Spinal cord injury following a mild trauma in homocystinuria-related bone frailty: neurorehabilitation and education on bone health management.

Homocystinuria (HCU) is a rare autosomal recessive disease characterized by the deficiency of cystathionine ß-synthetase, presenting with variable clinical features including micronutrient deficiency-related osteoporosis. Early-onset osteoporosis results in increased bone fragility, which is associated with low-impact fractures. To date, no traumatic myelopathy has ever been described in patients with HCU. This case report describes a 30-year-old male patient with HCU who was not aware that he was at high risk of sustaining debilitating bone fractures. After a mild trauma, he reported a T12 compression fracture with spinal cord injury. The patient underwent a tailored rehabilitation program, on the basis of multidisciplinary approach, and was educated about the increased risk of fractures, maintaining adherence to treatment and diet, having an active lifestyle, avoiding excessive weight loss, and preventing falls or other traumatic injury. To reduce the risk of fractures - with possible catastrophic consequences - patients with HCU, and their caregivers, should be educated about prevention of fractures.

Active music therapy approach in amyotrophic lateral sclerosis: a randomized-controlled trial.

This randomized controlled study assessed the efficacy of active music therapy (AMT) on anxiety, depression, and quality of life in amyotrophic lateral sclerosis (ALS). Communication and relationship during AMT treatment were also evaluated. Thirty patients were assigned randomly to experimental [AMT plus standard of care (SC)] or control (SC) groups. AMT consisted of 12 sessions (three times a week), whereas the SC treatment was based on physical and speech rehabilitation sessions, occupational therapy, and psychological support. ALS Functional Rating Scale-Revised, Hospital Anxiety and Depression Scale, McGill Quality of Life Questionnaire, and Music Therapy Rating Scale were administered to assess functional, psychological, and music therapy outcomes. The AMT group improved significantly in McGill Quality of Life Questionnaire global scores (P=0.035) and showed a positive trend in nonverbal and sonorous-music relationship during the treatment. Further studies involving larger samples in a longer AMT intervention are needed to confirm the effectiveness of this approach in ALS.

Development of the Italian version of the Neck Pain and Disability Scale, NPDS-I: cross-cultural adaptation, reliability, and validity.

STUDY DESIGN: Evaluation of the psychometric properties of the Neck Pain and Disability Scale (NPDS). OBJECTIVE: Translating, culturally adapting, and validating the Italian version of the NPDS-I. SUMMARY OF BACKGROUND DATA: Great importance is devoted to validated and comprehensive outcome measures to improve interventions for neck pain. A translated form of a functional scale in patients with neck pain has never been studied within the Italian population. METHODS: The Italian version of the NPDS questionnaire was developed involving forward-backward translation and final review by an expert committee to establish multidimensional correspondence with the original English form. Psychometric testing included factor analysis with Varimax rotation, reliability by internal consistency (Cronbach's alpha) and test-retest repeatability (Spearman's rank test), validity by comparing the Italian version of SF-36 to NPDS-I (Pearson's correlation); further subscales comparisons to single SF-36 domains were also conducted. RESULTS: The authors required a 4-month period before achieving a shared version of the NPDS-I. The form was administered to 157 subjects, presenting good acceptability. Factor analyses demonstrated 3 subscales (63% of explained variance), defined as neck dysfunction related to general activities (subscale 1, 8 items), neck pain and cognitive-behavioral aspects (subscale 2, 8 items), neck dysfunction related to activities of the cervical spine (subscale 3, 4 items). The questionnaire showed high internal consistency (NPDS: 0.942, subscale 1: 0.919, subscale 2: 0.856, subscale 3: 0.889) and good test-retest reliability (P < 0.001). Validity was explained by high correlations with SF-36 total score (r = -0.47, P < 0.001) and with single SF-36 domains scores, highly significant (P < 0.001) with the exception of physical role domain (r = -0.17, P = 0.035). Finally, the 3 subscales demonstrated good correlations when compared with selected SF-36 domains. CONCLUSION: The NPDS-I outcome questionnaire was successfully translated into Italian, showing good multidimensional and psychometric properties, supporting the results of the already existing versions of the scale. Its use is recommended in clinical and research practice.