Sudden death related to sexual activity: A multicenter study based on forensic autopsies (2010-2021).

INTRODUCTION AND OBJECTIVES: To investigate the epidemiological characteristics, clinic-pathological findings and recent use of substances of abuse and prescribed drugs in sexual activity-related sudden death (SArSD). METHODS: Multicenter population-based study on forensic autopsies conducted in 27 provinces of Spain over 12 years (2010-2021). RESULTS: Out of 18046 autopsied natural deaths, 64 cases (0.35 %) of SArSD were investigated (87 % males). Women were younger than males (50.5 ± 13.4 years vs 37.2 ± 14.2; p = 0.017). Sudden cardiac deaths (SCD) accounted for 87 % of cases. Ischemic heart disease was the predominant pathology (58 %), mainly affecting men ≥ 36 years of age. Cerebral haemorrhage (8 %) and asthma (5 %) were the leading non-cardiac causes. In young adults, SADS (36 %) and asthma (27 %) were the main causes The disease responsible of SCD was diagnosed in life in 7 subjects. In 64 % there were cardiovascular risk factors, mainly obesity. Toxicological analysis detected illicit drugs (23 %), mainly cocaine, medications for erectile dysfunction (9 %), and ethanol ≥ 0.5 g/L (8 %). Deaths occurred usually in the context of heterosexual intercourse and during or immediately after sexual activity. The most common location was at home (63 %). In 12 men the sexual partner was a sex worker. CONCLUSIONS: SArSD has a low incidence in the general population affecting middle-aged males during intercourse with a heterosexual partner. It is of cardiovascular origin, mainly due to ischemic heart disease that frequently remained silent during life. There is a frequent association with obesity, use of cocaine (and, to a lesser extent, medications for erectile dysfunction) and performing unconventional sexual practices. Forensic investigation is useful for developing prevention strategies.

Characterisation of spinophilin immunoreactivity in postmortem human brain homogenates.

Spinophilin is a multifunctional scaffold protein that regulates the formation and function of dendritic spines and plays a role in neuronal migration. The distinct roles of spinophilin depend on its localization and the direct interaction with other proteins, which may target spinophilin to specific locations within the cell. Several studies suggest a role of spinophilin in the pathophysiology of neurological or psychiatric diseases. However, the majority have been performed in animals or cultured cells. Thus, the aim of the present study was to characterise the regional and subcellular expression of spinophilin immunoreactivity by western blot in postmortem human brain. Two specific immunoreactive bands for spinophilin were observed: an intense band migrating at around 120kDa, which seems to correspond to the apparent molecular weight of spinophilin described by other authors, and a less intense band of around 95kDa. This second form seems to be a proteolysis or cleavage product of the ~120kDa spinophilin. Interestingly, the subcellular distribution of both bands was different. In membrane fraction, the ~120kDa spinophilin band was the most abundant, whereas in cytosol it was the ~95kDa form. Furthermore, a different regional distribution for ~120kDa spinophilin band was observed, with the highest expression in prefrontal cortex, followed by hippocampus and cerebellum, and the lowest in caudate nucleus. Altogether, these results constitute a useful reference for future studies of spinophilin in pathological and non-pathological human brain tissues.

[Alimentary and lifestyle changes as a strategy in the prevention of metabolic syndrome and diabetes mellitus type 2: milestones and perspectives]. / Cambios alimentarios y de estilo de vida como estrategia en la prevención del síndrome metabólico y la diabetes mellitus tipo 2: hitos y perspectivas.

A high caloric intake in today's nutrition and a sedentary lifestyle are the main causes of the notable increase in obesity in our society. In turn, this results in an increase in associated pathologies, such as metabolic syndrome and diabetes type 2. In the present work we review most recent studies and programs, which are significant due to their sample size and geographical diversity. It clearly shows that changes in alimentation and lifestyles are an effective instrument for combatting or delaying the onset of these diseases. In this sense, prevention is also key to avoiding serious consequences related to diabetes and metabolic syndrome, which can affect the life of the population.

