After the capture of Fortress Louisbourg by the New England Irregulars, Louis XV of France commissioned Jean Baptiste Louis Frédéric de la Rochefoucauld, due d'Anville, to command a massive offensive flotilla to retake Louisbourg, and ravage the eastern seaboard of North America south to Florida.

After arriving in Chibouctou (Halifax Harbour) following thirteen weeks at sea. due d'Anville collapsed. His doctor treated him with enemas and emetics, but he suddenly turned blue, convulsed, and died.

The report of his autopsy, which included the cranium, (and which is Canada's only Royal autopsy) had been lost for two centuries. It is now analysed, showing that although he had significant intracranial pathology, he died from medical therapy.

Giant axonal neuropathy, a generalised disorder or neurofilaments, presents as a chronic, progressive peripheral neuropathy in childhood. Evidence for central nervous system involvement is demonstrated in this study of four male patients with giant axonal neuropathy who had defective visual function and abnormal ocular motility. The visual system was studied by electroretinography, which showed normal retinal function, and by visual evoked potentials, which showed disease of both optic nerves and retrochiasmal visual pathways. The ocular motility disorder, studied by electrooculography, comprised defective pursuit, inability to maintain eccentric gaze with gaze paretic and rebound nystagmus, abnormal optokinetic responses and failure of suppression of the vestibulo-ocular reflex by fixation. These findings suggested involvement by giant axonal neuropathy of the cerebellar and brain stem pathways important in the control of ocular motility.

Mechanisms underlying focal motor seizures and the Jacksonian “march” have been explored on the basis of recent physiologic data concerning the “nested ring” spatial and functional organization of motor cortex. Focal motor seizures can be understood in terms of the focal representation at motor cortex of elements controlling movement of a limb part about a single joint. The preponderance of three foci of origin of motor seizures, first reported by Hughlings Jackson, are related to lower thresholds of excitability at these loci, as demonstrated for forelimbs in the case of subhuman primates, and to the resultant bias of peripheral and central input to activate these loci. The “march” phenomenon which typically involves specific patterns of spread in the forearm, is explained in terms of the recently discovered nested ring organization. Thus the centrifugal spread of excitation from a particular locus in motor cortex would demand the pattern of spread of convulsive movements generally observed in clinical situations. The latter involves spread from face or leg to shoulder, forearm, then to hand, or conversely.

The physiological basis of activation and/or inhibition of focal motor epilepsy by peripheral stimuli is also now available on the basis of current experimental evidence. Thus the point to point coupling of periphery and motor cortex as defined in terms of control of limb parts about single forelimb joints, provides a substantial understanding of many of the clinical events sometimes termed “reflex” epilepsy.

Seven members of a family succumbed to prolonged dementing illnesses. In two, whose dementia lasted 10 years, spongiform encephalopathy was found at autopsy. This duration of illness has not been a feature in other instances of familial Creutzfeldt-Jakob disease.

Foci of recent cell death were identified in 10 consecutive cases of Huntington's disease in the caudate nucleus and putamen. Indirect immunohistochemical staining procedures were performed on these areas and controls using fluorescein conjugated anti-globulin and peroxidase-conjugated anti-globulin. Positive results were found when human antiserum to Cytomegalovirus was used. The possibility of abnormal antigen presence within the dying neurons in this disease is discussed.

Occasionally patients in the stroke age-bracket over 40 have unexplained transient cerebral is chemic attacks in association with normal cerebral angiograms. From this group 120 have been collected in whom the transient episodes resembled the neurological accompaniments of migraine. According to symptoms, the patients were categorized as follows: Visual accompaniments (patients with only ordinary scintillating scotoma were excluded), 25; visual and paresthesias, 18; visual and speech disturbance, 7; visual, and brain stem symptoms, 14; visual, paresthesias, and speech disturbance, 7; visual, paresthesias, speech disturbance and paresis, 25; recurrence of old troke deficit, 9; miscellaneous, 8. In establishing the diagnosis angiography is advisable in all but classical cases. Typical of migrainous accompaniments are the buildup and migration of visual scintillations, the march of paresthesiae, and progression from one accompaniment to another, characteristics that do not occur in thrombosis and embolism. Diagnosis is facilitated when 2 or more similar episodes have occurred or migraine-like scintillations are present. Headache occurred in 50% of cases. Other cerebrovascular processes, coagulation disorders, and cerebral seizures must be ruled out.

