Examination of the effects of primary site kindling upon the development of secondary site amygdaloid kindling in cats showed that the former exerts powerful positive as well as negative after effects upon the latter. The positive after effect appears to be directed towards establishing a linkage between the secondary site and the Stage 6 generalized convulsive mechanism. The negative after effects were strikingly similar to those observed in animals with forebrain bisection subjected to amygdaloid kindling, suggesting that it is primarily directed towards functional accessibility of the cerebral structures interconnected by the forebrain commissure. The similarity of the findings in animals with anterior neocortical lesions supports such an assumption. The transient nature of the negative after effects was indicated by the development of a ‘normal’ clinical ictal pattern from the secondary site when it occurred spontaneously. Secondary site kindling exerted similar but less prominent negative after effects when the primary site was re-tested. Stage 6 seizure was elicited upon the first trial, however.

Determination of lipoprotein patterns and of major plasma lipids was carried out in 171 patients with strokes. Of these, 22 had hyperlipoproteinemia (HLIPR) by paper electrophoresis and by elevation of principal plasma lipids (either cholesterol over 300 mg/100 ml or triglycerides over 200 mg/100 ml or both these components). More than two-thirds of the patients had at least one close relative with elevated blood lipids. Using criteria of the World Health Organization, these patients were classified as follows: 5 had HLIPR type IIa, 8 had HLIPR type IIb, 3 had HLIPR type III and 6 had HLIPR type IV. Phospholipids showed relatively little change from the values of normal controls. The numerical distribution of patients with stroke and HLIPR into the four different types corresponds quite well with the approximate frequency of these types of HLIPR in the general population. Thus, this study does not indicate that the patients with a particular type of HLIPR are at a greater risk to have a stroke than those belonging to other types.

Resuscitation of the brain following total circulatory arrest may be impeded by difficulty in establishing cerebral tissue perfusion, a post-ischemic “low-flow” state. We have confirmed this hypothesis in a rat model of total cerebral ischemia and have demonstrated marked improvement in post-ischemic brain tissue perfusion following epinephrine injection. This is mainly due to the systemic vascular effects of epinephrine, resulting in improved central aortic pressure and cerebral perfusion pressure. Hyperkalemic induced vasoconstriction has also been postulated as a cause of the “low-flow”. We have, therefore, investigated the in vitro effects of increasing potassium ion concentration on cerebrovascular smooth muscle strips. Large arteries constrict, while small arteries dilate in response to hyperkalemia. The net effect on cerebral blood flow remains unsettled. Our research to date suggests that resuscitation of the totally ischemic brain, in animal models at least, is enhanced by epinephrine, mainly via its effects on central aortic pressure.

The autoradiographic findings using tritiated leucine are described in muscle biopsy material from five patients with progressive muscular dystrophy (P.M.D.), three with motor neuron disease (M.N.D.) and four with Charcot-Marie-Tooth disease (C.M.T.). In progressive muscular dystrophy there is a marked increase in uptake of leucine into cytoplasmic proteins and precursors, and reduced incorporation into structural protein. In Charcot-Marie Tooth disease muscle there is a significantly increased uptake into cytoplasmic elements and a normal uptake into structural protein. In motor neuron disease the uptake into cytoplasmic elements appears normal but is reduced into structural proteins.

The abnormal uptake in C.M.T. could be explained as a product of regenerative efforts associated with reinnervation. However, the abnormal uptake may represent the primary effects of gene action in the muscle, as seems probable in progressive muscular dystrophy.

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.

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.

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).

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.

A 38-year-old patient with the clinical picture of a progressive myopathy resembling limb girdle muscular dystrophy is presented. Muscle biopsy showed amyloid deposits in the walls of small endomysial blood vessels. There was no clinical or physiological evidence of peripheral nerve involvement, no plasma cell dyscrasia and no generalized amyloidosis. There was no muscle fiber hypertrophy, inflammation or neurogenic change. There was no response to steroid therapy.

The etiopathogenesis of this amyloid angiopathy is undetermined. The extensive vessel involvement with amyloid deposition and the absence of changes indicative of muscular dystrophy or inflammatory myopathy leads us to favor an ischemic basis for this patient’s myopathy.