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Biomedical subjects

J Berciano

Publications and source records attributed to J Berciano.

At least 127 records · Page 7Linked to original sources
PubMed1994

Experience in the diagnosis of headaches that start in elderly people.

The diagnoses of the 193 patients aged 65 and over who attended the service of neurology in the past 15 years with de novo headache as their initial and main symptom were specifically analysed. Headaches beginning in elderly people represented only 5.4% of headaches in all ages, whereas 12.1% of the population in this health area was 65 or over. Head pain appeared more often in women (63%), although post-traumatic, cluster, and benign cough headaches were almost restricted to men. Tension type headache (83 patients, 43% of the aged patients) and idiopathic trigeminal neuralgia (36, 19%) were the most frequent diagnoses. Fifteen per cent of the elderly patients v 1.6% of patients under 65 presented headache secondary to serious conditions, such as stroke, temporal arteritis, or intracranial neoplasm. Only one patient over 65 met migraine criteria. It is concluded that whereas the incidence of patients with headache attending a general hospital decreases with age, the risk of headaches due to serious conditions increases 10-fold after 65.

Age of Onset↗
PubMed1994

Ageusia associated with thalamic plaque in multiple sclerosis.

Ageusia and the cheirooral syndrome developed in a patient with a relapse of multiple sclerosis. Magnetic resonance imaging revealed an area of demyelination in the thalamus. This lesion presumably affected the most medial part of the ventralis posterior nucleus, where taste information is located. Given the proximity of the taste area and somatosensory representation of the hand and oral cavity in the ventralis posterior nucleus, we propose that a diagnosis of thalamic lesion should be considered when ageusia occurs with the cheirooral syndrome.

Adult↗
PubMed1994

Tegmental pontine hemorrhage with pure trigeminal motor impairment.

We report a patient with a right dorsolateral pontine syndrome including right masticatory weakness without facial hypesthesia. Brain computed tomography showed a right dorsolateral pontine hemorrhage. Blink reflex study indicated preservation of spinal tracts and sensory nuclei of the trigeminal nerve. Right masseter electromyography revealed denervation. This patient provides a good correlation between clinical, radiological and electrophysiological findings and nuclear organisation of the trigeminal nerve.

Aged↗
PubMed1993

Axonal form of Guillain-Barré syndrome: evidence for macrophage-associated demyelination.

We report on the clinical, electrophysiological, and pathological findings in a patient with pure motor and axonal Guillain-Barré syndrome, who died 29 days after onset. There was marked reduction of compound motor action potential amplitudes and denervation potentials in the tibialis anterior muscle. Motor and sensory conduction velocities of median nerve were normal. Peroneal nerve was inexcitable at the ankle but its latency from knee to tibialis anterior was normal. F waves were absent or delayed. The major burden of pathological changes fell on ventral spinal roots. Fundamental lesions included segmental demyelination, axonal degeneration, widespread endoneurial lipid-laden macrophage infiltrates, remyelination, and clusters of small regenerating fibers. These findings suggest that axonal damage in the axonal form of Guillain-Barré syndrome is secondary to demyelination.

Axons↗
PubMed1993

Friedreich's ataxia presenting with pure sensory ataxia: a long-term follow-up study of two patients.

We describe two patients with Friedreich's ataxia whose presenting symptomatology was for years progressive tabetic ataxia. Based upon the initial clinical, electrophysiological and nerve biopsy data, a diagnosis of idiopathic sensory neuropathy was established. Subsequent examination of the kin showed that three sisters of case 1 had Friedreich's ataxia. Upon serial clinical and electrocardiographic study, both patients eventually developed a florid Friedreich's ataxia, including cardiomyopathy. Our findings indicate that at onset Friedreich's ataxia may be indistinguishable from sensory neuropathy and also that serial examination and investigation of kinship are essential steps for accurate diagnosis.

Adult↗
PubMed1993

Autoradiographic demonstration of loss of alpha 2-adrenoceptors in progressive supranuclear palsy: preliminary report.

