False aneurysm of the intracranial arteries occurs infrequently as a complication of head injury and rarely as a consequence of other pathology. A case of false aneurysm of the supraclinoid internal carotid artery following minor intraoperative injury during clipping of a basilar-superior cerebellar aneurysm by the pterional approach is described. False aneurysm is a potential cause of recurrent intracranial hemorrhage after successful aneurysm clipping. Arterial bleeding which appears to come from the region of a branch origin should be viewed with concern even if it subsides spontaneously.

A 64-year-old man presented with a three day history of progressive Broca’s aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasie. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed periodic discharges on the left. At autopsy, there were marked cortical spongiform change, neuronal loss, and gliosis throughout the left hemisphere and in the right occipital cortex. Elsewhere in the right hemisphere, spongiform change was non-existent to minimal. There was moderate spongiform change in the molecular layer of the cerebellar cortex, much more marked on the left. Clinical and pathological unilateral cerebral predominance extended to the ipsilateral cerebellum. Creutzfeldt-Jakob disease is an important consideration in patients with rapidly progressive unilateral cerebral signs associated with a movement disorder.

Fifty-four per cent of 41 chronically institutionalized adult patients with epilepsy had ataxia of gait (wide mean stride width). None of the following correlated with stride width: serum phenytoin, previous phenytoin toxicity, seizure frequency, or status epilepticus. Seventeen of the 41 patients had computed tomographic head scans. Patients with radiological evidence of cerebellar atrophy had a wider mean stride width, later age of onset of seizures, greater peak serum concentrations of phenytoin than did those without cerebellar atrophy. Ataxia of gait was inconsistently associated with cerebellar atrophy. Elevated serum/plasma concentrations of phenytoin may be a risk factor for cerebellar atrophy, but seizure frequency or status epilepticus are not independently related to this complication.

The complicated pathophysiological and immunological changes in the central nervous system of patients with neurocysticercosis produce a variety of signs and symptoms, which complicate the clinical and surgical management of this disease. A complete and objective classification is needed, to improve the medical approach as a whole. We studied 336 patients, in whom we classified neurocysticerosis according to criteria of viability and location of the parasite in the CNS: active form (37.2%) when the cysticercus is alive, transitional form (32.8%) when it is in the degenerative phase, and inactive form (30%) when the parasite is dead. This classification establishes the correlation between the different forms of neurocysticerosis and its clinical manifestations, and can be used for planning therapeutic strategies.

Percutaneous retrogasserian glycerol rhizotomy (PRGR) was used during an 11-year interval in 53 patients with typical trigeminal neuralgia associated with multiple sclerosis. All patients had failed extensive medical trials prior to PRGR. Long-term (median follow-up, 36 months) complete pain relief (no further medication) was achieved in 29 (59%) of 49 evaluable patients. Eight patients (16%) had satisfactory pain control but required occasional medication. Twelve patients (25%) had initial unsatisfactory results with inadequate pain relief; nine underwent alternative surgical procedures. Sixteen patients (30%) subsequently required repeat glycerol rhizotomies to reachieve pain control. Twenty-seven patients (60% of 45 patients evaluated for this finding) retained normal trigeminal sensation after injection. Major trigeminal sensory loss developed in a single patient who had four glycerol rhizotomies over a 25-month interval. No patient developed deafferentation pain. We believe that PRGR is a low-morbidity, effective, and repeatable surgical procedure for the management of trigeminal neuralgia in the setting of multiple sclerosis.

A woman had a left superior cerebellar artery infarct associated with reduced hexamethyl-propileneamine oxime uptake on SPECT scan of the basal ganglia and frontoparietal areas of the opposite hemisphere performed poorly in some neuropsychological tests indicating right hemisphere dysfunction. There was a lengthening of reaction and movement times with the hand ipsilateral to the lesion. These deficits were temporary. A unilateral cerebellar lesion can produce neuropsychological deficits, possibly because of hypoperfusion in contralateral frontoparietal regions, but spontaneous neuropsychological remissions may occur.

