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Biomedical subjects

F Graus

Publications and source records attributed to F Graus.

At least 19 recordsLinked to original sources

[Neurologic syndromes associated with anti-Hu antibody. Study of 24 patients].

BACKGROUND: Twenty-four patients with neurologic involvement and anti-Hu antibodies were studied with the aim of defining the type of tumor associated, evaluating whether the clinico-pathologic picture agreed with the concept of paraneoplastic encephalomyelitis (PEM) and evaluating the treatments used. METHODS: The study was retrospective with the clinical histories being reviewed to define the neurologic syndromes, their evolution and response to the different treatments, time of appearance and type of tumor as well as the neuropathologic changes in the patients undergoing autopsy. RESULTS: In 18 patients a neoplasm was diagnosed as small cell pulmonary carcinoma (SCPC) in 89% of the cases. The neurologic picture preceded the tumor by an average of five months. The clinical pictures included: sensitive neuropathy (20 patients), cerebellous and truncus encephalicus involvement (8 patients), motor neuropathy (6 patients), cortical involvement (5 patients) and neurovegetative dysfunction (4 patients). In 55% of the patients more than one area was altered. Post mortem studies carried out on 5 patients demonstrated inflammatory infiltrates and neuronal loss in multiple areas of the nervous system. None of the patients improved with treatment. The 9 patients who only received immunodepressants evolved in a way similar to those who were not treated. In 7 of the 11 patients who received antitumoral therapy, the neurologic syndrome stabilized for at least 6 months. CONCLUSIONS: The clinicopathological picture and the associated tumor seen in patients with anti-Hu antibodies are identical to those seen in PEM. Antitumoral treatment seems to be more effective than immunodepressant treatment.

Adult

[Contribution of magnetic resonance imaging in the early diagnosis of epidural metastasis].

BACKGROUND: Vertebral bone metastases represent the most frequently affected region within the skeleton. They are of important relevance because of the risk of medullar compression. The diagnosis of medullar canal invasion is of particular interest in order to prevent neurological dysfunction. A prospective study was carried out to detect the frequency and degree of invasion of the spine. METHODS: Twenty-eight patients were studied (13 males and 15 females), with a median age of 61 years (range 35-85), with cancer diagnosis and vertebral bone metastases, and local or radicular pain, without signs or symptoms suggesting myelopathy. Neurological, physical examination, radiological study (anteroposterior and lateral) and magnetic resonance imaging (MRI) study was performed in all patients to detect medullar canal invasion. RESULTS: Local pain was present in 43% of patients (n = 12), and radicular in 57.1% (n = 16). The most frequent radiologic vertebral involvement was thoracic (46.4%), with 71% of vertebral body collapse. MRI showed epidural space invasion in 75% of patients (n = 21), with a degree superior to 50% in 43%. CONCLUSIONS: The beginning of vertebral pain in a patient with cancer diagnosis, with evidence of bone invasion after radiological study, represents a major indicator to perform a MRI study to detect epidural involvement.

Adult

[Primary cerebral lymphoma in 10 patients with AIDS. Comparative clinico-radiologic study with cerebral toxoplasmosis, cerebral tuberculoma and primary cerebral lymphoma in non-immunodepressed patients].

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is the second cause of cerebral masses in patients with the acquired immunodeficiency syndrome (AIDS). The present study evaluated the possible presence of clinical or radiologic signs permitting differentiation of AIDS patients and PCNSL from those with cerebral masses of other etiologies. METHODS: Clinical history and cranial computerized tomography (CT) of patients with PCNSL and AIDS from the Hospital Clinic i Provincial in Barcelona were reviewed. Results were compared with those of patients with PCNSL without evidence of immunosuppression and with those with AIDS and cerebral toxoplasmosis or tuberculoma diagnosed during the same period. RESULTS: Of 685 patients with AIDS, 10 were identified with PCNSL. The clinical picture was not different to that observed in patients with AIDS and cerebral toxoplasmosis or tuberculomas. In contrast to PCNSL in non immunodepressed patients, the cerebral CT in patients with PCNSL and AIDS demonstrated hyperdense lesions in only 44% and contrast enhancement was not homogeneous in any case. These characteristics were similar to those observed in the CT of patients with cerebral toxoplasmosis or tuberculoma with the exception that only 8% of the lesions by toxoplasmosis were spontaneously hyperdense. CONCLUSIONS: The clinical-radiological data of primary central nervous system lymphoma in patients with the acquired immunodeficiency syndrome are similar to those observed in other etiologies. However, the presence of a sole spontaneously hyperdense region in cranial computerized tomography is more suggestive of primary central nervous system lymphoma than cerebral toxoplasmosis.