Cambios alimentarios y de estilo de vida como estrategia en la prevención del síndrome metabólico y la diabetes mellitus tipo 2: hitos y perspectivas / Alimentary and lifestyle changes as a strategy in the prevention of metabolic syndrome and diabetes mellitus type 2: milestones and perspectives

El elevado aporte calórico de la alimentación actual y el sedentarismo, son los principales causantes del notable incremento de la obesidad en nuestra sociedad. A su vez, esto conlleva un aumento de las patologías asociadas a ella, como pueden ser el síndrome metabólico y la diabetes tipo 2. En el presente trabajo, han sido revisados los estudios y programas más recientes y significativos en cuanto a su tamaño muestral y a su diversidad geográfica, poniendo de manifiesto que los cambios en la alimentación y en los estilos de vida, son un instrumento efectivo para combatir o retrasar la aparición de estas enfermedades. En este sentido, la prevención también es clave para evitar las graves consecuencias, relacionadas con la diabetes y el síndrome metabólico, que pueden afectar a la calidad de vida de la población (AU)

A high caloric intake in today’s nutrition and a sedentary lifestyle are the main causes of the notable increase in obesity in our society. In turn, this results in an increase in associated pathologies, such as metabolic syndrome and diabetes type 2. In the present work we review most recent studies and programs, which are significant due to their sample size and geographical diversity. It clearly shows that changes in alimentation and lifestyles are an effective instrument for combatting or delaying the onset of these diseases. In this sense, prevention is also key to avoiding serious consequences related to diabetes and metabolic syndrome, which can affect the life of the population (AU)

Brain RGS4 and RGS10 protein expression in schizophrenia and depression. Effect of drug treatment.

RATIONALE: Regulator of G-protein signaling (RGS) proteins, RGS4 and RGS10, may be involved in the pathophysiology of schizophrenia. RGS4 has attracted special interest since the reports of genetic association between SNPs in RGS4 and schizophrenia. However, there is no information about the subcellular distribution of RGS4 and RGS10 proteins in psychiatric disorders. OBJECTIVES: Plasma membrane RGS4 and cytosolic RGS10 protein immunoreactivity in prefrontal cortex from schizophrenic subjects (n = 25), non-diagnosed suicides (n = 13), and control subjects (n = 35), matched by age, gender, and postmortem delay, was analyzed by western blot. A second group of depressed subjects (n = 25) and control subjects (n = 25) was evaluated. The effect of the antipsychotic or antidepressant treatments was also assessed. RESULTS: No significant differences in plasma membrane RGS4 and cytosolic RGS10 protein expression were observed between schizophrenic subjects, non-diagnosed suicides, and control subjects. However, RGS4 immunoreactivity was significantly higher (Δ = 33 ± 10 %, p < 0.05) in the antipsychotic-treated subgroup (n = 12) than in the antipsychotic-free subgroup (n = 13). Immunodensities of plasma membrane RGS4 and cytosolic RGS10 proteins did not differ between depressed and matched control subjects. CONCLUSIONS: Expression of RGS4 and RGS10 proteins at their predominant subcellular location was studied in the postmortem brain of subjects with psychiatric disorders. The results suggest unaltered membrane RGS4 and cytosolic RGS10 proteins levels in schizophrenia and major depression. Antipsychotic treatment seems to increase membrane RGS4 immunoreactivity. Further studies are needed to elucidate RGS4 and RGS10 functional status.

