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Biomedical subjects

F Vassella

Publications and source records attributed to F Vassella.

At least 55 records · Page 3Linked to original sources
PubMed1985

Heterogeneity of congenital motor and sensory neuropathies.

Six children suffering from a congenital motor and sensory neuropathy (CMSN) are described. Severe muscle hypotonia, areflexia and a delay of motor development are detectable in all of them. Sural nerve biopsies exhibited an almost complete absence of myelinated fibres and a correspondingly slow nerve conduction velocity (NCV) of less than 10 m/s was detectable in four patients. A few segments with hypermyelination adjacent to gross hypomyelination were seen in the fifth patient, and the NCV was 15 m/s. The sural nerve of the sixth patient showed a loss of thick myelinated nerve fibres, and his NCV was 25 m/s. These results demonstrate the histological heterogeneity of CMSN which was already detected by the NCV. The relation of our findings to the classification of HMSN by Dyck and Lambert (1968) is discussed.

Child↗
PubMed1985

Benign migraine-like syndrome with CSF pleocytosis in children.

Four children with symptoms consistent with complicated migraine had CSF pleocytosis, in three cases lymphocytic and in the fourth polymorphonuclear. In one case the CSF abnormality was found during two separate attacks of complicated migraine. On the basis of the 23 cases reported in the literature and these four personal cases, the authors conclude that, as a rule, pleocytosis is a secondary phenomenon of an attack of complicated migraine. However, in exceptional cases an infectious disease might produce both a migraine attack and CSF pleocytosis.

Adolescent↗
PubMed1984

Synopsis: gangliosidoses.

Gangliosidoses are very rare neurological diseases based on specific enzyme defects. They constitute models for the disruption of specific metabolic pathways and cellular functions with the ultimate consequence of manifest clinical symptoms. The investigation of the various steps involved in the generation of a given syndrome can therefore lead to a more profound understanding of the cell biology of the nervous system. In the present synopsis we try to briefly summarize some aspects of the present knowledge of pathophysiological mechanisms in GM1- and GM2-gangliosidoses.

Brain↗
PubMed1984

Electroencephalographic findings in children treated with cytotoxic agents.

106 EEG investigations were carried out in 17 children with various types of neoplastic disease without cerebral involvement during one or more courses of treatment with cytotoxic agents. EEGs were recorded before and 24 h after administration of the drugs. A transient slowing of the dominant frequency in the alpha-band by about 1 c/s and a decrease in the relative power of alpha-activity by 20-30% was observed in only 4 patients. These children did not receive the same antineoplastic treatment. 1 patient received very high dose methotrexate, 2 patients received vincristine combined with other cytotoxic agents, and the other patient received L-asparaginase. It is suggested that EEG changes in patients receiving intravenous cytotoxic treatment usually occur only where there is a preexisting impairment of the blood-cerebrospinal fluid barrier or the blood-brain barrier.

Adolescent↗
PubMed1983

Brain-stem auditory evoked potentials and early somatosensory evoked potentials in neurointensively treated comatose children.

Forty-three comatose children treated by invasive neurointensive care were examined by brain-stem auditory evoked potentials (BAEPs) and somatosensory evoked potentials (SEPs). The evoked potential (EP) results obtained were reviewed in conjunction with the clinical outcome. As a BAEP criterion, the V-I interpeak latency and, as an SEP criterion, the central conduction time (N20-N14 latency) were measured. A loss of BAEP and SEP components portended a poor prognosis. On the other hand, latency prolongations were seen in a third of patients who made a complete recovery. However, the SEP and BAEP have proved themselves as reliable methods in the judgment of the clinical state and the prognosis of intensively treated children with a hypoxic-ischemic encephalopathy or a head injury. The measurement of both BAEPs and SEPs was a more reliable prognostic aid than either EP measurement alone.

Adolescent↗
PubMed1983

Hereditary motor sensory neuropathies in childhood.