Acquired Immunodeficiency Syndrome

[Paragonimiasis and pulmonary tuberculosis].

Paragonimiasis (infestation by the Paragonimus species) in Spain is a very infrequent entity within the group of imported infectious diseases. A native, resident of Equatorial Guinea who was affected by pulmonary and probably extrapulmonary paragonimiasis together with active pulmonary tuberculosis is described. This association is relatively common and may complicate the diagnostic process. The identification of the parasite was established from samples of pulmonary secretion obtained by natural expulsion and by fiber bronchoscopy in which Kinyoun carbonfucsine dye, Giemsa dye and argentic impregnation were used. The possibility of neurological disease existing (medullar and cerebral) produced by the same parasite is also discussed. Antituberculous treatment and the use of praziquantel satisfactorily control both infections.

Adult

[Study of neurological and cognitive changes in patients in stage III and initial phases of stage IV of infection by human immunodeficiency virus type 1].

BACKGROUND: To evaluate the prevalence of neurological and cognitive disorders in patients with the human immunodeficiency virus type 1 (HIV-1). METHODS: A transversal prospective study was carried out in 56 patients with HIV-1 and in 18 seronegative subjects by a protocolized neurological and cognitive examination. RESULTS: Twenty-seven patients were intravenous drug users (IVDU) and 29 were male homosexuals. Nineteen were in stage III of HIV-1 infection and 37 were in the initial phases of stage IV. No significant differences were found in the cognitive performance of patients in stage III when the control group was compared with seropositive patients. Significant differences were observed in stage IV in the Rey figure complex tests the B trace test and the number key. Three stage IV patients presented dementia criteria. No significant differences were seen in test performance upon comparing the ex-iVDU seropositive subgroup with the male homosexuals nor in the stage III or stage IV groups. CONCLUSIONS: Cognitive performance of stage III patients did not significantly differ from that of the seronegative control group; a global diminution in cognitive performance was observed in the stage IV group and there were no differences in the cognitive state between the two risk groups analyzed.

Acquired Immunodeficiency Syndrome

Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients.

We studied 71 patients with "paraneoplastic" encephalomyelitis, sensory neuronopathy, or both associated with the presence of the anti-Hu antibody in their serum. Most (78%) had small-cell lung cancer. In 9 patients no tumor was detected. Fifty-two patients (73%) had signs and symptoms of multifocal involvement of the nervous system; in 28 (39%), 2 areas, and in 24 (34%), 3 or more areas were clinically affected. Sensory neuronopathy was present in 52 patients (74%), but in only 44 (62%) did it dominate the course of the disease. Other predominant findings were: motor neuron dysfunction (14 patients, 20%), limbic encephalopathy (14, 20%), cerebellar symptoms (11, 15%), brainstem encephalopathy (10, 14%), and autonomic nervous system dysfunction (7, 10%). The presence of the anti-Hu antibody prompted a search for the tumor in 60% of the patients; the tumor when found was usually small and remained localized until death, or was demonstrated only at autopsy. Treatment using steroids and plasmapheresis, immunosuppressants, or both, did not improve the paraneoplastic symptoms. Autonomic and respiratory failure, either of central origin or secondary to neuromuscular weakness, were the principal causes of death. Patients with rapidly developing sensory neuropathy or symptoms of encephalomyelitis should be studied for the presence of the anti-Hu antibody; if the antibody is found, the possibility of small-cell lung cancer should be investigated. If a tumor is not found in the initial search, one may become evident in several months.

Adolescent

Adult-onset mitochondrial myopathy.

Mitochondrial diseases are polymorphic entities which may affect many organs and systems. Skeletal muscle involvement is frequent in the context of systemic mitochondrial disease, but adult-onset pure mitochondrial myopathy appears to be rare. We report 3 patients with progressive skeletal mitochondrial myopathy starting in adult age. In all cases, the proximal myopathy was the only clinical feature. Mitochondrial pathology was confirmed by evidence of ragged-red fibres in muscle histochemistry, an abnormal mitochondrial morphology in electron microscopy and by exclusion of other underlying diseases. No deletions of mitochondrial DNA were found. We emphasize the need to look for a mitochondrial disorder in some non-specific myopathies starting in adult life.