Tumor disembrioplástico neuroepitelial: un caso de muerte súbita en un paciente joven epiléptico / Dysembryoplastic neuroepithelial tumor: a case of sudden death in a young epileptic patient

Las muertes súbitas por tumores intracraneales no diagnosticados en vida son muy raras. En el presente artículo se presenta el caso de un paciente joven que es hallado muerto durante el sueño. Como único antecedente de interés destaca la presencia desde la infancia de crisis epilépticas de difícil control medicamentoso. Las pruebas de imagen no habían mostrado en vida alteraciones reseñables. En la autopsia se evidencia un tumor de 0,5 x 1 cm en la corteza cerebral del lóbulo parietal izquierdo, sin efecto masa. A la vista de los hallazgos del estudio histopatológico e inmunohistoquímico se realiza el diagnóstico de tumor disembrioplástico neuroepitelial (TDN) simple. Se establece como causa de la muerte el trastorno epiléptico orgánico secundario al tumor. El TDN es un tumor cerebral de muy baja frecuencia (0,2-1% de los tumores cerebrales) y normalmente se asocia a crisis epilépticas fármaco-resistentes. Este caso demuestra que ante una muerte súbita e inexplicada asociada a epilepsia es fundamental la realización de una autopsia médico-legal a fin de reconocer la causa y los factores de riesgo relacionados con la epilepsia y la muerte (AU)

Sudden deaths due to intracranial tumors that have not been diagnosed in life are very rare. In this paper we present the case of a young patient who died during sleep. The only medical antecedent was drug-resistant epilepsy suffered since childhood. Neuroimage techniques did not show any alteration prior to death. The autopsy showed in the cerebral cortex of the left parietal lobe the presence of a tumor of 0.5 x 1 cm without mass effect. In view of the findings of histopathological and immunohistochemical studies a diagnosis of dysembryoplastic neuroepithelial tumor (DNT) simple was made. The cause of sudden death was attributed to an organic seizure disorder secondary to the tumor. DNT is very infrequent (0.2-1% of brain tumors) and it is usually associated with drug-resistant seizures. This case demonstrates that in the investigation of sudden unexplained death associated to epilepsy is crucial to perform a medico-legal autopsy in order to confirm the cause and the risk factors related to epilepsy and death (AU)

Embolismo pulmonar biliar no traumático fatal tras colecistitis litiásica / No disponible

No disponible

Postprandial insulin response and mitochondrial oxidation in obese men nutritionally treated to lose weight.

Obesity, hyperglycemia, and insulin resistance have been associated to an oxidative mitochondrial dysfunction. The aim of this research was to evaluate the relation between carbohydrate metabolism and mitochondrial oxidation, as affected by the weight status and the weight loss induced by a calorie-restricted diet. Lean control men (BMI<25 kg/m2, n = 6) and obese men (BMI>30 kg/m2, n = 14), who were characterized as insulin resistant (n = 6) or insulin sensitive (n = 8) based on HOMA index values, participated in the trial. Plasma insulin levels and mitochondrial oxidation estimated by the 2-keto(1-13C)isocaproate breath test, were measured after ingestion of a test meal during 3 h. Obese subjects repeated the breath test protocol after a 10-week caloric restriction diet to lose weight. Postprandial insulin secretion tended to be marginally higher (P = 0.059) in both obese groups than in controls, while the rate of postprandial mitochondrial oxidation was markedly decreased (P = 0.019) in the obese subjects as compared with lean individuals. The nutritionally induced weight loss produced a rise in the postprandial oxidative process in volunteers initially considered as insulin resistant (P = 0.036), while no statistical differences in the insulin-sensitive obese (P = 0.241) were found. Interestingly, the percentage of oxidized tracer was inversely related to postprandial insulin secretion (r = -0.56; P = 0.001). In conclusion, these results support the hypothetized relation between carbohydrate metabolism and mitochondrial oxidation at a postprandial state in obese subjects, raising interest about mitochondria stimulation as a target in the therapy of obesity.

[In vivo study of mitochondrial oxidation in obese patients treated by means of calorie restriction]. / Estudio in vivo de la oxidación mitocondrial en pacientes obesos tratados mediante restricción calórica.

