The clinical and histological features of two cases of giant cell tumor of the sphenoid bone are described. Both presented with similar symptoms and signs, comparable to previously described cases. The problems in histological differential diagnosis are discussed and radiotherapy as the treatment of choice is suggested.

The responsiveness of the visual cortex (VC) and superior colliculus (SC) was simultaneously compared following conditioning “ON” or “OFF” stimulation, in the rabbit.

Average evoked responses were recorded simultaneously from the visual cortex and superior colliculus. “ON” or “OFF” steps constituted the conditioning stimuli whereas the test stimulus consisted of optic nerve stimulation. All evoked responses exhibited a reversal of their polarity when the electrode was moved in the dorsoventral direction (Negative-Positive in the SC, Positive-Negative in the VC). This assured the somato-dentritic origin of the potentials. The results showed that responsiveness in both structures was significantly higher following an “OFF” stimulus than after an “ON” step. Collicular responsiveness was higher than in the VC when the same conditioning stimulus was applied. The spatial distribution of the source of “OFF” responses was circumscribed to the ventral part of the superficial layer of the superior colliculus. These results suggest specific properties associated with the brightening and dimming systems.

The propagation velocities and conduction times of nerve impulses responsible for muscle F-waves were measured in the proximal segments of 60 normal posterior tibial nerves and of 41 normal peroneal nerves. The results were compared with those of 25 patients having confirmed lumbosacral root compression due to disc degeneration. Using the peroneal nerve, 65 per cent of patients had a prolonged proximal conduction time; a similar abnormality was found in 56 per cent of patients when the posterior tibial nerve was studied. The yield of positive results rose to 85 per cent and 76 per cent respectively when the M- and F-latencies in given individuals were compared. It was also shown that in normal subjects the F-response has a longer latency, and slower conduction velocity than the H-reflex when both are obtained using the same stimulating and recording sites, in patients in whom the ankle jerks and H-reflexes are absent, the F-waves may still be recorded, indicating that the latter are mediated through motor fibers

The history of a patient with rheumatoid arthritis and necrotizing vasculitis affecting only the central nervous system is reported. Clinical and pathological involvement by this process was present in both cerebral hemispheres, the pons and spinal cord. Review of the literature revealed that cerebral vasculitis in rheumatoid arthritis has been reported rarely and spinal cord vasculitis not at all.

The ribonuclease activity of cerebrospinal fluid of 219 patients was studied. The normal level was 269 ±95 units/ml. Consistent elevations above 550 units/ml were found in. 1 Chronic cerebrovascular disease, 2 Spinal cord compression; 3. Tumors. The molecular weights of the ribonucleases in the cerebrospinal fluid are approximately 33,000; 21,000 and 15,000, the major species is the one with m w. 33,000.

Although the increase in the CSF ribonuclease activity is not disease specific, the measurement has provided corroborative help in cases when the CSF protein is normal.

The increase in CSF RNAase is not due to red or white blood cells and the immunologie data suggest that the CSF enzyme activity is derived from the blood stream Further studies are necessary to rule out a nerve cell origin of the CSF ribonuclease activity.

From an analysis of the electroencephalograms of 4,458 patients who underwent recording during both wakefulness and sleep, through the years 1969 to 1975, wicket spikes — recorded in 39 patients — may be described as follows:

They were found during both wakefulness and sleep, almost exclusively in adults. Their cardinal feature is a changing mode of occurrence through any single recording: from intermittent trains of more or less sustained, arciform, discharges resembling mu rhythm, to sporadic, unitary, single spikes. When occurring singly, wicket spikes can be mistaken for anterior or middle temporal spikes, since they predominate in either area, and since they share with them other characteristics such as amplitude (60 to 210 microvolts), polarity (surface negative) duration, and configuration.

Wicket spikes should not be considered interictal abnormalities; they do not correlate with epilepsy or with any particular symptom complex.

Electrodes were implanted into dorsal hippocampus (CAI), ventral CAI, dorsal dentate gyrus or ventral dentate gyrus. Epileptiform afterdisvharge (AD) thresholds were lower in dorsal areas than in ventral areas. Dorsal areas, however, required a greater number of stimulations to develop (“kindle”) a fully generalized convulsion than did ventral areas. Thresholds and kindling rates in the dentate gyrus were intermediate between dorsal and ventral CAI, except for the ventral dentate which had higher AD thresholds than ventral CAI.

Secondary sites within the hippocampus subsequently kindled within a few stimulations following completion of kindling in the primary site, regardless of which hippocampal area served as the primary site.

Intraventricular administration of a peptide from ovine pituitaries whose structure is identical to the 61-91 C-terminal portion of beta-lipotropic hormone (61-91 beta-LPH) induced catalepsy, muscular hypertonus and analgesia in rats. Naloxone inhibited both the analgesic and cataleptic effects. l-dihydroxyphenylalanine (l-DOPA) completely prevented the cataleptic effect. The cataleptic effect of 61-91 beta-LPH was potentiated by 1-5-hydroxytryptophan (5-HTP).