We assessed, by quantitative autoradiography in tissue sections, the density of alpha 2-adrenoceptors in several representative brain regions from a typical progressive supranuclear palsy (PSP) patient and in 9 matched brains. The full agonist 3H-bromoxidine was used as a ligand. The density of alpha 2-receptors was dramatically reduced in all the examined brain areas of this PSP patient as compared to the control group. The locus ceruleus degeneration observed here is the most plausible explanation for this loss of alpha 2-receptors. Our data show that abnormalities in the noradrenergic system may justify some clinical features of the PSP clinical picture, this supporting the idea of further study of the clinical effects of noradrenergic drugs in PSP.

Adrenergic alpha-Agonists↗
PubMed1993

Reactive astroglia-neuron relationships in the human cerebellar cortex: a quantitative, morphological and immunocytochemical study in Creutzfeldt-Jakob disease.

In order to investigate the role of neuron-glia interactions in the response of astroglial to a non-invasive cerebellar cortex injury, we have used two cases of the ataxic form of Creutzfeldt-Jakob disease (CJD) with distinct neuronal loss and diffuse astrogliosis. The quantitative study showed no changes in cell density of either Purkinje or Bergmann glial cells in CJ-1, whereas in the more affected CJ-2 a loss of Purkinje cells and an increase of Bergmann glial cells was found. The granular layer in both CJD cases showed a similar loss of granule cells (about 60%) in parallel with the significant increase in GFAP+ reactive astrocytes. GFAP immunostaining revealed greater reactivity of Bergmann glia in CJ-2 than in CJ-1, as indicated by the thicker glial processes and the higher optical density. Granular layer reactive astrocytes were regularly spaced. In both CJD cases there was strict preservation of the spatial arrangement of all astroglial subtypes--Fañanas cells, Bergmann glia and granular layer astrocytes. Reactive Fañanas and Bergmann glial cells and microglia/macrophages expressed vimentin, while only a few vimentin+ reactive astrocytes were detected in the granular layer. Karyometric analysis showed that the increase in nuclear volume in reactive astroglia was directly related with the level of glial hypertrophy. The number of nucleoli per nuclear section was constant in astroglial cells of human controls and CJD, suggesting an absence of polyploidy in reactive astroglia. Ultrastructural analysis revealed junctional complexes formed by the association of macula adherens and gap junctions. In the molecular layer numerous vacant dendritic spines were ensheathed by lamellar processes of reactive Bergmann glia. Our results suggest that quantitative (neuron/astroglia ratio) and qualitative changes in the interaction of neurons with their region-specific astroglial partners play a central role in the astroglial response pattern to the pathogenic agent of CJD.

Astrocytes↗
PubMed1993

Relief of cluster-tic syndrome by the combination of lithium and carbamazepine.

This paper presents a further case of cluster-tic syndrome. Cluster headache and trigeminal neuralgia have coexisted in our patient for 18 years. Carbamazepine has selectively relieved the tic douloureux, while lithium has completely controlled the cluster headache. Our case shows again that the simultaneous occurrence of cluster headache and trigeminal neuralgia seems to be more than coincidental.

Carbamazepine↗
PubMed1993

Hereditary "pure" spastic paraplegia: a study of nine families.

The genetic and clinical features of 46 patients in nine families with "pure" hereditary spastic paraplegia are described. Inheritance was autosomal dominant in seven families and autosomal recessive in two. In dominant kinships, five families corresponded to type I with onset below 35 years, and two to type II with onset over 35 years. In early onset dominant families, in spite of apparent complete penetrance before 20, variable expression and incomplete penetrance occurred. Irrespective of genetic type, serial evaluation revealed that the main symptom consisted of slowly progressive spastic gait, extremely variable in severity, associated in some patients with decreased vibratory sense and micturition disorders generally as late features. In dominant families, the disease tended to be more severe in late onset cases. No patient had symptoms in the upper limbs and plantar responses were flexor in six symptomatic patients. Central motor conduction time studied by transcranial magnetic stimulation was always normal in the upper limbs and increased in the lower limbs in five of the eight patients on whom it was performed. Monomorphic and stereotyped clinical pattern in this series does not support the concept of multisystem involvement of the central nervous system as a hallmark of the disease.

Adult↗
PubMed1993

A prospective study of stroke in young adults in Cantabria, Spain.

