Since the validation of carotid endarterectomy (CEA) as an effective means of stroke prevention, there has been renewed interest in its best indications and methods, as well as in how it compares to carotid angioplasty and stenting (CAS). This review examines these topics, as well as the investigation of carotid stenosis and the role of auditing and reporting CEAresults.

Brain imaging with CTor MRI should be obtained in patients considered for CEA, in order to document infarction and rule out mass lesions. Carotid investigation begins with ultrasound and, if results agree with subsequent, good-quality MRAor CTangiography, treatment can be planned and catheter angiography avoided. An equally acceptable approach is to proceed directly from ultrasound to catheter angiography, which is still the gold-standard in carotid artery assessment.

Appropriate patients for CEA are those symptomatic with transient ischemic attacks or nondisabling stroke due to 70-99% carotid stenosis; the maximum allowable stroke and death rate being 6%. Uncertain candidates for CEA are those with 50 - 69% symptomatic stenosis, and those with asymptomatic stenosis ≥ 60% but, if selected carefully on the basis of additional risk factors (related to both the carotid plaque and certain patient characteristics), some will benefit from surgery. Asymptomatic patients will only benefit if surgery can be provided with exceptionally low major complication rates (3% or less). Inappropriate patients are those with less than 50% symptomatic or 60% asymptomatic stenosis, and those with unstable medical or neurological conditions.

Carotid endarterectomy can be performed with either regional or general anaesthesia and, for the latter, there are a number of monitoring techniques available to assess cerebral perfusion during carotid cross-clamping. While monitoring cannot be considered mandatory and no single monitoring technique has emerged as being clearly superior, EEG is most commonly used. “Eversion” endarterectomy is a variation in surgical technique, and there is some evidence that more widely practiced patch closure may reduce the acute risk of operative stroke and the longer-term risk of recurrent stenosis.

Experience with this endovascular and less invasive procedure grows, and its technology continues to evolve. Some experienced therapists have reported excellent results in case series and a number of randomized trials are now underway comparing CAS to CEA. However, at this time it is premature to incorporate CAS into routine practice replacing CEA.

It has been shown that auditing of CEA indications and results with regular feed-back to the operating surgeons can significantly improve the performance of this operation. Carotid endarterectomy auditing is recommended on both local and regional levels.

Population-based studies are necessary to better understand the risk factors for developing seizure disorders and the impact of these conditions on children. We undertook an assessment of the prevalence of seizure disorders in a population of children on the basis of health care utilization records.

Using Manitoba’s population-based prescription and health care data for 1998/99, the prevalence of children with seizure disorders, on the basis of at least one physician visit or hospitalization for epilepsy or a prescription for an antiepileptic drug, was determined by age, urban/rural region and socioeconomic status. The latter was measured as neighbourhoods stratified by income quintiles according to Census data.

Age-specific prevalence rates for seizure disorders in Manitoba children, determined from health care administrative records, were similar to published data on the prevalence of epilepsy, with one exception. Prevalence rates in adolescents were higher than those reported in the literature. No statistically significant differences in prevalence rates were observed between urban and rural populations. However, a higher prevalence was found among children of all ages living in lower socioeconomic neighbourhoods in urban areas, which presented as a gradient of increased prevalence with decreased levels of income.

Population-based health care administrative data can be used to describe the geographical distribution of seizure disorders. Our data suggest that the burden of seizure disorders is not evenly distributed among children.

Superior cerebellar artery aneurysms are rare. We present a clinical series of twelve of these aneurysms that were treated exclusively with endovascular coils.

A retrospective analysis of a prospectively collected database of cerebral aneurysms treated with coil embolization was performed. Clinical notes and radiological images were reviewed.

Twelve superior cerebellar artery aneurysms were treated in eleven patients between 1992 and 2001. Seven patients presented with subarachnoid hemorrhage, two with neurologic deficit, and two had asymptomatic aneurysms. Coiling resulted in complete aneurysm obliteration in six patients and incomplete obliteration in the other six. No subsequent hemorrhage occurred with follow-up between 6 and 119 months (mean follow-up 50 months). Procedural morbidity was one superior cerebellar artery infarct with good recovery. Management morbidity was one middle cerebral artery embolus during a follow-up angiogram that required thrombolysis with a good clinical result. Nine out of 11 patients on follow-up were performing at Glasgow Outcome Scale (GOS) 5. One patient with GOS 3 presented with a poor grade subarachnoid hemorrhage and the other patient with GOS 4 presented with a parenchymal hemorrhage due to an arteriovenous malformation.

