The Parkinson's Disease Sleep Scale-2 (PDSS-2): Validation of the Spanish Version and Its Relationship With a Roommate-Based Version.

BACKGROUND: Because of the prevalence and impact of sleep disorders in Parkinson's disease (PD), valid instruments for their evaluation and monitoring are necessary. However, some nocturnal sleep disorders may go unnoticed by patients themselves. OBJECTIVES: To validate a pan-Spanish version of the Parkinson's Disease Sleep Scale Version 2 (PDSS-2) and to test the relationships between the PDSS-2 and a PDSS-2 roommate version. METHODS: PD patients (n = 399) from seven Spanish-speaking countries were included. In addition to the tested PDSS-2 scales, valid measures for sleep disorders and both motor and nonmotor manifestations were applied. Acceptability, dimensionality, reliability, precision, and construct validity were explored, as well as discrepancies and agreement between the PDSS-2 and the roommate version. RESULTS: PDSS-2 showed negligible floor and ceiling effects. Four factors (57% of the variance) were identified. Reliability parameters were satisfactory: alpha = 0.84; item homogeneity coefficient = 0.27; corrected item total correlation = 0.28 to 0.61; and test-retest reliability (average kappa = 0.70; intraclass correlation coefficient [ICC] = 0.83). The standard error of measurement was 5.84, and correlations with other scales assessing nocturnal sleep were high (rS = 0.62-0.56). In comparison to the patient-based total score, the by proxy total score showed no significant difference, high correlation (rS = 0.70), and acceptable agreement (ICC = 0.69), but there were discrepancies in two or more points in 18% of item scores. CONCLUSIONS: The Spanish version of the PDSS-2 has shown satisfactory clinimetric attributes. Acceptability and precision data are presented for the first time. The PDSS-2 roommate version could be useful to complement the patient-based evaluation, but additional studies are needed.

Experiencia clínica en pacientes con síndrome de Meige primario y disfonía espasmódica / Clinical experience with patients with spasmodic dysphonia and primary Meige síndrome

Introducción: El síndrome de Meige (SM) es considerado una distonía craneal segmentaria que afecta a dos o más músculos craneales. Específicamente, asocia blefaroespasmo a otra distonía craneal (oromandibular, cervical o laríngea). El objetivo de este artículo es presentar nuestra experiencia clínica en pacientes con disfonía espasmódica (DE) asociada a SM primario. Material y métodos: Estudio retrospectivo realizado entre mayo de 2010 y junio de 2015. Incluyó 8 pacientes. Las variables recogidas fueron: sexo, edad, distonías asociadas, electromiografía laríngea y tratamiento. Los resultados clínicos objetivados fueron realizados, siempre por el mismo investigador, con la escala GRBAS(i) y el cuestionario VHI-30. Resultados: Cincuenta y seis pacientes fueron tratados de SM por el servicio de Neurología. Ocho pacientes asociaron DE (prevalencia 14%). El 100% de nuestros pacientes tenían DE aductora. La mediana de edad para la aparición de DE fue de 71años. Todos los pacientes fueron tratados con infiltración intralaríngea de toxina botulínica bajo control electromiográfico. Se objetivó mejoría clínica tanto en la escala GRBAS(i) como en los cuestionarios VHI-30. Conclusión: En el estudio de la DE siempre debe tenerse presente su posible asociación con el SM. Desde el punto de vista otorrinolaringológico, el uso conjunto de la escala GRBAS(i) y los cuestionarios VHI-30 es útil, fiable y eficiente como método para evaluar evolución y respuesta a tratamiento. La infiltración laríngea de toxina botulínica bajo control electromiográfico es la alternativa terapéutica que provee mejores resultados clínicos. El manejo de la DE asociada al SM no difiere del de la DE aislada

Introduction: Meige syndrome (MS) is part of the group of segmental cranial dystonias, which affect more than two cranial muscle groups. Specifically, blepharospasm is associated with another cranial dystonia (oromandibular, cervical or laryngeal). The aim of this paper was to report our experience in patients with spasmodic dysphonia (SD) associated with primary MS. Material and methods: A retrospective study involving 8 patients between May 2010 and June 2015. Variables recorded were: age, sex, associated dystonia, electromyographic pattern in laryngeal muscles and treatment given. Outcomes after treatment were assessed using GRBAS(i) scale and VHI-30 questionnaire, always provided by the same examiner. Results: Fifty-six patients with MS were treated in the Neurology Department. Eight patients of 56 were diagnosed with SD (prevalence of 14%). All of our patients had adductor SD. The median age was 71years. All the patients were treated with intralaryngeal botulinum toxin under electromyographic control. Clinically relevant improvements were found after treatment on both the GRBAS(i) scale and the VHI-30 questionnaire. Conclusion: In the study of SD, we should always rule out an association with MS. From the point of view of otorhinolaryngology, the joint use of the GRBAS(i) scale and the VHI-30 questionnaire are useful, reliable and efficient methods for assessing progress and response to treatment. Laryngeal infiltration under electromyographic control with botulinum toxin is the therapeutic alternative that provides better results. The management of SD associated with MS does not differ from isolated SD

Clinical experience with patients with spasmodic dysphonia and primary Meige syndrome. / Experiencia clínica en pacientes con síndrome de Meige primario y disfonía espasmódica.

