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Biomedical subjects

J Berciano

Publications and source records attributed to J Berciano.

At least 109 records · Page 6Linked to original sources
PubMed1996

Schwann cell nuclear remodelling and formation of nuclear and coiled bodies in Guillain-Barré syndrome.

We have examined the reorganization of the cell nucleus in myelin-related Schwann cells (SCs) in a case of acute Guillain-Barré syndrome (GBS). Spinal root samples of the GBS case and human controls were processed for light and electron microscopy. The cytochemical EDTA method for ribonucleoproteins (RNPs) and a specific silver staining technique for nucleolar organizer regions were used on ultrathin sections. In SCs of the GBS case, we observed a significant increase in nuclear size (64.99 +/- 10.47 microns 2 in the GBS vs 35.07 +/- 8.74 microns 2 in the controls, mean +/- SD) accompanying partial decondensation of heterochromatin domains and elaboration of an extensive network of RNP-containing perichromatin fibrils. In addition, the formation of two types of nuclear structures, coiled bodies and nuclear bodies of Bouteille, was induced in SCs of the case of acute GBS. Free coiled bodies were observed in the nucleoplasm and were characteristically stained with both RNP and silver procedures. Typical "simple" and "complex" nuclear bodies were regularly found, sometimes in association with coiled bodies. On the basis of cell nucleus physiology, all of these changes are considered cytological indicators of enhanced transcription and cellular hyperactivity, and they seem to reflect a reactive response of SCs triggered by the constellation of cellular and humoral signals associated with acute GBS.

Aged↗
PubMed1996

Cough, exertional, and sexual headaches: an analysis of 72 benign and symptomatic cases.

We analyzed our experience with cough, exertional, and vascular sexual headaches, evaluated the interrelationships among them, and examined the possible symptomatic cases. Seventy-two patients consulted us because of headaches precipitated by coughing (n = 30), physical exercise (n = 28), or sexual excitement (n = 14). Thirty (42%) were symptomatic. The 17 cases of symptomatic cough headache were secondary to Chiari type I malformation, while the majority of cases of symptomatic exertional headaches and the only case of symptomatic sexual headache were secondary to subarachnoid hemorrhage. Although the precipitant was the same, benign and symptomatic headaches differed in several clinical aspects, such as age at onset, associated clinical manifestations, or response to pharmacologic treatment. Although sharing some properties, such as male predominance, benign cough headache and benign exertional headache are clinically separate conditions. Benign cough headache began significantly later, 43 years on average, than benign exertional headache. By contrast, our findings suggest that there is a close relationship between benign exertional headache and benign vascular sexual headache. We conclude that benign and symptomatic cough headaches are different from both benign and symptomatic exertional and sexual headaches.

Adolescent↗
PubMed1996

[Parkinsonism after lethargic encephalitis: possibly the first Spanish case in the second half of the century].

We present the case of a 38-year-old patient who developed symptoms are parkinsonian syndrome at the age of 14, after meningoencephalitis meeting the criteria for a diagnosis of lethargic encephalitis (LE). The extrapyramidal symptoms are asymmetric are quiescent and have responded successfully to levodopa therapy for over 20 years. This is the first such case of parkinsonism secondary to LE to have been described in the Spanish medical literature in recent decades.

Adult↗
PubMed1995

[Distribution of headache by diagnosis as the reason for neurologic consultation].

BACKGROUND: The aim of this study was to analyze the diagnostic distribution of patients consulting because of headache in a neurology department. METHODS: The clinical records of 3,498 patients who had consulted specifically due to headache between 1978 to 1993 were retrospectively studied. Diagnosis was reclassified according to the criteria of the new headache classification with data concerning the age of onset and sex distribution also being collected. RESULTS: The most frequent diagnoses were migraine (1,802 patients; 52%) tension-type headache (113; 32%), followed in decreasing order by trigeminal neuralgia, post injury headache, cluster headache, subarachnoid hemorrhage, analgesic-ergotamine abuse headache, tumoral headache, cervical disease headache, benign exertional headache, due to paranasal sinusal disorders and benign intracranial hypertension. Female predominance was observed in the whole series (68%) as in the groups with migraine, tension-type headache, neuralgia headache by analgesic-ergotamine drugs and benign intracranial hypertension. Cluster, post injury, tumor and provoked headaches (cough, exertional and sexual) were found to be more frequent in men. The age of onset was very specific for each kind of headache. The percentage of symptomatic headaches increased in direct relation to age, being 10 fold higher in patients over the age of 65. CONCLUSIONS: The diagnostic distribution of headache as a cause of medical consultation is very different to the prevalence of different headaches. This distribution by diagnosis largely depends on the age of the patient at the time of headaches onset [corrected].

