Mortalidad por enfermedad de Parkinson en Chile / Parkinson disease mortality rates in Chile

Background: Parkinson disease (PD) is the second more common neurodegene-rative disorder and determines a loss in quality oflife, caregiver burden and increased mortality in those affected. Aim: To determine the rates of mortality due to Parkinson disease in Chile. Material andMethods: Data were collected from death certificates between 1997 and 2008. Mortality rates were calculated and the mortality trend was established along the study period, and analyzed by age, sex and geographic región of Chile. Results: There was a steady increase in mortality over the years, particularly since2001 (r²= 0.85, p < 0.01). The increase in mortality rates was 0.25per 100.000 habitants/year (r²: 0.89, p < 0.01). Mortality was always higher in men and in those older than 80years. The highest rate (per 100,000 habitants) in Chile was observed in the región of Valparaíso (2.4) while the lowest was in Antofagasta (0.8) (t = 3.72, p < 0.05). Conclusions: Mortality associated with PD has increased progressively, consistent with the expected increase in prevalence ofthe disease in the population.

Avances en el tratamiento del accidente cerebrovascular isquémico / Advances in treatment of ischemic stroke

Stroke is a devastating disease with a high mortality and high degree of disability. In the nineties a new specific treatment was proved to be beneficial, the intravenous recombinant tisular plasminogen activator (IV r-TPA). The first notion of pharmacologic vessel revascularization marked a new era in stroke treatment. However, there are cases that need a more aggressive approach, such as large intracranial vessel occlusion. Recently, new endovascular approaches and an explosive development of devices to remove or suck the thrombus, bring a second revolution in the manner that we treat stroke.

Muerte cerebral o muerte encefálica: la muerte es una sola / Brain death or encephalic death: the death is only one

Historic aspects are described of the concept of death and alter brain death. We analyzed definition and characteristics of coma without answer, cerebral trunk activity absence. Theyare described and details the execution of the test of apnea and its limits. It is analyzed some differential diagnostics and movements that can be found in brain death. Some complementaryexams are indicated and by finalize legal aspects are discussed.

Manejo incial del accidente cerebrovascular (ACV) isquémico agudo: los detalles hacen la diferencia / Initial management of acute ischemic stroke: details make the difference

The cerebrovascular disease, especially the ischemic stroke, is the second cause of death in our country. In Chile every year we have 14.000 new cases of ischemic stroke; this number is going up due to the progressive aging of our population. Every action we do during the six first ours of the evolution of the disease are critical for the final outcome. A proper treatments made of a group of unspecific actions that can really change the prognosis of the disease: Multidisciplinary approach, quick stabilization, unspecific neuroprotection, CT in the emergency room and hospitalization in a Stroke Unit, prevent and treat infections, early physical therapy, diagnosis of underlying disease and secondary prevention. The current law in Chile guarantee to every patient with an ischemic stroke the right to be hospitalized under the suspect of a stroke and to be followed up by a Neurologist, Psychologist, Physiatrists, kinesiologist, Speech and swelling therapist and a physical therapist. This law can only be fulfilled if every hospital in our country, or at least one per region, has its own Stroke Unit.

Tendencia de la mortalidad por tumores cerebrales malignos en Chile: Análisis de tasas / Analysis of Chilean trends in mortality from malignant brain tumors

Objective: To analyse the Chilean trends in mortality from brain malignancies between 1985 and 1999. Methods: We calculated mortality rates from malignant brain tumors using data obtained from death certificates available at the National Statistics Office. The following International Classification of Diseases categories were selected: 191.0 to 191.9 (ICD-9), and C71.0 to C71.9 (ICD-10). The rates were adjusted using direct standardization. Prais-Winsten methodology was used for time correlation analysis. Results: Sex-specific rates varied from 0.9 to 1.75 per 100.000 in men and from 0.7 to 1.22 in women. The trend was toward a statistically significant increase in mortality from malignant brain tumors in both groups. The analysis by age group showed no statistically significant variation in those below 35 years old, and a statistically significant increase in those between 35 and 39 years old, and in those above 45 years old. Conclusions: The trend in mortality from malignant brain tumors, in Chile, shows a statistically significant increase in those between 35 and 39 years old, and in those above 45 years old.