Endovascular treatment of superior cerebellar artery aneurysms is an effective treatment strategy with low morbidity.

Patients with relapsing remitting multiple sclerosis on the disease modifying therapy of glatiramer acetate may be experiencing an adverse reaction of lipoatrophy at the sites of their subcutaneous injections. The purpose of this study was to complete a full examination of the injection site areas for users of glatiramer acetate, and to examine the relationship between lipoatrophy and patient characteristics.

Glatiramer acetate users were identified by means of chart review. Over six months, during regular clinic appointments, assessment included a full examination of injection site areas including visual inspection and manual palpation. Additional patient and clinical characteristics were obtained by means of chart review and patient questioning.

Seventy-six patients had been or were current users of glatiramer acetate. Of these, 34 (45%) had evidence of lipoatrophy in at least one injection site area. All were female, and five had severe, nine had moderate and 20 had mild lipoatrophy. In some cases, lipoatrophy occurred within months of therapy initiation. Case reviews are included for five of the 34 patients, along with photographs of the lipoatrophy, a magnetic resonance image and comments on skin biopsies.

Prevalence of lipoatrophy was much higher than expected. Possible reasons for this adverse reaction are explored and suggested treatment recommendations are reviewed. Lipoatrophy can be very disfiguring and is thought to be permanent, and the psychological impact can be significant. It is, therefore, important that patients be aware of the possibility of lipoatrophy, be able to identify it and discontinue injecting in areas where it is identified.

Quality of life instruments provide information that traditional outcome measures used in studies of multiple sclerosis do not. It is unclear if longer, disease-specific instruments provide more useful information than shorter, more general instruments, or whether patients prefer one type to another.

We conducted a cross-sectional study of quality of life in a multiple sclerosis clinic population using a mailed questionnaire that combined three different quality of life instruments; the SF-36, the Multiple Sclerosis Quality of Life Instrument-54, and the EuroQol EQ-5D. We assessed the feasability of using each instrument and patient preference for each, calculated correlation coefficients for the summary scores of each instrument and other measures of disease severity, and calculated odds ratios from proportional odds models comparing each instrument with the Expanded Disability Status Scale.

We did not find substantial differences between the three instruments. All were well-received by patients, and over 75% felt that the combination of the three instruments best assessed their quality of life. For each instrument there was substantial variability between patients with similar quality of life scores in terms of their disability (as assessed by the Expanded Disability Status Scale and their own perception of their disease severity and quality of life (on simple 1-10 scales).

Quality of life instruments are easy to use and well-received by patients, regardless of their length. There do not appear to be clinically important differences between general and disease-specific instruments. Each instrument appears to measure something other than a patient’s disability or perception of their own disease severity or quality of life.

Chronic daily headache (CDH: headache on fifteen days a month or more) is one of the most common forms of chronic pain. The relative efficacy of different treatment methods for these patients needs to be determined.

To compare treatment outcomes for patients with CDH treated in a traditional office-based pharmacological treatment program with a second group treated in a multidisciplinary management program.

Patient outcomes were measured using changes in the Headache Disability Inventory (HDI) and the Short-Form-36 (SF-36) over the treatment period. Outcomes from seventy patients treated in an office setting were compared to thirty-seven patients treated in a multidisciplinary headache treatment program. Both groups received similar pharmacological treatment. All patients treated in the office setting and the majority of patients in the multidisciplinary program had transformed migraine.

Even though a reduction in headache days per month occurred, mean headache related disability (measured by HDI) and mean Health Related Quality of Life (HRQoL measured by SF-36) did not improve for the patient group treated in the office setting but did improve significantly for the patient group treated in the multidisciplinary headache program.

For patients with CDH, headache-related disability and HRQoL is more likely to improve with management in a multidisciplinary headache treatment program as compared to the traditional specialist consultation - family physician office-based setting.