A patient with a remote infarct, seizures, mild hemiparesis, and dysphasia became obtunded over four months and died. Computerized tomography (CT) over 5 years showed a consistent, large, wedge-shaped left hemisphere hypodensity with a central calcification, but without signs of mass effect. This was interpreted as an infarct of the left middle cerebral artery territory. Post-mortem examination of the brain revealed the entire area appearing as infarct on CT was a gliosarcoma. We suspect that the unusual CT appearance of the lesion was likely caused by multiple pathologies: a low grade glioma transforming into a gliosarcoma that was able to spread throughout the area of infarct encephalomalacia without revealing a typical CT appearance of mass effect. The patient's brief period of deterioration probably coincided with transformation of the tumor into a gliosarcoma. The variable CT characteristics of gliosarcomas are reviewed.

Gilles de la Tourette syndrome is a neurological disorder characterized by the presence of motor and vocal tics. Other associated features include complex motor and vocal tics, obsessive compulsive behavior and attention-deficit disorder. Although initially thought to be a rare condition, it now appears that the gene for Tourette syndrome may be present in as many as one in a thousand people, and is transmitted as an autosomal dominant trait. However, many individuals carrying the gene may exhibit only minimal symptoms. Neuropathophysiology of Tourette syndrome is unknown but investigations have suggested that the cause may be an abnormality either in the dopaminergic or endorphin receptor system within the basal ganglia. Treatment of Tourette syndrome involves education and counseling of the patient and family. Medications such as neuroleptics, serotonin-reuptake inhibitors, and stimulants are available to treat the manifestations of Tourette syndrome and need to be individualized for each patient.

The use of the combination of fluoxetine, an anti-depressant serotonin uptake inhibitor, and selegiline, a monoamine oxidase -B inhibitor, was reviewed in a large population of patients with Parkinson’s disease. All records were reviewed from a Parkinson’s disease clinic to determine how many patients were treated simultaneously with selegiline and fluoxetine. Patient characteristics, duration and dose of treatment, side effects and reasons for discontinuation were noted. Twenty-three patients received both medications at the same time. No additional side effects were noted with the combination therapy that had not already been reported with each medication alone. No serious side effects were found. In this clinic population, fluoxetine and selegiline were used in combination without major side effects, but further observation is warranted.

To assess the frequency of subjective and objective dysphagia and its possible pulmonary sequelae, we prospectively studied 22 out-patients with Parkinson's disease; 15 spouses served as controls. All subjects answered a standard questionnaire concerning swallowing and respiratory functions and underwent barium swallow videofluoroscopy. Possible pulmonary infection was investigated by recordings of body temperature, ESR, leucocyte count, and chest X-ray. Patients had significantly more symptoms than controls, especially choking, piece-meal deglutition and regurgitation. Videofluoroscopy revealed tracheal aspiration in one patient, vestibular aspiration in one patient and in one control. Non-fluent swallowing movements were common in patients: abnormal bolus formation, delayed swallowing reflex, vallecular stasis, and piriform sinus residue. None of the subjects had signs of pulmonary infection. Both subjective and objective oro-pharyngeal dysfunction is frequent in ambulant Parkinson patients, but apparently does not produce demonstrable pulmonary infection.

Interest in vascular causes for cognitive impairment is increasing, in recognition that such causes are common, and possibly preventable. This has led to attempts to better define vascular dementia and its natural history. Several sets of criteria for the diagnosis of vascular dementia have been proposed. We provide a brief overview of the background to the initiation of a Canadian consensus conference, established by the Consortium of Canadian Centres for Clinical Cognitive Research (C5R) and report the conclusions reached at that conference. To date, no one set of criteria is demonstrably superior to another; we have therefore not endorsed any of the competing sets, nor have we recommended our own. Instead we suggest that empiric studies are required to establish valid criteria. A diagnostic checklist, which combines existing criteria and additional data, is attached for clinicians wishing to participate in such studies.

