Marcia Greenberg

Selective Control Assessment of the Lower Extremity (SCALE): development, validation, and interrater reliability of a clinical tool for patients with cerebral palsy

Normal selective voluntary motor control (SVMC) can be defined as the ability to perform isolated joint movement without using mass flexor/extensor patterns or undesired movement at other joints, such as mirroring. SVMC is an important determinant of function, yet a valid, reliable assessment tool is lacking. The Selective Control Assessment of the Lower Extremity (SCALE) is a clinical tool developed to quantify SVMC in patients with cerebral palsy (CP). This paper describes the development, utility, validation, and interrater reliability of SCALE. Content validity was based on review by 14 experienced clinicians. Mean agreement was 91.9% (range 71.4–100%) for statements about content, administration, and grading. SCALE scores were compared with Gross Motor Function Classification System Expanded and Revised (GMFCS‐ER) levels for 51 participants with spastic diplegic, hemiplegic, and quadriplegic CP (GMFCS levels I – IV, 21 males, 30 females; mean age 11y 11mo [SD 4y 9mo]; range 5–23y). Construct validity was supported by significant inverse correlation (Spearmans r=‐0.83, p<0.001) between SCALE scores and GMFCS levels. Six clinicians rated 20 participants with spastic CP (seven males, 13 females, mean age 12y 3mo [SD 5y 5mo], range 7–23y) using SCALE. A high level of interrater reliability was demonstrated by intraclass correlation coefficients ranging from 0.88 to 0.91 (p<0.001).

Lower-extremity selective voluntary motor control in patients with spastic cerebral palsy: increased distal motor impairment

Aim  Multiple impairments contribute to motor deficits in spastic cerebral palsy (CP). Selective voluntary motor control (SVMC), namely isolation of joint movement upon request, is important, but frequently overlooked. This study evaluated the proximal to distal distribution of SVMC impairment among lower extremity joints.

Pilot evaluation of wearable tactile biofeedback system for gait rehabilitation in peripheral neuropathy

Peripheral neuropathy (PN) is a significant public health concern, giving rise to abnormal gait biomechanics, diminished postural stability, and increased risk of falls. A wearable tactile feedback system previously developed for sensory augmentation of prosthetic limbs has been adapted for individuals with PN and evaluated in a pilot group of four subjects with idiopathic bilateral PN. Subjects were assessed both for their abilities to perceive and distinguish tactile stimuli, and for the effect of tactile biofeedback on gait, using optical motion capture and embedded force plate technology. Preliminary data indicate that participants could adequately perceive and localize tactile stimuli, as well as make meaningful modifications to their gait in real time, with minimal training. Observed gait modifications with feedback active included increases in walking speed, step cadence, step length, and peak joint powers. However, the variability of biofeedbacks effect on gait from subject to subject demands further investigation with the peripheral neuropathy patient population.

P.3.1 GNE myopathy functional activity scale (GNEM-FAS): Development of a disease-specific instrument for measuring function and independence

GNE myopathy or hereditary inclusion body myopathy (HIBM) is an autosomal recessive myopathy presenting with distal leg weakness in early adulthood. Progressive weakness results in greater dependence and disability over time. A disease-specific measurement of functional activity is needed to better understand the burden of illness, inform the design of clinical studies and optimize care. After clinical interview of patients, a 25-item questionnaire was developed to assess ability and independence in three domains: mobility, upper extremity (UE) use and self-care. Each item was rated from 0 to 4 with higher scores representing better function. Total scores range from 0 to 100; subscale scores range from 0 to 40 for Mobility, 0–32 for UE and 0–28 for Self-Care. The GNE Myopathy Functional Activity Scale (GNEM-FAS) was administered to 47 ambulatory subjects enrolled in a Phase 2 study of extended release sialic acid. Physical therapists completed the GNEM-FAS based on clinical observation and subject interview. Scores were compared to performance on volitional measures of strength and function, as well as scores on the Inclusion Body Myositis Functional Rating Scale (IBMFRS), a validated instrument for myositis. The mean GNEM-FAS total score was 69 out of 100 (23–94). Mobility subscores averaged 50%, UE, 81% and Self-Care, 82% of the maximum possible. Higher Mobility scores were associated with greater lower extremity strength ( r =0.83) and longer 6MWT distances ( r =0.83). A moderate association was seen between the UE domain scores and UE strength ( r =0.66). Self-care domain scores and the stair climb time were negatively related ( r =−0.68). There was a strong correlation between GNEM-FAS total scores and IBMFRS scores ( r =0.94). Mobility was limited more than UE or self-care function in this cohort of ambulatory subjects with GNE myopathy. Repeat administration in treated and untreated patients with varying degrees of severity is underway to further validate the instrument.

Reproductive healthcare experiences of women with cerebral palsy

BACKGROUND Little is known about pregnancy rates in women with disabilities in general and even less is known about women with child-onset disabilities such as cerebral palsy (CP). HYPOTHESIS We hypothesized that discussions about pregnancy with healthcare providers and pregnancy rates for woman with CP would be related to their functional levels. METHODS Survey methodology was used to gather information about demographics, function, whether women were asked about their desire for children, pregnancy outcomes, and services offered during pregnancy and postpartum. RESULTS Of the 375 women with CP who participated in the survey, 76 (20%) reported 149 pregnancies resulting in 100 live births. Using Chi square statistics, mobility, manual dexterity, and communication function were significantly higher in women who were queried about or who experienced pregnancy. More than half of the women experienced a loss of mobility during pregnancy but few received referrals for physical or occupational therapy. Few reported screening for postpartum depression. A higher rate of Cesarean sections (50.4%), preterm births (12.1%), low birth weight infants (15.7%), and very low birth weight infants (7.1%) was reported by women with CP compared to national statistics. CONCLUSIONS Pregnancy rates and discussions were related to functional levels. As 20% of women with CP surveyed experienced pregnancy, there is a need to increase awareness, education, support, and advocacy for achievement of optimal reproductive health. More research is needed to identify factors contributing to maternal and infant health in women with CP.

Characterization of Strength and Function in Ambulatory Adults With Gne Myopathy

Objective: To characterize the pattern and extent of muscle weakness and impact on physical functioning in adults with GNEM. Methods: Strength and function were assessed in GNEM subjects (n = 47) using hand-held dynamometry, manual muscle testing, upper and lower extremity functional capacity tests, and the GNEM-Functional Activity Scale (GNEM-FAS). Results: Profound upper and lower muscle weakness was measured using hand-held dynamometry in a characteristic pattern, previously described. Functional tests and clinician-reported outcomes demonstrated the consequence of muscle weakness on physical functioning. Conclusions: The characteristic pattern of upper and lower muscle weakness associated with GNEM and the resulting functional limitations can be reliably measured using these clinical outcome assessments of muscle strength and function.