Malignant optic nerve gliomas in adults.

Two patients with malignant optic nerve gliomas are described. The clinical courses and radiographic appearances are discussed, with emphasis on the fact that this condition should be considered in the differential diagnosis of rapid visual failure. It can now be strongly suspected preoperatively on the basis of computed tomographic scan appearances.

Bedtime cimetidine maintenance treatment: optimum dose and effect on subsequent natural history of duodenal ulcer.

Sixty patients, whose duodenal ulcers had healed endoscopically after six weeks of treatment with cimetidine 1 g/day in divided doses, were treated with maintenance cimetidine 800 mg at bedtime for six months. Eighteen relapsed endoscopically (30%). Of the 42 still in remission, 36 then completed a six month double-blind comparison of bedtime cimetidine 400 mg and placebo. Twelve of the 19 (63%) cimetidine-treated patients and 10 of 17 (59%) placebo-treated patients relapsed within six weeks (NS). This high relapse rate on cimetidine contrasts with our earlier trial, in which the six week relapse rate was only two out of 21 (10%) on bedtime cimetidine 800 mg and 16 out of 24 (66%) on placebo (P less than 0.0005). Apart from the difference in the dose of cimetidine, both our trials used the same experimental protocol during the double-blind part of the trial. In the earlier trial, however, there was no period of pretreatment with maintenance cimetidine as in the present trial. The pattern of placebo relapse was similar in both trials. We conclude that bedtime cimetidine maintenance treatment does not alter the long-term natural history of duodenal ulcer once it has been withdrawn; and that either tolerance to cimetidine develops during long-term maintenance treatment, or that bedtime cimetidine maintenance treatment in the conventional dose of 400 mg is not as effective as 800 mg in prevention of endoscopic relapse, although it does reduce symptoms.

Motor neurone disease with elevated cerebrospinal fluid protein.

The correlation between age and CSF protein in 38 patients with pathologically proven motor neurone disease was the reverse of that described for normal subjects by Tibbling et al in 1977. Eight had CSF protein ranging from 0.75 g/1 to 1.52 g/L. These patients were younger, but other clinical and gross and light microscopy pathological features were not significantly different from those with lower CSF protein. Transudation from serum may be only one of the mechanisms underlying this elevation in CSF protein.

The separation of pineocytoma from pineoblastoma.

From our study of eight pineoblastomas and five pineocytomas and a review of the literature, we have described two clinicopathologic syndromes that characterize these neoplasms. Pineoblastomas highly resemble the medulloblastoma-neuroblastoma group of tumors and occur mostly in young people. The tempo of progression of the disease is fast, the length of illness is short. These are infiltrating neoplasms that commonly spread via the cerebrospinal fluid. They are radiosensitive. Histologically they are also similar to the medulloblastoma-neuroblastoma group and are characterized by the scarcity of cytoplasmic processes and by the Homer Wright rosette. They contain giant cells. Pineocytomas are tumors of adults. The tempo of progression of the disease is slow, and the length of illness is long. They expand by compressing the surrounding tissues. Histologically they are characterized by the abundance of cytoplasmic processes and by the pineocytomatous rosette. They contain giant cells. Areas composed of neoplastic gangliocytes and astrocytes in various combinations are common variants in some of these neoplasms.

Pineocytoma with astrocytomatous differentiation.

A pineocytoma with astrocytomatous differentiation in a 57-year-old man is reported. The histogenetic and oncogenetic implications of this neoplasm are briefly discussed.

Low-cholesterol diet: enhancement of effect of CDCA in patients with gall stones.

Fifteen patients with gall stones who were taking chenodeoxycholic acid(CDCA) 15 mg/kg at bedtime participated in two separate experiments to investigate the effects of altering sterol intake on the cholesterol saturation index (SI) of fasting gall-bladder bile. In experiment I the 15 patients on an unrestricted diet had a SI of 0.87 +/- 0.04 (mean +/- SE of mean), which fell to 0.75 +/- 0.04 after one week in hospital on a diet of 100 mg cholesterol daily. In experiment II seven of the patients were given four different dietary regimens lasting one month each in random order as outpatients. On a diet of 600 mg of cholesterol daily the mean SI was 0.72 +/- 0.05, which fell to 0.67 +/- 0.05 when the patients were put on a 100 mg cholesterol diet. The addition of plant sterols (3 g daily) to both diets raised the mean SIs to 0.80 +/- 0.05 and 0.77 +/- 0.05 respectively. The percentage CDCA in bile was unaffected by alterations in the cholesterol and plant sterol intakes. We conclude that a low-cholesterol diet but not a high intake of plant sterols enhances the effect of CDCA in patients with gall stones.