Reports of a high prevalence rate for multiple sclerosis in Southern Alberta led to an epidemiologic study of this disease in the Crowsnest Pass and Cardston regions. In Cardston, the prevalence rate for multiple sclerosis was 88 per 100,000. In the Crowsnest Pass, the prevalence rate was 217 per 100,000. Previous epidemiologic studies of the prevalence rate of multiple sclerosis in Western Canada have shown rates between 93 and 111 per 100,000. Two prevalence studies of multiple sclerosis in Barrhead County, Alberta and Westlock County, Alberta show prevalence rates of 196 and 201 per 100,000. The prevalence rate in the Crowsnest Pass is comparable to the prevalence in Barrhead County and Westlock County, Alberta. However, there is no statistically significant difference between prevalence rates in the Cardston and Crowsnest Pass regions and our overall feeling is that the results of studies of small populations should be interpreted with caution.

The specialty of neurological surgery in this country began when Kenneth G. McKenzie was appointed to the Toronto General Hospital surgical staff in 1924, Canada's first dedicated neurosurgeon. In the years and decades that followed McKenzie and his successors established the Toronto General Hospital as one of the leading clinical and teaching neurosurgical units in the world. It was not without some sadness, therefore, that in 1990 neurosurgery left the walls of the Toronto General Hospital, the service transferred to join with the neurosurgical division of a sister hospital, the Toronto Western, during a merger which created the new, two-site, Toronto Hospital. The following story is of the men and women at the Toronto General Hospital who provided, advanced, and taught neurosurgical care. Many persons, among them orderlies, nurses and physicians, will not receive the mention they deserve in the pages that follow, but are no less remembered.

The swallowing movements of 22 ambulant patients with Parkinson's disease were examined by videofluoroscopy, 15 spouses serving as controls. The relation between bolus volume and movement of the hyoid bone was studied in the lateral view to assess the adaptation of the pharynx musculature. Although movement characteristics of the hyoid varied considerably both intra- and interindividually, mean values followed typical patterns. In controls, the position of the hyoid before the start of the swallow tended to be lower as the bolus volume increased, apparently to create space in the oral cavity by lowering the floor of the mouth. This trend was not seen in the group of Parkinson patients. We suppose that this specific lack of adaptation represents hypokinesia. The amplitude of the hyoid movement did not change with increasing bolus volumes, either in patients or in controls. Moreover, Parkinson patients had many more aborted swallowing movements (“hesitations”) than controls, probably representing defective switching from the voluntary phase of swallowing to the involuntary, automatic phase.

We present the results of a randomized double-blinded placebo controlled, multicenter trial, of low-dose mitoxantrone (MX), after one year, in 25 patients with relapsing-remitting multiple sclerosis, who had serial enhanced magnetic resonance imaging (MRI). Treatment groups were balanced for age, gender, duration of illness and neurological disability. Five of the 13 MX patients and 10 of the 12 placebo patients had exacerbations during treatment (p < 0.02). The mean change in the extended disability status scale was not significantly different between the MX and placebo treatment groups. Serial Gadolinium-DTPA enhanced MRI detected no significant difference between the MX treated and placebo groups in the mean total number of new, enlarging, or Gadolinium-DTPA enhancing lesions; there was a trend toward a reduction of new, enlarging and Gadolinium-DTPA enhancing lesions in MX patients. Despite this ameliorating effect, the results indicate that serial Gadolinium-DTPA enhanced MRI, performed over one year in a limited number of patients, could not provide conclusive evidence for a role of MX therapy in relapsing-remitting multiple sclerosis.

We describe a 31-year-old man with clinically definite multiple sclerosis who presented with intractable hiccup for one month prior to admission. The responsible lesion was confirmed by magnetic resonance imaging to be a plaque in the ventral paramedian portion of the medulla oblongata.

A patient with a three year history of POEMS syndrome-polyneuropathy, hepatosplenomegaly M-protein, skin changes, optic disc edema, peripheral oedema, pleuritis, lymphadenopathy, and osteosclerotic myeloma is described. The patient had a twenty year history of psoriasis and impotence was the first sign of the disease. The pathophysiology of this multisystem disorder is unknown, although the M-protein is essential. No improvement was found after radiation, chemotherapy and steroid treatment.