This report describes a freeze fracture study of rat dorsal sympathetic chain in normal, streptozotocin diabetic on normal diet, and a group fed a 1% myoinositol normal diet. In nonmyelinated fibres, the diabetic group had significant loss of particles on the P face ofjuxta-axonal Schwann cell membranes, whilst myelinated nerves showed a profound loss of particles on P and E faces. In the group with adjuvant myoinositol the particle numbers were normal in both types of nerve. Axonal plasma membranes showed increased numbers of particles in the P face of the nonmyelinated membranes in the diabetic, but were normal in the myoinositol group. Myelinated axonal membranes showed no significant difference between the diabetic and the normal. Rats on a myoinositol diet showed a significant decrease in the E face particles. These results are consistent with those described in the sciatic myelinated nerves by earlier authors and reinforce the view that myoinositol is an important membrane constituent as phosphatidyhnositol and is essential for the expression of normal protein particle numbers.

Clobazam is a new benzodiazepine recently introduced in Canada on an experimental basis. We report our experience with 27 children (average age 9.1 years) with severe intractable seizure disorders. All had mixed seizures (41% focal plus generalized, 59% mixed generalized) and 93% were mentally retarded. Eighty-five percent had multiple daily seizures and all had been on an average of 6 other anticonvulsants in the past. Forty-one percent of our patients responded to clobazam with a reduction of greater than 75% in seizure frequency and 15% had no further seizures. Tolerance to clobazam which could not be overcome by dose increases developed in 26% of patients. Fortyfour percent of non-responders had exacerbations of their seizures shortly after starting clobazam, however 43% of these patients had reductions in comedications simultaneously. Side effects of clobazam in responders were minimal. We conclude that clobazam shows sufficient promise to warrant further trials in more benign seizure disorders in children.

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.

Lymphocytic adenohypophysitis (LAH) is an uncommon disorder in the spectrum of pituitary disease. Twenty-three cases proven by biopsy or at autopsy have been reported since 1962. We report 2 further cases and review the etiology, immunology and pathology of the disease. The diagnosis should be considered in afemale patient who presents during the post-partum period with the clinical picture of a non-functional or prolactin cell pituitary adenoma and evidence of hypopituitarism.

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.

We describe a child with Brown-Sequard syndrome beginning at C8-T1, immediately following Harrington rod instrumentation for idiopathic scoliosis. We believe this is most likely related to either a common variation of the branches of the anterior spinal artery to one side or due to rupture of the ipsilateral spinal vein. This possible complication should be considered in patients undergoing Harrington rod instrumentation.

Chickens homozygous for the epi gene (epileptics) suffer from spontaneous seizures throughout their life, whereas heterozygous (carriers) are phenotypically normal. Seizures can also be evoked in epileptics by photic stimulation. In addition, epileptic chickens' brains are 25% heavier than those of carriers. We have investigated whether hyperplasia or hypertrophy of astrocytes or increased numbers of astrocytic processes are involved in the development of seizures and the megalencephaly in this model by quantitative comparison of sections immunocytochemically stained for glial fibrillary acidic protein (GFAP). No statistically significant differences between epileptics and controls were found in any of seven areas selected for comparison. In this model gliosis is not involved in the development of epilepsy, nor does it result from repeated seizures.

This 60 year old male developed a right hemiplegia and aphasia. A C.T. head scan showed a cerebral infarct which appeared hyperdense on a subsequent scan done 18 days after presentation. This was interpreted as indicating a hemorrhagic transformation resulting in discontinuation of anticoagulation therapy. At autopsy, the area of infarction in the left frontoparietal hemisphere appeared intensely green due to breakdown of the blood-brain barrier in the presence ofjaundice. A striking finding on microscopy was the presence of calcium salts throughout the area of infarction but most prominent in the grey matter at the periphery of the infarct corresponding to the areas which appeared hyperdense on the CT head scan and stained intensely with bilirubin. There was no evidence of recent hemorrhage. This case illustrates that calcification can occur within weeks after the onset of a recent cerebral infarct and should be considered when interpreting the development of C.T. scan hyperdensity in recent cerebral infarcts.

Wernicke disease (WD) is a complication of alcoholism and malnutrition and usually presents acutely and is characterized by disturbances of consciousness, paralysis of the external ocular muscles, and ataxia. The disease results from deficiency of vitamin B 1, or thiamine, an essential coenzyme in intermediate carbohydrate metabolism. On the other hand, Jakob-Creutzfeldt disease (J-C) results from infection with an unconventional agent with a long incubation period and is characterized by a rapidly progressive dementia and histologically by a spongiform encephalopathy associated with neuronal destruction and pronounced astrogliosis. Combination of both diseases has not been reported in the literature previously and their relationship is uncertain. We present 3 cases with this interesting association and consider their relationship.

