Patients with predominantly unilateral parkinsonian signs may provide a unique opportunity to evaluate the cerebral representation of cognitive functions characteristically affected in idiopathic Parkinson’s disease. Twenty hemiparkinsonian patients (ten left and ten right) and 10 healthy controls, matched for age and education, were studied with neuropsychological tests and positron emission tomography. Both right and left hemiparkinsonians evidenced impairments in visuospatial and verbal episodic memory function, but had no deficits in executive abilities, compared to controls. None of the neuropsychological test scores distinguished right from left hemiparkinsonians. Glucose metabolic profiles were identical for the three groups in all cortical areas assessed; in the subcortex however, lenticular hypermetabolism contralateral to the predominant side of motor involvement was evident in the left hemiparkinsonian group. Correlational analysis revealed that higher glucose metabolic rates in the basal ganglia of these hemiparkinsonians were associated with lower visuospatial test scores. In frontal and parietal cortex, decreasing glucose metabolism was positively associated with neurobehavioral function; in temporal cortex, measures of attention and memory decreased with increasing glucose metabolic rates.

The study investigated features of life-style and dietary habits early and late in life of patients with idiopathic Parkinson’s disease (IPD). Seventy-one patients and 103 controls were interviewed personally with a structured questionnaire. Living in villages during primary school time was significantly more frequent among patients, and in the urban environment patients had lived less frequently in larger-sized towns. Mushroom harvesting during childhood was more frequent among patients. No difference between patients and controls was found in childhood water supply, habits of fishing in the countryside or at the seaside, and eating such fish. Actual food preference in patients was greater for almonds and plums, while no difference was found in the actual intake of mushrooms, peanuts, oil-dressed salad, fish and animal offals. The study did not indicate a higher consumption of foods known to harbour heavy metals and pesticides in IPD patients either long before or during the disease. Reduced consumption of foodstuffs rich in vitamin E, as reported previously for premorbid patients, is no longer observed in patients with overt disease.

Five siblings with autosomal dominant oculopharyngeal muscular dystrophy (OPMD) underwent P-31 Nuclear Magnetic Resonance Spectroscopy studies of forearm flexor muscles. Mean values of PCr/(PCr + Pi) in the patients were reduced (p = 0.01) and pH elevated (p = 0.02) in resting muscle when compared to controls. During exercise PCr/(PCr + Pi) fell quickly to values less than controls (p < 0.0001) despite submaximal exercise output and developed exercise-induced acidosis which exceeded that of controls (p = 0.05). Acidosis recovered slowly despite relatively normal recovery of PCr/(PCr + Pi) following exercise. Within the patient group, however, one member had normal resting, exercise and recovery values. The studies suggest that OPMD is a more widespread disorder of striated muscle than clinically appreciated. The pattern of findings observed in OPMD differs from those identified in denervation, disuse and mitochondrial myopathy.

Twenty-five patients with spinal epidural abscess were treated at the University of Western Ontario hospitals between July 1980 and July 1990. There were eighteen males (72%) and seven females (28%), with a median age of 60 years. Concurrent illness resulting in immunocompromise was present in 60%. Eleven presented with complete myelopathy, thirteen had limb weakness, and one had no neurological deficit. In twenty cases the abscess consisted of frankly purulent material, while in five the epidural collection consisted of chronic granulation tissue. Staphylococcus aureus was isolated in 64% of the abscesses. Twenty-seven surgical procedures were performed on 21 patients. Ten cases occurred in the cervical spine (40%), seven in the thoracic spine (28%), three in both the cervical and thoracic spine (12%) and five in the lumbosacral spine (20%). Fourteen patients (56%) retained or recovered ambulation and there were five deaths (20%). The progression from back and radicular pain to weakness and eventual paralysis continues to be characteristic of spinal epidural infection. Morbidity and mortality remain unacceptably high because of delay in diagnosis and treatment. Magnetic resonance imaging is the radiological investigation of choice for the diagnosis of spinal epidural abscess. Prompt intervention, before the development of severe neurological deficits, can improve outcome. Immediate surgical drainage combined with antibiotics remains the treatment of choice.

