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Biomedical subjects

J Berciano

Publications and source records attributed to J Berciano.

At least 199 records · Page 11Linked to original sources
PubMed1985

Serial waking-sleep EEGs and evolution of somatosensory potentials in Creutzfeldt-Jakob disease.

We have analysed the serial sleep and waking EEGs of 3 patients with Creutzfeldt-Jakob disease (CJD), demonstrated by pathology studies. The presence of periodic complexes (PCs) was a constant finding in all the records, including those made on admission (1-3 months after the onset of symptoms) although at this stage the PCs were not as persistent of generalized as in the more advanced stages. The physiological states of sleep were replaced by cyclic changes which initially consisted of phases with PCs alternating with theta-delta activity. As the disease progressed, brief periods of suppression of background rhythm were added, followed by slower PCs. In case 3, the SEPs obtained on stimulating the median nerves were normal at the first test. However, 17 days later a further test revealed two types of abnormality. On the one hand, using a non-cephalic reference, the SEPs were of lesser amplitude and had become disorganized. On the other hand, with a cephalic reference, a potential of enormous amplitude was obtained unilaterally. On the contrary, the SEPs obtained with a cephalic reference on stimulating the posterior tibial nerves were of small amplitude and disorganized. These findings, in combination with the EEG anomalies, suggest a progressive dysfunction of the cerebral cortex. The recording of a giant potential only at C'3-Fz on stimulating the median nerve could be related to a pathological facilitation of long-loop transcortical stretch reflexes of the hand.

Aged↗
PubMed1985

Late onset epileptic seizures. A retrospective study of 250 patients.

A retrospective study of 250 patients with late-onset epilepsy was carried out. The ages ranged from 22 to 88. The seizures were partial in 104 patients and generalized in 146. The neurological examination was abnormal in 41 patients and normal in 209. The EEG studies and CT scan revealed abnormalities in 76.5% and 50.8% respectively. The most frequent CT scan findings were diffuse atrophy (19.2%), tumors (16.4%) and cerebral infarct (8.8%). The clinical parameters which best predicted the CT scan abnormalities were an abnormal neurological examination and simple partial seizures. In seven of the 45 patients with space-occupying lesions, the clinical examination and EEG were normal. The etiology of the convulsions was established in 201 patients, the most frequent cause being chronic alcoholism (62 cases), tumors (41 cases), postischemic vascular epilepsy (33 cases) and postraumatic epilepsy (28 cases). We conclude that a CT scan is essential in the assessment of patients with epileptic convulsions of late onset, even when the EEG and clinical examination are normal.

Adult↗
PubMed1984

Axon membrane remodeling in the lead-induced demyelinating neuropathy of the rat.

Single-teased fibers stained with the ferric ion-ferrocyanide method allowed us to study axonal remodeling in the lead-induced demyelinating neuropathy of the rat. Our findings, in agreement with recent physiological data, pointed to a transitory reorganization of the demyelinated axons to maintain impulse conduction until remyelination and formation of new cytochemically normal nodes had restored a secure saltatory conduction.

Animals↗
PubMed1983

Dominantly inherited motor and sensory neuropathy type I. Genetic, clinical, electrophysiological and pathological features in four families.

This report describes the genetic, clinical, electrophysiological and sural nerve biopsy features in 26 affected members and 21 unaffected relatives from 4 families with autosomal dominant inherited motor and sensory neuropathy (HMSN), Type I. In all age categories, the proportion of affected to unaffected individuals at risk did not significantly differ from the expected 50%. The peak age of onset was in the first decade. There was a complete concordance between nerve conduction velocity in the propositi and that in their affected relatives within each family. Marked slowing of conduction velocities was present as early as the age of 2.5 years, while precocious clinical signs and symptoms were quite subtle. Determination of conduction velocity is a valuable aid aid to the early diagnosis of the disease.

Biopsy↗
PubMed1983

Occipital dysplasia and Chiari type I deformity in a family. Clinical and radiological study of three generations.

Three generations of a family affected by a craniocervical malformation (CCM) were subjected to clinical and radiological studies. Occipital dysplasia (OD) and Chiari type I deformity (CD.I) were the main features, inheritance being autosomal-dominant. The malformation was variably expressed; it ranged from OD with basilar impression (BI) to OD without BI and from CD.I with OD to CD.I without obvious osseous malformation. Its pathogenesis, and that of other related familial disorders (Klippel-Feil syndrome and syringomyelia), is discussed, the conclusion being drawn that all were elements of one genetic disorder which finds expression in a very variable sequence. The value of high-resolution CT in the detection of asymptomatic carriers is emphasized.

Adult↗
PubMed1983

Voluminous benign osteoblastoma of the skull.

A case of benign osteoblastoma of the skull is reported. The clinical picture began when the patient was 15 years old, with exophthalmos and blindness in the right eye. After the enucleation of this eye, the only symptoms were repetitive convulsive seizures. Radiologic studies done when the patient was 76 years old, and a postmortem examination following her death at this age, revealed a voluminous osteoblastoma localized in the anterior cranial fossa and invading the structures below. The clinical characteristics of osteoblastomas of the skull are briefly reviewed.

Aged↗
PubMed1983

Livedo reticularis and cerebrovascular lesions (Sneddon's syndrome). Clinical, radiological and pathological features in eight cases.

Eight patients with diffuse livedo reticularis and cerebrovascular lesions (Sneddon's syndrome) are reported. The disorder was inherited by autosomal dominant transmission in 3 cases. Multiple occlusions in medium-sized arteries were demonstrated by cerebral and hand arteriograms. Digital artery biopsies showed intimal hyperplasia in 7 cases and recanalized thrombosis in one case. Our findings are compared with an extensive review of the literature. Differential diagnosis with other vascular disorders, especially cerebral thromboangiitis obliterans and the corticomeningeal angiomatosis of Divry and Van Bogaert is considered. We conclude that Sneddon's syndrome is a new genetic and progressive arteriopathy, occlusive and noninflammatory, involving medium-sized vessels. The pathogenesis has yet to be elucidated.

Adolescent↗