Five patients with progressive supranuclear palsy are described, in whom the ophthalmoplegia developed late in the course of the disease. In two, an internuclear component was identified in the ophthalmoplegia, and one patient had an alternating nystagmus of a type not previously described in this condition.

The late appearance of the ophthalmoplegia, with a corresponding delay in establishing the diagnosis, is compared to the similar experience of Pfaffenbach et al (1972) in six patients.

Other clinical features, previously seldom described, have been encountered. Dysphasia was seen in two cases, both of whom had evidence of cortical atrophy on neuro-radiological investigation. The evidence that cortical changes, in particular the presence of neurofibrillary tangles, may be a specific morphological characteristic of the disease rather than a chance association is discussed. Disorders of respiratory rhythm in four patients were similar to those described by Mastaglia et al. (1973), and indistinguishable from those occurring after encephalitis lethargica.

A review of cases resembling progressive supranuclear palsy in the early part of the century fails to show any with post-encephalitic features, nor does a search of reviews of eye movement disorders in encephalitis lethargica and post-encephalitic Parkinsonism provide comparable cases. None of the forty patients with post encephalitic Parkinsonism examined at the Highlands Hospital had a clinical picture resembling progressive supranuclear palsy.

It is suggested that neither on clinical nor pathological grounds is it justifiable to equate this disorder with known postencephalitic syndromes.

This is the first report of a method of sequential regional cerebral blood flow (rCBF) analysis, called Croma-Memo-Flow. This technique is a computerized modification of the initial slope method of regional cerebral blood flow (rCBF init.), allowing temporal resolution of the flow pattern by calculation of the slopes of sequential segments of the initial 1-2 minutes of the Xenon-133 washout curve. The same theoretical analysis applies to this method as to the rCBF init. method. Each flow calculation is based on the slope of a discrete 16 second segment of the initial washout; and each second the segment is advanced by one second. A new flow calculation is made each second and is displayed as a color coded map on a TV screen. Each map is labelled, indicating the time in seconds following Xenon injection, and sequential rCBF changes during the clearance period can be immediately visualized. This allows for almost instantaneous analysis and display of rapid or transient rCBF responses to activation and deactivation of the cerebral cortices.

The data is stored in a 35 channel memory for deliberate replay, photography, and analysis.

Functional tests may be applied during the initial washout period and both the magnitude and chronological relationships of the evoked regional cerebrovascular responses observed. A clinical study is presented to illustrate the possibilities of applying the technique to assess cortical reactivity.

One of the hazards of advancing age is the temptation to repeat old stories, but the worst that can happen is to forget.

“Happy moments come and go, our friends die, everything is in the end destroyed, but this could be endured if only a purer and sharper reflection were kept. If there is anything that we save and hoard up it is our memories. All the substance of our life is in them.” (Brennan, 1975).

This is a report of a series of patients who suffered major cerebral hemorrhage or infarction complicating industrial head injuries. The variable importance of trauma and of pre-existing vascular disease is exemplified. Accuracy of assessment of such complex problems is facilitated by modern applied neuroscientific techniques.

Neurological complications of brucellosis have seldom been reported. Typical cases of meningoencephalitis, myelitis, and peripheral nerve involvement due to brucellosis, which have been collected over the past ten years, are described. Only four cases of extradural brucellar abcesses have been published previously. We now add four new observations. A CSF electrophoresis was done in the cases of meningoencephalitis and meningomyelitis and showed a marked increased of gamma globulin. This has never been reported previously.

Multiple sclerosis patients treated with antithymocyte globulin (ATG) were re-evaluated after five years. No long term benefit was found. Notably, the group of patients with an elevated gamma globulin to total protein ration in their C.S.F. and who did particularly well after treatment with ATG also failed to show any long term benefit. Few long term detrimental effects of ATG immunosuppression were identified. The implications of the results are discussed as they relate to the use of immunosuppression in multiple sclerosis.

