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Biomedical subjects

F Vassella

Publications and source records attributed to F Vassella.

At least 73 records · Page 4Linked to original sources

Elevated CSF cyclic AMP concentrations in patients with inflammatory diseases of cerebral and extracerebral origin.

Cyclic 3'-5' adenosine monophosphate (c-AMP) concentrations were measured in cerebrospinal fluid (CSF) from children admitted to the hospital because of suspected meningitis. c-AMP levels were found to be markedly elevated (P < 0.001) during the acute phase of most of the purulent meningitis patients, as well as in patients with acute aseptic meningitis. In convalescent patients after purulent meningitis mean c-AMP concentrations remained elevated (P < 0.01) beyond the normalization of the routine parameters in CSF. In addition, a variety of febrile inflammatory conditions of extracerebral origin produced elevations (P < 0.001) of c-AMP although CSF by routine criteria was normal. The results suggest that c-AMP might serve as a sensitive indicator of transient cellular metabolic disturbance in the brain.

Adolescent↗

Complicated migraine (migraine accompagnée) in children. Clinical characteristics and course in 40 personal cases.

Forty cases of complicated migraine (c.m.) were analysed. The onset was before the age of 16 years, in the majority, however, after the 10th year. In 20 patients the first crisis of c.m. occurred in the absence of a previous history of migraine. 38 of the children had paresthesia during the crises, localized mostly to one hemisoma or a part thereof, in 3 bilateral from the beginning. In the majority of these patients the same hemisoma was always affected, the upper limb almost always. The preferred localisation of paresthesia in the face were the mouth and/or the tongue. A progression of paresthesia with a "march" of several minutes duration was frequent. In 5 children a paresis occurred, mostly at the upper part of one hemisoma, in another 4 children hemiplegia was present. In several cases signs of brainstem lesion occurred. Headache was mostly localized on the opposite side to the neurological signs. Vomiting and scotoma were frequent. In EEG done during the periods of crisis anomalies were demonstrated in the majority of our patients with a predominance of diffuse or focal slowing. In our patients there was a spontaneous tendency for c.m. to disappear upon reaching adult age. None of our 25 patients who had a neurological examination at the last check-up has shown residual deficits.

Adolescent↗

Hypodipsia-hypernatremia syndrome.

The pathogenesis of the rare hypernatremia, usually described in the literature as "neurogenic" or "essential" hypernatremia, consists of defective thirst mechanism either alone or in combination with impaired osmoregulation of ADH release. As etiology, disturbances of the neoplastic, vascular and degenerative type and malformations in the hypothalamic area are known. In patients with the hypodipsia-hypernatremia syndrome, dysfunction of the anterior pituitary lobe, obesity, abnormal regulation of body temperature, psychomotor retardation and episodic muscular weakness are frequently encountered as additional abnormalities. A 6-year-old patient is described with hypodipsia-hypernatremia syndrome manifest for 3 years. Besides hypernatremia, hypodipsia and the relative insensitivity of the osmoreceptors regulating ADH release, elevated body temperature, polyphagia and obesity, partial hypothalamic-hypophyseal dysfunction, lethargy and psychomotor retardation are the principal findings. An inflammatory lesion or one occupying an intracranial space was not demonstrable until now. Under forced water intake and hypocaloric diet the patient has progressed well with nearly complete normalization of the hypernatremia, body temperature and obesity.

Child↗

Combination chemotherapy with VM 26 and CCNU in primary malignant brain tumors.

Twenty-seven patients with inoperable or recurrent primary malignant brain tumors after previous resection and/or radiotherapy, were treated with corticosteroids and a combination chemotherapy consisting of VM 26 and CCNU. There were 15 (55%) patients that responded, three with a complete and 12 with a partial response with a mean duration of response of 13.4 months. Toxicity was mainly hematological and of acceptable degree. Combination chemotherapy with VM 26 and CCNU is well tolerated and can be administered on an out-patient basis. In our experience it is better than single agent chemotherapy.

Adolescent↗

[Double-blind study on the anti-convulsive effect of phenobarbital and valproate in the Lennox syndrome].