The energy restriction is the most common nutritional approach to treat obesity, whose efficiency depends on oxidative response against changes in body weight. In that context, the aim of the present work was to in vivo examine the mitochondrial oxidation of obese volunteers by the 2-keto[1-(13)C]isocaproate breath test, before and after weight loss. Thirty-two volunteers (men and women) participated: 16 controls (body mass index: 19.0-27.0 kg/m2), and 16 obese (body mass index: 30.0-41.6 kg/m2) who followed a caloric restriction program for 10 weeks (-500 kcal). Before and after dieting, the 2-keto[1-(13)C]isocaproate breath test was performed by ingestion of 1 mg/kg tracer and 20 mg/kg L-leucine, dissolved in 200 ml orange juice. Breath samples were recovered at baseline and at 10 min intervals for 2 h after ingestion. The 13C-enrichment in breath was measured by isotope ratio mass spectrometry and the percentage of mitochondrial oxidation of tracer (%13C) was calculated. The percentage of oxidized tracer marginally tended to be lower in obese than in controls (25.1 +/- 5.5%, vs 27.5 +/- 4.0%, p = 0.175). After the intervention, the mean of weight loss was -7.8% +/- 3% p < 0.001), and the mitocondrial oxidation of the tracer statistically increased (25.1 +/- 5.5% vs 34.3 +/- 5.2%, p < 0.001). In fact, the body weight and the percentage of oxidized 2-keto[1-(13)C]isocaproate were inversely related (r = -0.34, p = 0.018). Thus, the 2-keto[1-(13)C]isocaproate in vivo showed the mitochondrial adaptation of obese volunteers treated by caloric-restriction intake and provided new information about the weight loss process induced by an hypocaloric diet.

Daily ingestion of fermented milk containing Lactobacillus casei DN114001 improves innate-defense capacity in healthy middle-aged people.

Different lactic acid bacteria have often been administered as a dietary means to enhance immune system activity. Based on this statement, the aim of the current work was to test the effects of a Lactobacillus casei DN114001 fermented milk consumption on the immune response capacity in middle-age volunteers. Forty-five healthy volunteers, 24 women and 21 men (aged: 51-58 years), were randomized into two groups to receive three cups per day of a L. casei DN114001 (10(8)-10(10) ufc/g) fermented milk (n = 23), or placebo (n = 22), during an 8-week period. Measurements were performed before (day 0), and after the nutritional intervention (day 56). After the trial, no changes in immune cell proportions were detected, but the probiotic-treated group increased oxidative burst capacity of monocytes (probiotic group: p = 0.029; placebo group: p = 0.625), as well as NK cells tumoricidal activity (probiotic group: p = 0.023; placebo group: p = 0.125). Results showed that daily intake of fermented milk containing Lactobacillus casei DN114001 could have a positive effect in modulating the innate immune defense in healthy-middle-age people.

Monocyte function in healthy middle-aged people receiving fermented milk containing Lactobacillus casei.

BACKGROUND: Lactic acid bacteria have been suggested as a dietary strategy to enhance immune system activity. OBJECTIVE: The aim of the current work was to test the effects of a Lactobacillus casei fermented milk consumption on monocyte activity of middle-aged volunteers. DESIGN: Forty-five healthy volunteers, 24 women and 21 men (aged: 51 - 58 years), were randomized in two groups to receive three cups per day of a fermented milk containing L. casei DN114001 (108 - 1010/g) (n = 23), or placebo (n = 22), during 8 weeks. White blood cell count and the oxidative burst capacity of monocytes and granulocytes were examined with a FACScalibur. Measurements were performed at baseline and after the nutritional intervention, at day fifty-six. RESULTS: After the trial, no changes in immune cell proportions were detected in both groups, as well as in monocyte activity after the placebo consumption (p = 0.625). However, volunteers included in the probiotic-treated group increased (p = 0.029) their oxidative burst capacity of monocytes, and this increment inversely and significantly correlated with the intensity registered at baseline (r = -0.653, p = 0.004). CONCLUSIONS: Results showed that daily intake of fermented milk containing Lactobacillus casei was able to module the oxidative burst capacity of monocyte subset in healthy middle-aged people, particularly in subjects with lower initial levels. Thus, this nutritional strategy could be considered to maintain immune competence in ageing.

