Background: Oat cell carcinoma of the lung is the most common cause of paraneoplastic limbic encephalitis. Association with other malignancies, in particular Hodgkin’s disease, is very rare. Case Report: This 23-year-old male presented with a six month history of progressive alteration in mental status, which consisted of insomnia, short-term memory loss, depression and cognitive impairment. Gadolinium MRI of the head showed intense bilateral contrast enhancement affecting the medial aspects of the temporal lobes in the region of the amygdala and hippocampus. The brain biopsy showed minimal neuronal loss with intense perivascular lymphocytic cuffing and microglia] nodules. Polymerase chain reaction for herpes simplex and cytomegalovirus were negative. With prednisone treatment, the patient’s neurologic status stabilized but did not improve. Four months later, he presented with left axillary lymphadenopathy. Lymph node biopsy was diagnostic of Hodgkin’s disease. During the chemotherapy, his lymphadenopathy subsided and his neurologic and mental status improved. When seen last after completion of his chemotherapy, one year after presentation, he had resumed normal social activities and was enrolled in a university language course. Conclusion: This is the first reported case in the English literature of a biopsy proven paraneoplastic limbic encephalitis associated with Hodgkin’s disease. Hodgkin’s disease should be thought of as a possible cause of paraneoplastic limbic encephalitis in the appropriate clinical setting.

Background: Major neurological complications of sickle cell disease include cerebral infarction and intracranial hemorrhages. Methods: We describe the case of a 25-year-old man with sickle cell disease who developed a severe headache of acute onset, located at the vertex. There was associated pitting edema of the scalp. Results: Technetium and gallium bone imaging showed focal decreased uptake of the tracers, consistent with a recent fronto-parietal skull infarction. Management included intravenous hydration and analgesic medication. Conclusions: In addition to the more common intracranial vaso-occlusive complications, skull infarction should be considered as a cause of new-onset headache in patients with sickle cell disease, especially if scalp edema is present.

It is particularly fitting that at this reception of members of the Canadian Neurological and Clinical Neurophysiologists Societies at the American Academy of Neurology we should honour Dr. Herbert Jasper. He is, at the age of 90, a living legend in the neurosciences. It is a rare opportunity for the young people who have followed him to meet a pioneer of his generation, let alone somebody who continues to this day to exercise his wise counsel and his extraordinary grasp of the working of the human brain.

Herbert Jasper, like Wilder Penfield, was born in the west of the United States, and, like his friend and colleague, became a Canadian and a founder of Canadian Neuroscience.

Jasper started his career as a psychologist of physiological persuasion and studied with Lapique who is remembered for his studies on chronaxie, in Paris. He became aware of Berger’s writings on the electroencephalogram and realized early the enormous potential of being able to record the electrical activity generated by the human brain. In the 30s, two schools of epilepsy developed in North America: the medical with William Lennox in Boston, and the surgical with Wilder Penfield in Montreal.

Background: Increased intracranial pressure with encephalopathy has rarely been reported in Addison’s disease. Method: Case Study. Results: A 16-year-old female who presented with cerebral edema of unknown etiology was eventually diagnosed as having Addison’s disease. She had early morning headaches, fatiguability, diarrhea and deterioration in school performance. She was hyponatremic with a serum sodium of 128 mmol/L and hyperkalemic with a serum potassium of 5.9 mmol/L. She had a low serum osmolality (264 mosm), high urine osmolality (533 mosm) and high urine sodium (87 mosm). She had a postural drop in blood pressure and diffuse hyperpigmentation. An ACTH stimulation test revealed a low baseline Cortisol and no response to ACTH. Plasma renin activity was increased. Serum ACTH was elevated. She responded well to intravenous fluids and solu-cortef and was discharged on hydrocortisone and florinef. She remains well 18 months after the acute episode with no neurologic complaints or findings. Conclusion: Addison’s Disease should be considered in the differential diagnosis of symptomatic cerebral edema and idiopathic intracranial hypertension.

In Part 1 of this history of neurology in Toronto, the advent of neurology as a specialty in 1892 and its progress over the next 20 years was described (Can J Neurol Sci 1995; 22: 322–332). Donald Campbell Meyers, the first neurologist received his training from “founders” in Paris, Vienna and London and returned to Toronto to establish his own private neurological hospital and a special unit – “the Nervous Wards” – in the Toronto General, the major teaching hospital. These pioneering initiatives clashed with an alienist establishment, itself internally in conflict, and the “Nervous Wards” were lost in the ensuing competition for medical resources.