Sixteen cases of spontaneous dissection of the cervical internal carotid artery (6 verified) are described. The mean age was 45 years. The clinical picture varied from simply headache and a bruit to hemiplegia and aphasia. Eleven patients had transient ischemic attacks. Headache, facial pain, a subjective bruit, oculo-sympathetic palsy and transient monocular blindness were present in various combinations in two-thirds of cases and their presence suggested the correct diagnosis. Examples of suspected dissection of the intracranial internal carotid, middle cerebral, posterior cerebral and extracranial vertebral arteries are also presented. Spontaneous dissection is more common than the literature indicates.

The time response of the Electroretinogram (ERG) is an essential part of the examination. Peak time of response increases with dark adaptation and decreases with the intensity of stimulation. When working with constant amplitude during dark adaptation sensitivity increases but the intensity of stimulation decreases. It is essential to clarify the relationship of these two factors for valid interpretation of results. The present work suggests that the state of adaptation or retinal sensitivity affects amplitude, while peak time of the response is modified by the absolute value of the stimulating source whatever the state of adaptation. This observation has been done on rod responses and is valid as long as pigments remain unbleached and able to respond.

The general principles of bypass surgery as they affect the cerebral circulation are reviewed. The preliminary results of an extracranial intracranial bypass operation performed on a group of 19 patients suffering from cerebral ischemia are presented. The results of the surgery compare favorably with those published in the literature.

Using theANOPS-101 minicomputer, an automatic analysis of muscle action potentials (MAP) was performed on a total of 53 extensor digitorum brevis (EDB) and abductor pollicis brevis (APB) muscles in patients with other evidence of denervation. The results were compared with observations made on 27 control subjects and they were also matched against estimates of functioning motor units made by the incremental stimulating technique of McComas et al (1971). Although the incidence of abnormal MAP parameters was relatively low in both muscles, it was possible to improve the diagnostic yield by combining the results for individual muscles. The relative merits and disadvantages of motor unit counting and automatic MAP analysis are discussed.

Eleven patients with transient paralytic attacks of obscure nature are described. Paralysis could involve face or leg alone, face and hand, or face, arm and leg. The duration varied from two minutes to one day. Four patients had brain tumors, six probably had brain infarcts, and one a degenerative process. The differential diagnosis included TIAs, migraine accompaniments, and seizures. In the absence of good evidence for the first two, the cases are discussed from the standpoint of possibly representing nonconvulsive seizure paralysis (ictal paralysis, inhibitory seizure paralysis or somatic inhibitory seizure). Because of the difficulty in defining seizures as well as TIAs and migraine in their atypical variations, a firm conclusion concerning the mechanisms of the spells was not attained. Two cases of the hypertensive amaurosis-seizure syndrome have been added as further examples of ictal deficits.

Valproic acid is a new antiepileptic drug recently introduced in the United States for the treatment of absence seizures. In this study on patients with absence and other seizure types, the majority of patients achieved optimal control within four weeks of therapy. No patient responded to valproic acid who did not show an initial clinical response by four weeks of active therapy. Optimal response was generally achieved when plasma levels were greater than 55 μg/ml. Excellent clinical response was observed in the treatment of absence and myoclonic seizures. Twenty-two patients continued in a long term study have maintained the same degree of seizure control as observed at the time of optimal control.

Anesthetized rabbits were given intravenous injections of either beta-lipotropin (beta-LPH), betamelanotropin (beta-MSH) or betaendorphin. The postinjection concentrations of these peptides in plasma and cerebrospinal fluid (CSF) were measured by radioimmunoassay (RIA). The plasma disappearance half-times were 13.7 min for beta-LPH, 5.1 min for beta-MSH, and 4.8 min for beta-endorphin. Circulating beta-LPH is cleaved to peptides tentatively identified as gamma-LPH and beta-endorphin. Each of these peptides appeared in the CSF within 2 min postinjection. The maximum CSF to plasma ratios were 0.08 for beta-LPH, 1.48 for beta-MSH, and 0.23 for beta-endorphin.

An automated system, incorporating the ANOPS-101 mini-computer, has been used to analyse the EMG. The vastus medialis (VM) and biceps brachii (BB) muscles were studied in 28 controls, 16 patients with myopathies, and in 26 patients with denervating disorders. For each muscle mean values were computed for the durations and numbers of phases of muscle action potentials; the mean density and amplitude of the deflections in the interference pattern were also measured. A higher incidence of abnormalities could be detected in myopathic than in neuropathic disorders; for both conditions the incidence was significantly greater in BB than in VM. For the diagnosis of denervation the most useful measurement was that of potential duration; for the detection of myopathies amplitude determinations were also very useful. The present results have been compared with those of other published studies in which automatic EMG analysis has been employed.

