Abstract Details

Title Natural History of Hereditary Spastic Paraplegias on Functional Gait Parameters and Electromyography During 18 Months Follow-up

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) S46 - Neuromuscular and Clinical Neurophysiology (EMG): Motor Neuron/Charcot Marie Tooth (4:18 PM-4:30 PM)

Poster/Presentation
Number 005

Background HSP is a heterogeneous group of genetic diseases characterized by spastic hypertonia and muscle weakness in the lower limbs resulting from the degeneration of the distal portions of corticospinal tract.

Objective

To investigate the natural history of hereditary spastic paraplegias (HSP) on functional gait parameters and electromyography variables during 18 months of follow-up.

Design/Methods The MOVESPAST study is constituted of two phases, the first one being a cross-sectional study and the second one a cohort study. We have evaluated 32 HSP individuals, 25 of them were able to perform functional instruments (17 being SPG4), Eighteen months follow-up of 13 patients (42 ± 4.94 years old; 68 ± 23 kg; 161 ± 17 cm) on functional gait tests and surface electromyography (EMG) during gait on treadmill were analyzed. The 10-meter walk test (10MWT) and time up and go (TUG) on self-selected walking speed (SSWS) and maximal walking speed (MWS), and the six-minute walk test (6MWT) were s applied.

Results All outcomes of functional gait worsen after 18 months of baseline. However, differences were only statistically significant for 10MWTssws, TUGssws and TUGmws (p- value= 0.001). The lowest electromyography activity occurred in stages 0 and 1 of the disease in self-selected walking speed of tibialis anterior, gastrocnemius medialis, Vastus Medialis, Biceps femoralis longus. EMG on the other disease stages showed different behavior patterns for each muscle.

Conclusions Functional instruments were sensitive to the progression of HSPs after 18 months, with greater differences for 10MWTssws, TUGssws and TUGmws. Complete results of 18 months natural history of HSP on functional instruments and EMG and the minimal clinically important differences of studied variables by anchored and distributive methods will be presented.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file

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