Síndrome metabólico, resistencia a la insulina y metabolismo tisular / Metabolic syndrome, insulin resistance and tissue metabolism

Un metabólico recibe una gran atención sanitaria debido al elevado número de personas que lo sufren y que presentan un alto riesgo de padecer diversas complicaciones metabólicas (diabetes mellitus tipo 2, enfermedades cardiovasculares, etc.).Los criterios de diagnóstico, descritos por diferentes comités y organismos, normalmente son: alteración de la regulación de la glucemia, resistencia a la insulina, obesidad abdominal, alteración del metabolismo lipídico e hipertensión arterial. La prevalencia del síndrome metabólico, según el Grupo Europeo para el estudio de la Resistencia a la Insulina, se ha estimado para la población europea caucásica en un 16 por ciento. Asimismo, existen varias técnicas para determinar la resistencia a la insulina: técnica del clamp, el modelo mínimo del metabolismo de la glucosa y el test de supresión de la insulina. Por otra parte, el modelo HOMA es un modelo matemático sencillo aplicable a estudios epidemiológicos. Los individuos con este síndrome presentan hiperinsulinismo, lo que determina adaptaciones y alteraciones que afectan al metabolismo de la glucosa y ácidos grasos en diferentes órganos como el tejido adiposo, el hígado y el músculo esquelético. (AU)

[Sudden unexpected death in epilepsy in children and adolescents]. / Muerte súbita inesperada en epilepsia en niños y jóvenes.

INTRODUCTION: Sudden unexpected death in epilepsy (SUDEP) is an important cause of death in young epileptic patients. However, it is relatively unknown to neurologists. OBJECTIVE: To analyze the frequency and clinicopathological characteristics of SUDEP as a cause of sudden death (SD) in young persons. PATIENTS AND METHODS: An observational study of all SD due to SUDEP in persons aged 1 35 years occurring between 1991 and 1998 in Bizkaia, in whom medico legal autopsy was done. In all cases a full autopsy was done and the previous clinical history was investigated. RESULTS: Eight of the 107 cases of SD (7.5%) were due to SUDEP. Five were aged between 25 and 29 years and in this age group it was the commonest cause of death (22%). Six had been diagnosed as having idiopathic epilepsy and two as having posttraumatic epilepsy. Generalized convulsions occurred in 75%. Four patients died when they were alone. Two patients had epileptic seizures hours before their death. At necropsy no structural changes were seen in the brain except in the two patients with posttraumatic epilepsy. Pulmonary oedema was a frequent finding. In 50% of the dead patients, chemico toxicological study showed there to be no trace of the drugs they had been prescribed. CONCLUSIONS: Although SD is rare, SUDEP is an important cause of mortality in young epileptics. Violent death in relation to epilepsy causes problems in forensic medicine. Although the mechanisms of SUDEP are not clear, it would seem necessary to increase medical control of this disorder, especially in young people.

Muerte súbita inesperada en epilepsia en niños y jóvenes / Sudden unexpected death in epilepsy in children and adolescents

