An autopsy case involving a 12-year history of amyotrophic lateral sclerosis with CIDP-like polyneuropathy

Tetsuya Akaishi, Maki Tateyama, Kazuhiro Kato, Emiko Miura, Rumiko Izumi, Kaoru Endo, Naoto Sugeno, Naoki Suzuki, Toru Baba, Tatsuro Misu, Akio Kikuchi, Takafumi Hasegawa, Sachiko Konosu-Fukaya, Fumiyoshi Fujishima, Hiroyoshi Suzuki, Ichiro Nakashima, Masashi Aoki

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Demyelinating polyneuropathy associated with amyotrophic lateral sclerosis (ALS) is quite rare. We herein present the case of a woman patient with a 12-year history of chronic inflammatory demyelinating polyneuropathy (CIDP)-like polyneuropathy who later developed bulbar palsy and respiratory failure. The autopsy findings revealed neuronal loss in the anterior horn and primary motor cortex with degeneration of the corticospinal tracts. Diffuse phosphorylated TAR DNA-binding protein of 43 kDa inclusions were observed in the anterior horn and cerebral cortices, including the temporal lobe. The final diagnosis was ALS with CIDP-like polyneuropathy. Compared with other reports of ALS with CIDP-like polyneuropathy, the present patient was younger and followed a relatively long clinical course, with no upper motor neuron signs.

Original languageEnglish
Pages (from-to)1371-1375
Number of pages5
JournalInternal Medicine
Volume53
Issue number12
DOIs
Publication statusPublished - 2014

Keywords

  • Amyotrophic lateral sclerosis
  • Autopsy
  • Chronic inflammatory demyelinating polyneuropathy
  • Onion bulb formation
  • TAR DNA-binding protein of 43 kDa

ASJC Scopus subject areas

  • Internal Medicine

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