[A patient with Creutzfeldt-Jakob disease presenting with a motoneurone disease]. / Paciente con enfermedad de Creutzfeldt-Jakob que comenzó con una enfermedad de motoneurona.

TITLE: Paciente con enfermedad de Creutzfeldt-Jakob que comenzó con una enfermedad de motoneurona.

Incidence and subtypes of early-onset dementia in a geographically defined general population.

OBJECTIVE: To estimate the incidence of early-onset dementia (EOD) and to compare the clinical characteristics of EOD vs late-onset dementia (LOD) in a geographically defined area. METHODS: We used data from the Registry of Dementia of Girona (ReDeGi), an epidemiologic surveillance system of dementia. The ReDeGi is a standardized clinical registry of new dementia cases diagnosed in the 7 hospitals of the Health Region of Girona (Catalonia, Spain), which encompasses an area of 5,517 km(2) and 690,207 inhabitants. EOD cases were defined as those patients residing in the target area at the time of diagnosis who were diagnosed with dementia with an age at onset of symptoms before 65 years. RESULTS: The ReDeGi registered 2,083 patients between January 1, 2007, and December 31, 2009 (6.9% EOD). The incidence rate of EOD for the age range 30-64 was 13.4 cases per 100,000 person-years (95% confidence interval 11.3-15.8). Alzheimer disease was the most frequent cause of EOD (42.4%), followed by secondary dementia (18.1%), vascular dementia (13.8%), and frontotemporal dementia (9.7%). EOD cases at the time of diagnosis were less impaired on the Mini-Mental State Examination and had a greater score on the Blessed Dementia Rating Scale behavior subscale than LOD cases. The frequency of a personal history of depression was higher in EOD cases. CONCLUSIONS: The incidence of EOD was less than 6 cases per 100,000 person-years in the age group 30-49 years; in the age group 50-64 years, the incidence rate was 3-fold higher and doubled with each 5-year increase.

[Analysis of the demand for neurological health care generated by primary care in a geographical area within the districts of Girona]. / Análisis de la demanda asistencial neurológica generada por la atención primaria en una área geográfica de las comarcas de Girona.

AIM: To perform a descriptive analysis of the outpatient activity in a neurological department in terms of the frequency and type of neurological diseases that were attended. PATIENTS AND METHODS: A retrospective and cross-sectional study was conducted involving patients referred to the neurology outpatients department. The cases that visited for the first time during the years 2006 and 2007 were recorded consecutively. Medical information was evaluated based on computerised hospital registers and the following data were collected: health referral area, date of visit, age, sex and diagnosis according to the International Classification of Diseases, 9th edition, clinical modification (CIE-9-MC), reconverted into the International Classification of Diseases, 10th edition (CIE-10). RESULTS: The mean age was 60.6 +/- 20.9 years (range: 4-95 years) and 61.4% were females. The rate of frequency of visits was 4.3 requests/1000 inhabitants for the year 2006 and 3.9 for the year 2007; an increase was observed with age for the neurodegenerative diseases. As far as the CIE-10 is concerned, the diseases were classified as episodic and paroxysmal (25%), degenerative and demyelinating (18.6%), organic mental disorders (14.7%), extrapyramidal syndromes (10.5%), diseases affecting cerebral circulation (3.5%), stress-related disorders and somatomorphs (3.5%) and diseases of the inner ear (3.3%). The remaining pathologies did not reach 3% of the total. Diseases of the central nervous system were observed in 61% of the patients and psychiatric disorders were found in 20.3%. The most common diseases were cognitive disorders (31.5%), headaches (18.6%) and movement disorders (11.7%), followed by psychiatric diseases, epilepsies, cerebrovascular diseases and neuropathies. CONCLUSIONS: The frequency of visits increases with age and the order, in terms of frequency, was: episodic and paroxysmal, degenerative and demyelinating, psychotic and behavioural disorders, and extrapyramidal syndromes.

Análisis de la demanda asistencial neurológica generada por la atención primaria en una área geográfica de las comarcas de Girona / Analysis of the demand for neurological health care generated by primary care in a geographical area within the districts of Girona

Objetivo. Análisis descriptivo de la actividad ambulatoria en un servicio de neurología en relación con la frecuenciay tipo de enfermedades neurológicas atendidas. Pacientes y métodos. Estudio retrospectivo y trasversal de los pacientesremitidos a la consulta ambulatoria de neurología. Se registraron consecutivamente los casos visitados por primera vez durantelos años 2006 y 2007. Se evaluó la documentación médica a partir de los registros hospitalarios informatizados, recogiéndose:zona de salud de referencia, fecha de visita, edad, sexo y diagnóstico según la Clasificación Internacional de Enfermedades,9.ª edición, modificación clínica (CIE-9-MC), reconvertida a la Clasificación Internacional de Enfermedades, 10.ªedición (CIE-10). Resultados. La media de edad fue de 60,6 ± 20,9 años (rango: 4-95 años) y el 61,4% fueron mujeres. El índicede frecuentación fue de 4,3 solicitudes/1.000 habitantes para el año 2006 y de 3,9 para el año 2007, y se observó unincremento con la edad para las enfermedades neurodegenerativas. Respecto a la CIE-10, las enfermedades se clasificaroncomo episódicas y paroxísticas (25%), degenerativas y desmielinizantes (18,6%), trastornos mentales orgánicos (14,7%), síndromesextrapiramidales (10,5%), enfermedades de la circulación cerebral (3,5%), trastornos relacionados con el estrés y somatomorfos(3,5%) y enfermedades del oído interno (3,3%). Las demás patologías no alcanzaron el 3%. El 61% de los pacientespresentó enfermedades del sistema nervioso central y el 20,3%, trastornos psiquiátricos. Las enfermedades más frecuentesfueron los trastornos cognitivos (31,5%), las cefaleas (18,6%) y los trastornos del movimiento (11,7%), seguidas delas enfermedades psiquiátricas, epilepsias, enfermedades cerebrovasculares y neuropatías(AU)

Aim. To perform a descriptive analysis of the outpatient activity in a neurological department in terms of thefrequency and type of neurological diseases that were attended. Patients and methods. A retrospective and cross-sectional study was conducted involving patients referred to the neurology outpatients department. The cases that visited for the first time during the years 2006 and 2007 were recorded consecutively. Medical information was evaluated based on computerisedhospital registers and the following data were collected: health referral area, date of visit, age, sex and diagnosis according to the International Classification of Diseases, 9th edition, clinical modification (CIE-9-MC), reconverted into the InternationalClassification of Diseases, 10th edition (CIE-10). Results. The mean age was 60.6 ± 20.9 years (range: 4-95 years) and 61.4% were females. The rate of frequency of visits was 4.3 requests/1000 inhabitants for the year 2006 and 3.9 for the year2007; an increase was observed with age for the neurodegenerative diseases. As far as the CIE-10 is concerned, the diseases were classified as episodic and paroxysmal (25%), degenerative and demyelinating (18.6%), organic mental disorders (14.7%), extrapyramidal syndromes (10.5%), diseases affecting cerebral circulation (3.5%), stress-related disorders and somatomorphs (3.5%) and diseases of the inner ear (3.3%). The remaining pathologies did not reach 3% of the total. Diseases of the central nervous system were observed in 61% of the patients and psychiatric disorders were found in 20.3%. The mostcommon diseases were cognitive disorders (31.5%), headaches (18.6%) and movement disorders (11.7%), followed by psychiatric diseases, epilepsies, cerebrovascular diseases and neuropathies(AU)