Existe controversia en el aumento de la incidencia en las tasas de tumores primarios malignos de cerebro. Este incremento podría explicarse por el crecimiento exponencial en el número de Tomografías computarizadas. Objetivo: Evaluar la tendencia de la mortalidad por tumores cerebrales primarios malignos en Chile (TCM). Metodología: Estudio de tasas de mortalidad de datos obtenidos en índices demográficos (años 1985 a 1999). Se utilizó la población de Chile de los años estudiados y se ajustó a la población estimada de 1999. Se usó las categorías 191,0 a 191,9 y C 71-0 a C 71-9 de la Clasificación Internacional, correspondiendo al grupo de tumores malignos del SNC. Se estudiaron tasas específicas por edad y sexo. Se calculó la tendencia utilizando las tasas ajustada por edad y sexo. Se utilizó una prueba de regresión lineal (Prais-Winsten) para mediciones correlacionales en el tiempo (Stata 7). Resultados: Se obtuvo 2.304 TCM. Las tasas específicas por sexo varían entre 0,90 a 1,75 x 100.000 en hombres y de 0,7 a 1,22 en mujeres. La tendencia global de la mortalidad por tumores tiende al ascenso en ambos grupos y es significativamente más alta en hombres (0,47 95 % IC 0,18 a 0,42 p = < 0,005) El estudio por grupos etarios no muestra un aumento significativo en menores de 14 años, ni entre 15 y 34 años. En los grupos de 45 años y más es estadísticamente significativa. Conclusión: La tendencia a la mortalidad por TCM aparece en ascenso en los grupos etarios 35 a 39 años y 45 años y más.

Evaluación de una unidad de tratamiento del ataque cerebral en un hospital universitario / Evaluation of a stroke unit at a university hospital in Chile

Background: Stroke is the second specific cause of death in Chile, with a mortality rate of 48.6 per 100.000 inhabitans. It accounts for 6 percent of all hospitalizations among adults. Aim: To study the type of patients hospitalized at a Stroke Unit in a general hospital and the costs and benefits of such unit. Material and methods: A descriptive and retrospective study using a patient registry, developed in Access® that included separate sections for ischemic and hemorrhagic stroke. Established diagnostic criteria were used. The mean costs per patient and complications were also calculated. Results: During 2003, 425 stroke patients were admitted to our hospital and 105 (age range 30-89 years, 58 percent female) were hospitalized at the Stroke Unit. Eighty three percent had ischemic and 16 percent had hemorrhagic stroke. The most common etiologies were thrombosis in 41 percent, embolism in 36 percent, lacunar in 13 percent, arterial dissection in 5 percent and transient ischemic attack in 3 percent. Fifty eight percent of patients had partial anterior ischemic stroke (PACI), 73 percent had hypertension and 29.5 percent diabetes. Only 18 percent arrived to the Stroke Unit with less of 6 hours of evolution, 7 percent of patients were admitted within the 3 hours after the onset of symptoms and 18 percent, from 3 to 6 hours. The mean lenght of stay in the Stroke Unit was 6.6 days and at the hospital 9.9 days (p <0.01). The mean costs per patient at the Stroke Unit and at the hospital were US$ 5.550 and US$ 4.815, respectively (p =ns). Conclusions: The Stroke Unit decreases hospital stay days without raising costs importantly. The inclusion criteria for stroke patients admitted to the Unit were adequate and the stroke registry allowed a good assessment of the Unit operation.

Encefalomielitis aguda diseminada de presentación atípica: utilidad de la biopsia estereotáctica para su diagnóstico. Caso clínico / Diagnosis of acute disseminated encephalomyelitis using a stereotaxic biopsy: Report of one case

We report a 15 year-old female presenting with behavioral disturbances, headache, left hemiparesis and paresis of the vertical gaze. CAT scan and magnetic resonance showed an involvement of right thalamus, third ventricle and medial temporal lobe suggesting an encephalitis or lymphoma. 201Thalium SPECT suggested a lymphoma. A stereotaxic biopsy showed a subacute demyelinizing lesion, compatible with an acute disseminated encephalomyelitis. The patient was treated with Methylprednisolone with resolution of symptoms. She remains in good condition after one year of follow-up.

