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Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy

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Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy. / Godel, Tim; Bäumer, Philipp; Pham, Mirko; Köhn, Anja; Muschol, Nicole; Kronlage, Moritz; Kollmer, Jennifer; Heiland, Sabine; Bendszus, Martin; Mautner, Victor-Felix.

in: NEUROLOGY, Jahrgang 89, Nr. 12, 19.09.2017, S. 1274-1282.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Godel, T, Bäumer, P, Pham, M, Köhn, A, Muschol, N, Kronlage, M, Kollmer, J, Heiland, S, Bendszus, M & Mautner, V-F 2017, 'Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy', NEUROLOGY, Jg. 89, Nr. 12, S. 1274-1282. https://doi.org/10.1212/WNL.0000000000004396

APA

Godel, T., Bäumer, P., Pham, M., Köhn, A., Muschol, N., Kronlage, M., Kollmer, J., Heiland, S., Bendszus, M., & Mautner, V-F. (2017). Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy. NEUROLOGY, 89(12), 1274-1282. https://doi.org/10.1212/WNL.0000000000004396

Vancouver

Godel T, Bäumer P, Pham M, Köhn A, Muschol N, Kronlage M et al. Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy. NEUROLOGY. 2017 Sep 19;89(12):1274-1282. https://doi.org/10.1212/WNL.0000000000004396

Bibtex

@article{15307747af0344d09571f1cd19aa0327,
title = "Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy",
abstract = "OBJECTIVE: To evaluate functional and morphometric magnetic resonance neurography of the dorsal root ganglion and peripheral nerve segments in patients with Fabry painful neuropathy.METHODS: In this prospective study, the lumbosacral dorsal root ganglia and proximal peripheral nerve segments of the lower extremity were examined in 11 male patients with Fabry disease by a standardized 3T magnetic resonance neurography protocol. Volumes of L3 to S2 dorsal root ganglia, perfusion parameters of L5-S1 dorsal root ganglia and the spinal nerve L5, and the cross-sectional area of the proximal sciatic nerve were compared to healthy controls.RESULTS: Dorsal root ganglia of patients with Fabry disease were symmetrically enlarged by 78% (L3), 94% (L4), 122% (L5), 115% (S1), and 119% (S2) (p< 0.001). In addition, permeability of the blood-tissue interface was decreased by 53% (p< 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglion containing the cell bodies of the primary sensory neurons (p< 0.001). Spinal nerve permeability showed no difference between patients with Fabry disease and controls (p= 0.7). The sciatic nerve of patients with Fabry disease at the thigh level showed an increase in cross-sectional area by 48% (p< 0.001).CONCLUSIONS: Patients with Fabry disease have severely enlarged dorsal root ganglia with dysfunctional perfusion. This may be due to glycolipid accumulation in the dorsal root ganglia mediating direct neurotoxic effects and decreased neuronal blood supply. These alterations were less pronounced in peripheral nerve segments. Thus, the dorsal root ganglion might play a key pathophysiologic role in the development of neuropathy and pain in Fabry disease.",
keywords = "Adult, Fabry Disease, Ganglia, Spinal, Humans, Lumbar Vertebrae, Magnetic Resonance Imaging, Male, Middle Aged, Pain, Peripheral Nervous System Diseases, Prospective Studies, Sacrum, Sciatic Nerve, Young Adult, Journal Article",
author = "Tim Godel and Philipp B{\"a}umer and Mirko Pham and Anja K{\"o}hn and Nicole Muschol and Moritz Kronlage and Jennifer Kollmer and Sabine Heiland and Martin Bendszus and Victor-Felix Mautner",
note = "{\textcopyright} 2017 American Academy of Neurology.",
year = "2017",
month = sep,
day = "19",
doi = "10.1212/WNL.0000000000004396",
language = "English",
volume = "89",
pages = "1274--1282",
journal = "NEUROLOGY",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "12",

}

RIS

TY - JOUR

T1 - Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy

AU - Godel, Tim

AU - Bäumer, Philipp

AU - Pham, Mirko

AU - Köhn, Anja

AU - Muschol, Nicole

AU - Kronlage, Moritz

AU - Kollmer, Jennifer

AU - Heiland, Sabine

AU - Bendszus, Martin

AU - Mautner, Victor-Felix

N1 - © 2017 American Academy of Neurology.