Adolescent↗
PubMed1995

The gene for progressive myoclonus epilepsy of the Lafora type maps to chromosome 6q.

Progressive myoclonus epilepsy of the Lafora type (Lafora's disease) is an autosomal recessive disease characterized by epilepsy, myoclonus, dementia, and periodic acid-Schiff-positive intracellular inclusion bodies. The inclusion deposits consist of branched polysaccharides (polyglucosans) but the responsible biochemical defect has not been identified. Onset is during late childhood or adolescence and the disease leads to a fatal outcome within a decade of first symptoms. We studied nine families in which Lafora's disease had been proven by biopsy in at least one member. In order to locate the responsible gene, we screened the human genome with microsatellite markers spaced an average of 13 cM. We used linkage analysis in all nine families and homozygosity mapping in four consanguineous families to define the Lafora's disease gene region. Two point linkage analysis resulted in a total peak lod score of 10.54 for marker D6S311. Six additional chromosome 6q23-25 microsatellites yielded lod scores ranging from 5.92 to 9.60 at theta m = f = 0. An extended pedigree with five affected members independently proved linkage with peak lod scores over 3.8 at theta m = f = 0 for D6S292, D6S403, and D6S311. The multipoint one-lod-unit support interval covered a 2.5 cM region surrounding D6S403. Homozygosity mapping defined a 17 cM region in chromosome 6q23-25 flanked by D6S292 and D6S420 that contains the Lafora's disease gene.

Chromosome Mapping↗
PubMed1995

Clinical experience with headaches in preadolescent children.

We have reviewed the diagnoses of 654 children aged from 7 to 14 years who attended a neurologist for headache evaluation. Headaches beginning between the age of 7 and 14 represented a higher percentage (18.3%) than the proportion of preadolescent children in our health area (12.9%). Headaches were more common in girls; although cluster, posttraumatic, benign exertional headaches, and the only case of brainstem glioma were restricted to boys. Despite the female predominance, the proportion of males with migraine was significantly higher in the preadolescents than in the over 15 age group. Migraine accounted for the majority of diagnoses (609-93% of the total series), while tension-type headache (27-4%), and headache associated with sinus infection (7-1%) were the diagnoses which followed in frequency. There were only two headaches (0.3%) associated with intracranial masses. Even though, in terms of frequency, headache is a very common reason for neurology consultation, the present results show that the majority of preadolescents consulting because of headache suffer from benign conditions.

Adolescent↗
PubMed1994

Changes in aminergic receptors in a PSP postmortem brain: correlation with pathological findings.

The state of different aminergic receptors was assessed, by quantitative autoradiography in tissue sections, in several representative brain regions from a typical progressive supranuclear palsy (PSP) patient and from 9 matched brains. The densities of muscarinic receptors were within control limits in most of the brain areas of this PSP brain. Serotonin1 receptors were clearly reduced only in areas with very relevant neuropathological damage, such as locus niger and globus pallidus. The density of D1 dopamine receptors in the caudate-putamen and frontal cortex of the patient was within control limits. By contrast, nigral D1 and striatal D2 dopamine receptors were dramatically reduced in the patient as compared to controls. Finally, alpha 2-adrenoceptors were clearly reduced in all the examined areas of this PSP patient as compared to control group. Both the potential role of these receptor changes in the pathophysiology of the clinical features of PSP and their correlation with the neuropathological findings of this PSP patient are discussed.

Aged↗
PubMed1994

Primary leptomeningeal lymphoma presenting as cerebellopontine angle lesion.