INTRODUCTION: Meige syndrome (MS) is part of the group of segmental cranial dystonias, which affect more than two cranial muscle groups. Specifically, blepharospasm is associated with another cranial dystonia (oromandibular, cervical or laryngeal). The aim of this paper was to report our experience in patients with spasmodic dysphonia (SD) associated with primary MS. MATERIAL AND METHODS: A retrospective study involving 8 patients between May 2010 and June 2015. Variables recorded were: age, sex, associated dystonia, electromyographic pattern in laryngeal muscles and treatment given. Outcomes after treatment were assessed using GRBAS(i) scale and VHI-30 questionnaire, always provided by the same examiner. RESULTS: Fifty-six patients with MS were treated in the Neurology Department. Eight patients of 56 were diagnosed with SD (prevalence of 14%). All of our patients had adductor SD. The median age was 71years. All the patients were treated with intralaryngeal botulinum toxin under electromyographic control. Clinically relevant improvements were found after treatment on both the GRBAS(i) scale and the VHI-30 questionnaire. CONCLUSION: In the study of SD, we should always rule out an association with MS. From the point of view of otorhinolaryngology, the joint use of the GRBAS(i) scale and the VHI-30 questionnaire are useful, reliable and efficient methods for assessing progress and response to treatment. Laryngeal infiltration under electromyographic control with botulinum toxin is the therapeutic alternative that provides better results. The management of SD associated with MS does not differ from isolated SD.

Rheumatoid meningitis mimicking progressive supranuclear palsy.

INTRODUCTION: Rheumatoid meningitis is an uncommon manifestation of longstanding rheumatoid arthritis and few cases have been described. The clinical presentation is extremely variable as reported in medical literature. CASE REPORT: We report a 71-year-old woman with 15 years of seropositive rheumatoid arthritis who developed neurological complications: cognitive deterioration; hypomimia; limitation on vertical gaze; and axial stiffness, resembling progressive supranuclear palsy and seizures. Brain magnetic resonance imaging showed a diffuse dural plaque on both frontal and temporal lobes exhibiting homogeneous gadolinium enhancement. There was diffuse leptomeningeal enhancement and hyperintense white matter lesions. The final diagnosis made by image-guided biopsy showed rheumatoid pachymeningitis. After the definitive diagnosis, high doses of corticosteroids and immunosuppressive treatment were started. CONCLUSIONS: We emphasize the diagnostic importance of the biopsy in cases of chronic pachymeningitis and stress that diverse entities can cause progressive supranuclear palsy-like phenotypes.

The SCOPA-Motor Scale for assessment of Parkinson's disease is a consistent and valid measure.

OBJECTIVE: The SCOPA-Motor Scale (S-MS) for assessment of Parkinson's disease (PD), contains 21 items in three domains: Motor examination, Disability, and Complications. Our objective was to validate the S-MS Spanish version. STUDY DESIGN AND SETTING: This validation study was based on a multicenter, cross-sectional, one-point-in-time evaluation design. The applied measures were: Unified Parkinson's Disease Rating Scale-3.0 (UPDRS); S-MS; PD Global Evaluation (PDGE); and Clinical Global Impression of severity (CGI). Completeness of data collection, floor and ceiling effect, internal consistency, precision, and construct and discriminative validity were analyzed in 151 PD patients. RESULTS: Scores from S-MS were fully computable. Floor effect was high for Complications (43.7%). Cronbach's alpha was > 0.90 for every domain, and item-total correlation was > 0.70 except for Examination. Standard error of measurement (SEM) ranged from 0.40 to 2.4. Convergent validity with corresponding UPDRS sections yielded coefficients > 0.90. Discriminative validity across Hoehn and Yahr (HY) and CGI stages was significant (Kruskal-Wallis, P < .0001). Insofar as internal consistency was concerned, alpha-values of the Examination sections were marginally higher for the UPDRS than for the S-MS (a finding perhaps accounted for by redundancy in this part of the UPDRS). CONCLUSION: The S-MS is a consistent and valid scale, shorter by almost half than the UPDRS.