Change in MUNIX in Patients With CMT1A Undergoing a Home Ankle Strengthening Program Versus Standard of Care
Charcot-Marie-Tooth Disease
About this trial
This is an interventional diagnostic trial for Charcot-Marie-Tooth Disease focused on measuring MUNIX
Eligibility Criteria
Inclusion Criteria:
- Adult Patients 18 years or older with genetically confirmed CMT1A
- Adult Patients 18 years or older with a CMT1A genetically confirmed relative and a positive clinical exam or nerve conduction study consistent with CMT1A.
- Unaffected persons 18 years or older with no past medical history of peripheral neuropathy
Exclusion Criteria:
- Patients with a history of medical diseases that affect peripheral nerve function including diabetic neuropathy, uncontrolled thyroid dysfunction, amyloidosis, monoclonal gammopathy of uncertain significance or untreated vitamin deficiencies.
- Patients with a history of other neurologic disease which may affect peripheral nerve function or extremity strength or function including stroke, seizures with a history of Todd's paralysis, Parkinson's Disease, Dementia, Guillen-Barre Syndrome, Myasthenia Gravis, Lambert Eaton Myasthenia Gravis or hypothyroidism.
- Patients with ankle dorsiflexion strength of less than 3/5 in either limb on Medical Research Council scale.
- Patients enrolled in a clinical trial (excluding natural history studies) in the past 12 months.
- Patients who have undergone intense physical therapy, meaning more than 1 time per week for 6 or more weeks within the last 12 months.
- Patient who do resistance training of the lower extremity more than 2 times per week for more than 3 months.
- Patient in whom exercise would be consider dangerous including autonomic failure, postural orthostatic tachycardic syndrome (POTS), lower extremity deep vein thrombosis or pulmonary embolism, arterial insufficiency, uncontrolled pulmonary hypertension, uncontrolled hypertension or uncontrolled heart failure with reduced ejection fraction.
Sites / Locations
- Vanderbilt University Medical Center
Arms of the Study
Arm 1
Arm 2
Experimental
Experimental
Home Lower Extremity Strengthening
No intervention
Patients in this arm will undergo a 12 week strengthening program which focuses on ankle dorsi- and plantar- flexion. Clinical visits will occur at baseline, 6 weeks and 12 weeks from the start of study. At each visit all patients will undergo a clinical exam, answer questionaires and undergo MUNIX testing.
In this portion of the study patients will not be given any intervention. Clinical visits will occur at baseline, 6 weeks and 12 weeks from the start of study. At each visit all patients will undergo a clinical exam, answer questionaires and undergo MUNIX testing of both legs.