Pjb. Dyck et al., Microvasculitis in non-diabetic lumbosacral radiculoplexus neuropathy (LSRPN): Similarity to the diabetic variety (DLSRPN), J NE EXP NE, 59(6), 2000, pp. 525-538
Citations number
46
Categorie Soggetti
Neurosciences & Behavoir
Journal title
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
Diabetic lumbosacral radiculoplexus neuropathy (DLSRPN) has been shown to b
e due to ischemic injury from microvasculitis. The present study tests whet
her ischemic injury and microvasculitis are the pathologic cause of non-dia
betic lumbosacral radiculoplexus neuropathy (LSRPN), and whether the pathol
ogic alterations are different between LSRPN and DLSRPN. We studied distal
cutaneous nerve biopsies of 47 patients with LSRPN and compared findings wi
th those of 14 age-matched healthy controls and 33 DLSRPN patients. In both
disease conditions, we found evidence of ischemic injury (multifocal fiber
degeneration and loss, perineurial degeneration and scarring, characterist
ic fiber alterations, neovascularization, and injury neuroma) that we attri
bute to microvasculitis (mural and perivascular mononuclear inflammation of
microvessels, inflammatory separation, fragmentation and destruction of mu
ral smooth muscle, and previous microscopic bleeding [hemosiderin]). Teased
nerve fibers in LSRPN showed significantly increased frequencies of axonal
degeneration, segmental demyelination, and empty nerve strands. The segmen
tal demyelination appeared to be clustered on fibers with axonal dystrophy.
The nerves with abnormal frequencies of demyelination were significantly a
ssociated with nerves showing multifocal fiber loss. We reached the followi
ng conclusions: 1) LSRPN is a serious condition with much morbidity that mi
rrors DLSRPN. 2) Ischemic injury from microvasculitis appears to be the cau
se of LSRPN. 3) Axonal degeneration and segmental demyelination appear to b
e linked and due to ischemia. 4) The pathologic alterations in LSRPN and DL
SRPN are indistinguishable, raising the question whether these 2 conditions
have a common underlying mechanism, and whether diabetes mellitus contribu
tes to the pathology or is a risk factor in DLSRPN. 5) Both LSRPN and DLSRP
N are potentially treatable conditions.