In a double-blind crossover trial valproate was compared with phenobarbital with regard to anticonvulsive activity and tolerance in 17 epileptic children (mean age 55 +/- 26 months) with Lennox syndrome. Valproate in association with a phenobarbital dose reduced by about 40% proved to be to a statistically significant degree more active against epileptic seizures than phenobarbital alone. No difference in the effect on the EEG tracings was observed. Valproate appeared to be somewhat more active than phenobarbital with regard to behaviour, but the difference was not significant. Tolerance to both products was equally good.

Benzodiazepines↗

The syndrome of 'continuous muscle fiber activity.'.

A 7-year-old boy who suffered from increasing stiffness and contractures of the extremities had distally pronounced atrophy and absent tendon reflexes. Electromyography showed continuous electrical activity during rest, sleep, after intravenous injection of diazepam, and after peripheral nerve block. The H reflex was elicitable; the silent period after the reflex was absent. Histopathological examination of the peroneus muscle disclosed a marked preponderance of type I fibers and slight atrophy of the type II fibers. Electron microscopic examination of the endplates demonstrated a marked atrophy of the postsynaptic regions and widened synaptic clefts. After one year's treatment with phenytoin, 200 mg daily, the patient showed an almost normal muscle tone. As not all of these electrophysiological phenomena can be fully explained by disturbances of the nerve terminals or the endplates, a further anomaly proximal from the peripheral nerve block seems to have been present.

Child↗

Adjuvant chemotherapy with intraventricular methotrexate and CCNU after surgery and radiotherapy of medulloblastomas.

Seven patients were treated with a combination of intraventricular methotrexate (MTX) and oral CCNU after resection and whole CNS irradiation of medulloblastomas. 3 patients survived 3 or more years after the operation which is not different from reported series with postoperative radiotherapy only. Adjuvant chemotherapy is experimental and should be employed only in controlled studies. The inadequate bone marrow reserve after previous spinal irradiation is a major problem. Because of its delayed and unpredictable myelotoxicity, CCNU is in our experience not a suitable substance for adjuvant combination chemotherapy of medulloblastomas. No complication was observed with 81 intraventricular injections of MTX. It is a good agent and deserves further clinical trials in less myelotoxic combinations.

Brain Neoplasms↗

[Visual discrimination conditions in infants with increased risk for cerebral damage].

Infants judged at birth to be at risk for cerebral damage show at 2 to 4 months of age significant differences compared to normal controls with respect to visual behavior at presentation of geometric patterns: Children of the risk group discriminated less consistently between a striped pattern and a homogenous gray surface. Their average time of fixation is significantly longer, and they show increased variance within the test parameters. It was therefore concluded that parameters of visual fixation and discrimination could probably allow for more reliable prognoses for future cognitive development in an infant. A longitudinal study to test this hypothesis is already in execution.

Brain Damage, Chronic↗

Development of visual discrimination (pattern preference) in normal infants.

Visual discrimination for vertical stripes versus an unpatterned visual stimulus (plain grey) was studied longitudinally in 75 "normal" infants born at term, at monthly intervals from 1 to 6 months of age. Total fixation time was found to progressively decrease with increasing age, with a very sharp jump between the values of 2 and 3 months. Correspondingly, the mean duration of a single fixation decreased. The number of single fixations increased by age, doubling at 6 months in comparison to 1 month. A preferential fixation for stripes was already evident at the age of 1 month. A probabilistic method for assessing discrimination in individuals is proposed. The values will be correlated with follow-up results of cognitive development until school-age.

Age Factors↗

Generalized gangliosidosis: acid beta-galactosidase deficiency with early onset, rapid mental deterioration and minimal bone dysplasia.

This report concerns a 3-month-old girl with rapidly progressive psychomotor retardation, hepatomegaly, vacuolated lymphocytes, minimal bone dysplasia and normal excretion of acid mucopolysaccharides. A deficiency of acid beta-galactosidase was demonstrated in isolated leucocytes and in a liver biopsy. The diagnosis of generalized gangliosidosis due to deficiency of beta-galactosidase was also based on the absence of the enzyme activity from cultured fibroblasts. The diagnosis was confirmed on autopsy at 16 months by typical histology, electron microscopy and biochemistry of the organs. beta-galactosidase deficiency has been demonstrated in various clinical conditions ranging from generalized gangliosidosis with severe mental retardation to clinical pictures resembling Morquio's disease and normal intelligence. The heterogeneity of the clinical manifestations in beta-galactosidase deficiency could be explained by different residual activities of a structurally mutated enzyme towards its various substrates.

Bone Diseases, Developmental↗