Myasthenia gravis concurrent with Parkinson's disease in a Spanish cohort. Causation or correlation?

BACKGROUND: Comorbidity between myasthenia gravis (MG) and other autoimmune diseases is well-documented. However, concurrent MG and Parkinson's disease (PD) have rarely been described. This concurrence has mostly been considered coincidental in cases reported to date. MATERIAL/METHODS: We characterized patients with concurrent MG and PD within a cohort of 631 MG patients by gender, age, MGFA class, quantitative MG score at diagnosis, UPDRS score at diagnosis, and the DaTSCAN uptake pattern, to determine the frequency and the phenotype of individuals with these two concurrent entities. Meta-analysis of cases in the literature was used for comparison with our series. RESULTS: Eighteen cases were identified in which the two diseases were concurrent. The major characteristics of the phenotype are male prevalence, late-onset MG, and frequent initial symptoms of dropped head and oculobulbar involvement. DAT confirmed reduced bilateral uptake in eleven patients and reduced unilateral uptake in the others. CONCLUSIONS: To our knowledge, this is the largest reported series of concurrent MG and PD. This concurrence is more common than expected (2.85%). Either MG or PD may appear first. We found no iatrogenic relationship for the order of appearance. The overlapping of symptoms sometimes leads physicians to overlook the second disease, instead viewing it as a deterioration of the first. This study describes patients with well-documented diagnoses of both MG and PD, thus providing further indications of a shared etiology of these two diseases. Prospective studies including genetic, immunological, and environmental analysis are necessary to identify possible common pathogenic mechanisms.

Staging Parkinson's Disease Combining Motor and Nonmotor Symptoms Correlates with Disability and Quality of Life.

INTRODUCTION: In a degenerative disorder such as Parkinson's disease (PD), it is important to establish clinical stages that allow to know the course of the disease. Our aim was to analyze whether a scale combining Hoehn and Yahr's motor stage (H&Y) and the nonmotor symptoms burden (NMSB) (assessed by the nonmotor symptoms scale (NMSS)) provides information about the disability and the patient's quality of life (QoL) with regard to a defined clinical stage. MATERIALS AND METHODS: Cross-sectional study in which 603 PD patients from the COPPADIS cohort were classified according to H&Y (1, stage I; 2, stage II; 3, stage III; 4, stage IV/V) and NMSB (A: NMSS = 0-20; B: NMSS = 21-40; C: NMSS = 41-70; D: NMSS ≥ 71) in 16 stages (HY.NMSB, from 1A to 4D). QoL was assessed with the PDQ-39SI, PQ-10, and EUROHIS-QOL8 and disability with the Schwab&England ADL (Activities of Daily Living) scale. RESULTS: A worse QoL and greater disability were observed at a higher stage of H&Y and NMSB (p < 0.0001). Combining both (HY.NMSB), patients in stages 1C and 1D and 2C and 2D had significantly worse QoL and/or less autonomy for ADL than those in stages 2A and 2B and 3A and 3B, respectively (p < 0.005; e.g., PDQ-39SI in 1D [n = 15] vs 2A [n = 101]: 28.6 ± 17.1 vs 7.9 ± 5.8; p < 0.0001). CONCLUSION: The HY.NMSB scale is simple and reflects the degree of patient involvement more accurately than the H&Y. Patients with a lower H&Y stage may be more affected if they have a greater NMS burden.

[Movement disorders units and management of motor fluctuations in advanced Parkinson's disease]. / Unidades de trastornos del movimiento y tratamiento de las fluctuaciones motoras de la enfermedad de Parkinson avanzada.

INTRODUCTION: Advanced Parkinson's disease (PD) entails complications, such as motor fluctuations. In Spain, medical attention for such cases is often provided in movement disorder units (MDU). AIM: To gain further knowledge of the diagnostic resources and therapeutic approach of MDU. SUBJECTS AND METHODS: A descriptive cross-sectional study was conducted. The researchers designed an on-line questionnaire, addressed to neurologists from MDUs, containing 48 questions about the resources they have available, the number of patients with PD and motor fluctuations that have been attended to, as well as the therapeutic approach, according to the Hoehn and Yahr (HY) scale. RESULTS: Fifty-five neurologists participated. Structural neuroimaging is available to 100% of them; 89% have access to functional neuroimaging; 89% have acute pharmacological tests available for use; 78% have access to genetic tests; and 53% have transcranial ultrasound at their disposal. There are 2.5 neurologists and 1.2 nurses per unit. Of the patients with PD that they see, 19% of them are in HY stage 1, 59% are in HY stage 2-3 and 22% are in HY stage 4-5. Treatment consists, first of all, in monoamine oxidase type B inhibitors in HY stages 1 and 2, and levodopa in HY stages 3, 4 and 5. Twenty-four per cent of the patients have motor fluctuations, with 5.5 off episodes per day, lasting 44 minutes, with a total of seven off hours per day. Fourteen per cent of the patients under 70 years of age with more than three long-term off episodes per day are receiving invasive treatment for motor fluctuations. CONCLUSIONS: MDUs are well equipped with diagnostic and pharmacological resources. Pharmacological treatments are tailored to each patient with a wide range of combinations. Despite this optimisation, the prevalence of motor fluctuations is still high in advanced patients, and invasive therapies may be underused.

