PMID: 18717180Aug 23, 2008Paper

A nationwide survey of ALS patients on trachoestomy positive pressure ventilation (TPPV) who developed a totally locked-in state (TLS) in Japan

Rinshō shinkeigaku = Clinical neurology
Akihiro KawataHideaki Hayashi

Abstract

We conducted a nationwide survey of ALS patients on tracheostomy positive pressure ventilation (TPPV) who had developed a totally locked-in state (TLS) during the period from August to November 2006, in Japan. TLS occurred in 89 of 709 (13%) ALS patients on TPPV. On the second investigation, 29 of 41 patients with TLS showed complete palsy of more than two voluntary motor systems out of 4 motor systems [respiratory, pontine and medullar (bulbar), limb and external ocular motor systems] successively during a certain six months. The conventional classification of ALS based on the initial symptoms (bulbar, upper limb and lower limb type) was not found to be useful for predicting the onset of TLS. Seventy percent the patients developed TLS within 5 years after the start of TPPV. Thirty-seven (90%) patients finally developed total ophthalmoplegia at the onset of TLS, while one patient eventually developed complete bulbar palsy. One of 11 ALS patients with TLS, whom we experienced at Tokyo Metropolitan Neurological Hospital, also eventually showed complete palsy of the pontine (bulbar) motor system (inability to pull back the jaw). Due to the fact that TLS is a state of complete palsy of the voluntary motor systems for communication,...Continue Reading

References

Oct 1, 1989·Journal of the Neurological Sciences·H Hayashi, S Kato
Dec 24, 1997·Journal of Neurology·G D Borasio, R Voltz
Jan 14, 1999·Journal of Neurology, Neurosurgery, and Psychiatry·M I PolkeyP N Leigh
Jul 9, 2003·Neurology·Hideaki Hayashi, Edward Anthony Oppenheimer

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Citations

Mar 20, 2014·Neurodegenerative Disease Management·Susana Pinto, Mamede de Carvalho
Nov 11, 2014·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration
Jun 30, 2015·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Yuki NakayamaImaharu Nakano
Aug 19, 2016·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Dagmar KettemannThomas Meyer
Jan 11, 2017·The Cochrane Database of Systematic Reviews·Louisa NgMary Galea
Oct 6, 2017·The Cochrane Database of Systematic Reviews·Aleksandar RadunovicNaveed Mustfa
Jan 16, 2020·Journal of Neurology, Neurosurgery, and Psychiatry·Naoki HayashiGen Sobue
Jan 1, 2014·Journal of Neurodegenerative Diseases·U E WilliamsS K Oparah

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