INTRODUCTION: Charcot-Marie-Tooth (CMT) disease type 1A is the most common form of CMT. The main clinical features are distal weakness, sensory loss, and skeletal deformities. Although pain is a frequent complaint, small fiber involvement in CMT1A has not been studied extensively. METHODS: We assessed pain and small fiber involvement in 49 CMT1A patients using a variety of pain scales, pain questionnaires, and thermal thresholds. RESULTS: Forty-three of 49 patients (88%) complained of pain. The pain was localized to the feet in 61% of patients. Only 18% of patients had neuropathic pain. Cold and warm detection thresholds were elevated in 53% and 12% of patients, respectively. CONCLUSIONS: Our findings confirm that CMT1A patients have significant pain, which is more likely to be multifactorial in origin and suggests that a proportion of patients have small fiber dysfunction affecting mainly thinly myelinated Aδ fibers.
INTRODUCTION: Charcot-Marie-Tooth (CMT) disease type 1A is the most common form of CMT. The main clinical features are distal weakness, sensory loss, and skeletal deformities. Although pain is a frequent complaint, small fiber involvement in CMT1A has not been studied extensively. METHODS: We assessed pain and small fiber involvement in 49 CMT1A patients using a variety of pain scales, pain questionnaires, and thermal thresholds. RESULTS: Forty-three of 49 patients (88%) complained of pain. The pain was localized to the feet in 61% of patients. Only 18% of patients had neuropathic pain. Cold and warm detection thresholds were elevated in 53% and 12% of patients, respectively. CONCLUSIONS: Our findings confirm that CMT1A patients have significant pain, which is more likely to be multifactorial in origin and suggests that a proportion of patients have small fiber dysfunction affecting mainly thinly myelinated Aδ fibers.
Authors: Maria Nolano; Fiore Manganelli; Vincenzo Provitera; Chiara Pisciotta; Annamaria Stancanelli; Giuseppe Caporaso; Rosa Iodice; Michael E Shy; Lucio Santoro Journal: Neurology Date: 2014-12-24 Impact factor: 9.910
Authors: Anthony Tucker-Bartley; Jordan Lemme; Andrea Gomez-Morad; Nehal Shah; Miranda Veliu; Frank Birklein; Claudia Storz; Seward Rutkove; David Kronn; Alison M Boyce; Eduard Kraft; Jaymin Upadhyay Journal: Neurosci Biobehav Rev Date: 2021-02-10 Impact factor: 9.052