Prevention by bedtime cimetidine of duodenal-ulcer relapse.

45 patients with healed duodenal ulcers completed a six-month double-blind trial to compare the effects of cimetidine and placebo on the prevention of duodenal-ulcer relapse. A single bedtime dose of cimetidine (800 mg) was given and ulcer relapse was assessed endoscopically at regular intervals. At the end of the six-month trial, 16 of the 21 (76%) cimetidine-treated patients remained ulcer-free compared with only 3 of the 24 (12.5%) placebo-treated patients. No untoward effects of this treatment were observed.

Ocular myopathy (progressive external ophthalmoplegia) with neuropathic complications.

The condition known as Ocular Myopathy or Progressive External Ophthalmoplegia is reviewed. Three hundred and thirty-five published case reports have been analyzed and 13 personal cases are described. Histological and electron microscopical studies on one of our cases are reported. It is clear from these cases that, although in its simplest form the condition may consist merely of myopathic changes confined to the upper eyelids and external ocular muscles, myopathic changes may also occur in the muscles of the face, neck and proximal parts of the limbs. In addition, however, a variety of neurological disorders may also develop and degenerative lesions have been found in the peripheral nerves, spinal cord, brain stem, and basal ganglia. Other associated conditions such as perceptive deafness, pigmentary retinal degeneration, cardiomyopathy and red cell abnormalities may also occur. There is thus a wide spectrum of syndromes, all based on a condition which is possibly a general metabolic disorder. Recent work has directed attention to mitochondrial abnormalities in the affected muscle fibers and other cells. A positive family history may be present and an autosomal dominant mode of inheritance seems likely.

Cimetidine in duodenal ulcer. Controlled trial.

As part of a double-blind controlled clinical trial of cimetidine (1.6 g daily) in patients with endoscopically proven duodenal ulcer, repeat endoscopy has been carried out in 24 patients after two and/or six weeks' treatment. At six weeks, 9 out of 11 patients on cimetidine and 3 out of 12 patients on placebo had healed (P less than 0.025). A separate open pilot trial in 23 patients has shown no difference in ulcer healing at six weeks between patients taking 0.8 and 1.6 g daily. A total of 32 different patients received cimetidine in the two trials, and ulcer healing was observed in 21 (66%) at six weeks. No patients showed evidence of bone-marrow toxicity. A small but significant rise in mean S.G.O.T., S.G.P.T., and serum-creatinine occurred in 13 patients on cimetidine 1.6 g daily, but not in 13 patients on 0.8 g daily.

The cerebral pathways of optokinetic nystagmus: a neuro-anatomical study.

Abnormalities of optokinetic nystagmus are described in a patient with pathologically proven bilateral infarcts in the middle cerebral artery territories. There were no other central nervous system lesions. These abnormalities of eye movement are interpreted as indicating an inhibitory effect of the frontal eye fields upon the occipital lobes.

Some characteristics of the rabbit vermiform appendix as a secreting organ.

It has been confirmed that the rabbit vermiform appendix secretes spontaneously at a relatively rapid rate (1-12 ml.h(-1); 1.4+/-0.24 mul.min(-1).cm(-2)). The electrolyte composition is similar to that of ileal fluids and independent of the secretory rate. The transmural potential difference is about 12 mV, mucosa negative. Of the major electrolytes, only HCO(3) (-) is secreted grossly against its electrochemical potential difference. This finding plus the low hydraulic (or osmotic) permeability (L(p)) and high secretory pressures of the organ strongly suggest that the secretion is an active one. The passive permeability to Na(+) and Cl(-) appears to be, at most, somewhat less than for small bowel. Permeability to mannitol was estimated at 2.5 x 10(-7) cm.s(-1). On the basis of reasonable assumptions and results with luminal test solutions of differing osmolarities, it was concluded that (a) the L(p) of the appendiceal epithelium is in the lower range of values reported for small bowel and colon; (b) the L(p) is higher for osmotic absorption than for osmotic secretion; and (c) the rate of spontaneous secretion is insensitive to luminal anisotonicity over a wide range of values. But sufficiently hypotonic solutions can reverse net secretion to net absorption, more by inhibiting spontaneous secretion than increasing osmotic absorption. The rabbit vermiform appendix appears to be a useful model for the elucidation of intestinal secretory processes.