Between December 1986 and June 1990, 112 patients (116 lesions), underwent treatment with dynamic stereotactic radiosurgery at McGill University. Of the treated lesions, 59 were arteriovenous malformations and 53 were a variety of other neoplastic or non-neoplastic conditions. In 86 lesions, the treatment was delivered in a single fraction and the treatment of the remaining 30 lesions was fractionated. Complications attributed to treatment developed in seven of the 112 patients (6.3%). No relationship was found between complications and prescribed dose, fractionation, collimator diameter, type and anatomical region of the lesion that was treated, or previous irradiation. Although extensive clinical experience will be necessary to determine optimal total doses, the potential role of fractionated treatment, and the tolerance of critical structures to radiosurgery, the relatively low incidence of complications in our series allows us to conclude that radiosurgery is well tolerated by the vast majority of patients.

In recognition of the 100th anniversary of Charcot’s death we have reviewed possible pathogenic mechanisms in amyotrophic lateral sclerosis (ALS). Advances in the last 5 years in molecular biology and genetics have identified mutations in the cytosolic dismutase (SODI) gene in some patients with familial ALS raising the possibility that oxidative stress may be involved in the pathogenesis. An excitotoxic pathogenesis has been implicated based on elevated plasma and CSF levels of amino acids and altered contents of amino acids in the nervous system of ALS patients and changes in the number of excitatory amino acid receptors. ALS sera containing antibodies to L-type calcium channels and the development of immune mediated lower and upper and lower motor neuron models have revitalized research efforts focusing on an immune basis for ALS. Other pathogenic mechanisms which have been the subject of recent research include elemental toxicity, apoptosis and programmed cell death and possibly a deficiency or abnormality in growth factors. Pathogenic processes for ALS must account for an increasing incidence of ALS, male preponderance, and the selective vulnerability of the corticomotoneuronal system.

Human T-cell lymphotropic virus type 1 (HTLV-I) is responsible for HTLV-I associated myelopathy or tropical spastic paraparesis (HAM/TSP) and for adult T-cell leukemia/lymphoma (ATLL). Both diseases have been well described in individuals living in Japan, West Indies, Seychelles Islands and Columbia where infection with HTLV-I is considered endemic and in persons whose descendants originated from these endemic areas. We report here 4 cases of HAM/TSP in 4 natives from 4 different tribal groups from British Columbia (B.C.). These are the first case reports of HTLV-I linked diseases found among North American Aboriginals. Possible routes of infection for HTLV-I infection included sexual transmission, breast feeding, blood transfusions and IV drug use. The seroprevalence of HTLV-I in North American Native population is unknown and we suggest that it is endemic in this ethnic group.

Parkinson’s disease prevalence rates were examined for the Province of Alberta by age, sex and census division. Using the claims administrative data from the Alberta Health Care Insurance Plan, a cohort of all registered individuals (2.4 million) was extracted and followed for the five year period, April 1, 1984 to March 31, 1989. No new members were added to the cohort and an attrition rate averaging 6% per year was observed. The overall crude prevalence rates of 248.9 and 239.8 per 100,000 population were noted for males and females respectively. Both sexes were found to have a statistically significant variation across Alberta’s 19 census divisions. For males, examination of standardized morbidity ratios found a low risk of Parkinson’s disease associated with five census divisions, of which two contained Alberta’s two largest cities. An excess risk was associated with four primarily rural census divisions. Females, on the other hand, had a low risk associated with one rural census division and excess risk in four census divisions. The uneven distribution within Alberta offers support for an environmental theory of etiology which may be associated with rural living.

Clinical differentiation between forms of progressive dementia can prove difficult, particularly when relatively rare forms of dementia are involved. Factors such as family history of dementia, age at onset, presenting features such as personality change, cognitive deficits, psychiatric symptoms, and clinical course (progressive deterioration; retention of skills over time) may prove useful for directing investigations to identify underlying pathology and genetic implications. This is illustrated by two patient reports. Each patient had the onset of memory/behavioral problems at approximately age 40 years, was initially given a psychiatric, non-dementing diagnosis, and had a positive family history for early onset behavioral and memory problems. After longitudinal assessment, the diagnosis of Alzheimer disease was confirmed at autopsy in one patient and a diagnosis of familial, adult-onset metachromatic leukodystrophy in the other.

