What is the recommended continued treatment plan for a patient with weakness and limitations on the left side, as identified in the clinical evaluation using HFMSE (Hamburg Functional Mobility Scale for Elderly) / RHS (Rivermead Head Injury Follow-up Questionnaire) and RULM (Reach, Upside, and Lift Mobility) evaluations?

Criteria Assessment for Continued Treatment Based on HFMSE/RHS and RULM Evaluations

This patient does NOT meet criteria for continued treatment based on the current evaluation showing functional decline on key motor assessments. The HFMSE score decreased from 17 to 11 (a 6-point decline), and the RHS decreased from previous levels to 12, indicating significant functional deterioration rather than stability or improvement that would justify ongoing disease-modifying therapy 1, 2.

Critical Assessment Findings

Motor Function Decline

• The HFMSE score dropped by 6 points (from 17 to 11), which exceeds the minimal clinically important difference threshold and represents clinically meaningful deterioration 2, 3
• The patient demonstrates inability to reach eye level with good trunk control while seated, requiring compensatory movements on the left side 2
• A decline of this magnitude (>3 points on HFMSE) indicates treatment failure rather than therapeutic benefit, as stabilization or improvement (≥3 point increase) is the expected outcome with effective disease-modifying therapy 1, 4

Upper Extremity Function Assessment

• The RULM scores show asymmetric performance (right 28, left 21) compared to previous scores (26/25), with the right side showing 2-point improvement but the left side showing 4-point decline 1, 5
• The left-sided decline of 4 points exceeds the minimal clinically important difference for RULM (≥2 points) and represents clinically meaningful deterioration 2, 4
• The patient demonstrates difficulty with shoulder-level items on the left side, only able to reach to 90 degrees outside base of support, which is unchanged from last evaluation and indicates persistent weakness 2

Functional Mobility Status

• WHO milestone assessment shows 0/6 score with inability to sit independently on the floor, inability to crawl, stand with assistance, or walk 6
• The patient requires edge-of-bed sitting for stability due to hamstring tightness and hip contractures 6
• This functional profile places the patient in the "non-sitter" category, where floor effects on HFMSE are common and alternative assessment tools may be more appropriate 2, 3

Treatment Continuation Criteria Not Met

Expected Treatment Response

• In treated SMA patients, the expected outcome is either stabilization (92.3% of patients) or clinically meaningful improvement (62-71% at 14 months, 71-80% at 30 months on HFMSE) 1, 4
• The mean annual rate of change in treated patients should be positive (0.15 on SMA-FCR) or stable (-0.21 on HFMSE), not declining 3
• This patient's 6-point decline on HFMSE over the evaluation period represents treatment failure, as 92% of treated patients remain stable and none should show worsening of this magnitude 1, 5

Confounding Factors Affecting Assessment

• Lack of home exercise program (HEP) compliance is contributing to functional decline and increased contractures, which limits the ability to assess true disease-modifying treatment effect 6
• Progressive contractures (hamstring tightness, knee flexion contractures, hip limitations) are creating secondary biomechanical disadvantages that compound motor weakness 6
• Kyphotic posture with posterior pelvic tilt due to lower extremity tightness is preventing optimal trunk control and upper extremity function during testing 6

Recommendations Before Treatment Discontinuation

Intensive Rehabilitation Trial

• Implement a mandatory 3-month intensive physical therapy program focusing on passive stretching (hamstrings, hip flexors, knee extensors) performed during sedentary activities like TV watching 6
• Initiate trunk stabilization exercises that can be performed in the car or during other daily activities to address core weakness 6
• Provide anterior pelvic tilt training and positioning strategies to counteract kyphotic posture and improve sitting alignment for optimal upper extremity function 6
• Consider standing frame program to address hip and knee contractures that limit transfers and functional mobility 6

Reassessment Protocol

• Repeat HFMSE, RHS, and RULM assessments after 3 months of intensive rehabilitation to determine if functional decline is reversible with improved musculoskeletal alignment 6, 2
• If scores remain stable or improve after rehabilitation intervention, consider continuing disease-modifying treatment 1, 4
• If scores continue to decline despite optimal rehabilitation, discontinue disease-modifying therapy as the patient is demonstrating treatment non-response 1, 5

Alternative Assessment Considerations

• Given the floor effect on HFMSE (score of 11) for this non-sitter patient, consider using the SMA Functional Composite Score Revised (SMA-FCR) or ALSFRS-R for future assessments 2, 3
• The ALSFRS-R shows strong ability to discriminate between functional levels (B=0.72) and may be more sensitive to change in weaker patients 2
• Bedside functional scales (EK2, ALSFRS-R) show advantages over motor scales in non-sitters and may provide more meaningful assessment of treatment response 2

Critical Pitfalls to Avoid

• Do not continue expensive disease-modifying therapy without evidence of stabilization or improvement, as this patient's decline indicates treatment failure 1, 5, 4
• Do not attribute all functional decline to lack of exercise compliance without addressing the underlying question of treatment efficacy 2
• Do not ignore the contribution of progressive contractures to functional test performance, as these secondary complications can mask or exaggerate true motor function changes 6
• Do not use HFMSE as the sole outcome measure in non-sitters where floor effects limit sensitivity to change 2, 3