Aged

Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies.

We retrospectively evaluated the effect of plasmapheresis (PE) in seven patients with paraneoplastic encephalomyelitis (PEM), small-cell lung carcinoma, and anti-Hu antibodies, and four patients with paraneoplastic cerebellar degeneration (PCD), ovarian or breast cancer, and anti-Yo antibodies. In addition to PE, patients received prednisone (nine), cyclophosphamide (eight), or treatment of the tumor (five). All but one patient were severely disabled by the time PE began. The clinical outcome was compared with that of five patients (PEM, four; PCD, one) who only had treatment of the tumor. Only one of these five patients had a severe neurologic deficit at the onset of the antineoplastic treatment. No patient improved. Two patients treated with PE and antineoplastic therapy and three who only received treatment of the tumor remained stable for at least 6 months. Four of the five patients with a stable course started the treatment when the neurologic deficit was not severe. We conclude that the efficacy of PE with other immunosuppressive therapies in the stabilization of the neurologic deficit is uncertain.

Aged

[Paraneoplastic encephalomyelitis and Lambert-Eaton syndrome].

In two men presenting with muscle weakness and disturbances of equilibrium neurophysiological examination by repeated stimulations revealed responses suggestive of Lambert-Eaton syndrome. In the first month of the disease very high levels of anti-Hu antibody were found in the serum and CSF, betraying a malignant lesion. This was confirmed by autopsy 4 months later in one patient and by bronchial biopsy 16 months later in the other patient. Both had small-cell lung carcinoma associated with paraneoplastic encephalomyelitis.

Aged

Clinical and pathological advances on central nervous system paraneoplastic syndromes.

In the last decade, several features have improved our knowledge of CNS paraneoplastic syndromes. Patients with paraneoplastic cerebellar degeneration (PCD) and breast or ovarian cancer, but not with other tumors, harbor an antibody against Purkinje cells (called anti-Yo). Clinical features of anti-Yo positive and negative PCD are similar but the latter may have a less progressive clinical course with occasional remissions. In addition to the association of opsoclonus with neuroblastoma, this syndrome has been identified in patients with breast or small-cell lung cancer (SCLC). Patients with opsoclonus and breast cancer have an antineuronal antibody (called anti-Ri) not present if opsoclonus is associated with SCLC or neuroblastoma. Paraneoplastic encephalomyelitis (PEM) is almost always associated with SCLC. Most patients present with sensory neuronopathy, limbic or brainstem encephalitis but involvement of multiple levels is usual. An antibody (called anti-Hu) against neuronal nuclear antigens is present in patients with PEM and SCLC. Autopsy studies demonstrate deposits of anti-Hu specific IgG in the neurons and a predominance of T cells in the inflammatory infiltrates. Treatment of the tumor and immunosuppressors are effective in opsoclonus whereas patients with PCD or PEM with circulating antibodies do not improve.

Brain Diseases

[Incidence of Borrelia burgdorferi infection in isolated facial paralysis. A prospective study].

Between the causes of facial paralysis, similar to Bell's palsy, recently are included those due to some families of spirochaeta (Borrelia burgdorferi) transmitted through tick-bite. These bacteria, besides de facial palsy, may produce several degrees of lymphocytic meningitis and subsequently the paralysis of the face, sometimes clumsy or recurrent. The treatment being the antibiotic drugs, never the corticoids. In the paper are explained prospectively the incidence of this disease in the AA's environment.

Adolescent

[Tropical spastic paraparesis and HTLV-I. The first case in Spain].

The first patient with myelopathy associated with T-cell leukemia/lymphoma virus infection (HTLV-I) in Spain is reported. Although this condition is endemic in several areas from the Caribbean Sea, South America, Africa and Japan, some cases have been reported in European countries, generally in immigrant patients, residents in endemic zones or patients having received transfusions. In the present case the infection was probably acquired in a tropical zone through blood transfusion. The patient was a 60-year-old female who had been living in Peru during the last 25 years. In that country aortocoronary bypass was carried out for ischemic heart disease. During operation she received blood transfusion. One and a half year later she developed progressive spastic parapesia with impaired sphincter control. Other causes were ruled out, and the diagnosis was confirmed by HTLV-I antibody determination with ELISA and Western blot.

Female