Cerebral vasospasm adversely impacts the outcome of those suffering aneurysmal subarachnoid hemorrhage (SAH). Prediction of vasospasm could improve outcomes. We hypothesized that preclinical vasospasm would be heralded by an increase in cerebral oxygen extractions (AVDO2) which could be detected by jugular bulb oximetry. A pilot study was conducted to address this hypothesis.

Fourteen consenting patients with aneurysmal SAH, undergoing early surgery, were entered into the study. Four patients were withdrawn from the study secondary to failure of catheters or religious belief. At the time of craniotomy, a jugular bulb catheter was placed. Post-operatively, arterial and jugular bulb blood samples were taken every 12 hours to calculate AVDO2. As this was an observational study, no change in management occurred based on measurements.

Four of 10 patients had clinical vasospasm. These patients had a significant rise in AVDO2 approximately one day prior to the onset of neurologic deficits (P<0.001). Symptoms resolved along with a significant improvement in AVDO2 on instituting hypertensive, hemo-dilutional, and hypervolemic therapy in these patients. The six patients who did not exhibit clinical vasospasm did not demonstrate significant rise in AVDO2.

Jugular bulb oximetry is simple and cost effective. Increases in AVDO2 using this technique were predictive of clinically evident vasospasm in the subsequent hours to days. This investigation supports a larger study to assess the utility of jugular bulb oximetry in predicting vasospasm in aneurysmal SAH.

The role of neural transplantation as a restorative strategy for spinal cord injury continues to be intensely investigated. Ideally, the tissue source for transplantation must be readily available, free of disease and able to survive and mature following implantation into the adverse environment created by the injury. We have studied the use of a commercially available cell line of cultured human neurons (hNT neurons) as a tissue source for neural transplantation in spinal cord injury.

Following a left lateral thoracic hemisection, 54 immunosuppressed, female Wistar rats were randomly allocated into different treatment groups; hemisection only or hemisection and hNT cell transplantation (via a bridge, double or triple graft). Grafting occurred three days after spinal cord injury. After thirteen weeks the animals were sacrificed and tissue sections were stained with human neuron specific enolase and human specific neural cell adhesion molecule.

Immunohistochemical evidence of graft survival was displayed in 66.7% of the surviving, grafted animals. Fibre outgrowth, greatest in the bridge and triple grafts, was observed in both rostral and caudal directions essentially bridging the lesion. Double grafts were smaller, displaying less fibre outgrowth, which did not cross the lesion. Long fibre outgrowth was evident up to 2 cm from the graft as assessed by tracing and immunohistochemical studies.

Bridge and triple grafts displayed greater growth and enabled the hNT graft to essentially bridge the lesion. This suggests that hNT neurons have the potential to structurally reconnect the proximal and distal spinal cord across the region of injury.

Intravenous rt-PA (IV rt-PA) for acute stroke has raised many concerns, including its inadvertent use in patients presenting with acute stroke-like symptoms as the expression of their somatoform disorder. Diagnosis of the somatoform disorder is often delayed, and thrombolytics in these patients for their stroke-like presentation subjects them to risk for hemorrhage.

The presentation, neurological findings, and the therapeutic decision making was audited in 85 patients who received IV rt-PA for a diagnosis of acute stroke. All the surviving patients were re-examined neurologically at least three months after IV rt-PA. Baseline and follow-up brain CT scans were re-reviewed by a neuroradiologist who was blinded to clinical presentation and outcome. Patients whose clinical presentation, brain CT and neurological outcome did not fit into known or expected anatomical and clinical patterns of stroke underwent psychological assessment using the Minnesota Multiphasic Personality Inventory-2.

In two patients three stroke-like presentations of somatoform disorder inadvertently were treated with IV rt-PA. This was primarily caused by abbreviated neurological examination and narrow differential diagnosis.

Patients with somatoform disorder may present with symptoms mimicking acute stroke. Under the time constraints of IV rt-PA use, a diagnosis of somatoform disorder can be missed, subjecting such patients to the potential complications of thrombolytics.

Fewer than 30 cases of oligodendroglioma or anaplastic oligodendroglioma metastatic to bone are reported in the literature. Prolonged survival even with therapy is uncommon.

