Two patients with pure motor hemiplegia were regaining strength in the affected limbs when a pure motor stroke developed on the opposite side. At the same time as the new hemiplegia appeared, the recovering side became re-paralyzed, suggesting that activity in the contralateral corticospinal tract had participated in the recovery process. Pathological studies were confirmatory. The literature pertaining to contralateral motor compensation is reviewed.

We report experience with 11 patients misdiagnosed for years, on the basis of computed tomography (CT) and angiography, as harbouring brainstem tumours in whom magnetic resonance imaging (MRI) demonstrated cavernous angiomas. Seven had undergone external irradiation, 2 had a ventriculo-peritoneal shunt, 2 developed aseptic femur necrosis following corticosteroid treatment, 1 had undergone a biopsy with a pathological diagnosis of glioma. CT had depicted ill-defined, hyperdense, faintly enhancing lesions. Angiography was normal, or showed an avascular mass or subtle venous pooling. MRI delineated discrete lesions, typical of cavernous angiomas, with a mixed hyperintense, reticulated, central core surrounded by a hypointense rim. Six patients subsequently underwent stereotactic radiosurgery without changes in clinical status or lesion. Although hemorrhagic neoplasms may mimic the clinical course and MRI appearance of cavernous angiomas, MRI is useful in the diagnosis of brainstem cavernous angiomas and should be performed in patients with suspected brainstem tumours.

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 following review indicates that the impact of cobalamin on neurologic disease extends far beyond the traditional myelopathy of classical pernicious anemia. The delineation of a broad spectrum of inherited disorders of cobalamin processing has served to illustrate and precisely define each step in the normal absorption, transport and intracellular metabolism of this essential vitamin. Recent clinical work has extended the boundaries of acquired cobalamin deficiency to encompass a variety of neuropsychiatric disturbances without identifiable concomitant hematologic derangements and emphasized the utility and sensitivity of new laboratory tests. These findings will demand increased vigilance from clinicians so that atypical and subtle cobalamin deficiency states will be readily diagnosed. The wide range of neurologic dysfunction observed in both inherited and acquired disorders of cobalamin metabolism challenges basic scientists to delineate cobalamin’s presumed important role in the normal development and homeostasis of the nervous system.

The addition of a D2 agonist such as bromocriptine to L-Dopa therapy can often improve the response of patients with Parkinson’s disease dramatically. Simultaneous activation of D1 and D2 dopamine receptors can produce a synergistic effect on locomotion in rats and primates. However, despite the importance of this addition of a D2 agonist to the D1/D2 agonist L-Dopa, little is known of the sites of action of these agents. Recent work suggests that, in addition to D1 and D2 dopamine receptor sites in the striatum (caudate-putamen), L-Dopa and D1 agonists have important effects at D1 dopamine receptors in the substantia nigra. Animal experiments suggest that D1 and D2 dopamine receptor agonists probably also affect different outflow pathways from the striatum. An understanding of these pathways and how dopamine agonists affect them gives insight into some of the clinical problems experienced in treating Parkinson’s disease (the “on-off phenomenon, for example). D1/D2 dopamine receptors also differentially affect gene expression and regulation in the striatum. An understanding of the anatomical and biochemical location of the actions of dopamine receptor agonists will be important in maximizing the beneficial effects and minimizing the side-effects of both presently-used drugs and new treatments.

The purpose of this investigation was to compare three methods of assessing the excitability of lower motoneurones—TA-reflex, H-reflex and F-wave—in 120 patients with spastic hemiparesis following a stroke. The H-reflex was recorded from the soleus muscle after submaximal electrostimulation of the tibial nerve. The T-Achilles (TA) reflex was recorded from the soleus muscle after percussion of the Achilles tendon. The F-wave was recorded in the distal limb muscles after supramaximal electrostimulation of the median, ulnar, fibular and tibial nerves. The patient’s healthy side was used as a control. The TA-reflex, H-reflex and F-wave showed increased amplitudes on the spastic side. All amplitude ratios: TA/M, H/M, Fmax/M and Fmean/M were increased. The H-reflex thresholds were decreased. The F-wave duration, persistence and number of phases were also increased on the spastic side. Despite clinically decreased muscle tone, there were no changes in TA or H-reflex parameters after treatment. On the other hand, F-wave parameters tended to normalize after treatment in all groups. In conclusion, the F-wave is a more sensitive method than the TA and H-reflexes in assessing the excitability of the lower motoneurone.

The rationale for sustained release oral levodopa preparations is to deliver levodopa in the areas of maximal intestinal absorption in a slow and predictable way, leading to stable plasma levodopa levels and brain dopamine levels, therefore resulting in a lengthened duration of action. Sinemet CR is the prototype of such preparations, with demonstrated efficacy in decreasing periods of akinesia in parkinsonian patients with mild to moderate motor fluctuations. Total doses of levodopa are raised 10 to 30% because of the lowered bioavailability; diphasic dyskinesias may increase at the end of the day. Tolerance is good in de novo patients and studies are in progress to establish if early treatment with Sinemet CR delays the onset or attenuates the severity of motor fluctuations as compared to standard Sinemet.

Spreading depression has been implicated in the pathophysiology of a number of diseases such as migraine, stroke and epilepsy. The characteristics of this phenomenon were explored in neocortex of anesthetized rats. Spreading depression was produced in 10 of 15 animals using mechanical, electrical and chemical stimulation. Mean amplitude of the DC shift was -9.3 mV, mean duration at any one electrode 65 sec and rate of spread 2-5 mm/min. Spreading depression was facilitated by focal interictal spike activity induced by penicillin and completely blocked by the N-methyl-D-aspartate (NMDA) receptor antagonist, DL-2-aminophosphonovaleric acid (APV), providing further evidence that excitatory amino acid neurotransmission is a critical element in the development or propagation of the phenomenon.