Introducción. La muerte súbita inesperada en la epilepsia (MSIEP) es una causa importante de mortalidad en pacientes epilépticos jóvenes. Sin embargo, su existencia es escasamente conocida por los neurólogos. Objetivo. Analizar la frecuencia y características clinicopatológicas de la MSIEP como causa de muerte súbita (MS) en jóvenes. Pacientes y métodos. Estudio observacional de todas las MS ocurridas por MSIEP en personas de 135 años desde 1991 a 1998 en Bizkaia que fueron objeto de autopsia médico-legal. En cada caso se efectuó una autopsia completa y se investigaron los antecedentes patológicos. Resultados. Ocho de los 107 casos de MS (7,5 por ciento) fueron debidos a MSIEP. Cinco tenían entre 25 y 29 años, siendo en este grupo quinquenal la causa más frecuente (22 por ciento). Seis estaban diagnosticados de epilepsia idiopática, y 2 de postraumática. Un 75 por ciento tenían crisis generalizadas. En 4 la muerte no fue presenciada. Dos pacientes sufrieron una crisis epiléptica horas antes del fallecimiento. El estudio necrópsico no mostró alteraciones estructurales del encéfalo, salvo en los 2 pacientes con epilepsia postraumática. Hubo una alta frecuencia de edema pulmonar. En el 50 por ciento el estudio químico-toxicológico fue negativo para los fármacos antiepilépticos prescritos. Conclusiones. Dentro de la rareza de la MS, la MSIEP constituye una causa importante de mortalidad en epilépticos jóvenes. Para la Medicina Forense la muerte de carácter violento relacionada con la epilepsia plantea mayor conflictividad. Aunque los mecanismos de la MSIEP no son bien conocidos, parece necesario reforzar el control médico de esta enfermedad especialmente en la juventud (AU)

[Sudden death caused by atheromatous coronary disease in the young]. / Muerte súbita por enfermedad ateromatosa coronaria en jóvenes.

INTRODUCTION AND OBJECTIVES: Atheromatous coronary disease (ACD) is the most frequent cause of sudden death (SD) in adults. Few studies have focused in SD due to ACD in those patients under 35 years old. The aim of this study is to analyze the incidence, and clinical and pathological characteristics of ACD as a cause of death in young people. MATERIAL AND METHODS: We reviewed all SD cases in people from 1-35 years old which occurred in Bizkaia and in which there was a legal-medical autopsy from 1991 to 1998. A complete autopsy was performed in each case. SD due to ACD were analyzed, including pathological antecedents. RESULTS: 19 out of 107 SD (18%) occurred by ACD. All of them were males from 27 to 35 years of age. According to the male population from 30 to 35 years, the incidence was 3.7/100,000/year. In two patients ACD was diagnosed before death. Coronary risk factors were obtained in 10 cases and prodromal symptoms were described in 5 (chest pain in 4). In 79% death occurred during routine activity. None of the 19 patients arrived alive to hospital. In 6 cases multiple coronary disease was observed; coronary thrombosis in 8; recent acute ischemic myocardial necrosis in 4 and old ischemic damage in 7. 18 cases showed cardiac hypertrophy. CONCLUSIONS: ACD is an important cause of SD in young males, frequently being the first manifestation of the disease. As identification of groups at risk is so difficult and death occurs so quickly, primary prevention of ACD, rapid intervention of emergency services and educational programs in cardiopulmonar resuscitation for normal population are fundamental in reducing the mortality.

[Incidence and causes of sudden death in persons less than 36 years of age]. / Incidencia y causas de muerte súbita en menores de 36 años.

BACKGROUND: Epidemiological, clinical and pathological study of sudden death in children and young adults under 36 years old. SUBJECTS, MATERIAL AND METHOD: Sudden deaths between 1 and 35 years that occurred in Bizkaia (North of Spain) from 1991 to 1998 were analyzed. In all cases a complete forensic autopsy including histopathological, toxicological and occasionally microbiological studies were performed. Also, pathological antecedents were investigated. RESULTS: 107 sudden death cases between 1 and 35 years occurred, being the mortality rate of 2.4/100,000/year. Males were threefold at risk of sudden death than females. Mortality rate was higher in youngs than children. 4.5% of natural deaths were sudden deaths.43% were sudden cardiac deaths, and atheromatous coronary disease was the most frequent over 30 years. Myocardial diseases and conduction system abnormalities were common between 15 and 29 years. 39% were extracardiac sudden deaths. Infections were frequent in children, and epilepsy and asthma between 15 and 29 years. Intracraneal hemorrhage was also frequent. In 19 cases (18%), specially in children, the cause of death was unexplained. Cause of death was different according to gender and age. CONCLUSIONS: Sudden death is infrequent in children and youngs, and causes are numerous. This study supports the importance of complete forensic autopsies in order to encourage epidemiological and preventive studies on sudden death.