Background: Periodontoid synovial cysts are rare lesions which may produce symptomatic cervico-medullary compression. Method: We report such a patient, whose progressive neurological deterioration required surgical treatment by transoral odontoidectomy and decompression. Results: The diagnostic and theraputic interventions are described, including a lumbar puncture which precipitated a transient loss of consciousness and respiratory arrest. Surgery achieved clinical improvement, without complications or need for operative stablization. Detailed neuropathology is presented, as well as a literature review. Conclusion: Appropriate neuroradiological assessment is required in patients with suspected cervico-medullary compression, and symptomatic periodontoid synovial cysts may respond well to transoral surgical decompression.

The 100th anniversary of the discovery of the extensor plantar response will be celebrated in 1996. It was Joseph François Félix Babinski who became known worldwide for the sign that bears his name. In order to help Joseph in establishing his career, brother Henri gave up his aspirations and abandoned engineering. Clovis Vincent, ‘father’ of French neurosurgery and pupil of Joseph, stated: “Joseph Babinski lived for science, and Henri lived for his brother; without Henri Babinski, Joseph would not have accomplished that much”. However, Henri’s name became famous in all Paris for a cookbook Gastronomie Pratique written under the pseudonym of ‘Ali-Bab.’ Throughout Joseph’s career his surname remained distorted despite his own efforts to spell and pronounce it correctly. Several people can claim the name Babinski, but in neurology and neurosurgery there is only one, Joseph.

Neurology has a proud tradition, built on the established principles of medicine, the scientific method of the 17th century and the clinical approach of the French and later the English schools of neurology in the 19th century and the first half of this century. In the post World War II era neurology was advanced by the development of neurological training centers, the discovery of new pharmacological and neurosurgical therapies, and most recently by in the exciting advances in neurogenetics. These changes have broadened and advanced the scope of our discipline by focusing on the disease processes that affect the nervous system. I would like to suggest an additional conceptual framework to broaden it further. That concept is a population health perspective that should place our various approaches in closer relationship to the communities they serve.

Background: A 20-year-old woman with a right occipital condylar fracture and bilateral hypoglossal nerve injury is presented. Only 17 cases of condylar fracture have been reported in the literature. Methods: The patient was evaluated with plain films, coronal and axial cut CT, and MRI. Results: MRI showed a severely distorted but otherwise normal medulla and a displaced condylar bone fragment. Conclusion: Condylar fracture may cause twelfth nerve palsy by injuring the central or peripheral nerve.

In the hope of eventually using replication-competent, neurovirulence-attentuated Herpes simplex type 1 virus in the treatment of brain tumors in humans, we analyzed the effect of such a mutant in human malignant meningioma transplanted into nude mice. Two models were used, one subcutaneous, the other intracranial subdural; nine or ten animals were used in each group. A 1 mm tumor mass was implanted. In the subcutaneous model, after 7 or 12 days, the tumors had reached size of 6 or 10 mm, respectively. At this time either saline or the attenuated, lac Z HSV-1 mutant G207 (5 × 106 PFU/50 μ1) were injected into the tumor; for the 10 mm tumors, the identical dose and 5 × 106 PFU/50 μl were given. Animals were sacrificed up to 28 days later. The ratio of tumor volume between untreated and treated groups was compared by the t-test. For all three treatment groups, tumor growth was markedly inhibited (p < 0.0001). In the intracranial model, either saline or G207 at a dose of 2 × 106 PFU/2 μl was injected into the tumor implant on day 7, with 9 animals in each group. By day 31, all animals in the control group had died; in the treated group, three were still alive at day 66; a significant difference in survival was found (Wilcoxon test, p < 0.01).

George A. Savoy was born in Cohoes, New York, in 1873. He left the U.S.A. in 1921 to manage the Canadian branch of a large manufacturer of ledgers and looseleaf registers. This company was asked to supply Professor Jasper’s laboratory with rolls of plain unlined paper and it was George Savoy who later developed fanfolded and lined EEG paper, which was first used at the Montreal Neurological Institute. He also had personal contacts with Wilder Penfield concerning their mutual interest in the needs of patients with epilepsy. He was a successful industrialist involved with several charitable organizations funding programmes for people with epilepsy. He was opposed to the sectarianism then prevalent in Quebec, which was unfamiliar to him, and in reaction built his own institution, Dieppe House, a home for people with epilepsy, later renamed «Foyer Savoy». It was to operate without regard to race, language or religion. In 1971, his son Harold and other generous donors decided to create a foundation to support research in epilepsy. The Foyer Savoy was sold in 1988 and the proceeds used to increase the endowment of the foundation. His grandson George M. Savoy is the current president. The fourth generation is also represented by Caroline Savoy, daughter of the president, who joined the board of directors in 1992. The foundation will distribute from $300,000 to $400,000 yearly to researchers from many different countries working in the field of epilepsy in universities and hospitals throughout Canada.