Glycosaminoglycans of brain tumors transplacentally induced by ENU in the rat.

GAGs accumulate in the oligodendrogliomas and in the oligodendroglial component of mixed gliomas induced in rats by transplacental ENU. We studied the period between birth and the appearance of the earliest tumoral lesions and did not find any accumulation preceding the early neoplastic proliferations. These findings, together with the observation that GAGs are present only in the oligodendroglial component of tumors, are in agreement with our previous interpretation that the tumoral transformation of oligodendroglia takes place after it has acquired the ability to interfere with GAG metabolism during myelinogenesis.

The significance of minimal alterations in muscle biopsy of Duchenne carriers.

In order to define the significance of minimal histological and ultrastructural abnormalities in Duchenne carriers, 18 normal healthy volunteers were examined by muscle biopsy. Light microscopy evidenced occasional internal nuclei and less frequent small round and angular fibres. Variability of fibre size, increase in connective tissue, necroses and basophilia found in carriers were not present in controls.

Tissue alterations induced by radio- and chemotherapy in brain with malignant gliomas.

35 brains with malignant gliomas treated by surgery, radiotherapy and chemotherapy and processed by means of the "complete study" technique have been studied. The preliminary results are reported. Many types of changes have been observed.

Motor neuron disease following poliomyelitis. Bioptic study of five cases.

5 cases of late motor neuron degeneration following poliomyelitis are presented. CPK, CSF, EMG and spine radiology were studied. In all cases, muscle biopsy evidenced neurogenic alterations both in previously affected limbs and in newly affected ones.

Pathology of human malignant gliomas after radiation and chemotherapy.

The pathological effects of radio- and chemotherapy have been studied in 31 malignant gliomas. The brains have been examined by means of the complete study technique. Many histological features have been related to surgery, localization, preoperative duration, postoperative survival, irradiation, and chemotherapy.

Experimental brain tumors by transplacental ENU. Multifactorial study of the latency period.

Experimental cerebral tumors have been induced by transplacental ENU. The morphologic study of the brains of treated rats revealed that cellular hyperplasias appear at the 30th day of extrauterine life in the paraventricular white matter, i.e.

Mitochondrial abnormalities of late motor neuron degeneration following poliomyelitis and other neurogenic muscular atrophies.

A case of late motor neuron degeneration following poliomyelitis with abnormal mitochondria in muscle fibers is presented with two additional cases of systemic neurogenic muscular atrophy (Charcot-Marie-Tooth disease). Muscle biopsy revealed a neurogenic pattern of variable severity in all cases. Subsarcolemmal zones of hyperactivity and hyperpositive intermyofibrillar collections of granular material present in a variable proportion of type I fibers were demonstrated by oxidative enzymes.

[Roussy-Levy's syndrome: clinical aspects (author's transl)].

A case of non familial Roussy-Lévy syndrome is presented: optic atrophy, gait ataxia, bilateral hypotrophy of thenar and hypothenar muscles, weak deep tendon reflexes of the upper limbs, weak knee jerks, absent ankle jerks, bilateral Babinski, anapallaesthesia from the bisiliac spine distally, bilateral pes cavus. The literature on Roussy-Lévy syndrome is discussed and whether this syndrome should be considered as a precise entity among heredo-degenerative diseases. The overlapping of differing clinical features may suggest an intermediate form between Friedreich's and Charcot-Marie-Tooth's diseases.

Analysis of some factors effecting survival in malignant gliomas.

Many factors, including the histological aspect, are known to effect the survival of patients with malignant gliomas. The relation between survival and diagnoses such as primary and secondary glioblastoma, anaplastic astrocytoma, etc., is not definitely clear.

Cerebral tumours induced by ENU; changes of adenylate cyclase activity in the tumour latency time.

Tumours of the nervous system have been induced by transplacental ENU. Until the fourth month of life the tumoural lesions appear as mixed glial proliferations or oligodendroglial foci. From the fourth month on they develop as glial micro- and macrotumours or as isomorphic and polymorphic oligodendrogliomas.

[Chemotherapy of cerebral tumours: data on malignant gliomas (author's transl)].

The Authors, after briefly reviewing the literature on chemotherapy of malignant gliomas, present their own experience on forty-four patients with such tumors. All of them had been previously operated and irradiated. The clinical course was controlled by various parameters which are described.

Human malignant gliomas treated with chemotherapy: a pathological study.

Twenty-one malignant cerebral tumors treated with chemotherapy after operation or clinical diagnosis were studied. The brains were cut in coronal slices that were embedded in paraffin. The histological picture of the whole tumor and of the adjacent normal tissue was carefully investigated, and it was compared with the histological features noted on biopsy.

Acute muscle necrosis after chronic overdosage of phenformin and fenfluramine.

A case of acute muscle necrosis with probable myoglobinuria is presented. The patient had been taking excessive doses of phenformin and fenfluramine, and a toxic etiology is suggested. Muscle necrosis is tentatively attributed either to the combined effect of these drugs on some underlying biochemical abnormality of muscle or to a defect of oxygen utilization secondary to myoglobin alterations.

Central core disease: histochemical and ultrastructural study of muscle biopsies of father and daughter.

Two cases of central core disease, father and daughter, of a family with dominant autosomal inheritance, are presented, one with bilateral congenital dislocation of the hip. Muscle biopsy was performed in both cases. Oxidative enzymes evidenced only type I fibers, most of them presenting a central core and not uncommonly more than one.

Cerebral tumors induced by transplacental ENU: study of the different tumoral stages, particularly of early proliferations.

Experimental cerebral tumors have been induced by transplacental ENU. A systematic diachronic study of the brains has been performed starting from the 15th day of extrauterine life. The tumoral lesions firstly appear as "early stage proliferations" or oligodendroglial foci and develop as glial micro- and macrotumors or as isomorphic and polymorphic oligodendrogliomas respectively.

Sporadic oculopharyngeal myopathy with abnormal mitochondria.

A case of late onset ophthalmoplegia and dysphagia is presented. Serum enzymes, ECG, EEG, thyroid function and edrophonium test were normal. Muscle biopsy revealed the presence at oxidative enzymes of 5% of fibres displaying subsarcolemmal hyperactivity and a coarse network pattern, mainly involving type II fibres.

Histochemical and ultrastructural findings in a case of centronuclear myopathy.

A case of centronuclear myopathy is presented. The presence of central nuclei in almost all fibres, the existence of type I fibres only, the histochemical pattern of a negative central zone with a perinuclear halo and a hyperactive rim with oxidative enzymes and the ultrastructural data are discussed in the light of the previous literature. The possible relationships with other myopathies are taken into consideration as well as the fact that central nuclei may be a non-specific change in several conditions.

Chemotherapy studies of various nitrosoureas: prevention of development of ethylnitrosourea-induced tumors in the nervous system.

Of four nitrosoureas administered to F344 rats that were offspring of mothers treated with ethylnitrosourea during pregnancy, 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea and its methyl derivative were particularly effective in blocking tumor development.