We report a case of anaplastic oligodendroglioma metastatic to bone with a dramatic and durable response to temozolomide therapy. A retrospective case review, molecular analysis, and literature search were performed.

The patient presented with a right frontal mass in 1990. Progression led to resection of the lesion in 1995. Histology revealed an anaplastic oligodendroglioma and the tumour was found to have allelic loss of heterozygosity (LOH) of chromosome 1p (1p-). He received standard radiation therapy. In 2000 he developed hip and pelvic pain. A bone scan showed multiple skeletal lesions. Magnetic resonance imaging of the brain showed stability of intracranial disease. Resection of one lesion found metastatic anaplastic oligodendroglioma with identical morphology to the patient’s original tumour, including glial fibrillary acidic protein expression. The patient was started on standard temozolomide chemotherapy and celecoxib with prompt pain relief, and rapid normalization of serum alkaline phosphatase. He received a total of 12 cycles of combined therapy with no toxicity and no evidence of progression until increasing pain marked disease recurrence. The patient underwent palliative chemo- and radiation therapy but eventually succumbed.

Loss of heterozygosity 1p- is associated with prolonged survival in anaplastic oligodendroglioma and may increase the cumulative risk for development of systemic metastases. We speculate that metastases from oligodendroglioma harbouring loss of heterozygosity at chromosome 1p- retain the chemosensitivity of the initial lesion.

Methylphenidate is a central nervous system stimulant used for the treatment of attention deficit hyperactivity disorder and narcolepsy and like other psychostimulants has a potential for abuse.

A young man with a cerebral lacunar infarction following chronic oral abuse of methylphenidate is presented.

The experience of our patient and a review of the literature suggest that cerebral infarction is a potential side effect of chronic consumption of methylphenidate.

Fabry’s disease is an X-linked disorder, caused by a deficiency of the lysosomal enzyme α-galactosidase A which results in the accumulation of the glycosphingolipid, ceramide trihexose in the vascular endothelium and can lead to cerebral infarction. Male hemizygotes are generally more severely affected than heterozygote females. Clinical disease in females is thought to be due to unequal X chromosome inactivation.

A 19-year-old woman, who was previously well, presented with neurological deficits secondary to basal ganglia and pontine infarction. Extensive cardiac, arterial and hematologic investigations did not identify the etiology of her stroke. Muscle biopsy revealed endothelial lysosomal aggregates most consistent with a diagnosis of Fabry’s disease. The diagnosis was confirmed on the basis of molecular genotype analysis.

Inherited causes of stroke such as Fabry’s disease should be considered in young patients with stroke if an etiologic diagnosis is not reached after complete investigations. Muscle biopsy can assist with the diagnosis and guide further investigations. This report summarizes the biochemical and histological features of Fabry’s disease and the associated genetic abnormalities.

Metastatic renal cell carcinoma to the choroid plexus is a rare condition and can be easily confused with meningioma.

A 61-year-old female presented with progressive neurologic deterioration and MRI findings of obstructive hydrocephalus and a homogeneously contrast enhancing 3 cm oval mass in the trigone of the left lateral ventricle.

Despite radiologic, intraoperative gross features, and frozen pathology all consistent with meningioma, the final pathology revealed metastatic renal cell carcinoma.

Renal cell carcinoma metastatic to the choroid plexus can mimic intraventricular meningioma. We present a review of the literature and comparison of the radiological features of meningiomas and metastatic renal cell carcinoma. We also discuss the use of an under-utilized technique, the contralateral transcallosal approach, in the surgical treatment of this intraventricular lesion.

The lateralizing value of the motor automatisms is generally doubted in most patients with temporal lobe epilepsy. However, subgroup analysis of the seizures of temporal lobe origin suggests a role for motor automatisms in discriminating seizures of neocortical versus mesial temporal lobe origin.

Video-EEG of a patient with well-defined neocortical temporal lobe epilepsy was reviewed to assess the localizing value of motor automatisms.

We report a patient with left upper extremity motor automatisms and clonic movements of the proximal left lower extremity with altered awareness as the sole manifestations of right temporal neocortical seizures.

Early onset unilateral motor automatisms without dystonic posturing can localize the seizure origin to the contralateral temporal lobe neocortex.