Migraña con aura: Una mirada molecular a un problema hereditario / Migraine with aura: A molecular view to a hereditary disorder

Migraine with aura (MA) is a common neurological disorder characterized by severe episodes of headache, generally unilateral, which are preceded by a focal reversible neurological deficit. The studies on MA reveal the existence of familiar aggregation compatible with a high degree of heritability and a complex multifactorial mode of transmission. The genetic factors of MA are unknown. An association to a locus in the long arm of chromosome 4 at the level of 4q22-q25 was recently reported in families with MA in Finland. Objetive: To analyze the genomic DNA of 5 Chilean families with MA to determine if there is linkage to the locus described in Finish families. Metodology: Families with MA were selected applying the diagnostic criteria of the International Headache Society (ICD-10), in which the index case or a member of the family should have MA. The genomic DNA was extracted from peripheral lymphocytes of members of each family (n = 25). Highly polymorphic genomic markers were used for the systematic analysis of the locus on 4q22-q25. Results and Discussion: The LOD score analysis of the 5 Chilean families investigated showed absence of linkage to the marker D4S1578 (maximum 0,35; p = 0.24). Due to the complexity of MA heritability it is possible that one or more loci different from the studied region are involved in the pathophysiology of MA. The study will continue with the inclusion of more family members and isolated MA cases, with the purpose of comparing Chilean and German families in an independent sample.

La migraña con aura (MA) es una patología común, caracterizada por ataques severos de cefalea generalmente unilateral, precedidos por un déficit neurológico focal reversible. Los estudios en MA revelan la existencia de agregación familiar compatible con un alto grado de herencia y un modo de transmisión multifactorial complejo. Los factores genéticos de la MA con herencia compleja son desconocidos. En familias finlandesas con MA se encontró recientemente ligamiento a un locus en el brazo largo del cromosoma 4 a nivel de 4q22-q25. Objetivo: Analizar el DNA genómico de 5 familias chilenas con MA para determinar si hay ligamiento al locus descrito en familias finlandesas. Metodología: Se seleccionaron familias con MA aplicando los criterios diagnósticos de la Sociedad Internacional de Cefalea (ICD-10), donde el caso índice o algún miembro de la familia debían tener MA. El DNA genómico se extrajo de leucocitos en sangre periférica de miembros seleccionados de cada familia (n = 25). Se usaron marcadores genómicos altamente polimórficos (microsatélites) para el análisis sistemático del locus 4q22-q25. Resultados y Discusión: El cálculo del LOD score mostró ausencia de ligamiento en las 5 familias analizadas (máximo de 0,35; p = 0,24) con el marcador D4S1578. Dado el carácter complejo de herencia en la MA es posible que existan uno o varios loci involucrados en su etiopatogenia, distintos al de la región estudiada. El estudio se continuará a través de la ampliación de las familias estudiadas y de la recolección de casos aislados de MA, con el objetivo de realizar futuros estudios de asociación para comparar los hallazgos en familias chilenas con los de familias alemanas en una muestra independiente.

Pseudodemencia como forma de presentación de la enfermedad de Creutzfeldt-Jakob / Pseudodementia as presentation form of the Creutzfeldt-Jakob disease

Se presentan dos casos de enfermedad de Creutzfeldt-Jakob que fueron diagnosticadas como pseudodemencias. Eran mujeres con trastornos de conducta; el examen mostró ausencia de signos neurológicos categóricos, actitudes anormales y discordancias en los rendimientos neuropsicologicos; las tomografías computadas cerebrales (TAC) y, en un caso el EEG fueron normales. El seguimiento clínico, EEG y con resonancia nuclear magnética cerebral permitieron diagnosticar enfermedad de Creutzfeldt-Jakob probable. Se postula que la pseudodemencia puede ser considerada un síndrome con importantes factores psicosociales, a los cuales puede sumarse como desencadenante una patología cerebral de magnitud moderada.

Two female patients with Creutzfeldt-Jakob’s disease whose initial diagnosis was pseudodementia are reported. Both of them began with abnormal behaviour that could have had relation to biographic circumstances. They had normal or slightly abnormal neurological signs, abnormal attitudes and significant discordances in the neuropsychological performance. The CT scan (and the EGG in the second d case) were normal. However, the clinical course, serial EGG and MRI led us to diagnose probable Creutzfeldt-Jakob’s disease. It is concluded than pseudodemencia is a syndrome with important psycho-social factors; occasionally a low magnitude cerebral pathology may act as a triggering factor.