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Biomedical subjects

O Franch

Publications and source records attributed to O Franch.

13 recordsLinked to original sources
PubMed2002

SUNCT syndrome: priming of symptomatic periods and worsening of symptoms by treatment with calcium channel blockers.

We report a SUNCT patient who showed both a precipitation and worsening of symptomatic periods after treatment with L-type calcium channel blockers. This pharmacological response may provide us with important clues for understanding the pathophysiology of SUNCT, and hopefully to find a remedy for the victims of this syndrome. This observation could also support a verapamil trial in SUNCT patients as a precipitating of attacks.

Aged↗
PubMed1992

[Wilson's disease. A retrospective analysis of 12 cases].

We reviewed retrospectively 12 patients with Wilson's disease diagnosed during a 16-year period (1974-1989). The prevalence rate was 0.6 per 100,000 individuals. Clinical onset was hepatic (50%) or neurologic (50%), but at diagnosis (6.4 years later) 67% of patients showed several clinical manifestations: hepatic, neurologic, renal and haematologic. Among the essential diagnostic indices we find false negative results for Kayser-Fleischer ring (25%), serum ceruloplasmin (8%) and total serum copper (34%). Ten patients were treated with penicillamine. This drug was effective and well tolerated, although one patient (10%) developed membranous nephritis and required to change successively to BAL and trien. In a 61 months follow-up 5 patients (42%) died from severe liver failure. Patients with poor prognosis had a diagnostic delay and a liver failure degree significantly greater than patients with good prognosis. Our results suggest the following conclusions: a) in Spain the prevalence rate of Wilson's disease is near the lower reported rate; b) the early diagnosis of Wilson's disease is rare; c) diagnosis should be made only when several essential indices are positive; d) early hepatic transplantation showed carried out in patients with acute or chronic severe liver failure.

Age Factors↗
PubMed1987

Chromatolytic changes in the central nervous system of patients with the toxic oil syndrome.

Five patients died of a severe neuromyopathy months after the ingestion of adulterated rapeseed oil. These patients were selected for this study due to the presence of striking chromatolytic lesions in symmetric and scattered nuclei of the brain stem, including the locus coeruleus, midline raphe, lateral reticular nuclei of the medulla and cuneate nuclei. Two of the five cases, in addition to these topographic levels of involvement, had remarkable chromatolysis, vacuolar degeneration and heavy silver impregnation of the swollen perykarya and proximal dendrites in the nuclei of the basis pontis. In this paper we analyze the features of the chromatolytic lesion and suggest that the neuronal pathology observed in these cases is an example of irreversible chromatolysis involving vacuolization and filamentous proliferation as final events of the chromatolytic process. The cause of the cell degeneration in the toxic oil syndrome (TOS) is yet undetermined. Chromatolysis in this disease may be the result of a neurotoxic action of the toxic factor in the adulterated oil.

Brassica↗
PubMed1987

Subcortical arteriosclerotic encephalopathy (Binswanger's disease): a report of five patients.

Five patients with variable clinical symptoms were diagnosed as having--subcortical arteriosclerotic encephalopathy (Binswanger disease) based on the presence of lacunar infarcts in basal ganglia, various abnormalities of subcortical white matter and severe thickening and hyalinization of penetrating arteries and arterioles. One case had a classical clinical picture while in the others the course of the disease was short and was associated with severe systemic abnormalities. The variability of the clinical features, the identify of "classical" clinical symptoms with other forms of cerebral arteriosclerosis, the similarity between "atypical" cases and other entities, and the high frequency of associated conditions makes it difficult to characterize the clinical pathological entity called subcortical arteriosclerotic encephalopathy.

Aged↗
PubMed1986

Neurotransmitter changes in cerebrospinal fluid in the Spanish toxic oil syndrome: human clinical findings and experimental results in mice.

Patients with Spanish Toxic Oil Syndrome (TOS) complaining of neuromuscular symptoms had increased levels of homovanillic acid and 5-hydroxyindoleacetic acid (5-HIAA) in cerebrospinal fluid. Severity of pain and muscle cramps correlated with the magnitude of increment in levels of monoamine metabolites. Mice treated with oleyl anilide, a putative toxic compound found in some stocks of the toxic rapeseed oil, did not present clinical or anatomical findings compatible with TOS. However, biochemical studies in these mice revealed a depletion of serotonin and an elevation of 5-HIAA levels. Our findings suggest that the unidentified toxic agent of the TOS and oleyl anilide induce pharmacological changes in monoamine neurons of the brain.

Anilides↗
PubMed1981

Glycogen storage disease in skeletal muscle. Morphological, ultrastructural and biochemical aspects in 10 cases.

We analyzed clinical, histological and biochemical findings in 10 patients with glycogen storage disease in skeletal muscle. Four patients were deficient in acid-alpha-glucosidase (Glycogenosis type II), three of them with late infantile onset and one patient adult form. Five patients, two of them siblings, were deficient in myophosphorylase (glycogenosis type V, McArdle's disease). One patient was a newborn with phosphofructokinase deficiency (glycogenosis type VII, Tarui's disease). Of the study of our cases we would like to outline the following features: in the glycogenosis type II the deposit is fundamentally intralysosomal in the late infantile form, storage of mucopolysaccharides and deposit in interstitial fibroblasts were found, while in the adult form glycogen storage is minimal. In the glycogenosis type V the storage of glycogen is free and of a small amount. In two patients we have observed enzymatic activity in regenerating fibres. In glycogenosis type VII the storage is free, of considerable quantity and the interstitial cells are also affected; no storage is observed in the satellite cells.

Adolescent↗
PubMed1981

Neuromuscular changes in hypertrophic cardiomyopathy.

Clinical, electrophysiological and histological studies of skeletal muscle were performed in 12 patients with primary hypertrophic cardiomyopathy trying to add more information about the skeletal muscle affectation in this disease. Eight cases showed mild increase in serum enzymes. All of the patients showed slight abnormalities in the electrophysiologic studies: four had abnormal conduction velocities in peripheral nerve, another four a myopathic pattern in the EMG and four a mixed pattern. Histologically only 2 patients showed signs of a denervation atrophy. The most common alterations found were non-specific (isolated fibre atrophy, disorganization of the myofibrillar network and type II atrophy).

Adolescent↗
PubMedDate not supplied

[Evaluation of epilepsy surgery].

INTRODUCTION: Neuropsychology is a discipline included in the neurosciences which, over the past twenty years, has shown its value in the assessment and rehabilitation of cognitive functions in different disorders. In particular, in epilepsy it has permitted the description of the different factors which affect cognitive deterioration; permitted the establishment of profiles of cognitive deterioration and also the evaluation of the adverse effects of some anti-epileptic drugs on cognition. DEVELOPMENT: In this paper we confirm the value of neuropsychology in an Epilepsy Unit and describe different procedures for evaluation, as well as the cognitive consequences of the surgery of epilepsy.

Anticonvulsants↗