Abstract Details

Title Gait Patterns in Pediatric Charcot-Marie-Tooth Disease (CMT)

Topic Peripheral Nerve

Presentation(s) IN1 - (-)

Poster/Presentation
Number 007

Objective

Background Inherited peripheral neuropathy, or Charcot-Marie-Tooth disease (CMT), is among the most frequent pediatric neuromuscular diseases with variable clinical presentation and progression. Ambulation is a significant quality of life indicator in CMT.

Design/Methods In this retrospective study of 33 patients (16 males and 17 females) included with a diagnosis of CMT. Eleven of the 33 had the most common type 1A, six had type 2A, one had type 2B and the rest were undetermined. All of the patients underwent a comprehensive motion analysis including: passive range of motion, muscle strength, gait kinematics, surface electromyography and foot pressures using a VICON 512 motion measurement system (Lake Forest, CA).

Results Results showed three distinct ankle kinematic patterns consistent with combinations of ankle plantar flexor and dorsiflexor weakness and plantar flexor contracture. The three groups included: a) decreased dorsiflexion, b) normal peak dorsiflexion and c) excessive peak dorsiflexion. All patients showed delayed peak dorsiflexion in stance. There were no correlations found between the age of patients, onset of gait problems and the genetic type of CMT and the gait patterns.

Conclusions A better understanding of gait in pediatric CMT is the first step towards understanding the clinical progression in ambulation. Furthermore, it helps defining the treatment options. The forms of gait abnormalities in our cohort appear to be related to the progression of foot/ankle deformity. A prospective natural history of gait pathology and a correlation with other functional measures (e.g. CMTPeds) would be desirable.

Authors/Disclosures
Gyula Acsadi, MD, FAAN (Connecticut Children'S Medical Center/UConn) PRESENTER	Dr. Acsadi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Acsadi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Avexis. Dr. Acsadi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Serepta. Dr. Acsadi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Acsadi has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen.
	No disclosure on file
Seemant Chaturvedi, MD, FAHA, FAAN (University of Maryland)	Dr. Chaturvedi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astra Zeneca. Dr. Chaturvedi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for University of Calgary. Dr. Chaturvedi has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Heart Association. Dr. Chaturvedi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Ramar & Paradiso. Dr. Chaturvedi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Cole, Scott, Kissane. The institution of Dr. Chaturvedi has received research support from NINDS.

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