A group of six subjects with intractable spinal spasticity completed a double-blind cross-over paradigm in which they received two intrathecal bolus injections of baclofen solution five hours apart on two different days and two intrathecal bolus injections of placebo saline five hours apart on two other days. Each subject was repeatedly tested with a battery of clinical and physiological tests. In contrast to the placebo injections, the group responded to the baclofen injections with subjective and objective, clinically significant improvement in parameters of spasticity in their lower limbs, including muscle tone, frequency of spasms, hyperreflexia and passive range of joint motion. Furthermore, this improvement was maintained following thirty consecutive days of intrathecal bolus injections of baclofen at a fixed dose.

Eight cases of basal skull fracture with transverse fracture of the petrous temporal bone with medial extension to the internal carotid artery and lateral extension of the structures of the middle ear are described. Injuries in all cases were due to major blunt impact to the head usually occurring in a motor vehicle accident. General autopsy revealed major blood loss without any obvious external or internal site of hemorrhage suggesting that exsanguination was a complication of the head injury. The internal carotid arteries at the most medial extension of the fractures were lacerated or transected in all cases. In selected cases, the cervical internal carotid arteries were perfused and perfusate escaped rapidly from the ear(s) with the majority of fluid bypassing the cerebral venous system. Magnetic resonance image reconstruction of sequential sections of the fractured base of the skull confirmed the laceration of the internal carotid arteries and disruption of the middle ear. Based on this evidence, we propose that some displaced fractures of the base of the skull produce carotid-middle ear continuities which act as arterial shunts, resulting in rapid fatal exsanguination through the ear.

Vitamin E (d-α-tocopherol) has proven to be a useful adjunct to anticonvulsant drugs in clinical studies. Improvement has occurred even in patients with complex partial seizures, which are often resistant to drug therapy. In animals, vitamin E is effective against ferrous chloride seizures, hyperbaric oxygen seizures and penicillin-induced seizures. It has failed, however, to show anticonvulsant effects in the standard animal models used for drug screening – the maximal electroshock and threshold pentylenetetrazol tests. The present experiments were designed to further explore the anti-epileptic actions of vitamin E in animals. Three models related to complex partial epilepsy were used: 1) the development of amygdala-kindled seizures; 2) the development of electrically-induced status in kindled animals; and 3) the development of kainic-acid seizures. Vitamin E failed to produce significant effects in any of the models.

We report the first comparison of Chewable and Regular Carbamazepine (CBZ) tablets in children with epilepsy. Forty-four children receiving chronic monotherapy CBZ participated. In month 1 children received regular CBZ; in month 2, the same dose of Chewable CBZ. Once per week fasting predose CBZ and CBZ epoxide serum levels were determined. In a subset of 15 children, at the end of each month serum levels were obtained every 2 hours for 12 hours beginning pre-dose. Standards for CBZ and CBZ epoxide were tested in each centre. Overall, weekly levels showed no consistent differences between the month on chewable CBZ and regular CBZ. Seizure control and rates of reported side effects were similar. In five patients chewable CBZ produced higher peak CBZ levels while five had higher peaks with regular CBZ. In conclusion, regular and chewable CBZ often have unpredictable differences in peak but not trough levels of CBZ suggesting that peak level side effects with one form of CBZ might be alleviated by changing to the other.

Over the past fifty years considerable clinical evidence has accrued to demonstrate involvement of the cerebral cortex in cardiac function. Hemispheric stroke is often associated with electrocardiographic (ECG) evidence of cardiac repolarisation abnormalities. In addition strokes of all types are associated with specific pathological changes in the ventricular myocardium (myocytolysis). These effects are not attributable to concomitant cardiac ischemic disease in the majority of cases. The insular cortex has recently been shown to contain a site of cardiac representation. Prolonged stimulation of this region in the rat produces ECG and cardiac pathological changes similar to those observed after human stroke. It is suggested that middle cerebral artery stroke in certain cases either directly or indirectly leads to insular disinhibition, and increased autonomic activity represented by cardiac changes which significantly influence prognosis.

The term radiosurgery has been used to describe a variety of radiotherapy techniques which deliver high doses of radiation to small, stereotactically defined intracranial targets in such a way that the dose fall-off outside the targeted volume is very sharp. Proton, charged particle, gamma unit, and linear accelerator-based techniques appear to be equivalent from the standpoint of accuracy, dose distributions, and clinical results. However, capital and operating costs associated with the use of linear accelerators in general clinical use are much lower. Radiosurgery has an established role in the treatment of arteriovenous malformations and acoustic neurinomas. Interest in these techniques is increasing in neurosurgical and radiation oncological communities, as radiosurgery is rapidly assuming a place in the management of several other conditions, including craniopharyngiomas, meningiomas, and selected malignant lesions.

