Proprioception and Sensorimotor Control in Hereditary Sensory and Autonomic Neuropathy

Study Overview
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Brief Summary
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This project comprises three sets of physiological studies - testing eight specific hypotheses - that will contribute new knowledge on proprioception and motor control in a genetic disorder that affects specific components of the sensory nervous system. I: To investigate the neurophysiological basis for disturbed motor control in Hereditary sensory and autonomic neuropathy (HSAN) III II: To investigate the effects of enhancing cutaneous feedback on motor control in HSAN III III: To investigate the cortical representation of proprioceptive inputs in HSAN III

Detailed Description
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HSAN III patients (n=15) and healthy control subjects (n=15) will lay supine on an MRI bed and a tungsten microelectrode inserted percutaneously into a muscle or cutaneous fascicle of the right common peroneal nerve at the fibular head, according to standard techniques employed by Prof Macefield. Neural activity will be acquired, RMS-processed (200 ms) and analysed on computer. The subject's head will be tightly enclosed in a standard clinical 32-channel SENSE head coil and headphones will be provided to minimize noise and to allow communication with the subject. The subject will be placed in the bore of a 3T whole-body scanner for 60-90 minutes.

Official Title
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Proprioception and Sensorimotor Control in Hereditary Sensory and Autonomic Neuropathy

Conditions
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Atunomic Neuropathy

Intervention / Treatment
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* Other: kinesiology tape around the hip, knee and ankle joints
* Other: No kinesiology tape around the hip, knee and ankle joints


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Confirmed genetic diagnosis of HSAN III will be recruited from the Dysautonomia Center at NYU Exclusion Criteria: homeless active drug or alcohol dependence evidence of neurological disorder or diabetes exposure to neurotoxic drugs that in the investigator's opinion may compromise results Pregnant women

Ages Eligible for Study
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Minimum Age: 16 Years
Maximum Age: 40 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Basic Science
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: HSAN III<br> | Other: kinesiology tape around the hip, knee and ankle joints<br>* We will also demonstrate that taping the skin increases the central representation of cutaneous afferent input in HSAN III.<br>Other: No kinesiology tape around the hip, knee and ankle joints<br>* Investigators will demonstrate that taping the skin increases the central representation of cutaneous afferent input in HSAN III.<br>|
| Active Comparator: Control Subjects<br> | Other: kinesiology tape around the hip, knee and ankle joints<br>* We will also demonstrate that taping the skin increases the central representation of cutaneous afferent input in HSAN III.<br>Other: No kinesiology tape around the hip, knee and ankle joints<br>* Investigators will demonstrate that taping the skin increases the central representation of cutaneous afferent input in HSAN III.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement of gait in HSAN III |  | 120 Minutes | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Hereditary Sensory Neuropathy, Autonomic Neuropathy, Familial Dysautonomia, proprioception, motor control, sensory nervous system., HSAN III, IKBKAP gene mutation