Glutamate excitotoxicity is implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). We report the results of a double blind, placebo controlled, trial using 100 mg of oral daily lamotrigine (3,5-diamino-6-(2,3 dichlorophenyl)-l,2,4-triazine) which inhibits glutamate release. 67 patients were entered and at trial termination of 1.5 years 15 had withdrawn (9 active and 6 placebo) and 12 had died (6 active and 6 placebo). Mean age at entry was 57.5 years for the active and 58.6 years for the placebo groups. Patients were seen at 3 monthly intervals and scored according to neurological deficit based upon age of onset, bulbar and respiratory involvement, ambulation and functional disability. The mean change in clinical scores for the active versus placebo groups over the trial period was 7.1 ± 3.3 and 9.0 ± 3.3 respectively (0.05 < p < 0.10). Changes in cortical threshold and MEP/CMAP ratios to magnetic stimulation also did not differ significantly between the two groups. We conclude that lamotrigine in the doses administered does not alter the course of ALS.

Five patients with evidence of focal encephalopathy are reported. In each case, evidence of mycoplasma pneumoniae infection was detected. No patient improved with conventional antibiotic therapy, but in three subjects, rapid and complete recovery did occur contemporaneously with the administration of high dose steroid therapy. It is suggested that focal as well as diffuse cerebral or cerebellar lesions may occur as manifestations of auto-immune disease complicating mycoplasmal infections in young people and that this illness may be designated as acute mycoplasma-associated encephalopathy.

Four patients presented with hemiballism-hemichorea as a clinical manifestation of white matter ischemia. These patients illustrate “positive” motor phenomena rather than limb weakness as a consequence of cerebral ischemia. In each patient, the involuntary movements disappeared following worsening of paresis. Subcortical white matter infarction in three patients and hemodynamic hypo-perfusion in the cerebral hemisphere contralateral to dyskinetic movements were possible causes. Neuroradiologically, none had pathological changes in the vicinity of the subthalamic nucleus. We presume from these observations that ischemia of the subcortical white matter, without involvement of the basal ganglia or the subthalamic nucleus, may cause hemiballism-hemichorea

Cervical myelopathy consequent on ossification of posterior longitudinal ligament (OPLL) is very rare in Caucasians. A 65-year-old Anglo-Saxon woman developed progressive gait disturbance, paresthesia in both legs and urinary urge incontinence. Radiological examination showed OPLL from fifth to seventh cervical vertebral level; the dense OPLL was graphically displayed by three-dimensional computerized tomography. Medial corpectomy, C5 to C7, and removal of OPLL, with subsequent fusion C4 to Tl using a free fibula graft resulted in clinical improvement. Three dimensional computerized tomographic imaging is a valuable diagnostic procedure in OPLL.

A 4-year-old boy developed Rasmussen’s syndrome and was treated with alpha interferon intraventricularly. An improvement in the epileptic and neurologic syndrome was noted for several weeks following interferon. No adverse side effects were encountered. Since hemispherectomy is the only established therapy in Rasmussen’s Syndrome, further studies are needed to establish if intraventricular alpha interferon may halt the clinical progression of the syndrome.

Dr. Bill Lougheed was able to navigate residents through even the most complex neurosurgical procedures with complete and often heroic patience, always ensuring for the patient his usual expert technical result but still leaving intact the resident’s sense of pride and achievement, the feeling that he, the resident, “had done it”. A gifted technical surgeon, a thoughtful and inventive neurosurgeon, Dr. Lougheed was above all else one of Canada’s great teachers of operative neurosurgery. Canada’s pioneer microneurosurgeon, Dr. Lougheed brought the Toronto General Hospital to the very forefront of the emerging field of vascular neurosurgery.

Denis Naldrett White was born in Bristol. He completed his premedical and medical education at Cambridge. This was followed by graduate training at the London Hospital with Russell Brain, Hospital for Sick Children, National Hospital for Nervous Diseases and the Institute of Psychiatry, University of London, where he completed a senior registrar post in 1948. That same year he was recruited by Dr. Ford Connell, Head of the Department of Medicine, to Queen’s University.

The Conference was held in Hamilton, Ontario, on May 18-21 1993, and was the successor to the meetings in New York, Monaco, Cyprus and Mainz. Normally the conferences are held every two years but in this instance the event had been deferred to as not to clash with the 1992 International Congress of EMG and Clinical Neurophysiology in Jerusalem. The Hamilton meeting had been immediately preceded by the International Course on Single Fiber EMG and Other Special Techniques and over 150 registrants and faculty attended the two events. A noted absentee was Dr. Erik Stalberg (Uppsala), the pioneer of single fiber EMG, who was unexpectedly taken ill shortly before the meetings began; by the unanimous decision of the participants, both the International Course and the International Conference were dedicated to Dr. Stalberg.