PY - 2017/9/19

Y1 - 2017/9/19

N2 - OBJECTIVE: To evaluate functional and morphometric magnetic resonance neurography of the dorsal root ganglion and peripheral nerve segments in patients with Fabry painful neuropathy.METHODS: In this prospective study, the lumbosacral dorsal root ganglia and proximal peripheral nerve segments of the lower extremity were examined in 11 male patients with Fabry disease by a standardized 3T magnetic resonance neurography protocol. Volumes of L3 to S2 dorsal root ganglia, perfusion parameters of L5-S1 dorsal root ganglia and the spinal nerve L5, and the cross-sectional area of the proximal sciatic nerve were compared to healthy controls.RESULTS: Dorsal root ganglia of patients with Fabry disease were symmetrically enlarged by 78% (L3), 94% (L4), 122% (L5), 115% (S1), and 119% (S2) (p< 0.001). In addition, permeability of the blood-tissue interface was decreased by 53% (p< 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglion containing the cell bodies of the primary sensory neurons (p< 0.001). Spinal nerve permeability showed no difference between patients with Fabry disease and controls (p= 0.7). The sciatic nerve of patients with Fabry disease at the thigh level showed an increase in cross-sectional area by 48% (p< 0.001).CONCLUSIONS: Patients with Fabry disease have severely enlarged dorsal root ganglia with dysfunctional perfusion. This may be due to glycolipid accumulation in the dorsal root ganglia mediating direct neurotoxic effects and decreased neuronal blood supply. These alterations were less pronounced in peripheral nerve segments. Thus, the dorsal root ganglion might play a key pathophysiologic role in the development of neuropathy and pain in Fabry disease.

AB - OBJECTIVE: To evaluate functional and morphometric magnetic resonance neurography of the dorsal root ganglion and peripheral nerve segments in patients with Fabry painful neuropathy.METHODS: In this prospective study, the lumbosacral dorsal root ganglia and proximal peripheral nerve segments of the lower extremity were examined in 11 male patients with Fabry disease by a standardized 3T magnetic resonance neurography protocol. Volumes of L3 to S2 dorsal root ganglia, perfusion parameters of L5-S1 dorsal root ganglia and the spinal nerve L5, and the cross-sectional area of the proximal sciatic nerve were compared to healthy controls.RESULTS: Dorsal root ganglia of patients with Fabry disease were symmetrically enlarged by 78% (L3), 94% (L4), 122% (L5), 115% (S1), and 119% (S2) (p< 0.001). In addition, permeability of the blood-tissue interface was decreased by 53% (p< 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglion containing the cell bodies of the primary sensory neurons (p< 0.001). Spinal nerve permeability showed no difference between patients with Fabry disease and controls (p= 0.7). The sciatic nerve of patients with Fabry disease at the thigh level showed an increase in cross-sectional area by 48% (p< 0.001).CONCLUSIONS: Patients with Fabry disease have severely enlarged dorsal root ganglia with dysfunctional perfusion. This may be due to glycolipid accumulation in the dorsal root ganglia mediating direct neurotoxic effects and decreased neuronal blood supply. These alterations were less pronounced in peripheral nerve segments. Thus, the dorsal root ganglion might play a key pathophysiologic role in the development of neuropathy and pain in Fabry disease.

KW - Adult

KW - Fabry Disease

KW - Ganglia, Spinal

KW - Humans

KW - Lumbar Vertebrae

KW - Magnetic Resonance Imaging

KW - Male

KW - Middle Aged

KW - Pain

KW - Peripheral Nervous System Diseases

KW - Prospective Studies

KW - Sacrum

KW - Sciatic Nerve

KW - Young Adult

KW - Journal Article

U2 - 10.1212/WNL.0000000000004396

DO - 10.1212/WNL.0000000000004396

M3 - SCORING: Journal article

C2 - 28835396

VL - 89

SP - 1274

EP - 1282

JO - NEUROLOGY

JF - NEUROLOGY

SN - 0028-3878

IS - 12

ER -