Extensive recent literature on drugs used to enhance cognitive functioning, reflects the growing social problem of dementia. Many clinical trials have been undertaken with variable success. In most cases the disorder studied has been Alzheimer's disease. The pharmacological approach has been designed to rectify the presumed pathophysiological processes characteristic of the condition. Agents tested include cerebral vasodilators, cerebral metabolic enhancers, nootropics, psychostimulants, neuropeptides and neurotransmitters with a special emphasis on drugs used to enhance cholinergic function. Ethical and practical issues concerning clinical drug trials in dementia will be discussed.

Central nervous system (CNS) involvement in systemic lupus erythematosus is a major source of patient morbidity and mortality. The recognition of nervous system lupus is hampered by the diagnostic insensitivity and non-specificity of the various testing modalities that are currently available. A review of the effectiveness of diagnostic tests for CNS lupus is presented. Areas of current research in this area are examined. Because of the diversity of neurologic manifestations in this disorder and their complex pathogenesis, no single test is sufficient to establish the diagnosis rapidly and accurately in all cases, now or for the foreseeable future.

The validity and reliability of clinical instruments, including clinical scales, need to be determined. This paper presents guidelines for development, validation, and reliability assessment of stroke assessment scales.

Magnetic resonance imaging (MRI) has been shown to be a good method of visualizing the lesions in MS. We have studied several applications of MRI to the evaluation of patients and experimental models. In diagnosis, MRI is the most sensitive test for the demonstration of dissemination of lesions in space. Pathological correlation studies show that MRI reliably measures the extent of chronic demyelination. Experimental studies show that MRI detects acute inflammatory lesions and measures their evolution. MRI also is a reliable measure of the extent of the MS process, serial MRI scans detect evidence of disease activity in MS not always disclosed by clinical evaluation. MRI will have an enormous future impact on the evaluation of patients in clinical studies and in understanding the evolution of pathological processes.

It is easy to imagine a new bumper sticker with the following message:

“If you think medical research is expensive, try disease.” Specifically in today's context, it might well read:

“If you think neurological research is expensive, try the diseases of the nervous system.”

That is precisely what we are doing as a country - “trying disease”! Canada spends $36 billion per year on health care, or $700 million per month. Every hour Canada spends $4 million on "health care" and only $16,000 on medical research.

Seventy-six patients with blepharospasm (mean age 56.9 years) received 248 injection treatments with botulinum A exotoxin (mean 3.1 treatments per patient): 87.0% of treatments led to total relief of spasms for a mean interval of 14.1 weeks. The average duration of response remained fairly constant over the first six injection series, although patients with the most severe spasms had shorter intervals than patients with less severe symptoms. Twenty patients with hemifacial spasm (mean age 56.9 years) received 44 treatments (mean 1.9 treatments per patient): In 93.1% of cases there was total relief of periocular and perioral spasms, with a mean interval of 17.4 weeks. The average duration of response for the third series of treatments was much shorter than the mean durations for the first two treatments. Side effects were always transient and included ptosis (23.3%), dry eyes (18.1%), tearing (5.5%), and strabismus (1.4%). No patient had a systemic reaction to the drug. Chronic benign eyelid fasciculations were also successfully treated in 3 patients with single treatments.

Changes in anticonvulsant serum levels during intercurrent illness may cause toxicity or decreased seizure control in children with epilepsy. We studied prospectively the effect of intercurrent illness and its treatment in 111 children being treated with AC monotherapy. Free fraction and total serum AC levels were determined when the child was well, on the fifth day of any illness with fever and one month after recovery. There were 55 episodes of febrile illness in 39 children during the study period. Twelve illnesses were associated with significant increases or decreases in serum AC levels; 7 children became clinically toxic; 1 child had increased seizures during illness. The mechanisms of AC level changes appeared to include interaction with antibiotics, with antipyretics or with viral illness. Amoxycillin and acetaminophen did not appear to interact with the AC's used. Physicians caring for children with epilepsy should be aware of the frequency and complexity of potential interactions between intercurrent febrile illness and anticonvulsant medication.

A visual psychophysical adaptation procedure was used on patients with Multiple Sclerosis (MS) in an attempt to induce a temporary and local exacerbation of subclinical visual impairment. Using a flicker detection task, sensitivity before and after adaptation to a flickering stimulus was measured in 9 MS patients and 9 control subjects. Although only 22% of patient eyes had abnormal flicker sensitivity prior to adaptation, visual deficit was observed in 83% of eyes studied after adaptation. Of the 7 MS eyes studied for which no other sign or symptom of visual involvement was present, 5 were found to have visual deficits after flicker adaptation. In addition, 10 of the 11 eyes affected by MS showed an abnormal response to flicker adaptation. Recovery from the effects of adaptation was complete in all patients within 2 minutes. The results suggest that partial demyelination of visual pathway neurons may exist in patients without signs or symptoms of visual involvement. The prolonged stimulation provided during adaptation may produce a temporary fatiguing or conduction blockade of such neurons which may lead to reductions in sensory sensitivity.

