Rasmussen's encephalitis is a progressive disease characterized by severe progressive neurological and cognitive deficits. It is a difficult condition to treat with conventional medical treatment and requires, in most of the cases, surgical intervention.

We present the case of a patient with Rasmussen's encephalitis who presented with epilepsia partialis continua, who did not respond to antiepileptic treatment and was evaluated for possible surgical intervention.

The electroencephalographic studies revealed bihemispheric involvement during seizures, although magnetic resonance imaging (MRI) showed a marked right hemispheric atrophy. Hyperperfusion over the right hemisphere was seen with the performance of ictal single photon emission computed tomography (SPECT), which finally guided a right functional hemispherectomy and subsequent seizure control.

Ictal SPECT could be a useful tool in the armamentarium of tools used for the localization of the epileptogenic focus in patients with Rasmussen's encephalitis and other localization-related epilepsies.

Clobazam has been used successfully in adults and children with partial epilepsy. The purpose of this study was to evaluate the safety and efficacy of clobazam as add-on therapy in children with epileptic encephalopathy.

This was a retrospective study conducted at the pediatric epilepsy clinic of our university hospital. Children less than 18-years of age with epileptic encephalopathy were included in the study. Clobazam was introduced as add-on therapy, starting with 5 mg/Kg/day and increased in minimally effective doses, up to the maximum tolerated dose. Data were obtained from clinical files and follow-up visits.

Ninety-seven patients were included in the study (39 girls), aged between 1 and 17-years-old (mean = 9.9). Twenty-six patients had Lennox-Gastaut syndrome, seven had myoclonic astatic epilepsy, nine had West syndrome and, in 57 patients, the type of epileptic encephalopathy could not be determined. Clobazam dosage ranged from 5 to 60 mg/day (mean = 37.5 mg/day). Forty (41%) patients presented with adverse events, most of which were mild and transitory, and clobazam needed to be withdrawn in only 11 patients. Nine (9.2%) patients were seizure-free after clobazam adjunctive therapy. In 11 (11.3%) patients seizure improvement was >75%, in 16 (16.5%) it was >50%, in 17 (17.5%) improvement was <50% and in 44 (45.5%) there was no change in seizure frequency. Three patients were lost to follow-up. In 85% of the patients with seizure improvement, the results lasted for more than one year.

Clobazam is safe and effective in the treatment of epileptic encephalopathies of childhood.

To determine the effectiveness of an Acute Stroke Triage Pathway in reducing door to needle times in acute stroke treatment with IV t-PA.

A previous study at our tertiary referral centre, examining IV t-PA door to needle times, was completed in 2000. The median door to needle time was beyond the recommended National Institute for Neurological Disorders and Stroke (NINDS) standard of 60 minutes. In November 2001, an Acute Stroke Triage Pathway was introduced in the emergency room (ER) to address this issue. The goal of this pathway was to rapidly identify patients eligible for treatment for IV t-PA, so that CT scans and lab studies could be arranged immediately upon ER arrival. Our hypothesis was that the Triage Pathway would shorten door to CT and door to needle times.

Using retrospective data, pre (n=87) and post (n=47) triage pathway times were compared. The door to CT time was reduced by 11 minutes (p=0.015) and door to needle time was reduced by 18 minutes (p=0.0036) in a subgroup of patients that presented directly to our hospital.

These results indicate that the Acute Stroke Triage Pathway is effective in reducing Door to CT and Door to Needle Times in patients presenting directly to our ER. However, a majority of treatment times were still beyond NINDS recommendations. Stroke Centers require periodic review of their efficiency to ensure that target times are being obtained and may benefit from the use of an Acute Stroke Triage Pathway.

A 61-year-old woman with Parkinson's disease, receiving pergolide 1.75 mg four times daily, was admitted with progressive dyspnea.

Investigations revealed mitral and aortic regurgitation. She underwent surgical mitral replacement and aortic repair, but had a postoperative course characterized by repeated bouts of congestive heart failure.

Severe tricuspid valve (TV) regurgitation developed within one month after the TV was reported on echocardiography to be relatively normal. Subsequent discontinuation of pergolide was associated with symptomatic improvement.

This case illustrates the severity and rapidity with which cardiac valvular abnormalities can develop in patients receiving pergolide.