Agarose and/or cellulose acetate electrophoresis was performed on the CSF of one thousand patients. In patients with clinically definite multiple sclerosis (MS) (N = 267) 92.8% had oligoclonal banding (O.B.). In patients with possible MS (N - 283) O.B. was present in 31.1%. In patients with other neurological diseases (N = 450) O.B. was present in 8% (N = 36). Nineteen non-MS patients with positive O.B. had serum bands or disorders known to be associated with local immune response. The remaining 17 patients had no explanation for the oligoclonal banding. In the majority of these MS had not been a diagnostic consideration.

CSF electrophoresis is the single most reliable laboratory lest in multiple sclerosis and deserves incorporation into the diagnostic criteria for the disease.

The uptake of dopamine (DA) by platelet rich plasma was assayed in 11 patients with Huntington's chorea (H. C). The results confirmed the previous observation that platelets from H.C. patients take up, at equilibrium, more dopamine than do platelets from normal control subjects. The mean difference was 50% higher at DA substrate concentrations of 0.11 mM.

However, attempts to confirm the higher Na+ - K+ ATPase activity of erythrocyte ghosts from Huntington's chorea patients were unsuccessful.

GABA receptor binding in fifteen regions of cat central nervous system was investigated immediately postmortem and at twelve, twenty-four, and 72 hours postmortem. In each of the supraspinal areas studied, GABA binding increased with time-after-death. Changes after 24 hours occurred in the cerebellum, the visual cortex, the sensorimotor cortex. and the amygdala where more than a twofold increase in binding was observed. Increases were also noted in the thalamus, caudate nucleus, hippocampus, and hypothalamus. The results suggest that caution should be exercised in the interpretation of GABA binding data obtained from human brains that have not been treated in a similar postmortem manner.

From 123 hospital charts with a diagnosis of migraine, seen between the years 1974 and 1978, ten cases have been selected of complicated migraine and three cases of migraine with special features. A review, and a discussion of the clinical characteristics, and of the treatment of these unusual migraines has been made. The three cases with special features include migraine in association with hyperlipidemia, with Raynaud's disease and with meningioma.

A variety of peripheral nerve disorders may be associated with chronic renal failure. The polyneuropathy due to uremic toxins is a distal, motor and sensory polyneuropathy in which there is segmental demyelination, axonal degeneration, and segmental remyelination. The nature of the uremic toxin and the underlying mechanism of these changes is unknown.

The incidence in patients with “endstage” renal disease has fallen in recent years, severe cases now being rare, perhaps due to refinements in chronic hemodialysis, transplantation, and other therapies. However, while chronic hemodialysis stabilizes uremic neuropathy, manipulation of hemodialysis schedules may not alter its course, according to current assessment. Successful renal transplantation improves both the clinical and electrophysiological signs, even in severe uremic neuropathy.

Six cases of acute cerebellar infarction seen on a neurosurgery service in a general hospital during a twenty-six month period are reviewed. The clinical presentation, course, and treatment are presented and discussed. This is an important clinical syndrome which requires a high level of clinical suspicion for detection. The diagnosis rests primarily upon the clinical signs and symptoms. The C.T. Scan may provide useful confirmatory evidence and clarifies the differential diagnosis. Surgial treatment by resection of the infarcted tissue — mass lesion is urgently required for those patients who deteriorate progressively.

A 26 week gestation fetus with bilaterally symmetrical encephaloclastic lesions in the cerebral hemispheres is described. Information about gestation allows dating of the cerebral insult to the 3-4th fetal month. The history and morphology in this case suggest that insults occurring at or before the 3-4th fetal month interfere with normal neuroblast migration, producing a cerebral scar containing a band of neuroblasts arranged without order or lamination.

