ABSTRACT
INTRODUCTION: Reduced facial expression or amimia is one of the most typical characteristics of Parkinson's disease (PD). Despite being described in classic texts, its significance, physiopathology and correlation with motor and non-motor symptoms is largely unknown. PATIENTS AND METHODS: We have studied facial bradykinesia in a group of 84 de novo PD patients prospectively evaluated for five years. We also studied the relationship of facial bradykinesia with depression in a subgroup of 30 patients. RESULTS: Baseline and follow-up assessments were performed with the Unified Parkinson's Disease Rating Scale (UPDRS). Baseline facial bradykinesia was rated according to item 19 of UPDRS. Baseline facial bradykinesia correlated with total and motor baseline UPDRS. In addition, baseline bradykinesia correlated with total and motor UPDRS at five years. However baseline bradykinesia did not influence the presence of motor (motor fluctuation, dyskinesias and freezing of gait) or non-motor complications (delusion, behavior abnormalities and dementia) at five years. Finally a subgroup of 30 patients completed the self-report version of the Quick Inventory of Depressive Symptoms (QIDS-SR16) questionnaire, facial bradykinesia did not correlate with QIDS-SR16 scores. CONCLUSION: Our study suggests that baseline facial bradykinesia correlates with general baseline situation in PD and even might predict the motor and functional status at five years.
TITLE: Amimia en la enfermedad de Parkinson. Significado y correlacion con la clinica.Introduccion. La amimia o reduccion de la expresion facial es una de las caracteristicas mas tipicas de la enfermedad de Parkinson (EP), y se puede definir como bradicinesia facial. A pesar de ser un elemento clasico, la amimia no se conoce bien, no se sabe con precision su fisiopatologia, su significado patologico ni su correlacion con otros sintomas motores o no motores, incluyendo la depresion. Pacientes y metodos. Se ha analizado la amimia en un grupo de 84 pacientes con EP evaluados de forma prospectiva desde su diagnostico hasta el quinto año de evolucion, y tambien la correlacion entre la amimia basal y la depresion en un subgrupo de 30 pacientes con EP. Resultados. La valoracion basal (antes del tratamiento) y las evaluaciones de seguimiento se realizaron mediante la Unified Parkinson's Disease Rating Scale (UPDRS). La amimia se evaluo mediante el item 19 de la UPDRS. La amimia basal se correlaciono con la UPDRS basal total y motora. Ademas, la amimia basal se correlaciono con la UPDRS total y motora a los cinco años de evolucion. Sin embargo, la amimia basal no se relaciono con la presencia de complicaciones motoras (fluctuaciones motoras, discinesias o bloqueos) o no motoras. La correlacion entre amimia y depresion se analizo mediante el Quick Inventory of Depressive Symptoms (QIDS-SR16). La amimia no se correlaciono con las puntuaciones del QIDS-SR16. Conclusion. Este estudio sugiere que la amimia basal se correlaciona con la situacion basal general (UPDRS) e incluso con la valoracion clinica a los cinco años, aunque no predice la tasa de complicaciones a medio plazo.
Subject(s)
Facial Expression , Hypokinesia/etiology , Parkinson Disease/complications , Humans , Middle Aged , Parkinson Disease/diagnosis , Parkinson Disease/physiopathology , Prospective StudiesABSTRACT
TITLE: Enfermedad de Parkinson senil benigna.
Subject(s)
Parkinson Disease/diagnosis , Age of Onset , Aged , Female , Humans , Male , Middle Aged , Prospective Studies , Severity of Illness IndexABSTRACT
INTRODUCTION: Huntington's disease (HD) is an autosomal dominant hereditary disease caused by triplet repetition in exon 1 of the huntingtin protein located in chromosome 4. Medium spiny neurons in the striatum are selectively affected. Clinical manifestations include progressive behavioural, motor and cognitive disorders. There is no treatment available today capable of modifying the natural course of the disease. A great amount of research work is being carried out, much of which involves animal models of the disease. DEVELOPMENT: We reviewed the articles published in PubMed on basic research into HD and analysed the most frequently used models. Transgenic mouse models, excitotoxic models, transgenic fly models and cell cultures are all used in studies into HD. The advantages and disadvantages of each of them are highlighted. CONCLUSION: The contribution made by each model of HD must be known in order to draw up a correct design in experimental studies of the disease.
Subject(s)
Disease Models, Animal , Huntington Disease , Animals , Cells, Cultured , Humans , Huntington Disease/pathology , Huntington Disease/physiopathology , Mice , Mice, Transgenic , PubMed , Review Literature as TopicABSTRACT
BACKGROUND AND METHODS: The Parkinson's Disease Questionnaire (PDQ-39) was the first specific instrument for evaluation of the "health-related quality of life" (QoL) in Parkinson's disease patients. The PDQ-39 has been subjected to adaptation to Spanish language and culture (PDQ-39 Spanish version, PDQ-39SV) and this version has been validated in aspects of internal consistency and construct validity. The present study assess the test-retest reliability and the convergent validity of the PDQ-39SV with a generic QoL instrument (SF-36). RESULTS: Most of the PDQ-39 dimensions showed an adequate consistency-Cronbach's alpha > 0.7 for six dimensions. As a whole, test-retest reliability resulted satisfactory. Two dimensions-activities of daily living and emotional well-being- showed a low grade significant difference (paired Student t-test, p < 0.05) due to improvement in the second survey (at 10 to 14 days from the first one) perhaps related to adjustments of the treatment at the first visit. A strong association (Spearman r, p < 0.001), indicative of convergent validity, was obtained for the PDQ-39 dimensions and the relevant SF-36 scales, as well as for the physical and mental component summary scores of the SF-36. CONCLUSIONS: Taking into account these results and previous studies, it is concluded that the PDQ-39 SV is a reliable measure that has construct validity.
