Evidence of small-fiber neuropathy in neurofibromatosis type 1
| Autor: | Jeanna M. McCuaig, Paul Kongkham, Alon Abraham, Catherine Maurice, Suganth Suppiah, Gelareh Zadeh, Vera Bril, Tayir Alon, Carolina Barnett, Raymond H. Kim |
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| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Adult Male medicine.medical_specialty Neurofibromatosis 1 Intravital Microscopy Physiology Corneal nerve Small Fiber Neuropathy Neural Conduction Nerve fiber 030105 genetics & heredity Cornea 03 medical and health sciences Cellular and Molecular Neuroscience Young Adult 0302 clinical medicine Physiology (medical) Ophthalmology medicine Laser-Doppler Flowmetry Humans Thermosensing Abnormal nerve conduction Neurofibromatosis Skin Microscopy Confocal business.industry Electrodiagnosis Mean age Laser Doppler velocimetry Middle Aged medicine.disease Vasodilation medicine.anatomical_structure Sensory Thresholds Female Neurology (clinical) Nerve conduction business 030217 neurology & neurosurgery |
| Zdroj: | MusclenerveREFERENCES. 60(6) |
| ISSN: | 1097-4598 |
| Popis: | INTRODUCTION Large-fiber neuropathy is rare in neurofibromatosis type 1, but small-fiber neuropathy has not been studied. METHODS Patients with neurofibromatosis type 1 underwent nerve conduction studies for large-fiber assessment. Small-fiber tests included quantitative thermal thresholds, laser Doppler flare imaging, intraepidermal nerve fiber density, and corneal nerve fiber length. RESULTS Of the 52 patients enrolled, 31 (60%) were female and the mean age was 33.0 ± 12.3 years. Four (8%) patients had abnormal nerve conduction studies. Small-fiber tests were frequently abnormal: thermal thresholds in 7 (13%); laser Doppler flare imaging in 10 (19%); intraepidermal nerve fiber density in 11 (22%); and corneal nerve fiber length in 27 (52%). The mean corneal nerve fiber length was below normative level (10.1 ± 2.7 mm/mm3 ). DISCUSSION Small-fiber neuropathy may be common in neurofibromatosis type 1, and should be investigated in symptomatic patients. |
| Databáze: | OpenAIRE |
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