Different strategies have been employed to recanalize acutely occluded middle cerebral and internal carotid arteries (ICA) in the setting of acute stroke including intravenous and intra-arterial tPA. However, pharmaceutical thrombolysis alone, may not be effective in patients with a large amount of clot volume (complete M1, terminal internal carotid artery). We report our initial experience with endovascular clot disruption using a soft silicone balloon in addition to intravenous or intraarterial thrombolysis with tPA.

This is a retrospective review of nine patients with symptoms of acute stroke from clot in the middle cerebral or internal carotid territories who were treated with intracranial balloon angioplasty. All patients presented with symptoms of acute anterior circulation stroke less than six hours from onset. Patients in whom computed tomography (CT) angiography confirmed the presence of large vessel clot (terminal ICA, M1 or proximal M2) were included in the study. A CT perfusion was performed providing maps of cerebral blood volume, flow and mean transit time. If the patient presented less than three hours from onset then intravenous tissue plasminogen activator (tPA) was also administered. Intra-arterial tPA was delivered into the clot. If the volume of clot was judged to be significant by the treating neurointerventionist, then a limited trial of tPA was administered intraarterially followed by balloon angioplasty of persistant clot. The time from imaging to vessel recanalization was recorded. Clinical outcomes were assessed using the modified Rankin scale and Barthel Index.

Diagnostic CT perfusion studies were performed in 7 (78%), all of which showed a significant amount of salvageable tissue as judged by the treating neurointerventionist and neurologist. Recanalization (TIMI 2 or 3) was possible in 8 (89%). There were no cases of symptomatic intracranial hemorrhage and 2 (22%) asymptomatic hemorrhages. The average time from performance of the initial emergency CT to vessel recanalization was 2.1 hours with mean time from symptom onset to vessel recanalization of 4.1 hours. Five (56%) patients had good outcomes, 1 (11%) had mild and 3 (33%) had moderate to severe disability.

Clot angioplasty can potentially shorten recanalization times in well-selected patients and can be an effective complimentary procedure in patients with tPA resistant clot. Angioplasty can be performed with a very low complication rate using the technique described and may be associated with good outcomes.

Invasive monitoring for the investigation of medically intractable epilepsy may be associated with undesirable morbidity. We performed a review of our recent experience to determine the incidence of major complications.

We reviewed the clinical records of all patients who underwent invasive EEG monitoring at our institution between 2000 and 2004.

One-hundred and sixteen patients (57 males, 59 females) with a mean age of 32 years of age underwent intracranial placement of electrodes for epilepsy surgery investigation. Subdural strips were placed in 115 patients with a mean of eight strips per patient. Subdural grids were inserted in 11 patients and depth electrodes in five. Fourteen of the 15 patients with grids or depth electrodes also had strips. Coverage was unilateral in 37 patients and bilateral in 79 patients. Electrodes were placed over the frontal lobe in 78 cases, temporal in 93, parietal in 24, and occipital in 27 patients. The average duration of investigation was 12.3 days (range 3-29). The evaluation led to the performance of a surgical resection in 85 patients (74%). Complications were seen in four patients with subdural strips (3%), and in two patients with grids (13%), characterized by clinical infection, intracranial hemorrhage, aseptic meningitis, transient neurological deficits, and status epilepticus. Mortality was nil.

In comparison with previously published literature on the topic, the major complication rate in this group of patients appears to be low.

To examine demographic characteristics and clinical features of headache patients referred to neurologists specializing in headache in Canada.

Demographic and clinical data were collected at the time of consultation for 865 new headache patients referred to five headache-specialty clinics in Canada. The Headache Impact Test (HIT-6) and Migraine Disability Questionnaire (MIDAS) were used to measure headache impact and disability. Data were analyzed as part of the Canadian Headache Outpatient Registry and Database (CHORD) Project.

The average age of the patients was 40 years and the majority were female (78%). Most were employed either full time (49%) or part time (13%). The majority of patients were diagnosed with either migraine or tension-type headache (78%). Over a third of patients experienced headache every day, and half had experienced a headache in the previous month which was of severe intensity. Most (80%) scored in the “very severe” category of the HIT-6 and over half (55%) were severely disabled as measured by the MIDAS.

Patients referred to headache specialists in Canada are severely disabled by their headache disorders. These patients are in the most productive phase of their lives in terms of age and employment. It is important to provide the best available treatment to headache patients in order to minimize the disability and impact of their headache disorders.