The effects of bilateral common carotid artery occlusion en brain metabolism and arterial acid-base balance were studied in normotensive and experimental renovascular hypertensive rats.

One hour after carotid occlusion in hypertensive rats, supratentorial lactate increased to 383% and lactate-pyruvate ratio to 280% of the controls, while adenosine triphosphate (ATP) decreased to 69%. These metabolic changes were thought to be due to cerebral ischemia. Arterial pC02 was lowered and the pH was raised in the hypertensive animals due to cerebral ischemia induced hyperventilation. In the normotensive rats, carotid occlusion had minimal effects on cerebral metabolism and arterial acid-base balance.

These results suggest that hypertensive rats are more susceptible to cerebral ischemia caused by carotid occlusion than normotensive rats. Increased cerebrovascular resistance in hypertension is discussed as a casual factor in cerebral ischemia.

Recent observations are considered to provide further evidence for an abnormality involving motoneurones in DMD. The dystrophic process appears to take place in two stages of which the first occurs during early embryonic life. This stage is thought to involve faulty inductive actions of the neural tube upon mesoderm and upon itself. The neural consequences vary among individuals and are manifested as mental retardation, EEG abnormalities and losses of functioning motor units. While the first two abnormalities are non-progressive, a further loss of motor units, associated with striking reductions in the numbers of excitable muscle fibers, takes place in trunk and large limb muscles at 9-12 years. The latter process, the cause of which is uncertain, constitutes the second stage of DMD.

The aim of this study was to compare the b- and d- waves of the ERG, evoked respectively by ON and OFF stimuli. The experiements were carried out on frogs. The d-wave is facilitated by light adaptation and relatively high stimulus intensity. The depth profiles of b- and dwaves are different. Aspartic acid abolished only the b-wave whereas nembutal affected the d-wave. These results suggested that b- and d-waves were produced by separate neuronal mechanisms, even though both appeared as positive deflection in the corneal ERG and were sensitive to K+ ion.

Broken cell preparations derived from rat cerebral cortical grey matter were studied cytochemically to localize adenylate cyclase (AC) activity in subcellular organelle membranes. AC activity was localized by visualizing reaction product in brain particulate fractions by electron microscopy. Activity was found in the endoplasmic reticulum, on the inside of the inner mitochondrial membrane and on both leaflets of the nuclear membrane. Reaction product was found in the postsynaptic density area of most synapses. The reaction product tended to be more prominent in the presence of flouride. A synaptosome-rich fraction was shown to have NE stimulated AC activity which was blocked in vitro by both a α-and an β-blocker and in vivo by propranolol.

Spinal curves may be produced in fetal lambs with three surgical techniques. These procedures vary from mere exposure of the costo-vertebral junction of three ribs through a paravertebral incision, to resection of the head and part of the adjacent shaft of three ribs. The fetal age varies from forty-nine to seventy-three days. The degree of curvature present at birth seems to increase in severity with decreasing fetal age at the time of surgery, but the type of surgical procedure does not appear to influence the severity of the curve, suggesting that the mechanical presence of the ribs does not prevent the development of scoliosis in these animals.

Histological studies of the m. longissimus dorsi at the apices of the curves reveal two main types of abnormality in the muscle fibers. Both Type I and Type II fibers were significantly reduced in size in the biopsies taken from the side on which the surgery was performed, and there was marked alteration in the proportion of one fiber type to the other in most biopsies taken from both operated sides when compared with biopsies from unoperated twin animals.

The fetal age and amount of surgical trauma appeared to play no role in the degree of muscle alteration, suggesting that even minimal surgical trauma to the paraspinal region at any fetal age between 49–73 days is sufficient to produce significant muscle fiber abnormality and spinal curvature.

A parallel is drawn between these muscle findings and those in a number of human musculoskeletal diseases, and suggests the possibility of a developmental defect in the pathogenesis of these diseases.