Wild-Type Transthyretin Amyloidosis Occurring in the Ligamentum Flavum of the Cervicothoracic Spine.

Abstract:

BACKGROUND:Wild-type transthyretin amyloid (ATTRwt) has been noted to deposit in the ligamentum flavum of the spine. Prior studies have focused on ATTRwt in the lumbar region, but studies discussing its presence in other levels of the spine are lacking. We report on the presentation of patients with confirmed amyloid in the cervicothoracic regions and discuss the literature to date. METHODS:We retrospectively identified patients at a single institution who underwent surgery for spinal stenosis and had pathologic specimens sent for amyloidosis testing with Congo red staining. ATTRwt was confirmed by the presence of transthyretin amyloid by typing and the absence of mutations in the TTR gene sequence. A final study group of patients with ATTRwt and spinal involvement was established (n = 27). RESULTS:Of 27 patients with amyloid in the spine, 24 (89%) had amyloid present in the lumbar region, 2 (7%) had amyloid in the cervical region, and 1 (4%) had amyloid in the thoracic region. The median age at which patients in the study underwent surgery was 71 years (interquartile range: 9). Spinal stenosis was the indication for surgery in 26 of 27 (96%) patients. Surgery involved 1 or 2 spinal levels in 24 of 27 (89%) patients. CONCLUSIONS:ATTRwt amyloid predominantly deposits in the lumbar region, but it can also be present in the cervical and thoracic regions. While the lumbar regions should remain a focus for evaluation of ATTRwt amyloidosis, the cervicothoracic region should not be ignored.

journal_name

World Neurosurg

journal_title

World neurosurgery

authors

George KM,Dowd RS,Nail J,Yu A,Mastroianni M,Wang AY,Arkun K,Patel A,Kryzanski J,Comenzo R,Riesenburger RI

doi

10.1016/j.wneu.2020.06.228

subject

Has Abstract

pub_date

2020-10-01 00:00:00

pages

e325-e330

eissn

1878-8750

issn

1878-8769

pii

S1878-8750(20)31506-0

journal_volume

142

pub_type

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