Ten patients with multiple sclerosis were found to have lived in close proximity in a Nova Scotia farming community of 150 people. All had drunk unpasteurized milk as children, were teetotallers, ate a high animal fat diet, and were well educated. Of greater interest was the observation that six of the ten cases were related in two family groups.

The only time all patients lived in the community at the same time was in 1951 and 1952 during a polio outbreak. The relationship of polio to multiple sclerosis bears further study.

The average age of the patients when they had measles was 11.8 years. Evidence suggests a link between risk of multiple sclerosis and both late onset of measles and pubertal age. Late onset of measles may be important in this cluster. Further epidemiological studies are needed to examine the age of onset of measles in M.S. cases.

The post-mortem examination of the nervous system of a patient with Shy-Drager syndrome successfully treated with levodopa (Sharpe et al, 1972) revealed features of striato-nigral degeneration and amyotrophic lateral sclerosis, a cerebellar system degeneration and a loss of approximately 75% of sympathetic preganglionic neurons. Lewy bodies were not present and no detectable changes were observed in the sympathetic prevertebral ganglia.

While the limited and transient beneficial effect of levodopa on the bradykinesia in our case is possibly due to the progressive loss of striatal dopaminergic receptors seen in striatonigral degeneration, we propose that in Shy-Drager syndrome, levodopa therapy benefits orthostatic hypotension because of a suppression of the central depressor action of this drug. This suppression is attributable to functional disconnection of sympathetic ganglia secondary to the loss of preganglionic neurons or to degeneration of central autonomic catechoiaminergic systems.

The clinical and biochemical findings are presented of two brothers suffering from McArdle's Disease (Myophosphorylase Deficiency). Tissue enzyme estimations and lactate levels were done in affected and non-affected members of the family. Affected members showed absence of phosphorylase enzyme by histochemical and quantitative estimation. No quantitative abnormalities were found in other enzyme systems of glycolytic pathways in the family investigated. Various other aspects of clinical features, biochemical abnormalities and inheritance are discussed.

A consistent feature in dementia is an overall intellectual deterioration. The present study investigated the intellectual performance of patients with Alzeheimer's disease (AD) and multi-infarct dementia (MID) using the Wechsler Adult Intelligence Scale (WAIS). For reliability and generality purposes two independent samples of patients were collected. Significant differences in Education Level (EDU) and Performance IQ (PIQ) were obtained for the first sample, with the AD group having a significantly higher EDU level. The MID group obtained a higher PIQ. No significant differences were found in the second sample, but EDU level approached significance with the AD group again having a higher EDU level. A discriminant function analysis classified 81% of the patients in the first sample and 100% diagnostic accuracy was obtained for the second sample using the II predictor variables. A maximum R stepwise regression was performed in order to detect the “best” model of variables discriminating between the diagnostic groups. For the first sample the “best” model was the two variable model, including EDU and Full Scale IQ, accounting for 40% of the variance. The simplest model for the second sample was the one variable model including EDU, accounting for 20% of the variance. Quantitative, differences were found between the AD samples. Time since onset of the disease was offered as a possible influence in the quantitative differences in the AD samples. Sampling biases in the behavioral study of dementia are discussed. Successful replication was obtained.

The first description of the placing reaction (PR) in 12 human adult cases is given. The optimum position for eliciting the placing reaction (PR) is the dangling leg posture, i.e. the same as for the forward groping of the foot. There are three forms of PR quite similar to those noticed in animal physiology, i.e. the visual-PR, the dorsum-PR and the sole-PR. The term contact placing is preferred for the last two forms. The PR is encountered only in patients displaying a forward and medial groping of the foot as well as a groping of the hand on the same side. The PR is usually ipsilateral to the main cerebral lesion but there was no single case with a well limited unilateral lesion. An involvement of retrorolandic areas seems to be necessary for the occurrence of PR. It is concluded that both groping phenomena and the PR are highly coordinated reflexes subserving self-preservation and belonging to antigravity mechanisms, i.e. the standing posture.

We have reported three cases of intracranial dysgerminoma. The origin of these tumors has been controversial for a long time. It is now accepted that they develop from germ cells. The diagnosis is often difficult to confirm because of the variety of signs. If their origin is now accepted, the best way to deal with these lesions is still controversial. They can be macroscopically removed if they are in the pineal region. Such treatment is not possible for suprasellar tumors. Since these tumors can metastasize, radiation should be administered as soon as a pathological diagnosis is obtained. It is our belief that it should be administered to the whole spinal axis.

A case of congenital, intraspinal, extradural cyst is reported with pain as the only presenting feature. In the past, the lack of pain has been considered one of the characteristics of these rare lesions. In other reported cases, when pain has been present, it was minimal and never the presenting feature. The clinical and radiographic features and the surgical treatment are described. The pathogenesis is discussed and the literature reviewed. An addition to the surgical technique is given whereby one of the post operative complications may be obviated.

A case is reported where the appearance of acute, diffuse, disseminated sclerosis on computer tomography (Ct scan) is described and the literature is reviewed. This disease may give rise to multiple, small areas of diminished X-ray absorption which may decrease in size during the course of the disease. The histological features of one of the lesions was correlated with the radiological findings. It is suggested that, perhaps, only during the active stage of demyelination can the lesions be detected on the Ct scan.