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.

[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.

Mechanism of syncope in patients with bundle branch block and negative electrophysiological test.

BACKGROUND: In patients with syncope and bundle branch block (BBB), syncope is suspected to be attributable to a paroxysmal atrioventricular (AV) block, but little is known of its mechanism when electrophysiological study is negative. METHODS AND RESULTS: We applied an implantable loop recorder in 52 patients with BBB and negative conventional workup. During a follow-up of 3 to 15 months, syncope recurred in 22 patients (42%), the event being documented in 19 patients after a median of 48 days. The most frequent finding, recorded in 17 patients, was one or more prolonged asystolic pause mainly attributable to AV block; the remaining 2 patients had normal sinus rhythm or sinus tachycardia. The onset of the bradycardic episodes was always sudden but was sometimes preceded by ventricular premature beats. The median duration of the arrhythmic event was 47 seconds. An additional 3 patients developed nonsyncopal persistent III-degree AV block, and 2 patients had presyncope attributable to AV block with asystole. No patients suffered injury attributable to syncopal relapse. CONCLUSIONS: In patients with BBB and negative electrophysiological study, most syncopal recurrences have a homogeneous mechanism that is characterized by prolonged asystolic pauses, mainly attributable to sudden-onset paroxysmal AV block.

[Recurrent spontaneous carotid dissection]. / Disección carotídea espontánea recurrente.

INTRODUCTION: Arterial dissection is the cause of 20% of the stroke occurring in adults under the age of 45. The existence of recurrence has been discussed in recent studies, and the overall frequency estimated as 4% to 8%, with a risk of 1% per year. The course of the condition is usually oligosymptomatic, so that a high index of suspicion is necessary for diagnosis to be made. We consider that different connective tissue disorders and anomalies of the vascular wall predispose to dissection. It would seen reasonable to think that these same anomalies may lead to recurrence. However, this cannot always be demonstrated. A family history of dissection is also an important factor in recurrence. CLINICAL CASES: We present two cases of recurrent spontaneous dissection of the carotid artery from a series of 22 patients with dissection, during the period 1990-1997. In the first case, the second dissection occurred 15 days after the first and in the second case, seven months later. In both cases the recurrence was in the contra-lateral carotid artery. In the second case the vascular tree was noted to have been formed of ecstatic, tortuous vessels. CONCLUSIONS: Our series shows results similar to others published. In one of these, an underlying arteriopathy which predisposed to the condition was shown. Both followed satisfactory courses. In case 2 a high index of clinical suspicion was necessary, since the recurrence presented as headache alone.

[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.

[Familial internal carotid dissection]. / Disección de carótida interna de presentación familiar.

Cervicocerebral arterial dissections is responsible for 20% of first strokes in young adults. Several diseases of the connective tissue and wall vessels anomalies are considered predisposing of arterial dissection. Familiar cases have been reported in 5%, according to hereditary primary arteriopathy, such as fibromuscular dysplasia. However, the nature of this arteriopathy has been rarely proved. We present two sisters with spontaneous dissection of the internal carotid artery with details suggestive of an atypical way of fibromuscular dysplasia in one of them.

[Subacute combined degeneration of the spinal cord: manifestations of esophageal epidermoid carcinoma]. / Degeneración combinada subaguda espinal: manifestación de un carcinoma epidermoide esofágico.

INTRODUCTION: Subacute combined degeneration of the spinal cord associated with vitamin B12 deficit is well known. However a paraneoplastic induced cyanocobalamin malabsorption responsible for either subacute combined degeneration of the spinal cord, has not been reported. Moreover, the possibility of either subacute combined degeneration of the spinal cord may be manifestation of a cancer, it's unknown. CLINICAL CASE: We present the case of a 73-year-old woman with cutaneous paleness and clinical manifestations of a subacute combined degeneration of the spinal cord. Laboratory analysis showed a megaloblastic anemia and Schilling test showed cyanocobalamin malabsorption corrected by intrinsic factor. Anti-intrinsic factor antibodies were positive. Chronic atrophic gastritis and immunological diseases associated with pernicious anemia or with anti-intrinsic factor positive, were excluded. A hidden esophageal squamous cell carcinoma was discovered. Necropsy confirmed both, subacute combined degeneration of the spinal cord and esophageal carcinoma in the absence of chronic atrophic gastritis. CONCLUSIONS: Anti-intrinsic factor antibodies were the only found factor which could explain cyanocobalamin malabsorption. A esophageal squamous cel carcinoma was discovered. In our case, vitamin B12 malabsorption had a paraneoplastic origin mediated by anti-intrinsic factor antibodies. Subacute combined degeneration of the spinal cord may be the manifestation of a hidden esophageal squamous cell carcinoma.

[The current status of the prevention and treatment of sudden death]. / Estado actual de la prevención y tratamiento de la muerte súbita.

Sudden cardiac death is a major medical problem. The techniques to identify high risk patients have a limited value. Only betablockers, and perhaps amiodarone, are useful for primary prevention of sudden cardiac death. Prospective studies being carried out today with implantable cardioverter-defibrillator, will show us if these are the best way of treatment (secondary prevention) of sudden cardiac death.

[Prolonged asystole requiring resuscitation techniques during head-tilt table test]. / Asistolia prolongada que requiere maniobras de resucitación durante un test de basculación.

We describe a case of a patient with recurrent syncope without cardiac disease and with a normal electrophysiological study. The patient underwent a head-up tilt table test as a diagnostic method. During the test the individual suffered a twenty-nine seconds cardiac asystole. He recovered the cardiac rhythm nineteen seconds after having adopted the supine position and with resuscitation maneuvers. In the consulted bibliography, there is no evidence of such a long asystole induced during a head-up tilt table test, where the patient required a chest thumping. Commonly the spontaneous recuperation of the cardiac rhythm takes place immediately after the patient adopts a horizontal position. This case supports the hypothesis that the asystole provoked by a cardioinhibitor reflex in patients suffering from a vasovagal syncope, can become potentially lethal and therefore lead to sudden cardiac death.