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Biomedical subjects

E Fernandez-Alvarez

Publications and source records attributed to E Fernandez-Alvarez.

25 records · Page 2Linked to original sources

[Electroencephalographic morphology of seizures in full-term newborn infants].

e studied the morphology of the electrical discharges of 63 newborns who presented an electrical or electro-clinical status of known aetiology. The seizures have been classified as: discharges of beta, alpha, theta and delta frequencies; discharges of diphasic spikes; discharges of "saw waves"; positive spikes or slow waves, burst-suppressions and extremely polymorph discharges. We confirm the existing opinion in the current literature that the discharges are really changeable and polymorphic, are modified during seizure and vary in morphology, voltage, frequency and polarity. A typical or characteristic pattern of the seizures of newborn does not exist although theta and delta frequencies are predominant. A correlation between voltage, frequency and duration of the electrical discharge seems to exist; the higher the frequency of the electrical discharge, the lower is its voltage and its duration and vice versa. The cortical immaturity of the newborn plays an important role in the morphological changes of the electrical discharges.

Alpha Rhythm↗

Chronic progressive encephalitis in children with x-linked hypogammaglobulinemia.

This report is on six cases of a chronic relentlessly progressive encephalitis occurring in boys with congenital hypogammaglobulinemia presumably of the x-linked type, which are thought to represent a separate neurological entity. Intellectual deterioration, dysarthria, spasticity, ataxia, optic atrophy and an increase of lymphocytes in the cerebrospinal fluid, were the main clinical signs. The pathological picture was that of a viral encephalitis, but all virological investigations on brain biopsies and CSF were negative. The significance of intra-cisternal tubuloreticular inclusions in brain endothelial cells, similarities with chronic rubella encephalitis, and the role of the immunological deficiency are discussed. Sofar, the cause of this new type of encephalitis remains obscure.

Adolescent↗

Uncommon case of type II glycogenosis.

The authors report an uncommon case of type II glycogenosis. An 8-year-old boy developed a slow progressive myopathy. Biopsy of skeletal muscle showed scarce lesions under the optic microscope but in 50% of the fibers the presence of vacuoles filled with glycogen under the electron microscope. Ultrastructural analysis of fibroblasts in culture showed numerous vacuoles filled with glycogen, characteristic of type II glycogenosis. Enzymatic analysis revealed that acid-alpha-glucosidase activity was normal in muscle tissues but deeply deficient in leukocytes and fibroblasts in culture. This is, as far as we know, the first case with such a discrepancy in the distribution of the enzymatic activity, and it underlines the necessity of investigating several tissues in atypical cases.

Biopsy↗

"Moya-moya' disease caused by cranial trauma.

A case of "moya-moya" disease of a 12-year-old boy is reported. The clinical history started at 3 years 2 months after cranial trauma. The patient developed mental retardation, hemiparesis and seizures.

Arterial Occlusive Diseases↗

[Lissencephaly: agyria. A study using the Golgi technic].

A case of lissencephaly (agyria) is reported in which the Golgi stain was used to study the fronto-parietal cortex. The external cellular layer, the so-called true cortex, was shown to be made up of neurons from the 5th and 6th layers of the normal cortex. The neurons in the much less cellular layer were shown to be large pyramidal cells with well-developed dendritic branching and spines. The deeper cellular layer was a neuronal pool without well-defined layering. All the neural forms from the normal cortex were represented. In this layer the inner neurons showed a less advanced stage of development than did those in the outer groups. In the external cellular layer there was a disorganization of neuronal disposition as well. Otherwise typical large and medium-sized pyramidal cells from the outer third showed apical dendrites directed towards deeper structures, and the basal dendrites ran through the marginal layer. Neurons with doulbe dendritic branching and fusiform neurons were present in an abnormal rotated position. In both cases, the axons always showed a descending direction. These findings add to existing knowledge concerning the anatomy of the lissencephalic cortex, although the exact interpretation of the abnormalities found in the external cellular layer remains speculative.

Cerebral Cortex↗