In recent years there has been a tendency to abandon repeat cerebral angiography in patients with subarachnoid hemorrhage (SAH) if the initial angiogram is normal because prognosis is said to be excellent. Our experience does not support such a conclusion. We report our experiences in 25 patients with SAH, who had a normal initial angiogram. Aneurysms were seen in five of 20 cases when angiography was repeated. On subsequent review of the initial angiograms, the aneurysm was apparent in four of the five cases. When these four initial angiograms were shown to a second observer, unaware of the results of the second study, three of the four aneurysms were correctly identified. We suggest that if, under strong clinical suspicion of SAH, the angiogram is reported as normal the study should be reviewed by a second neuro-radiologist before proceeding to repeat angiography.

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.

The surgical results in 40 patients with syringomyelia, treated with a syringosubarachnoid shunt or other procedures are reviewed. The principal indication for surgery was that of significant neurological deterioration. There were 12 patients with idiopathic syringomyelia without tonsillar ectopia, 12 with an associated Chiari malformation, 11 with post-traumatic syringomyelia and five patients with spinal arachnoiditis. There were 38 syringosubarachnoid shunts performed in 35 patients, and an excellent or good result was achieved in 26 patients (74.3%). In terms of the type of syringomyelia, the best results were obtained in the idiopathic group without tonsillar ectopia and in the post-traumatic group. A short duration of pre-operative symptoms favoured a better outcome, and in our opinion, early surgical treatment is indicated for all patients with neurological deterioration. All eight patients in whom a posterior fossa decompression was performed as the initial surgical procedure required a second operation, either a syringosubarachnoid or syringoperitoneal shunt to achieve neurological improvement or stabilization. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for patients with syringomyelia, particularly for the idiopathic and post-traumatic groups. More than one surgical procedure may be required to achieve cessation of deterioration. Overall, excellent or good results were achieved in 29 (72.5%) of the 40 patients.

Subclavian “steal”, when blood siphons from one vertebral artery to the other, has been suggested as a cause of brain stem ischaemia and stroke. We investigated 33 patients using transcranial Doppler to determine the direction and velocity of basilar blood flow. All patients had severe subclavian stenosis with reversed vertebral blood flow in the ipsilateral artery previously demonstrated by extracranial Doppler. Basilar flow was normal in direction in all cases, but its velocity was significantly increased (p<0.0008) compared to age- and sex-matched controls. These findings, in conjunction with previous observations using extracranial Doppler techniques, suggest that subclavian steal is little more than a harmless haemodynamic phenomenon.

We report the case of a patient with meningio-angiomatosis, a rare disorder of the cerebral cortex often associated with Von Recklinghausen's neurofibromatosis. Previous reports have described a variety of radiological findings but in general they have been of little value in the diagnosis of this disorder. We describe the magnetic resonance image, which identified a well-defined lesion. The imaging techniques are reviewed.

The history of the provision of artificial vision by electrocortical stimulation has its origin in observations over 50 years ago. However, the realistic modern stimulation of human visual cortex is only 20-years-old. No useful device has been devised as yet and it therefore remains purely experimental. The primary objective of such a prosthetic device is that of independent mobility. This would require the appreciation of half-tone pictures, the detection of depth and discontinuities in outlines, and a significant quality of resolution. Some of the problems which threaten resolution in such a device and the factors which must be overcome in order to achieve a useful visual prosthesis are discussed.

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.

The records of fifteen patients referred for neurological assessment and found to have lupus anticoagulant or elevated anticardiolipin antibodies were reviewed. The mean age for females in the group was 29.4 years and for males was 35. A diagnosis of migraine, either as an acute or chronic problem, was made in 10 (66%) of these patients. Seven of the 15 patients had ischemic stroke and two patients had other thrombotic complications associated with lupus anticoagulant. Three of the nine female patients with migraine had histories of spontaneous abortions. All migraine patients experienced transient or more prolonged neurological deficits with their headaches. An association between lupus anticoagulant and migraine can only be suggested. Data on the incidence of migraine in patients with lupus anticoagulant in the general medical population does not exist. Furthermore the prevalence of lupus anticoagulant in migraine sufferers is unknown. Therefore further studies are required to investigate this possible association.