Sudden unexpected non-violent death between 1 and 19 years in north Spain.

AIMS: To study the epidemiological, clinical, and pathological characteristics of sudden unexpected non-violent deaths between 1 and 19 years. METHODS: Population based observational study of all sudden unexpected non-violent deaths between 1 and 19 years, from 1990 to 1997, in a north Spain county (Bizkaia). In each case, clinical information and circumstances of death were obtained and a complete forensic autopsy was performed. RESULTS: There were 34 sudden unexpected non-violent deaths. The mortality rate was 1.7 per 100 000 persons per year (representing 9% of the mortality rate of all non-violent deaths). In 10 cases the cause of death was cardiac, in 13 cases extracardiac, and 11 cases were unexplained. In 17 cases there were no pathological antecedents and in nine cases death occurred in patients with known disorders. Syncopes prior to death were present in five cases. Exercise related deaths occurred in seven cases (mainly associated with cardiac causes). Eight cases were "death in bed".

[Sudden death due to an anomalous origin of the left anterior descending coronary artery from the pulmonary trunk in a 9-year-old boy]. / Muerte súbita debida a origen anómalo de la coronaria izquierda descendente anterior desde el tronco pulmonar en un niño de 9 años.

A 9-year-old healthy boy is presented, who complained of dizziness and to being exhausted after climbing numerous stairs, collapsing immediately after. Advanced resuscitation efforts were unsuccessful. At autopsy an isolated congenital anomaly in the origin of the left anterior descending coronary artery from the pulmonary trunk was found, with the origin of the right and left circumflex coronary arteries from the aorta, accompanied by with cardiac hypertrophy and chronic ischemic changes in the myocardium irrigated by the anterior descending artery. This represents a very infrequent variant that allows a longer survival and less functional repercussion if compared with those cases in which the main left coronary is originated from the pulmonary trunk.

[Arrhythmogenic cardiomyopathy as cause of sudden death in Spain. Report of 21 cases]. / Miocardiopatía arritmogénica como causa de muerte súbita en España. Presentación de 21 casos.

INTRODUCTION AND OBJECTIVES: Arrhythmogenic cardiomyopathy is a myocardial disease of unknown origin characterized by progressive replacement of right and/or left ventricular myocardium by fibrofatty tissue. Young adult people are more frequently affected and symptoms include palpitations, syncopes or sudden death. The objective of this study is to assess the frequency and characteristics of arrhythmogenic cardiomyopathy as a cause of sudden death in a series of cases in Spain. MATERIAL AND METHODS: Among the sudden death cases studied at the Toxicology Institute of Madrid between 1991 and 1997, those with arrhythmogenic cardiomyopathy diagnosis were analysed. RESULTS: 21 cases were diagnosed (0.62% of all sudden deaths and 6.8% of sudden deaths under 35 years). There were 20 males and one female (mean age 24.5 +/- 9.37 years); eleven (52.3%) died during sport activity and 8 (38%) had previous exercise related symptoms. In only two cases other members of the family had died suddenly and only one had been diagnosed in life. Biventricular involvement was observed in 13 cases; isolated involvement of the left ventricle in 4, and isolated involvement of the right ventricle in 4. CONCLUSIONS: Arrhythmogenic cardiomyopathy is a relatively frequent cause of sudden death in the young population in Spain. It is very often the first manifestation of the disease. The myocardial involvement is more frequently biventricular than isolated in the right ventricle. The diagnosis could be difficult for cardiologists, pathologists and forensic doctors.