Afasia de instauración aguda como forma de presentación inicial de una enfermedad de Creutzfeldt-Jakob / Acute-onset aphasia as the presenting symptoms in a case of Creutzfeldt-Jakob disease

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[Progressive multifocal leukoencephalopathy in elderly immunocompetent patients. Report of 2 cases]. / Leucoencefalopatía multifocal progresiva en pacientes ancianos inmunocompetentes. A propósito de dos casos.

INTRODUCTION: Progressive multifocal leukoencephalopathy is a disorder which is rare in immunocompetent patients. OBJECTIVES: We report the cases of two elderly patients with serology, in one case positive for hepatitis C, and in the other with anti-DNA antibodies, and discuss the part these might play in causing progressive multifocal leukoencephalopathy. CLINICAL CASES: Case 1. An 86 year old man had been found on serology investigations to be positive for hepatitis C virus. In November 1996 he complained of dysarthria and left hemi-negligence following an accidental fall. Since his clinical condition became worse he was admitted to hospital for further investigation. On neuroimaging studies the intracerebral lesions were increased. The only other finding confirmed was that of positive serology for hepatitis C virus. The patient deteriorated progressively and died 50 days after admission. Case 2. A 70 year old woman began to show progressive cognitive impairment and left hemiparesia in June 1996. Se was investigated in another centre and provisionally diagnosed as having vasculitis of the CNS, in view of her positive anti-DNA antibody and right frontoparietal hypodense lesion. Treatment had been started with corticosteroids. She was admitted to our hospital when her neurological deficits worsened. The immunological alterations were confirmed. On MRI the lesions in the white matter were seen to have progressed. The patient slowly improved. She was discharged from hospital in February 1997 in a semiconscious state, able to follow persons and things with her eyes, with global aphasia and with spastic tetraparesia which was mainly left-sided. She remains stable. CONCLUSION: Progressive multifocal leukoencephalopathy is a condition which should be remembered when dealing with immunocompetent patients.

Acute delirium as a manifestation of obstructive sleep apnea syndrome.

Cognitive deficits and psychiatric manifestations such as depression and psychosis have been associated with obstructive sleep apnea (OSA) syndrome. We report a patient with OSA admitted to our center because of acute delirium of sudden onset at night, during sleep, and which impelled the patient to jump out of the window of his home. After exhaustive study, no other causes were found for the delirium, which resolved when nasal continuous positive airway pressure (nCPAP) was initiated. We believe that it is clinically important to be aware of this association, since it identifies a new, treatable cause of delirium.

[Value of cranial MRI in the follow-up of dural sinus thrombosis]. / Valor de la RM craneal en el seguimiento de las trombosis de senos durales.

INTRODUCTION: At the present time it is well established that cranial magnetic resonance (MR) is the imaging technique of choice for diagnosis of thrombosis of the dural sinuses. However, few studies have been done to evaluate its role in follow-up in this cerebrovascular disorder. OBJECTIVE: To assess the alterations in resonance signal in follow-up of patients with thrombosis of the dural sinuses and the clinical correlation of this. MATERIAL AND METHODS: We reviewed the clinical histories of 12 patients diagnosed on cranial MR as having thrombosis of the dural sinuses; 8 cases were also seen again six months later. The cranial MR study was done using a 1.5 or 1.0 T superconductive apparatus to do measured spin-echo sequences (SE) in T1 and T2 in sagittal, transverse and coronal planes. The time elapsed between diagnosis on MR and clinical diagnosis was between 24 hours and 6 months. RESULTS: All patients had stronger resonance signals in T1 and T2 indicating a subacute phase of the thrombosis (oxidative conversion of deoxyhaemoglobin to methoxyhaemoglobin) and complete absence of blood flow in the sinuses. The eight patients seen again after six months were found to be normal on neurological. On cranial MR partial recanalization was seen in 5 of these (62%). CONCLUSION: Changes in the resonance signal persist for a long time both with and without signs of recanalization. This finding does not affect the medium term clinical course.

Value of immunologic testing in stroke patients. A prospective multicenter study.

