title: An Autopsy Case of Amyotrophic Lateral Sclerosis with Diaphragm Pacing.
abstract: Respiratory insufficiency is a critical problem in amyotrophic lateral sclerosis (ALS) patients. We herein present the case of an autopsied patient with sporadic ALS who underwent diaphragm pacing (DP). The pathology showed several localized adhesions with a markedly atrophied diaphragm. A marked loss of motor neurons with Bunina bodies and phosphorylated TDP-43 positive inclusions was found in the spinal cord and primary motor cortex. Mild hyalinization and a few multinucleated giant cells were present around the electrode tracks in the diaphragm. However, no infiltration of inflammatory cells was detected. Our findings suggest that full-time DP might not cause severe damage to adjacent diaphragm tissue.
authors.affiliation: Department of Neurology, Shonan Fujisawa Tokushukai Hospital, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Ito
authors.forename: Tetsumasa
authors.initials: T
authors.lastname: Kamei
authors.forename: Sanae
authors.initials: S
authors.lastname: Odake
authors.forename: Masayuki
authors.initials: M
authors.lastname: Nakano
authors.forename: Riki
authors.initials: R
authors.lastname: Okeda
authors.forename: Shunsaku
authors.initials: S
authors.lastname: Kohriki
authors.forename: Jun
authors.initials: J
authors.lastname: Kawachi
authors.forename: Raymond P
authors.initials: RP
authors.lastname: Onders
authors.forename: Fumihito
authors.initials: F
authors.lastname: Yoshii
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D001344:Autopsy; D003964:Diaphragm; D004599:Electric Stimulation Therapy / Q000379:methods*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology