Calidad de vida relacionada con la salud en cuidadores de pacientes con enfermedad de Alzheimer / Health-related quality of life in caregivers of patients with Alzheimer's disease

Introducción: Los cuidadores de pacientes con enfermedad de Alzheimer (EA) tienen un deterioro de su calidad de vida relacionada con la salud (CVRS). La CVRS es una medida de resultado centrada en el usuario cada vez más usada. Evaluamos de forma longitudinal la CVRS en cuidadores de pacientes con EA, antes y después, durante un periodo de 12 meses. Métodos: Se incluyó en el estudio a 97 pacientes diagnosticados de EA según criterios NINCDS-ADRDA (Instituto Nacional de Trastornos Neurológicos y de la Comunicación y Accidente Cerebrovascular y Asociación de Enfermedad de Alzheimer y Trastornos relacionados) y sus respectivos 97 cuidadores principales. Se analizaron datos sociodemográficas de ambos, clínicos de los pacientes, y datos en relación con el cuidado en la visita basal. La CVRS se midió con el cuestionario SF-36 en la visita basal y a los 12 meses. Resultados: Las 8 dimensiones de la escala SF-36 en los cuidadores principales empeoraron de forma significativa a los 12 meses, salvo las dimensiones «Función física» y «Función social», que lo hicieron de forma no significativa. Las puntuaciones en la visita basal fueron menores que las correspondientes a la población general. La dimensión que presentó peor puntuación fue «Vitalidad». Conclusiones: La CVRS en cuidadores de pacientes con EA empeora a lo largo de la evolución de esta y es peor que la de la población general, para su edad y género (AU)

Introduction: Informal caregivers of patients with Alzheimer's disease (AD) have a poor health-related quality of life (HRQOL). HRQOL is an increasingly common user-focused outcome measure. We have evaluated HRQOL longitudinally in caregivers of AD patients at baseline and at 12 months. Methods: Ninety-seven patients diagnosed with AD according to the NINCDS-ADRDA (National Institute of Neurological and Communicative Disorders and Stroke, and Alzheimer's Disease and Related Disorders Association) and their 97 respective primary caregivers were included in the study. We analysed the following data at the baseline visit: sociodemographic data of both patients and carers, patients’ clinical variables, and data related to the healthcare provided to patients by carers. HRQOL of caregivers was measured with the SF-36 questionnaire at baseline and 12 months later. Results: At 12 months, primary caregivers scored lower in the 8 subscales of the SF-36 questionnaire; differences were statistically significant in all dimensions except for ‘physical function’ and ‘social function’. Baseline scores in our sample were lower than those of the general population. ‘Vitality’ is the dimension that presented the lowest scores. Conclusion: HRQOL in caregivers of patients with Alzheimer's disease deteriorates over time and is poorer than that of the age- and sex-matched general population (AU)

Health-related quality of life in caregivers of patients with Alzheimer's disease. / Calidad de vida relacionada con la salud en cuidadores de pacientes con enfermedad de Alzheimer.

INTRODUCTION: Informal caregivers of patients with Alzheimer's disease (AD) have a poor health-related quality of life (HRQOL). HRQOL is an increasingly common user-focused outcome measure. We have evaluated HRQOL longitudinally in caregivers of AD patients at baseline and at 12 months. METHODS: Ninety-seven patients diagnosed with AD according to the NINCDS-ADRDA (National Institute of Neurological and Communicative Disorders and Stroke, and Alzheimer's Disease and Related Disorders Association) and their 97 respective primary caregivers were included in the study. We analysed the following data at the baseline visit: sociodemographic data of both patients and carers, patients' clinical variables, and data related to the healthcare provided to patients by carers. HRQOL of caregivers was measured with the SF-36 questionnaire at baseline and 12 months later. RESULTS: At 12 months, primary caregivers scored lower in the 8 subscales of the SF-36 questionnaire; differences were statistically significant in all dimensions except for 'physical function' and 'social function'. Baseline scores in our sample were lower than those of the general population. 'Vitality' is the dimension that presented the lowest scores. CONCLUSION: HRQOL in caregivers of patients with Alzheimer's disease deteriorates over time and is poorer than that of the age- and sex-matched general population.

Perfil de consumo de antiepilépticos en los pacientes epilépticos del Área Oeste de Málaga / Consumption patterns of antiepileptic drugs among epileptic patients in the Western Málaga district

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Síndrome «painless limbs and moving extremities». Aportación de 2 casos y revisión de la literatura / Painless limbs and moving extremities syndrome. Presentation of 2 cases and a literature review

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Parkinsonismo-distonía unilateral sensible a levodopa por lesión traumática de la sustancia negra / Levodopa-responsive parkinsonism-dystonia due to a traumatic injury of the substantia nigra

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Síndrome de hiperinsulinismo-hiperamoniemia y epilepsia mioclónica grave de la infancia / Hyperinsulinism and hyperammonaemia syndrome and severe myoclonic epilepsy of infancy

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Réplica. Ausencias y ‘pseudoausencias’ frontales / Frontal absences and ‘pseudo-absences’. Reply

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Deterioro cognitivo rápidamente progresivo como inicio de un tumor bitalámico / Rapidly progressing cognitive impairment as the presenting symptom of a bithalamic tumour

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Ausencias frontales: presentación de un caso / Frontal absences: a case report

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Esclerosis lateral amiotrófica y anticuerpos anti-CV2. ¿Asociación paraneoplásica? / Amyotrophic lateral sclerosis and anti-CV2 antibodies. Paraneoplastic association?

Introducción. La esclerosis lateral amiotrófica (ELA) es una forma de enfermedad de motoneurona que afecta primariamente a las motoneuronas superior e inferior. Es de etiología desconocida, curso progresivo y a menudo fatal. Muy ocasionalmente se ha descrito su aparición como síndrome paraneoplásico (SPN). Determinados patrones clínicos de enfermedad de motoneurona sugieren esta asociación. Los anti-CV2 son un tipo de anticuerpo onconeuronal, asociado invariablemente a tumor, y descritos en distintos SPN como encefalomielitis paraneoplásica, degeneración cerebelosa y neuropatía periférica.Caso clínico. Varón de 29 años con criterios de ELA probable. Por su debut atípico (edad del paciente) se solicitó anticuerpos onconeuronales, detectándose anti-CV2+. Tras 3 años de seguimiento y búsqueda tumoral exhaustiva, con progresión de la enfermedad, no hay evidencia en la actualidad de cáncer asociado.Discusión. El estudio de la enfermedad de motoneurona/ ELA como síndrome paraneoplásico, por su rareza, ha sido motivo de revisiones al objeto de verificar dicha relación. Ante una ELA debemos descartar asociación a tumor cuando se trate de una presentación precoz (< 30 años) o tardía (>70 años), cuando asocie otros síntomas/signos neurológicos (síntomas sensitivos, ataxia, etc.), ante la presencia de anticuerpos anti-Hu u otros y/o ante la presencia de paraproteinemia y/o pleocitosis-hiperproteinorraquia en LCR. Presentamos un caso de ELA probable con anti-CV2+, sin evidencia de cáncer subyacente. Tras una búsqueda bibliográfica extensa no hemos encontrado descrita esta asociación. Tampoco tenemos conocimiento de la existencia de anticuerpos anti-CV2 fuera del contexto tumoral, por lo que pensamos que nuestro paciente, probablemente, presente una neoplasia oculta