We report a primary leptomeningeal lymphoma (PLML) presenting as a cerebellopontine angle lesion. CT showed slight enlargement of the ventricular system, obliteration of the basal cisterns and a dense lesion in the left cerebellopontine angle which enhanced with contrast medium. Cerebrospinal fluid abnormalities included sterile lymphocytic pleocytosis without malignant cells, low sugar and high adenosine deaminase levels. An erroneous diagnosis of tuberculous meningitis was made, but autopsy revealed a leptomeningeal B-cell lymphoma with infiltration of the middle cerebellar peduncle giving the appearance of a cerebellopontine angle lesion. Seven cases of cerebellopontine angle lymphoma have previously been described, only one of which could be classified as PLML.

Adult↗
PubMed1994

Adrenergic receptors in the cerebellum of olivopontocerebellar atrophy.

Using autoradiographic techniques we studied the changes that in adrenergic receptors occurred in the cerebellum of two olivopontocerebellar atrophy (OPCA) patients as compared with a control group. In OPCA cerebellum the densities of total beta-adrenoceptors were reduced along the cortex but increased in the white matter. Although mainly the beta 1 subtype was decreased along the cerebellar cortex, the increase of beta-receptors over the white matter was due to a selective raise in the beta 2 subtype. These findings suggest a postsynaptic neuronal location for the beta 1 subtype and a glial location for the beta 2-adrenoceptor. On the other hand, alpha 2-adrenoceptors were clearly reduced all along the cerebellar cortex of these OPCA brains, this probably being secondary to the loss of presynaptic adrenergic terminals arising from the locus coeruleus. These results help clarify both the subcellular location of adrenoceptors in human cerebellum and the neurochemical pathophysiology of OPCA.

Aged↗
PubMed1994

Isolated headache as the presenting clinical manifestation of intracranial tumors: a prospective study.

We prospectively studied over two years the incidence of headache as the initial and isolated clinical manifestation of adult patients suffering from intracranial tumors (n = 183). Fifteen patients (8%) exhibited headache as their first and isolated clinical manifestation. Age, sex, neoplasm localization, or pathological diagnosis did not correlate with the presence of headache. Posterior fossa location and hydrocephalus, though not reaching statistical significance, were more frequent in patients who presented with headache as the first symptom. At the moment of diagnosis, 59 (31%) of the patients admitted to headache, though only 1 out of the 15 patients starting as headache still had this symptom as the only manifestation. From our experience in adults, isolated headache for longer than 10 weeks will only exceptionally be secondary to an intracranial neoplasm.

Adolescent↗
PubMed1994

Guillain-Barré syndrome in Cantabria, Spain. An epidemiological and clinical study.

Seventy-one patients with Guillain-Barré syndrome (GBS) were retrospectively selected from within a defined area (Cantabria) in northern Spain, from 1975 to 1988. Excluding two non-resident cases, epidemiological analysis was based on 69 cases. The annual incidence rates were stable during the 14-year period of study with an average incidence of 0.95 (age-adjusted, 0.86) cases per 100,000 population. No significant difference was found for sex, urban or rural residence and there was no significant seasonal clustering. Antecedent event were recorded in 57% of patients, the most frequent events being upper respiratory infection and gastroenteritis. No association between use of gangliosides and the syndrome was found. Eight patients had variant syndromes including Fisher's syndrome (2 cases), and axonal (4 cases) and sensory (2 cases) GBS. Recurrences occurred in 3 cases. Excluding nine patients with incomplete follow-up and two with Fisher's syndrome, clinical analysis was based on 60 cases. Patients were divided into three groups as a function of their peak weakness. Significant features of the severe group were a requirement for ventilation, presence of bulbar palsy or dysautonomia and a longer duration of the plateau phase. However, it was not possible at an early stage of the clinical course to predict future motor deficit. Four (6.7%) patients belonging to the severe group died during the acute phase of the disease. No specific treatment for GBS was given. Outcome was assessed by means of serial examination up to 24 months after the onset of symptoms using a functional scale. At 3, 6 and 24 months 70%, 46% and 12% of patients, respectively, had a poor outcome.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