Deep brain stimulation for pantothenate kinase-associated neurodegeneration.

Pantothenate kinase-associated neurodegeneration (PKAN) is usually associated with dystonia, which is typically severe and progressive over time. Pallidal stimulation (GPi DBS) has been carried out in selected cases of PKAN with drug-resistant dystonia with variable results. We report a 30-month follow-up study of a 30-year-old woman with PKAN-related dystonia treated with GPi DBS. Postoperatively, the benefit quickly became evident, as the patient exhibited a marked improvement in her dystonia, including her writing difficulty. This result has been maintained up to the present. GPi DBS should be considered in dystonic PKAN patients provided fixed contractures and/or pyramidal symptoms are not present.

Psychometric characteristics of the Spanish version of instruments to measure neck pain disability.

BACKGROUND: The NDI, COM and NPQ are evaluation instruments for disability due to NP. There was no Spanish version of NDI or COM for which psychometric characteristics were known. The objectives of this study were to translate and culturally adapt the Spanish version of the Neck Disability Index Questionnaire (NDI), and the Core Outcome Measure (COM), to validate its use in Spanish speaking patients with non-specific neck pain (NP), and to compare their psychometric characteristics with those of the Spanish version of the Northwick Pain Questionnaire (NPQ). METHODS: Translation/re-translation of the English versions of the NDI and the COM was done blindly and independently by a multidisciplinary team. The study was done in 9 primary care Centers and 12 specialty services from 9 regions in Spain, with 221 acute, subacute and chronic patients who visited their physician for NP: 54 in the pilot phase and 167 in the validation phase. Neck pain (VAS), referred pain (VAS), disability (NDI, COM and NPQ), catastrophizing (CSQ) and quality of life (SF-12) were measured on their first visit and 14 days later. Patients' self-assessment was used as the external criterion for pain and disability. In the pilot phase, patients' understanding of each item in the NDI and COM was assessed, and on day 1 test-retest reliability was estimated by giving a second NDI and COM in which the name of the questionnaires and the order of the items had been changed. RESULTS: Comprehensibility of NDI and COM were good. Minutes needed to fill out the questionnaires [median, (P25, P75)]: NDI. 4 (2.2, 10.0), COM: 2.1 (1.0, 4.9). Reliability: [ICC, (95%CI)]: NDI: 0.88 (0.80, 0.93). COM: 0.85 (0.75,0.91). Sensitivity to change: Effect size for patients having worsened, not changed and improved between days 1 and 15, according to the external criterion for disability: NDI: -0.24, 0.15, 0.66; NPQ: -0.14, 0.06, 0.67; COM: 0.05, 0.19, 0.92. VALIDITY: Results of NDI, NPQ and COM were consistent with the external criterion for disability, whereas only those from NDI were consistent with the one for pain. Correlations with VAS, CSQ and SF-12 were similar for NDI and NPQ (absolute values between 0.36 and 0.50 on day 1, between 0.38 and 0.70 on day 15), and slightly lower for COM (between 0.36 and 0.48 on day 1, and between 0.33 and 0.61 on day 15). Correlation between NDI and NPQ: r = 0.84 on day 1, r = 0.91 on day 15. Correlation between COM and NPQ: r = 0.63 on day 1, r = 0.71 on day 15. CONCLUSION: Although most psychometric characteristics of NDI, NPQ and COM are similar, those from the latter one are worse and its use may lead to patients' evolution seeming more positive than it actually is. NDI seems to be the best instrument for measuring NP-related disability, since its results are the most consistent with patient's assessment of their own clinical status and evolution. It takes two more minutes to answer the NDI than to answer the COM, but it can be reliably filled out by the patient without assistance. TRIAL REGISTRATION: Clinical Trials Register NCT00349544.

Evaluation of timed tests in advanced Parkinsonian patients who were candidates for subthalamic stimulation.

UNLABELLED: The evaluation of Parkinson disease relies on the use of clinical scales, mainly the UPDRS. However, especially for those candidates for functional surgery, other objective methods are also considered, including the use of timed tests. METHODS: The authors studied the motor performance of 33 patients with advanced Parkinson disease (PD) who were candidates for subthalamic nucleus (STN) stimulation. Presurgical motor evaluation included UPDRS and the 4 timed tests of the CAPIT protocol, including pronation-supination (PS), finger dexterity (FD), movement between 2 points (MTP), and the walking test (WT). A clinical evaluation was performed during patients' OFF condition and during their best ON state. Fifteen patients were implanted with STN stimulation and were evaluated at 6 months with the same protocol described for the presurgical evaluation. RESULTS: At baseline, all 4 timed tests significantly correlated with total and motor UPDRS scores, in the OFF and ON states, especially MTP. All timed tests, save WT, significantly improved after surgery in the OFF state (especially MTP; P = 0.002). After surgery, all timed tests, save FD, significantly correlated with total and motor UPDRS scores in the OFF state. Timed tests, especially MTP, maintained an excellent correlation with UPDRS in both OFF and ON states before and after surgery.

Hernias discales cervicales: regresion espontanea / Cervical discal Hernias: spontaneous regression

Objetivo: evaluar la regresion espontanea de las hernias de disco cervicales (HDC) en resonancia magnetica (RM) y la mejoria clinica. Metodo: se analizaron retrospectivamente 15 pacientes con cervicobraquialgia causada por una HDC que no fueron operados. El tipo de hernia (medial o central) y la regresion (parcial o completa) fue evaluada con RM. La mejoria clinica se dividio en completa o parcial. Se compararon el tipo de hernia, su regresion y la mejoria clinica. Resultados: la regresion y mejoria clinica fueron completas en 9 casos. Las hernias laterales tuvieron mayor tendencia a la regresion. Conclusion: las HDC pueden regresar espontaneamente con mejoria clinica, sobre todo si son laterales

Hernias discales cervicales: regresion espontanea / Cervical discal Hernias: spontaneous regression

Objetivo: evaluar la regresion espontanea de las hernias de disco cervicales (HDC) en resonancia magnetica (RM) y la mejoria clinica. Metodo: se analizaron retrospectivamente 15 pacientes con cervicobraquialgia causada por una HDC que no fueron operados. El tipo de hernia (medial o central) y la regresion (parcial o completa) fue evaluada con RM. La mejoria clinica se dividio en completa o parcial. Se compararon el tipo de hernia, su regresion y la mejoria clinica. Resultados: la regresion y mejoria clinica fueron completas en 9 casos. Las hernias laterales tuvieron mayor tendencia a la regresion. Conclusion: las HDC pueden regresar espontaneamente con mejoria clinica, sobre todo si son laterales (AU)