BACKGROUND AND PURPOSE: The aims of this prospective and multicenter study were to determine the frequency of anticardiolipin and antinuclear antibodies in an unselected ischemic and hemorrhagic stroke population and to evaluate the clinical significance of these autoantibodies. METHODS: Over a 1-year period, we collected plasma from 481 consecutive patients with ischemic or hemorrhagic stroke attending four different hospitals. Blood (10 mL) was drawn from each subject into a citrated glass tube. Plasma was obtained immediately by centrifugation and was stored at -70 degrees C until use. Concentrations of IgM and IgG anticardiolipin antibodies were measured at room temperature in normal (not heat-treated) plasma by standardized enzyme-linked immunosorbent assay. All sera were treated by indirect immunofluorescence on mouse liver and kidney sections for antinuclear antibodies. RESULTS: A total of 481 patients (325 men, 156 women) 16 to 90 years in age (mean age, 61 years) were studied. Anticardiolipin antibodies were present in 5 of 481 (1.04%) patients. One patient was IgG positive and four patients were IgM positive. Of 481 patients, 35 (7.2%) were positive for antinuclear antibodies. Anti-DNA antibodies were not demonstrable in any patient. CONCLUSIONS: The frequency of anticardiolipin antibodies in a heterogeneous stroke population is possibly lower than reported. The routine screening of anticardiolipin and antinuclear antibodies in a stroke population is of questionable value.

[Pituitary apoplexy and empty sella]. / Apoplejía hipofisaria y silla turca vacía.

Pituitary apoplexy (PA) is currently considered as one of the causes of empty sella (ES). However, in most reported cases the diagnosis of PA was based on anamnesis. A prospective study of three patients diagnosed during the PA episode was carried out. Periodical evaluations of hormone concentrations and cerebral computed tomography images were carried out. Initially, the three patients had a global anterior pituitary developed ES within 6 and 18 months. Surgical therapy was not undertaken in any case. Owing to the rarity of the disease definitive conclusions are difficult to obtain; however, the present study suggests that after an episode of PA global anterior pituitary failure develops, with subsequent ES. Early medical therapy with glucocorticoids can avoid surgical therapy.

Cisternographic pattern of spontaneous liquoral hypotension.

We describe five cases of headache due to spontaneous liquoral hypotension, the syndrome comprising sudden, intense and oppressive orthostatic holocranial headache. The headache improved in the recumbent position and was accompanied by nausea and sometimes vomiting. There was no history of lumbar puncture or previous trauma. CSF tension was low or negative. The CSF showed a raised protein content and increased red and white cell counts. CT scan was normal or showed a slit ventricular system. Improvement was complete three to eight weeks from onset. The treatment consisted of bed rest and oral and parenteral fluid replacement. An isotope cisternography carried out in all patients while the headache was present showed a cisternographic pattern characterized by a combination of premature elimination and failure to detect the isotope at the cerebral convexity. Scan images did not show CSF leakage at any site. This stereotyped reaction pattern suggests that CSF hyperabsorption is the most likely pathophysiological mechanism of this entity.

Ischemic stroke in young adults. I. Analysis of the etiological subgroups.

An ischemic stroke (IS) group including 386 patients under 50 years old is analysed taking into account different etiological subgroups and comparing risk factors against a control group of 100 people. The series points out the presence of 66.1% patients included in the inconclusive-atherothrombosis group, of which 22.7% had defined criteria of atheromatosis, while 11.6% were diagnosed of lacunar infarct. 13.5% of cases were considered as cardiac origin embolisms, and 14.1% were affected of mitral valve prolapse. The migraine group includes 4.9% of the patients while 17.6% belong to the miscellaneous group. The comparison of each of these groups with the control group showed significant differences for family history of stroke, personal history of peripheral arteriopathy, tobacco, arterial hypertension and previous IS.

[Carotid artery disease induced by irradiation]. / Enfermedad de la arteria carótida inducida por irradiación.

We have evaluated 39 patients with ischemic strokes secondary to atherosclerotic disease of the extracranial carotid artery. Seven of them had been treated in the past with cervical radiotherapy for neoplastic diseases (radiotherapy group); the remaining 32 patients had not received radiation therapy (non-radiotherapy group). When the prevalence of risk factors was compared between the two groups, there was a significantly higher prevalence of peripheral arterial disease (p less than 0.05), hypertension (p less than 0.05) and atherogenicity index lower than 21.5% (p less than 0.05) in the non-radiotherapy group. Regarding angiographic data, the radiotherapy group showed a significantly higher occurrence of localized findings than the non-radiotherapy group (p less than 0.005). It is concluded that radiation-induced carotid disease is a clinical condition which may be individualized from the remaining patients with atherosclerotic carotid artery disease.