METHODS
  We undertook a clinicopathological investigation of sporadic ALS in three patients who maintained the ability to communicate after approximately 30-year survival on LTMV by tracheostomy.
  
  
  RESULTS
  The age of onset and duration of disease was 48 years and 31 years in patient 1, 55 years and 29 years in patient 2, and 31 years and 33 years in patient 3, respectively. Each patient displayed slow disease progression. In all patients, both upper and lower motor neurons were markedly degenerated, while other neuronal systems and the brainstem tegmentum were spared. A few normal-looking motor neurons remained in the anterior horn of the spinal cord. There were no TAR DNA-binding protein 43-immunoreactive inclusions in the lower motor neurons in any patient and only occasional inclusions in the cerebral cortex of one patient.
  
  
  CONCLUSIONS
  The clinicopathological findings of these three patients suggest that there is a distinct subgroup of ALS patients characterized by the above-mentioned features.
authors.affiliation: Department of Pathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan; Department of Neurology, Tokyo Metropolitan Kita Medical and Rehabilitation Center for the Disabled, Tokyo, Japan. Electronic address: mochi@nihon-u.ne.jp.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Mochizuki
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Hayashi
authors.affiliation: Laboratory of Nursing Research for Intractable Disease, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Nakayama
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Shimizu
authors.affiliation: Emergency Department of Atsugi City Hospital, Kanagawa, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kamide
authors.affiliation: Division of Integrated Care and Whole Person Care, Department of Comprehensive Medicine, Research and Development Center for New Medical Frontiers, Kitasato University School of Medicine, Kanagawa, Japan.
authors.forename: Mieko
authors.initials: M
authors.lastname: Ogino
authors.affiliation: Department of Pathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Komori
authors.affiliation: Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
authors.forename: Masato
authors.initials: M
authors.lastname: Hasegawa
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Isozaki
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000628:therapy; D003142:Communication; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D009410:Nerve Degeneration / Q000175:diagnosis* / Q000628:therapy; D012121:Respiration, Artificial / Q000639:trends*; D013997:Time Factors
keywords: Amyotrophic lateral sclerosis; Communication ability; Long-term survival; Neuropathological findings; Tracheostomy invasive ventilation