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Axon reflex-mediated vasodilation is reduced in proportion to disease severity in TTR-FAP.
Calero-Romero, Irène; Suter, Marc R; Waeber, Bernard; Feihl, Francois; Kuntzer, Thierry.
Afiliación
  • Calero-Romero I; Division of Clinical Pathophysiology (I.C.-R., B.W., F.F.), Department of Anaesthesiology, Pain Centre (M.R.S.), and Nerve-Muscle Unit, Neurology Service, Department of Clinical Neurosciences (T.K.), Lausanne University Hospital (CHUV), University of Lausanne, Switzerland.
  • Suter MR; Division of Clinical Pathophysiology (I.C.-R., B.W., F.F.), Department of Anaesthesiology, Pain Centre (M.R.S.), and Nerve-Muscle Unit, Neurology Service, Department of Clinical Neurosciences (T.K.), Lausanne University Hospital (CHUV), University of Lausanne, Switzerland.
  • Waeber B; Division of Clinical Pathophysiology (I.C.-R., B.W., F.F.), Department of Anaesthesiology, Pain Centre (M.R.S.), and Nerve-Muscle Unit, Neurology Service, Department of Clinical Neurosciences (T.K.), Lausanne University Hospital (CHUV), University of Lausanne, Switzerland.
  • Feihl F; Division of Clinical Pathophysiology (I.C.-R., B.W., F.F.), Department of Anaesthesiology, Pain Centre (M.R.S.), and Nerve-Muscle Unit, Neurology Service, Department of Clinical Neurosciences (T.K.), Lausanne University Hospital (CHUV), University of Lausanne, Switzerland.
  • Kuntzer T; Division of Clinical Pathophysiology (I.C.-R., B.W., F.F.), Department of Anaesthesiology, Pain Centre (M.R.S.), and Nerve-Muscle Unit, Neurology Service, Department of Clinical Neurosciences (T.K.), Lausanne University Hospital (CHUV), University of Lausanne, Switzerland.
Neurol Genet ; 4(4): e251, 2018 Aug.
Article en En | MEDLINE | ID: mdl-30109266
OBJECTIVE: To evaluate the area of the vascular flare in familial amyloid polyneuropathy (FAP). METHODS: Healthy controls and patients with genetically confirmed FAP were prospectively examined, on the upper and lower limbs, for thermal sensitivity (Medoc TSA-II thermal analyzer) and for axon reflex-mediated flare. The latter was induced by iontophoresis of histamine on the forearm and leg on 2 different visits. We used laser Doppler imaging (LDI) to measure the flare area (LDIflare). RESULTS: Six patients had FAP of variable severity; 1 had generalized analgesia secondary to leprosy (used as a positive control). The median Neurological Impairment Score-Lower Limbs (NIS-LLs) was 6 (0-27). The warmth detection thresholds in the feet were higher in patients (median 43°, interquartile range 39.0°-47.6°) compared with controls (37.4°, 35.3°-39.2°), indicating small fiber impairment. On the leg, LDIflare was smaller in the patients on 2 consecutive visits (controls: median 13.0 and 13.3 cm2, IQR 9.7-22.8 and 8.3-16.9; patients 6.9 and 8.0 cm2, 2.6-10.8 and 6.4-12.1; p = 0.011). LDIflare on the leg was correlated with NIS-LL (Spearman rank correlation 0.73, p = 0.09 on the first visit; Spearman rank correlation 0.85, p = 0.03 on the second visit). CONCLUSIONS: Our study underscores that histamine-induced axon reflex-mediated vascular flare on the leg is reduced in proportion to disease severity in patients with FAP.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Neurol Genet Año: 2018 Tipo del documento: Article País de afiliación: Suiza Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Neurol Genet Año: 2018 Tipo del documento: Article País de afiliación: Suiza Pais de publicación: Estados Unidos