We recorded somatosensory evoked potentials (SEP) in 15 patients affected by Friedreich's ataxia (FA) and in 9 patients with progressive early onset cerebellar ataxia (PEOCA). Brainstem auditory evoked potentials (BAEP) were also recorded in 14 FA patients and in five PEOCA patients. SEP results showed clear differences between groups of FA, evidence of peripheral involvement was seen in all patients, with absence of the N9 potential or a major reduction of its amplitude. In patients in whom central responses could be recorded, conduction velocity was normal or near normal up to the brainstem but was reduced from brainstem to cerebral cortex. Four patients with PEOCA had SEP abnormalities similar to those seen in FA. In the five other patients, the amplitude and latency of N9 were normal but conduction velocity was reduced from brainstem to cerebral cortex. In FA, BAEP were abnormal in all patients with a disease duration of four years or more but were normal in four of the five PEOCA patients. Systematic evoked potential recording is useful in the investigation of hereditary ataxias.

High-resolution CT scans of the brain and posterior fossa were performed on 106 phenytoin (PHT)- treated epileptics, 28 de novo epileptics and 43 control subjects. A higher incidence of cerebellar and brainstem (CBS) atrophy was observed in chronic PHT- or PHT+ phenobarbital-treated epileptics compared to the two other groups. Some control subjects and de novo epileptics presented mild CBS atrophy, whereas moderate to severe atrophy was noted exclusively in chronically-treated patients. In attempting to delineate the etiology of CBS atrophy, epileptic patients were divided in three groups: 55 subjects with normal CT scans, 30 with both cerebral and CBS atrophy, and 49 with pure CBS atrophy. Their ages, length of illness, number of generalized seizures, number of other seizures, and amount of PHT received during their lifetime were assessed. Statistical analysis revealed that posterior fossa atrophy in epileptics was significantly correlated with both the length of the illness and the amount of PHT ingested during the patient's lifetime. The number of seizures appears to not be related to CBS atrophy.

The initial presentation and clinical course of this 60-year old woman suggested a diagnosis of myasthenia gravis. The subsequent development of tongue fasciculations and the lack of response to treatment made a diagnosis of amyotrophic lateral sclerosis (ALS) more likely despite the presence of conjugate gaze paresis and the absence of many of the typical clinical and electromyographic (EMG) findings seen in this condition. The pathological findings were consistent with either a motor neuronopathy or an unusual variant of ALS. We review the clinical and pathological features of this unusual case in this report.

An unusual case of sphenoethmoidal sinusitis complicated by cavernous sinus thrombosis, meningitis and pontine and cerebellar infarction is described. The patient presented with advanced intracranial complications which in retrospect caused delay in recognition and treatment of the underlying sphenoethmoidal sinusitis. Surgical drainage of the sinusitis was ultimately required. The pathogenesis of these complications is discussed, and the topic of sphenoid sinusitis reviewed in order to emphasize the numerous neurological manifestations of this disease.

Involvement of the peripheral nervous system by amyloidosis is common. It is less well recognized that amyloid can directly infiltrate and weaken skeletal muscle. We report a case of a 73-year-old woman, known to have cardiac amyloidosis, who developed profound weakness secondary to amyloid myopathy. Review of the 8 other well documented cases in the literature has revealed a rather homogeneous syndrome. Proximal weakness, muscle stiffness, pseudohypertrophy and myalgia constitute the principal features. This syndrome usually develops in cases with well recognized generalized amyloidosis. Amyloid is deposited within the basal lamina of blood vessels and muscle fibers.

En premier lieu, je tiens à féliciter les principaux responsables de l'organisation de cette première journée des sciences neurologiques de l'Université de Montréal, soit les docteurs Guy Bouvier, Pierre Duquette, Yves Lamarre, Gérard Mohr, Pedro Molina-Negro et Serge Rossignol. Grâce à eux, à Madame Hélène Auzat, secrétaire, et à bien d'autres, cette journée s'est avérée un franc succès.

Basic research helps to understand clinical knowledge which in turn leads to new fundamental research. Two examples are taken from the studies of the anomalies existing in the brain of patients suffering from Parkinson disease or deafferentation Pain. The researches performed to solve these two problems for the past 40 years (in particular by the author's working group) are replaced in the international context. In Man the precision of stereotaxic technique was improved by using the organisation of the somato-sensory thalamic projection described in animals by the physiologists. In the thalamus of Parkinsonian patients, the presence of cells bursting at tremor frequency led to search for the relations existing between the striato-nigral system and the motor thalamus. In the patients suffering from deafferentation Pain, a special role is attributed to the medial thalamus, whose characteristics were formerly studied in animals. The origin of the deafferentation Pain is nowadays studied in an animal model of this disease.