Introduction. Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease that primarily affects upper and lower motor neurons. Its etiology is unknown, it has a progressive course and is often fatal. Very rarely, its appearance as paraneoplastic syndrome (PNS) has been described. Certain clinical patterns of motor neuron disease suggest this association. The anti-CV2 are a type of onconeuronal antibody, invariably associated to tumor and described in different PNSs as paraneoplastic encephalomyelitis, cerebellar degeneration and peripheral neuropathy.Clinical case. 29 years old male with criteria of probably ALS. Due to his atypical onset (patient's age), onconeuronal antibodies were requested, detecting anti- CV2+. After three years of follow-up and exhaustive search for tumors, with progression of the disease, there is currently no evidence of associated cancer.Discussion. The study of the motor neuron/ALS disease as paraneoplastic syndrome, due to its rareness, has led to reviews in order to verify this relationship. When ALS exists, we should rule out association to tumor when the presentation is early (< 30 years) or late (> 70 years), when it is associated to other neurological symptoms/signs (sensory symptoms, ataxia, etc.), when anti-Hu antibodies or others are present and/or when there is paraproteinemia and/or pleocytosis-high protein levels in cerebral spinal fluid. We present a case of probable ALS with anti-CV2+, with no evidence of underlying cancer. After an extensive bibliographic search, we have found no description of this association. We also have no knowledge of the existence of anti-CV2 antibodies outside of the tumor context, so that we believe that our patient probably has an occult neoplasm

[Amyotrophic lateral sclerosis and anti-CV2 antibodies. Paraneoplastic association?]. / Esclerosis lateral amiotrófica y anticuerpos anti-CV2. ¿Asociación paraneoplásica?

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a form of motor neuron disease that primarily affects upper and lower motor neurons. Its etiology is unknown, it has a progressive course and is often fatal. Very rarely, its appearance as paraneoplastic syndrome (PNS) has been described. Certain clinical patterns of motor neuron disease suggest this association. The anti-CV2 are a type of onconeuronal antibody, invariably associated to tumor and described in different PNSs as paraneoplastic encephalomyelitis, cerebellar degeneration and peripheral neuropathy. CLINICAL CASE: 29 years old male with criteria of probably ALS. Due to his atypical onset (patient's age), onconeuronal antibodies were requested, detecting anti- CV2+. After three years of follow-up and exhaustive search for tumors, with progression of the disease, there is currently no evidence of associated cancer. DISCUSSION: The study of the motor neuron/ALS disease as paraneoplastic syndrome, due to its rareness, has led to reviews in order to verify this relationship. When ALS exists, we should rule out association to tumor when the presentation is early (< 30 years) or late (> 70 years), when it is associated to other neurological symptoms/signs (sensory symptoms, ataxia, etc.), when anti-Hu antibodies or others are present and/or when there is paraproteinemia and/or pleocytosis-high protein levels in cerebral spinal fluid. We present a case of probable ALS with anti-CV2+, with no evidence of underlying cancer. After an extensive bibliographic search, we have found no description of this association. We also have no knowledge of the existence of anti-CV2 antibodies outside of the tumor context, so that we believe that our patient probably has an occult neoplasm.

[Spontaneous intracranial hypotension]. / Hipotensión intracraneal espontánea.

INTRODUCTION AND CLINICAL CASE: A 38 year old woman, with no previous history of trauma, presented complaining of interscapular pain followed by pulsating headache clearly related to posture, alleviated on lying down and worse on standing up. Subsequently, she also complained of diplopia. On examination there was paresia of the left sixth cranial nerve. Low opening pressure on lumbar puncture confirmed the presence of intracranial hypotension. The protein level of the cerebrospinal fluid was slightly raised. On CT the cortical sulci and small ventricles had disappeared. Cerebral MR (without gadolinium) showed marked diffuse meningeal hyper-intensity and apparent absence of the basal cisterni. Isotopic cisternography showed a pattern compatible with hypotension, without signs of fistulas. On spinal MR no spinal meningeal defects were seen. With conservative treatment the patient improved in a few days and the headache and diplopia disappeared. The absence of traumatism or spinal operations mean that the hypotension may be considered to be spontaneous. We discuss the CSF, neuroimaging and cisternography findings characteristic of the spontaneous intracranial hypotension syndrome. CONCLUSION: Unawareness of this syndrome, the particular neuroimaging changes and the usual CSF anomalies may lead to confusion over diagnosis, leading to the use of invasive techniques unsuitable for a condition which often has a good prognosis and in which the symptoms resolve in a few days or weeks with conservative treatment.