TITLE: Unidades de trastornos del movimiento y tratamiento de las fluctuaciones motoras de la enfermedad de Parkinson avanzada.Introduccion. La enfermedad de Parkinson (EP) avanzada conlleva complicaciones, como fluctuaciones motoras. Su atencion sanitaria en España se realiza frecuentemente en unidades de trastornos del movimiento (UTM). Objetivo. Conocer los recursos diagnosticos y el abordaje terapeutico de las UTM. Sujetos y metodos. Estudio descriptivo, transversal. Se diseño un cuestionario en linea, dirigido a neurologos de UTM, de 48 preguntas sobre recursos disponibles, numero de pacientes atendidos con EP y con fluctuaciones motoras, y abordaje terapeutico segun el estadio de Hoehn y Yahr (HY). Resultados. Participaron 55 neurologos. Disponen de neuroimagen estructural el 100%; neuroimagen funcional, el 89%; tests agudos farmacologicos, el 89%; tests geneticos, el 78%, y ecografia transcraneal, el 53%. Hay 2,5 neurologos y 1,2 enfermeras por unidad. Atienden a un 19% de pacientes con EP en estadio de HY 1, un 59% en estadio de HY 2-3 y un 22% en estadio de HY 4-5. Utilizan en primer lugar los inhibidores de la monoaminooxidasa B en los estadios de HY 1 y 2, y levodopa en los estadios de HY 3, 4 y 5. Un 24% de los pacientes tiene fluctuaciones motoras, con 5,5 episodios off diarios, de 44 minutos, con un total de siete horas off diarias. Un 14% de los pacientes de hasta 70 años con mas de tres episodios off diarios de larga duracion recibe tratamiento invasivo para las fluctuaciones motoras. Conclusiones. Las UTM estan bien dotadas de recursos diagnosticos y farmacologicos. Los tratamientos farmacologicos se individualizan con gran variedad de combinaciones. A pesar de esta optimizacion, la prevalencia de fluctuaciones motoras es todavia alta en pacientes avanzados, y las terapias invasivas pueden infrautilizarse.

[Clinical and psychopathological factors associated with impulse control disorders in Parkinson's disease]. / Factores clínicos y psicopatológicos asociados a los trastornos del control de impulsos en la enfermedad de Parkinson.

INTRODUCTION: Impulse control disorders (ICD) constitute a complication that may arise during the course of Parkinson's disease (PD). Several factors have been linked to the development of these disorders, and their associated severe functional impairment requires specific and multidisciplinary management. The objective of this study was to evaluate the frequency of ICDs and the clinical and psychopathological factors associated with the appearance of these disorders. METHODS: Cross-sectional, descriptive, and analytical study of a sample of 115 PD patients evaluated to determine the presence of an ICD. Clinical scales were administered to assess disease severity, personality traits, and presence of psychiatric symptoms at the time of evaluation. RESULTS: Of the 115 patients with PD, 27 (23.48%) displayed some form of ICD; hypersexuality, exhibited by 14 (12.2%), and binge eating, present in 12 (10.1%), were the most common types. Clinical factors associated with ICD were treatment with dopamine agonists (OR: 13.39), earlier age at disease onset (OR: 0.92), and higher score on the UPDRS-I subscale; psychopathological factors with a significant association were trait anxiety (OR: 1.05) and impulsivity (OR: 1.13). CONCLUSIONS: ICDs are frequent in PD, and treatment with dopamine agonists is the most important risk factor for these disorders. High impulsivity and anxiety levels at time of evaluation, and younger age at disease onset, were also linked to increased risk. However, presence of these personality traits prior to evaluation did not increase risk of ICD.

Eighteen month study of continuous intraduodenal levodopa infusion in patients with advanced Parkinson's disease: impact on control of fluctuations and quality of life.

Symptom control, daily "on" time, and quality of life (QoL) of nine patients with Advanced Parkinson's Disease was assessed following 18-months treatment with Continuous Intraduodenal Levodopa Infusion (CIDLI). Patients had severe motor fluctuations and dyskinesias and had previously received treatment with oral levodopa and dopamine agonists. There were significant improvements in patients' symptoms on the Unified Parkinson's Disease Rating Scale, and QoL (Parkinson's Disease QoL Questionnaire; Schwab & England Capacity for Daily Living Scale; p < 0.05). Mean (+/-SD) daily "on" time increased from 6.1 +/- 1.9 to 12.0 +/- 3.4 h (p < 0.05). Improved QoL in APD was associated with CIDLI-related improvements in symptom control and increase in daily "on" time.