The clinico-pathological features of five patients with vascular amyloid restricted to the central nervous system are presented.

In three normotensive patients, intracerebral hemorrhage was the dramatic manifestation of amyloid angiopathy. In two other cases, one of amyloid in an arteriovenous malformation, the other of amyloid following therapeutic radiation, amyloid deposition was asymptomatic.

Clinically, amyloid angiopathy must be considered in the different diagnosis of intracerebral hemorrhage, independent of the presence of dementia. Pathologically, a factor common to the syndrome of cerebrovascular amyloid appears to be locally increased vascular permeability resulting from a variety of previous tissue injuries.

Clinical and angiographic findings in migraine are briefly reviewed in relation to cerebral hemodynamic changes shown by regional cerebral blood flow (rCBF) studies. Three cases of migraine studied by the intracarotid xenon 133 method during attacks are reported.

In classic migraine, with typical prodromal symptoms, a decrease in cerebral blood flow has been demonstrated during the aura. Occasionally, this flow decrease persists during the headache phase. In common migraine, where such prodr.omata are not seen, a flow decrease has not been demonstrated.

During the headache phase of both types of migraine, rCBF has usually been found to be normal or in the high range of normal values. The high values may represent postischemic hyperemia, but are probably more frequently secondary to arousal caused by pain.

Thus, during the headache phase rCBF may be subnormal, normal or high. These findings do not exclude the possibility of distension of the larger intracranial arteries during migraine headache, but the angiographic evidence, however limited, does not support this speculation.

This presentation includes six cases of chronic bilateral pure motor hemiplegia, one of these with pathological findings; one clinical case of chronic pure motor quadriplegia and one pathologically-studied case of chronic pure motor paraplegia. These cases may illustrate a spectrum of pure corticospinal disorders that heretofore has not been fully recognized.

Two patients had lumbosacral radiculopathy following radiation treatment of cancer. Twenty previously reported cases were similar. The clinical picture is one of progressive motor and sensory loss in the legs, usually appearing within a year after radiation, but sometimes delayed up to several years. Experimental studies quoted indicate greater vulnerability of peripheral nerves to ionizing radiation than has been previously recognized. Lumbo-sacral radiculopathy is readily produced in the experimental animal (rat) and affords an experimental model closely resembling the human cases reported.

A study of 200 patients referred to a community neurologist showed that 87.5% of the patients were seen in the office and only 12.5% in hospital. Neurological signs were present in 52% and 28.5% had neurological signs which materially affected the diagnosis. A questionnaire sent to several teaching centers showed that only one center sent students and residents to community neurologists' offices at all and in most centers the resident spent just 10 to 20% of his time seeing out-patients. It was felt that the balance of in-patient/out-patient teaching for students and residents was wrong, and that more emphasis should be placed on the neurological history than on the examination. Proposals are made to involve the community neurologist as well as the academic neurologist in the training of students and residents which would benefit all four groups.

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.

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.

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.