Migraine and cerebral infarction in young people.

The objective of this study is to investigate relationship between migraine and cerebral infarction in young people. Patients aging 16-44 years, referred for stroke and age- and gender-matched controls were investigated for migraine following the International Headache Society criteria. Included people were 314 strokes and 314 controls. Each group consisted of 150 men and 164 women. Of the 105 persons with migraine (16.7%), 57 had migraine with aura (9.1%). In women, migraine with aura was related to stroke [35 women among strokes (21.3%) vs. 9 among controls (5.5%), P < 0.0001], whereas migraine without aura was not. After multivariate analysis, migraine with aura remained independently associated with stroke together with hypertension, and the estro-progestinic utilization. In men, migraine was not associated with stroke. In conclusion, migraine with aura appears to be associated with ischemic stroke in young women, independently from other common risk factors.

The transient syndrome of headache with neurological deficits, cerebrospinal fluid pleocytosis and acute confusional state: a case report.

The syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) now referred to in the IHS ICHD-II code 7.8, is only infrequently accompanied by a confusional state and severe agitation. We report the case of a 34-year-old man who suffered from three episodes of headache with transient focal neurological deficits that were consistent with HaNDL but that were accompanied by an intense, confusional agitated state that required admission, in the first episode, in a psychiatric unit.

Different mutations in the LMNA gene cause autosomal dominant and autosomal recessive Emery-Dreifuss muscular dystrophy.

Emery-Dreifuss muscular dystrophy (EMD) is a condition characterized by the clinical triad of early-onset contractures, progressive weakness in humeroperoneal muscles, and cardiomyopathy with conduction block. The disease was described for the first time as an X-linked muscular dystrophy, but autosomal dominant and autosomal recessive forms were reported. The genes for X-linked EMD and autosomal dominant EMD (AD-EMD) were identified. We report here that heterozygote mutations in LMNA, the gene for AD-EMD, may cause diverse phenotypes ranging from typical EMD to no phenotypic effect. Our results show that LMNA mutations are also responsible for the recessive form of the disease. Our results give further support to the notion that different genetic forms of EMD have a common pathophysiological background. The distribution of the mutations in AD-EMD patients (in the tail and in the 2A rod domain) suggests that unique interactions between lamin A/C and other nuclear components exist that have an important role in cardiac and skeletal muscle function.

Endocarditis infecciosa en pacientes drogadictos endovenosos: presentación clínica y evolución intrahospitalaria / Infective endocarditis in intravenous drug users

Este estudio analizó las características de cuarenta episodios de endocarditis infecciosa en 38 pacientes con drogadicción intravenosa como factor predisponente. Tenían una edad promedio de 28,9 años; 90 por ciento eran de sexo masculino, con compromiso de válvula sana en 82,5 por ciento de los casos; 20 por ciento de los pacientes habían tenido un episodio o más de endocarditis previa; 77,5 por ciento tuvieron afectación derecha; el agente causal más frecuente fue el Staphylococcus aureus, con 70 por ciento de hemocultivos positivos y 90 por ciento de incidencia de HIV. Las complicaciones más frecuentes fueron la insuficiencia cardíaca y el tromboembolismo de pulmón.La mortalidad hospitalaria fue más elevada que la descripta habitualmente en esta población

Endocarditis infecciosa en pacientes drogadictos endovenosos: presentación clínica y evolución intrahospitalaria / Infective endocarditis in intravenous drug users

Este estudio analizó las características de cuarenta episodios de endocarditis infecciosa en 38 pacientes con drogadicción intravenosa como factor predisponente. Tenían una edad promedio de 28,9 años; 90 por ciento eran de sexo masculino, con compromiso de válvula sana en 82,5 por ciento de los casos; 20 por ciento de los pacientes habían tenido un episodio o más de endocarditis previa; 77,5 por ciento tuvieron afectación derecha; el agente causal más frecuente fue el Staphylococcus aureus, con 70 por ciento de hemocultivos positivos y 90 por ciento de incidencia de HIV. Las complicaciones más frecuentes fueron la insuficiencia cardíaca y el tromboembolismo de pulmón.La mortalidad hospitalaria fue más elevada que la descripta habitualmente en esta población (AU)

[Abdominal propriospinal myoclonus of unknown etiology]. / Myoclonies propriospinales abdominales d'étiologie inconnue.

We report the case of a 77-year-old woman with a two-year history of abdominal non rhythmic myoclonus. Neurological examination was normal. Bursts of myoclonic activity were recorded from the rectus abdominis muscle and then from the external oblique muscle after a delay of 40 ms. Magnetic resonance imaging of dorsal spine revealed T7-T8 and T9-T10 disc protrusions without root compression. Electroencephalography, back-averaging-EEG, brain computed tomography scan, motor and sensory evoked potentials revealed no abnormality, thus ruling out the possibility that the myoclonic jerks might be of cortical origin. Electromyography of the rectus abdominis and external oblique and of the T7-T8 paraspinal muscles did not show signs of denervation. Therefore a peripheral origin of the myoclonus could be excluded. In our patient the difference in latency of the EMG activity between the external oblique and the rectus abdominis muscles was possibly due to the caudal propagation of the myoclonus by slowly conducting pathways, supporting the hypothesis for its propriospinal origin, even though a known ethiologic factor could not be identified.

[Cardiac involvement in scleroderma]. / Afectación cardiaca en esclerodermia.

It has been analyzed seven patients, five females and two males, affected by progressive systemic sclerosis. Their ages range from 35 to 60 years. Three of them correspond to the CREST syndrome named variant. There were made: physical examination, electrocardiogram, echocardiogram and radionuclide techniques such as: Thallium perfusion (T201) and ventriculography (Tc99). The cardiac examination of all the patients showed: Left myocardiopathy in six patients, as well right myocardiopathy in two of them. Four patients were affected by chronic cor pulmonale, due to pulmonary hypertension. All the patients had developed diffuse thallium perfusion defects as well, having abnormal resting left ventricular function in three patients and function abnormality from right ventricle in two patients. It is necessary to point out the importance of making such study systematically, even in the case cardiac symptoms absence.

[Cardiac repercussions in the CREST syndrome]. / Repercusión cardiaca en el síndrome de CREST.

Two females patients with CREST syndrome associated with cardiac involvement are reported. One of them has pericardial effusion just shown by echocardiography with patterns of cardiomyopathy of ventricular cavities, the latter as primary myocardial involvement, in the absence of pulmonary or systemic hypertension. The second patient has chronic cor pulmonale secondary to pulmonary hypertension verified by electrocardiography and echocardiography. It is emphasized the utility of the echocardiography and the importance of those anomalies that can lead to the patient's death, transforming the CREST towards a syndrome with potentially but late, severe systemic involvement.

Biochemical and clinical evaluation of a new cellulose diacetate secondary filter for cascade filtration.

We have performed 24 cascade filtration treatments in 8 patients with hyperviscosity syndrome (2 cases), essential mixed cryoglobulinemia, post-hepatitic cryoglobulinemia, Sjogrens disease, rheumatoid vasculitis, Miller-Fisher syndrome and chronic dysimmune polyneuropathy. New cellulose diacetate filters were employed, giving a satisfactory performance. At 1.5 L plasma treatment, the rejection rate for macromolecular plasma components was close to 90%, whereas albumin recovery was close to 70%. Treatments were clinically effective, confirming that cascade filtration is an alternative to conventional plasma exchange in patients with IgM or immune complex mediated diseases.