BACKGROUND AND PURPOSE: The aim of this study was to determine the incidence, type, and prognosis of stroke in young adults in Cantabria, Spain. METHODS: We investigated prospectively all patients aged 50 years or below who were admitted with the diagnosis of a stroke to the University Hospital "Marqués de Valdecilla" from April 1, 1986, to March 31, 1988. This is the main hospital of the region to which all patients with neurological problems are referred. These patients underwent a complete clinical and laboratory assessment for stroke and had 1-year mean follow-up. RESULTS: The total series included 81 patients. The annual age-specific crude incidence rates of stroke were 17.3 and 10.4 per 100,000 for males and females, respectively. Twenty-four patients (30%) were diagnosed as having nonembolic cerebral infarction, 14 (17%) embolic cerebral infarction, 20 (25%) subarachnoid hemorrhage, 22 (27%) spontaneous cerebral hemorrhage, and one case (1%) cerebral venous thrombosis. Eighteen patients (22%) died within 30 days of the cerebrovascular event, and two others died during the follow-up period. Seventy-nine percent of the survivors recovered and were completely self-sufficient. CONCLUSIONS: The incidence of stroke in the young found in Cantabria is comparable with that in previous studies. The initial hospital mortality was not negligible, but the prognosis among the survivors was favorable.

Adolescent↗
PubMed1993

Choreas, hereditary and other ataxias, tics, myoclonus, and other movement disorders.

Developments in the field of Huntington's disease have focused on the potential benefits of predictive testing. Markers have been described for autosomal dominant cerebellar ataxia and for certain subtypes of Friedrich's ataxia. Argentophilic neuronal and glial inclusions appear to be the first specific pathologic hallmark of multiple system atrophy. "Pure" hereditary spastic paraplegia is not a multisystem disorder of the central nervous system, but a monomorphic and stereotyped disease. Advances in Tourette's syndrome are limited because the presumed gene eludes identification. A new type of myoclonus, propiospinal myoclonus, has been described. Clinical and electrophysiologic criteria for defining primary orthostatic tremor have been proposed. Understanding of the neurophysiologic substrate of essential tremor and myoclonus is improving. New neurologic disorders presenting clinically with prominent movement disorder continue to be described.

Ataxia↗
PubMed1992

Duplication of part of chromosome 17 is commonly associated with hereditary motor and sensory neuropathy type I (Charcot-Marie-Tooth disease type 1).

Hereditary motor and sensory neuropathy type I (HMSNI), also known as Charcot-Marie-Tooth disease type 1 (CMT1), has been shown to be genetically heterogeneous. A major gene maps to chromosome 17 (CMT1A). A set of loci, D17S122, D17S125, and D17S124, show tight linkage to the CMT1A locus, and a duplication of D17S122 has been detected in some families. We show that the locus D17S122 is duplicated in affected individuals from 7 informative families with HMSNI. The duplication was demonstrated either by differences in hybridization densities between two bands of a restriction fragment length polymorphism or by the presence of all three alleles. No normal individual had the duplication. A single recombinant exists between the MspI polymorphism of D17S122 and the duplicated band, suggesting that the duplication is of considerable size. Patients with HMSN type II do not show the duplication. These findings will have considerable impact on the diagnosis of chronic demyelinating neuropathies, in patients with or without similarly affected relatives.

Charcot-Marie-Tooth Disease↗
PubMed1992

Dopamine D1 and D2 receptors in progressive supranuclear palsy: an autoradiographic study.

Dopamine D1 and D2 receptors were studied in brain tissue sections from a typical patient with progressive supranuclear palsy and in 7 age-matched brains. The density of D1 receptors in the caudate-putamen and frontal cortex of the patient was within control limits. By contrast, the density of nigral D1 receptors and striatal D2 receptors was dramatically reduced in the patient as compared to the control brains. This work shows again that the loss of striatal D2 receptors is the most plausible explanation for the poor response to dopaminergic drugs in patients with progressive supranuclear palsy. While the loss of nigral D1 receptors can be explained by the loss of nigral neurons, it seems that neurons bearing striatal D1 receptors are spared in progressive supranuclear palsy. The clinical effects of selective D1 agonists are worth testing in this devastating disorder.

Atrophy↗