About 10% of patients consulting a family practitioner have neurological complaints, and about 2% ultimately receive a neurological diagnosis As it is not possible to train enough neurologists for these patients, graduating medical students must be competent and confident in assessing neurological problems, particularly those that are common, treatable or require emergency management.

An evaluation was made of the neurological problems commonly seen in family practice, the difficulties in managing neurological problems by the family practitioner and the criteria for referral to a neurologist. To evaluate the teaching of medical students and house staff, the types of problems seen in a teaching hospital neurology service were examined.

As a result of this study an approach to the training of physicians is outlined to assist them to handle confidently and competently the neurological problems they will see in daily practice.

The histories of seven consecutive cases of Gilles de la Tourette's syndrome are presented to exemplify the range of clinical manifestations in this disease and to collate preliminary results with the new benzodiazepine, clonazepam, as a possible adjuvant therapy of this disorder. Controlled trials with clonazepam alone and in association with haloperidol are now justified. Five of our 7 patients had a positive family history of tics, and 2 a confirmed family history of gout. Because clonazepam improves myoclonia and tics and because its mechanism of action possibly involves serotonin, we thought it worthwhile to study simultaneously the relative roles of serotonin and dopamine metabolism in the production of tics, and their relationship to possible defects in purine metabolism in Gilles de la Tourette's syndrome.

Uptake of 14C-dopamine by human platelets has been studied in two diseases, namely Gilles de la Tourette's syndrome and Huntington's chorea, in which abnormal metabolism of dopamine has been implicated. Platelets from untreated Huntington's chorea patients showed a small increase in Km and Vmax.; platelets from patients in all other groups showed an uptake identical with the controls. Haloperidol (10 -5M) was also shown to be a strong non-competitive inhibitor of 14C-DA uptake by platelets. This property is probably unrelated to the drugs' action in ameliorating the symptoms of Huntington's chorea which is likely related to the increase in cholinergic neuronal activity produced by neuroleptic blockade of dopamine receptors.

A 65-year-old female patient with Paget’s disease displayed dementia, a bilateral Parkinsonian syndrome, gait ataxia, bilateral grasp reflex of the feet, urinary incontinence and a left hemicerebellar syndrome. She had a marked basilar impression; the radionuclide cisterno gram was suggestive of a communicating hydrocephalus with ventricular dilatation. After a shunting procedure there was improvement in both neurological and mental function.

Multivariate data analysis statistical procedures were applied to identify regional cerebral blood flow (rCBF) patterns in patients with multi-infarct dementia (M.I.D.) and Alzheimer’s disease (A.D.) when compared to a control group (C). A fronto-temporal-parietal pattern was identified for the A.D. group. The M.I.D. group demonstrated a predominant temporal-parietal pattern. A discriminant function analysis classified 96% of the A.D. and C. patients correctly. One hundred percent hit rate was obtained in discriminating between M.I.D. and C. Discrimination of A.D. and M.I.D. obtained 82% diagnostic accuracy. When the three groups were compared the hit rate was 93%. Mean rCBF and mean relative weight of the gray matter (Wg) differences were found when each dementia group was compared with C.M.I.D. and A.D. did not differ in mean rCBF but significant differences were found for Wg, with M.I.D. having greater reduction. The performance on standardized psychological tests of two independent samples of A.D. and M.I.D. were compared. The Memory Quotient from the Wechsler Memory Scale was consistently more impaired for the A.D. group. No correlation between degree of psychological impairment and rCBF or W g reduction was found.

A six year old girl complained of sudden severe headache, became hemiplegic and unconscious. A right carotid arteriogram revealed an obstruction of the right anterior cerebral artery and many sylvian branches. Death occurred four days later. At autopsy, a recent softening of nearly all the right middle cerebral arterial territory was found. Thrombus filled the sylvian artery and its main branches. Histologic examination of the vessel walls showed a dissecting infiltration of blood between the internal elastic lamina and the media. This particular form of dissecting aneurysm, occurring in young subjects, in the absence of atherosclerosis, high blood pressure and idiopathic medial necrosis, represents a distinct nosologic entity that has been called “Obstructive parietal hemodissection of intracranial vessels.” The pathogenesis of the disease is unknown: trauma has been mentioned, also congenital defects in the elastic lamina or other morphologic abnormalities ofthat lamina.

A case of pigment variant of Kufs’ disease is presented. The nature of the extra-neuronal pigment is discussed. Despite some of the histochemical discrepancies that existed between this pigment and the material that had accumulated in the nerve cells, they seemed to he ultrastructurally related. The hepatocytes contained numerous heterogeneous cytosomes, some of which resembled the storage material of Niemann-Pick’s disease.

Clinically the syndrome may present with progressive ataxia, spontaneous and reflex, coarse myoclonic jerks and eventual mental deterioration as well as epilepsy and muscle wasting. The pigment variant cannot be distinguished from Kufs’ disease except pathologically.