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 stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome.

This is a descriptive study of 19 families with glial tumors. Twelve were identified prospectively from 178 consecutive, unrelated adults and children with newly diagnosed gliomas seen at a regional cancer center between 01 Jan 89 and 31 Mar 91 (6.7%). There were 45 affected members (42 confirmed); 30 males, 15 females, ages 4 months-78 years (median, 44.5 years; mean, 38.9 years). Two families had four affected members, three families had three, and the others two. All confirmed tumors were supratentorial and all, save one, contained an astrocytic element. Three additional members of two families had other brain or neuroectodermal tumors. These families were not unusually cancer prone and did not appear to have neurofibromatosis, tuberous sclerosis, or colonic polyposis. There was no consistent pattern of inheritance.

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.

Previous studies using subjective tools to measure pain have shown that muscle exercise can have analgesic effects in man. The nociceptive leg flexion reflex (or RIII reflex) is a useful objective tool for assessing human pain. In this study, the pain threshold was assessed using the nociceptive flexion reflex in six high-level athletes 1) at rest in comparison with 8 control subjects and 2) after exercise requiring the production of a 200-Watt force over a period of 20 minutes. The nociceptive flexion reflex threshold at rest was found to be spontaneously higher in the athletes than in the controls. Physical activity resulted in a significant increase (+53%) in the threshold of the nociceptive reflex in the athletes. The role of stress-induced analgesia, the reduction in perceived intensity of stimuli during movement, and the release of opioids are discussed.

The use of the dopamine receptor agonists in Parkinson’s disease has a compelling logic. These agents are supposed to act independently of the dying cells of the substantia nigra directly on the cells of the striatum. Early clinical trials in advanced disease were only mildly impressive. Later they were found to be beneficial in early disease but their effectiveness waned. Their ultimate failure may reflect the fact that the majority of current agents do not stimulate D1 and D2 receptors in a physiologic ratio. The drugs may act presynaptically and with the eventual loss of the anatomic relationships between nigra and striatum the drugs fail. There is, however, a rationale to their current use. When used along with L-Dopa in early disease the development of late-stage fluctuations are reduced with the same anti-parkinsonian benefits. Merging this concept with the demonstrated effect of selegiline in slowing the course of the disease, the current practice of triple therapy with selegiline, L-Dopa and a dopamine receptor agonist emerges.

Spontaneous disappearance of an intracranial aneurysm after subarachnoid hemorrhage is an uncommon event and usually associated with severe cerebral vasospasm, giant aneurysms or the use of antifibrinolytics. We present a young woman who suffered a grade 5 subarachnoid hemorrhage with severe vasospasm caused by a small anterior communicating artery aneurysm. The patient underwent a slow recovery and two years later requested surgery. Angiography demonstrated complete disappearance of the aneurysm. The neurosurgeon should be aware that spontaneous thrombosis of cerebral aneurysms can occur and ensure that angiography is repeated when surgery is significantly delayed.

A case of central neurocytoma treated surgically is described. The authors review the literature. Emphasis is placed on radiological and pathological features not previously described. In particular, the intra-operative ultrasound appearance is described. The role of adjunctive radiotherapy is also discussed.

Malignant gliomas of optic nerve and chiasm are rare, rapidly fatal neoplasms of adulthood. This report documents the occurrence of a malignant astrocytoma of the optic nerve in an 11-year-old boy who 9 years previously had a cerebellar medulloblastoma treated with surgery and irradiation. This malignant optic nerve glioma followed the same aggressive clinical course as that seen in adults, with death 9 months after diagnosis despite surgery and chemotherapy: Radiation may have been an important factor in the development of this malignant tumor which is almost never seen in the pediatric age group.

A prevalence study of multiple sclerosis (MS) was carried out in the town of Barrhead and surrounding county of Barrhead, in Alberta, Canada. The prevalence rate for clinically probable/definite multiple sclerosis on January 1, 1990 was 196/100,000. The average annual incidence rates for patients living in the area at onset were 1.31/100,000 for 1950–59, 4.97/100,000 for 1960–69, 3.77/100,000 for 1970–79, and 4.22/100,000 for 1980–89. Fifty per cent of the patients were relapsing-remitting. Sixty per cent were still walking without assistance. The female-to-male ratio was 1:1. Mean current age, age at onset and duration of illness were 49, 27 and 22 years respectively. The majority of patients (40%) experienced multiple symptom onset. Fifty per cent were of single ethnic origin (either British or German); the rest were predominantly North European combinations. Forty per cent of patients reported another MS relative. MS had affected the work status of 60% of the patients, 15% of whom were confined to an extended care centre.

New methods of drug delivery and slowing down the progression of Parkinson’s disease (PD) are the major goals of research. More steady drug levels in the blood are possible by means of controlled-release preparations of levodopa and long-acting dopamine agonists, as well as transcutaneous duodenal tubes and pumps for controlled subcutaneous infusion. Patches containing dopamine agonists absorbed through the skin may be developed. The role of D1 agonists as compared with D2 agonists remains to be elucidated. Agonists on autoreceptors of dopaminergic neurons may potentially reduce excessive stimulation of the intact neurons and this may slow down the rate of neuronal death in PD. Monoamine oxidase-B inhibitors may have a potentially protective action on neurons. Investigations are being carried out to evaluate this claim. Catechol-o-methyl-transferase inhibitors may be helpful in theory. There is also recent interest in inhibitors of excitatory amino acids, which may contribute to neuronal loss in PD.

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.