Consecutive craniotomies (118) drawn from major hospitals, and performed for disorders other than epilepsy or acute trauma were reviewed. The final diagnosis included tumor (70), subdural hematoma (13), aneurysm (10), arteriovenous malformation (7), and miscellaneous lesions (18). Eighty-seven (73.7%) patients had not experienced seizures prior to neurosurgery, II of these (12.6%) had a seizure within the first week, in six the attack occurred within 24 hours, and of these three had further attacks. In contrast, of the 31 patients (26.3%) that had one or more seizures prior to operation, II patients (35.5%) had seizures within the first week. In ten patients seizures occurred within the first 24 hours and of these seven had one or more recurrences later in the week. Anticonvulsant drugs were administered to 72 patients before operation, including all those with a history of seizures, hut loading doses were not utilized to ensure therapeutic levels. In patients with predisposing factors to postoperative seizures, anticonvulsant drugs should be administered before or immediately following craniotomy in adequate dosage to rapidly achieve and maintain effective plasma levels. Phenytoin, owing to its minimal sedative effects is the drug of choice. A loading dose of18 milligrams per kilogram can be safely administered as an admixture to an intravenous infusion of 0.9% saline with careful monitoring of cardiopulmonary function.

Continuous 48-hour polygraphic recordings of sleep/waking patterns were performed on 14 patients with narcolepsy-cataplexy before and after 7-10 days of treatment of their nocturnal sleep with gamma-hydroxybutyrate (GBH). GBH improved the quality of night sleep by increasing the amount of slow wave sleep, reducing stage I, increasing sleep efficiency (percentage of time in bed spent asleep), and reducing the number of periods of short sleep under 15 minutes. Also nighttime REM sleep was reduced in latency and became less fragmented. The daytime period contained less slow wave sleep and REM sleep, and fewer episodes of prolonged sleep. Patients experienced reduction or loss of daytime attacks of irresistible sleep, cataplectic attacks, and other auxiliary symptoms. Residual daytime drowsiness subsequently improved on low doses of methylphenidate. Tolerance did not develop and there were no serious toxic side-effects. Four of the patients had been refractory to previous combinations of antidepresents and high doses of stimulants.

Using the Xel33 inhalation technique, measurements of the blood flow to the left and right parietal and temporal regions of the cerebrum were obtained in 5 healthy individuals while simultaneously recording their EEGs. Up to 3 measurements were obtained from each of the subjects the first while they were mentally at rest and the others while they were engaged in prescribed forms of mental activity. Relationships between the measured blood flow through grey matter, initial slope index, relative grey weight, percent grey flow and power in the delta, delta-theta, alpha, beta and gamma rhythms of the EEG were examined. The results showed that for the subject group as a whole there was a strong correlation between the power present in the low frequency components of EEG and the grey flow and relative grey weight parameters of blood flow. On an individual basis, the observed relationships were highly variable particularly at high flow rates and at low relative grey weights, but became much more definitive at low flows and high weights. The results as they relate to previous work of this kind are discussed.

Two patients with previous cerebral damage and seizures and three patients with acute inflammatory cerebral lesions developed status epilepticus. They were unresponsive to standard anticonvulsants, but anesthetic barbiturates (thiopental and pentobarbital) stopped the seizures promptly.

This study is based on 28 personal cases. Twenty seven cases of infantile hemiplegia and one case of Sturge- Weber syndrome, with uncontrollable epilepsy and severe behavior disturbance, were subjected to hemispherectomy. Any evidence of a defect in the other hemisphere was a contraindication to operation. In nine cases the left cerebral hemisphere was removed. Following the operation, epilepsy and behavior disturbances were abolished in nearly all of the cases. In those with left hemispherectomy there was no speech disturbance before or after the operation.

It is postulated that at birth all cerebral funtions including consciousness, speech, and movement are bilaterally represented, but to unequal degrees. With training and education these functions become more lateralized. Thus, we can explain the greater plasticity of the brain in childhood.

A thirty-seven year old, East Indian female was admitted to St. Michael's Hospital on July 26, 1979 complaining of headache, diplopia, nausea and vomiting.

In April of 1978 she was referred to a neurologist for investigation of headache. Neurological examination was normal. An X-ray of her skull revealed a lytic lesion, 2 cms. in diameter, in the right frontal area. A bone scan showed no abnormality. Further study was refused. A left facial “birth mark” was noted.