Pathological observations of a long spinal cord lesion in a patient with multiple sclerosis.
anti-aquaporin-4 antibody
autopsy
long spinal cord lesion
multiple sclerosis
neuromyelitis optica spectrum disorder
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Jun 2022
Jun 2022
Historique:
revised:
29
10
2021
received:
01
07
2021
accepted:
27
11
2021
pubmed:
17
2
2022
medline:
7
6
2022
entrez:
16
2
2022
Statut:
ppublish
Résumé
We report an autopsy case of multiple sclerosis (MS) manifesting as a long spinal cord lesion. The patient was a Japanese woman. At the age of 59 years, she presented with a one-month history of progressive paraplegia, dysesthesia in the lower extremities, and urinary retention. Magnetic resonance imaging revealed a long, hyperintense lesion on T2-weighted images that extended from the inferior portion of the medulla oblongata to the cervical segments of the spinal cord and an isolated lesion at the T6 level. Cerebrospinal fluid (CSF) examination revealed the presence of oligoclonal bands and increased myelin basic protein levels (999 pg/mL). Serum antibody against aquaporin 4 (AQP4) was undetectable in this patient. She was diagnosed as having atypical MS and experienced symptom improvement following immunotherapy with corticosteroids and plasma exchange. She died of pneumonia and renal failure at the age of 62 years. Postmortem examination revealed a long demyelinating lesion that extended from the inferior portion of the medulla oblongata to the sacral segments of the spinal cord. The lesion was comprised of numerous demyelinating plaques with inflammatory cell infiltration. A long spinal cord lesion is usually indicative of neuromyelitis optica spectrum disorder (NMOSD), and there are limited reports of postmortem observations of long spinal cord lesions among patients with anti-AQP4 antibody-seronegative MS. Our findings suggest that the pathomechanisms of such long spinal cord lesion formation differ between anti-AQP4 antibody-seronegative MS and NMOSD.
Substances chimiques
Aquaporin 4
0
Autoantibodies
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
212-217Informations de copyright
© 2022 Japanese Society of Neuropathology.
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