The pathogenic basis of the association between adult polycystic kidney disease (APKD) and cerebral aneurysms is unknown. We have compared cerebral aneurysms in 79 patients with APKD gleaned from the literature to the sporadic aneurysm cases reported by the Cooperative Study to determine if there are significant biological differences between these two groups. Sixty-eight patients had a single aneurysm and 11 (14%) had multiple aneurysms. In APKD patients with subarachnoid hemorrhage from a single aneurysm there was a significant over-representation of males (72%, p < 0.01); and the APKD group had more aneurysms of the middle cerebral artery (37%, p < 0.05). The peak decennial incidence and mean age of rupture of APKD-associated aneurysms was younger (mean age 39.7 years, p < 0.01) and over 77% of APKD-associated aneurysms had ruptured by age 50 versus 42% for sporadic aneurysms (p < 0.001). Cerebral aneurysms co-existed with APKD in the absence of hypertension in 25% of 45 cases where the presence or absence of hypertension was recorded. These biological differences and the occurrence of aneurysms in normotensive APKD patients suggests an etiology which may be independent of hypertension and that APKD-associated aneurysms may be genetically determined. It is hypothesized that cases of inherited, familial cerebral aneurysms could be linked to a genetic defect resembling that which occurs on chromosome 16 in APKD.

Clinically non-functioning pituitary adenomas are morphologically classified into two groups, those which have hormone immunoreactivity and ultrastructural features of known adenohypophysial cell types but are clinically silent, and those composed of cells that do not resemble nontumorous adenohypophysial cell types. Among the fomer are the silent somatotroph adenomas, silent corticotroph adenomas and silent gonadotroph adenomas; the latter include the silent type III adenomas, null cell adenomas and oncocytomas. We review their histological, immunohistochemical and ultrastructural features, the results of in situ hybridization to determine hormone synthesis by these tumors and data obtained from tissue culture characterizing their hormone release in vitro. Non-functioning adenomas represent a heterogeneous group. The discrepancies between morphology, immunoreactivity and lack of endocrine activity of silent adenomas are not clear. Oncocytomas are variants of null cell adenomas. We suggest that null cell adenomas and oncocytomas originate in uncommitted pluripotent precursor cells capable of undergoing multidirectional differentiation. The progenitor cells differentiate most frequently toward FSH / a-subunit producing cells; the mechanism of preferential differentiation is obscure.

We report six patients with partial, predominantly paramedian, tegmental pontine hemorrhages. Constant clinical manifestations consisted of: ipsilateral miosis, horizontal gaze paresis, lower motor neuron facial paresis, contralateral hemisensory loss and mild and transitory hemiparesis, dysarthria and mild or no compromise of consciousness. Five out of six were hypertensive. All patients survived with mild sequelae, oculomotor disturbances being the most persistent deficit. We found in our patients that a transverse diameter of less than 17 mm, unilaterality of the injury and absence of coma were the major indicators of a favorable outcome.

We describe a unique case of chronic lymphocytic leukemia (CLL) in a patient who presented with bitemporal hemianopsia, adrenal insufficiency, and gonadotrophin deficiency. Studies revealed an enhancing intrasellar mass with suprasellar extension and displacement of the optic chiasm. Peripheral blood and cerebrospinal fluid (CSF) studies disclosed a monoclonal IgM kappa expressing B-cell CLL. Biopsy of the pituitary mass revealed dense infiltration of the pituitary gland by leukemic cells. This is, to our knowledge, the first reported case of CLL presenting as a pituitary mass lesion.

Despite the fact that the teratogenic effects of phenytoin have been suggested in several case reports, the evidence for a possible oncogenic potential of phenytoin has not been widely recognized. Recently, neuroblastoma as well as other neuroectodermal and non-ectodermal tumors has been seen in several children exposed to phenytoin prenatally. Previous cases have been almost uniformly associated with the features of “fetal hydantoin syndrome” and none have been developmentally normal. We report a developmentally-normal boy of 2½ years with an abdominal neuroblastoma whose mother had been on phenytoin (as well as carbamazepine) throughout gestation. We review the various neoplasms which have been reported in the offspring of mothers receiving phenytoin.