Clinical data on 24 patients with hereditary motor sensory neuropathies, with onset in the paediatric period, and of their relatives, is reported. Electrophysiological studies were done in all patients and in 15 relatives. The patients were divided into two groups (Types I and II) and their hereditary trait was determined. In 11 patients a sural nerve biopsy was performed and revealed different patterns of histological alterations. The nerve biopsy always confirmed the value of conduction velocity in distinguishing between Types I and II. A genetic discordance was observed, both in regard to the phenotype and the conduction velocity, and there was increased slowing of the conduction velocity as individuals grew older. Thus the classification of these disorders in childhood can be particularly difficult. The rôle of sural nerve biopsy is discussed.

Adolescent↗
PubMed1982

Conventional and spectral EEG analysis in children treated with cytotoxic agents.

One hundred and six EEG investigations were carried out in 17 children with various types of neoplastic disease without cerebral involvement during one or more courses of treatment with cytotoxic agents EEGs were recorded before and 24 hr after administration of the drugs. The EEGs were evaluated visually and by spectral analysis. A transient slowing of the dominant frequency in the alpha band by about 1 Hz and a decrease in the relative power of alpha activity by 20-30% was observed in only 4 patients. These children did not show any clinical or biochemical signs of neurotoxicity. The children did not receive the same antineoplastic treatment. One patient received very high dose methotrexate, 2 patients received vincristine combined with other cytotoxic agents and the other patient received L-asparaginase. It is suggested that EEG changes in patients receiving intravenous cytotoxic treatment usually occur only where there is pre-existing impairment of the blood--cerebrospinal fluid barrier or blood-brain barrier. No clinical signs of epilepsy, new epileptiform waves in the EEG or long-term changes in the background activity of the EEG were observed in this pilot study.

Adolescent↗
PubMed1981

Phenytoin therapy for epileptic children: evaluation of salivary and plasma concentrations and of methods of assessing compliance.

Monitoring phenytoin therapy in children is difficult because they fear blood sampling, yet frequent control of phenytoin levels may be necessary because of the age-dependence of phenytoin metabolism. In 22 patients aged between six and 15 years, phenytoin concentrations were studied in mixed saliva and parotid saliva and were compared with plasma levels from blood obtained at the same time. For mixed saliva, the saliva: plasma concentration ratio was 0.11 +/- SD 0.04 and in parotid saliva it was 0.10 +/- 0.06. In addition, compliance was studied in 13 children, each receiving between three and seven different quantities of phenytoin. Clinical judgement about compliance agreed well with the two laboratory measures. A graphic analysis of the relationship between plasma concentration and dosage is proposed as practical method of assessing compliance, because it appears to be both simple and reliable, and therefore suitable for routine use.

Adolescent↗
PubMed1981

Neurological complications in hemophilia.

The clinical data of 59 patients with hemophilia A or B are reviewed. Intracranial bleeding was observed in 6 patients and a minor bleeding episode was assumed in a further 8 patients. Neurosurgical evacuation of the hematoma was necessary in 2 cases and the remaining patients were treated solely with factor VIII or IX. In 10 patients a peripheral nerve lesion was observed, paresis of the femoral nerve being the most frequent (5 cases). Two patients showed a lesion of the lumbar and sacral plexus, 2 patients a lesion of the radial nerve and one patient a lesion of the cutaneous femoris lateralis nerve.

Adolescent↗
PubMed1981

Adjuvant chemotherapy with procarbazine, vincristine and prednisone for medulloblastomas. A preliminary report.

The survival of 20 children with medulloblastoma who received adjuvant chemotherapy with procarbazine, vincristine and prednisone after resection and craniospinal irradiation is compared with the preliminary results of the Children's Cancer Study Group (CCSG) and the international Society of Pediatric Oncology (SIOP) medulloblastoma studies. Survival with the chemotherapy used in the SPOG study ws not superior to the survival of children who received craniospinal irradiation only.

Cerebellar Neoplasms↗