TY - JOUR
T1 - Hereditary sensory and autonomic neuropathy types 4 and 5
T2 - Review and proposal of a new rehabilitation method
AU - Yozu, Arito
AU - Haga, Nobuhiko
AU - Funato, Tetsuro
AU - Owaki, Dai
AU - Chiba, Ryosuke
AU - Ota, Jun
N1 - Funding Information:
This research was supported in part by the Japan Society for the Promotion of Science KAKENHI (# 25870170, 15K01361, and 26120008). Wiley-JAPAN permitted us the reuse of their figures and tables.
Publisher Copyright:
© 2015 Elsevier Ireland Ltd and the Japan Neuroscience Society.
PY - 2016/3/1
Y1 - 2016/3/1
N2 - Although pain is unpleasant, it should serve as a reminder for individuals to avoid similar damaging incidents in the future. Hereditary sensory and autonomic neuropathy (HSAN) includes genetic disorders involving various sensory and autonomic dysfunctions. They are classified by the mode of inheritance, clinical features, and related genes. HSAN type 4 (HSAN-4) and type 5 (HSAN-5) are characterized by insensitivity to pain and thermal sensation. Further, HSAN-4 is accompanied by decreased sweating and intellectual disabilities. These characteristics of HSAN-4 and -5 result in many clinical features, such as pediatric, psychiatric, orthopedic, oral, dermatological, and ophthalmological problems. Orthopedic problems include destructive injuries such as multiple fractures and joint dislocation. Studies on gait have shown greater speed and higher heel contact angular velocity in HSAN-4 and -5 patients compared with controls. Studies on grasp-lift-holding tasks have shown higher grasp force and fluctuations in acceleration of the object. We believe that these findings represent outcomes of deficient motor learning. We propose a new rehabilitation method for patients with HSAN-4 and -5, with the aim of decreasing their destructive injuries.
AB - Although pain is unpleasant, it should serve as a reminder for individuals to avoid similar damaging incidents in the future. Hereditary sensory and autonomic neuropathy (HSAN) includes genetic disorders involving various sensory and autonomic dysfunctions. They are classified by the mode of inheritance, clinical features, and related genes. HSAN type 4 (HSAN-4) and type 5 (HSAN-5) are characterized by insensitivity to pain and thermal sensation. Further, HSAN-4 is accompanied by decreased sweating and intellectual disabilities. These characteristics of HSAN-4 and -5 result in many clinical features, such as pediatric, psychiatric, orthopedic, oral, dermatological, and ophthalmological problems. Orthopedic problems include destructive injuries such as multiple fractures and joint dislocation. Studies on gait have shown greater speed and higher heel contact angular velocity in HSAN-4 and -5 patients compared with controls. Studies on grasp-lift-holding tasks have shown higher grasp force and fluctuations in acceleration of the object. We believe that these findings represent outcomes of deficient motor learning. We propose a new rehabilitation method for patients with HSAN-4 and -5, with the aim of decreasing their destructive injuries.
KW - Congenital insensitivity to pain
KW - Hereditary sensory and autonomic neuropathy (HSAN)
KW - Motor learning
KW - Multiple fractures
KW - Nerve growth factor (NGF)
KW - Rehabilitation
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U2 - 10.1016/j.neures.2015.10.011
DO - 10.1016/j.neures.2015.10.011
M3 - Review article
C2 - 26562335
AN - SCOPUS:84962855682
SN - 0168-0102
VL - 104
SP - 105
EP - 111
JO - Neuroscience Research
JF - Neuroscience Research
ER -