38 Chapter 3 (hip 70º flexed); knee flexion and extension; ankle plantar and dorsiflexion with knee extended (gastrocnemius) and knee flexed (soleus).22 Vibration sense is evaluated using a tuning fork on bilateral patella, lateral malleolus, and at the first metatarsophalangeal joint. Trunk control is assessed using the Trunk Control Measurement Scale (TCMS).23 Lastly, coordination is examined via (i) heel-to-knee test, (ii) toe tapping and (iii) leg agility.24 Primary outcome The primary outcome is gait adaptability as measured with the obstacle subtask of the Emory Functional Ambulation Profile (E-FAP). Participants are asked to negotiate a 10-meter course in which two wooden blocks (100(l) x 10(w) x 5(h) cm) and a bin are placed along the walkway. The instruction given is to complete the task as fast as possible but keep your own safety in mind. The number of seconds needed to complete the task is registered. A faster time score indicates better gait adaptability. The obstacle subtask of the E-FAP has previously been used as an outcome measure for gait adaptability in several neurological populations.14, 15 The full protocol is available online and via Wolf et. al.25 Secondary outcomes Secondary outcome measures comprise of the following clinical tests: 10-Meter Walk Test (10MWT) The 10MWT is a standardized and recommended measurement of walking velocity. Participants walk thirteen meters in a straight line; first three times at comfortable speed and then three times as fast as possible. Participants have three meters to accelerate to the requested speed. When the first foot crosses the 3-meter line, the timer starts. The timer stops when the first foot crosses the 13-meter line. Like this, the number of seconds it takes to walk ten meters is recorded.26 The test has been found reliable, valid and sensitive in neurological populations,26 and has been used in people with HSP.9, 13, 27, 28 Mini Balance Evaluation Systems Test (miniBEST) The mini-BEST test is a 14-item, 3 points ordinal rating scale (0-2 points) to evaluate balance capacity in 4 subcategories: anticipatory postural control, reactive postural control, sensory orientation, and gait stability. The attainable sum scores range from 0-28 points, a higher score indicating better balance capacity. Participants perform the test barefoot. The full protocol is available online and has been described by Franchignoni et.al.29 The mini-BEST is often used in neurological populations, has been found valid, reliable and responsive,30-32 and has been recommended for use in people with HSP.4
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