Mitochondrial tRNA(Cys) gene mutation (A5814G): a second family with mitochondrial encephalopathy.

We report an Italian family with maternally inherited encephalomyopathy including progressive external ophthalmoplegia, seizures, and neurophysiological evidence of brainstem dysfunction. Mitochondrial DNA analysis showed a heteroplasmic point mutation at position 5814 in the tRNA gene for cysteine (A5814G), previously reported in a 5-year-old girl of Portuguese origin. The mutation was very abundant (> 95%) in both muscle and blood from the proposita and was present in lower proportions (average 85 +/- 6%) in blood from three less severely affected maternal relatives. This observation confirms pathogenicity for the A5814G mutation.

The transdural extension of gliomas.

The extraneural spreading of gliomas is an infrequent occurrence which is not necessarily related to either tumor histology or site. This paper reports two cases, a glioblastoma and an oligodendroglioma, both presenting extradural diffusion. In the first case, where there was severe intracranial hypertension, the tumor found its way out from the neurocranium, far from the site of the operation, perforating the dura and the bone of the cranial base. In the second case, the operation may have facilitated the extraneural invasion. This unusual behaviour of glial tumors is probably less rare than presumed. It may go unnoticed if the attention is concentrated on the usually severe neurological syndrome which is present in these patients.

Lonidamine in the combined treatment of malignant gliomas. A randomized study.

Lonidamine (LND) is a drug that interferes with energy metabolism of cancer cells, principally inhibiting aerobic glycolytic activity, by its effect on mitochondrially-bound hexokinase (HK). In such way LND could impair energy-requiring processes, as recovery from potentially lethal damage, induced by radiation treatment and by some cytotoxic drugs. A randomized study started in November 1983, to evaluate the efficacy of LND in association with radiotherapy as first line treatment in malignant gliomas, after surgical procedure. LND was also used in association with Lomustine (CCNU) at the moment of documented clinical and neuroradiological recurrence. At the present time 60 patients entered the study, and 47 are evaluable. Present preliminary results are not statistically significant, however indicate that LND tends to prolong the median survival time and the rate of one year survivors.

Surgical treatment of paediatric malignant neuroepithelial brain tumors.

The role of the neurosurgeon in dealing with malignant neuroepithelial brain tumors, both supra- and infratentorial, in paediatric age, is stressed; this because surgery is the main therapy, and also it is mandatory, in order to achieve the pathological diagnosis, essential for planning any kind of complementary treatment. Open surgery versus stereotactic biopsy is considered, with emphasis on brain stem tumors. The treatment of malignant tumor recurrencies is also discussed, and the indications of a second surgical look in patients harboring recurrent medulloblastoma is proposed for discussion, when long disease-free time occur and combined modality treatments are performed.

Comparative non-blind trial of ceftriaxone and gentamicin in the treatment of complicated urinary tract infections.

Forty hospitalized patients with complicated urinary tract infections were treated with gentamicin 240 mg die i.m. or ceftriaxone, a new parenteral cephalosporin, 2 g die i.v. for 7 days. Ceftriaxone gave good clinical and bacteriological results which were better than those obtained with gentamicin, showing also an appreciable activity in cases of infection due to problematic organisms.