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.

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.

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.

Regional cerebral blood flow measurements and neuropsychological testing were conducted before and after venesection on 6 patients with polycythemia secondary to chronic obstructive pulmonary disease. Venesection resulted in lowered viscosity and hematocrit, and an accompanying improvement in cerebral perfusion and mental function. Blood flow was significantly increased in the left cerebral hemisphere following phlebotomy, and there was significant improvement in sensory I mental function. Cerebral function would appear to be related to blood flow alterations influenced by the viscosity of the blood.

We present the first case of central pontine myelinolysis (CPM) confirmed by high resolution computerized tomography (CT) in which auditory brainstem responses (A BR) revealed impaired conduction beyond the mid pons. The combined use of CT and A BR in the diagnosis of central pontine myelinolysis is discussed.

A case of intraventricular hemorrhage secondary to a ruptured aneurysm of the posterior inferior cerebellar artery in a child is presented. Treatment is discussed and the literature is reviewed.

The cerebral pathology in a case of tricyclic antidepressant overdose by amitriptyline is described. A review of the literature of similar cases is summarized. The relation of the cerebral lesions to the development of a hypertensive episode possibly induced by amitriptyline is discussed.

A patient exhibited confusion, dysphasia, and striking asterixis 18 hours after metrizamide myelography. The symptoms lasted for a day and a half. While metrizamide is a useful contrast medium, side effects are not unusual and can, on occasion, be quite severe.

A rare abnormality of the vertebral artery is described in a 63-yearold male. The right vertebral artery consisted of several minute endothelial lined channels which failed to perfuse at autopsy. The anterior spinal artery showed areas of muscular media dysplasia. These anomalies were associated with large posterior communicating arteries. The terminal event was precipitated by thrombotic occlusion of the left vertebral artery. The embryological aspect of these anomalies are discussed.

The authors present a simple system for disability scoring and functional staging of an ataxic patient, based on modifications of a previous scheme advocated by De Falco and collaborators (1979). This system was tested under field conditions in 47 ataxic subjects and found to be useful and functional.

We have studied a large family of which seven members suffer from a progressive disease with onset in the first decade. The first symptoms were gait ataxia and clumsiness in all cases, followed by progressive development of severe distal amyotrophy reminiscent of Charcot-Marie-Tooth disease. In four patients a postural tremor which was relieved by pharmacological agents was also evident in the limbs or head.

Cerebellar atrophy was confirmed on CT scan. Motor nerve conduction velocities were in the low normal range, while sensory nerve conduction was markedly decreased. All patients had impaired proprioception and vibration sense. The laboratory investigation revealed a normal CSF protein level and elevated serum bilirubin.

The patients reported in this study apparently suffer from an original recessive form of spinal and olivocerebellar degeneration associated with a neuronal form of Charcot-Marie-Tooth disease.

We report a clinical and biochemical survey of 23 patients with Friedreich's ataxia from southern Italy. They were studied clinically and by means of a clinical rating scale devised by us (Inherited Ataxias Clinical Rating Scale). Laboratory tests, based on the Quebec Cooperative Study, were also performed on our patients. No major clinical or biochemical differences were found between Italian and Canadian patients. Investigation of CSF monoamine metabolites showed that HVA decreased after probenecid and metoclopramide loading

Eighteen patients with the presumptive diagnosis of Friedreich's ataxia were studied. Clinical, neurophysiological and biochemical data were concordant in 14 patients and led to the diagnosis of typical Friedreich's ataxia in this group of patients. The remaining 4 patients differed from the typical patients in several respects, but mainly in the cardiological findings. It is concluded that no single clinical or laboratory finding is typical of F.A. Multidisciplinary approaches are essential to the diagnosis of Friedreich's ataxia.

Dynamic muscle function was evaluated in nine patients with Friedreich's ataxia (FA) and eight with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). The measurement of torque throughout maximum voluntary isokinetic knee movements was used to quantitatively describe muscle weakness in the ataxic patients. Both FA and ARSACS patients were shown to have decreased dynamic strength in comparison to normal values during knee extension and flexion movements at 30% /s. In the FA patients a lower torqueproducing capacity was seen in the older patients.

The electromyographic (EMG) activity was recorded in lower extremity muscles during the movements. In the vastus lateralis (VL), deviations from the normal EMG activation pattern were described in both groups of patients. A reduced amplitude in the EMG activity in the medial hamstrings (MH) was seen in the majority of the patients. An index of coactivation was defined by comparing the EMG activity when a muscle lengthened (antagonistic) to the EMG activity when the same muscle shortened (agonistic) during the isokinetic contractions. In comparison to normal values increased coactivation indexes were present in the VL and MH in patients of both groups. The characteristics of dynamic muscle strength and the activation of agonistic and antagonistic muscles described in the present study will provide the basis of evaluation for the effects of therapy in these patients.

We evaluated 15 patients with Friedreich's ataxia (FA) to define the incidence of myocardial involvement and the type of cardiomyopathy observed.

All patients with FA had either ECG, vectocardiographic or echocardiography abnormalities, suggesting some degree of myocardial involvement. In contrast to reports indicating that asymmetric septal hypertrophy (ASH), often obstructive, is associated with FA, symmetric, concentric hypertrophic cardiomyopathy (SCH) was the predominant abnormality (sixty-seven percent of patients). Echocardiograms should be performed periodically in all FA patients since this technique allows the detection of cardiac hypertrophy.

The preliminary results based on a one year study on the evolution and management of scoliosis are presented. Twenty-one patients were followed in the Neuro- Muscular Disease Clinic at Ste- Justine Hospital where standardized spinal radiographs were taken periodically with the Scoliosis Chariot and the Throne. The short period of observation as well as the relatively small number of Friedreich ataxia patients followed requires that these results and the following remarks be interpreted with caution.

Pathomechanics — Between the age groups I (5- 10 years) and 11(10-15 years), a substantial increase in the Cobb values occurs. Associated with it, an increase was observed in the thoracic and thoraco lumbar projected surface area indices. The relative rotation between the thoracic and lumbar segments was presumed to be the cause of the sudden increase in the Cobb measurements.

For the non-ambulatory patients, a decrease in the lumbar lordosis towards a thoraco-lumbar kyphosis as well as a sudden increase in the sacral angle and a drop in the lumbosacral angle were associated with the seated posture assumed by the patient.

Management — Prevention of the progression of established curves was our main objective. Careful examination of the spine, depending on the age of the child, in our preliminary study, stimulated early orthopaedic treatment in any curve of 20° or more. There was always concern for curves of 30° or more. In the growing child, bracing was recommended.

In the older child, the curve was usually stable after sixteen years of age. Surgery was usually attempted in curves over 40° in the growing child. The same curve was usually stable after the growth period.

For the non-ambulatory patients, the present study suggested the prescription of a molded seat with the following characteristics: i) a posterior lumbar support, ii) low thoracic lateral supports and iii) a slight inclination of the seating system. This was presumed to be beneficial in maintaining stability of the spine. Presently, an evaluation of such a device is under investigation.