We present information regarding the standardization, reliability and clinical validity of two versions of the Behavioural Neurology Assessment (BNA). The BNA-Long Form consists of 24 subtests within separate domains: Attention, Memory, Language, Visuospatial Function, Executive Function, and Praxis. The BNA-Short Form consists of 13 subtests within the domains of Attention, Memory, Naming, Visuospatial Function and Executive Function. In addition to individual domain indices, a Grand Total score was calculated for both BNA versions.

To standardize the administration and scoring and validate the BNA for detection of dementia.

Standardized normative data were obtained on 115 healthy subjects ranging in age from 50 to 95. Test-retest stability was obtained on 19 subjects and clinical validity was investigated by administering the BNA and Mini-Mental Status Examination (MMSE) to 29 patients with dementia and 29 age-matched healthy subjects (controls).

Age had a significant effect on all but the Visuospatial and Praxis indices of the BNA-Long Form and an effect on Naming and Grand Total score of the Short-Form. Internal consistency (Cronbach's coefficient a) was .87 and .67 for the Long and Short Forms (.95 and .96 for dementia and control groups combined). Test-retest stability was acceptable. Grand Total indices of both BNA versions showed significant, positive correlations with the MMSE. Both BNA versions had superior sensitivity to dementia relative to the MMSE (.93 versus .79). Specificity was equivalent to the MMSE (.93 versus .97).

Positive predictive values of the BNA and MMSE are equivalent but the BNA provides superior negative predictive value.

Pulmonary edema (PE) can occur in the early or late period following subarachnoid hemorrhage (SAH). The incidence of each type of PE is unknown and the association with ventricular dysfunction, both systolic and diastolic, has not been described.

Retrospective chart review of 178 consecutive patients with SAH surgically treated over a three-year period. Patients with pulmonary edema diagnosed by a radiologist were included. Early onset SAH was defined as occurring within 12 hours. Cardiac function at the time of the PE was analyzed using hemodynamic and echocardiographic criteria of systolic and diastolic dysfunction. Pulmonary edema was observed in 42 patients (28.8%) and was more often delayed (89.4%). Evidence of cardiac involvement during PE varied between 40 to 100%.

Pulmonary edema occurs in 28.8% of patients after SAH, and is most commonly delayed. Cardiac dysfunction, both systolic and diastolic, is commonly observed during SAH and could contribute to the genesis of PE after SAH.

The goal of this study was to provide a general descriptive and cognitive portrait of a population with traumatic brain injury (TBI) at the time of their acute care stay.

Three hundred and forty-eight TBI patients were assessed. The following data were collected for each patient: age, level of education, duration of post-traumatic amnesia, Galveston Orientation Amnesia Test score, Glasgow Coma Scale score, results of cerebral imaging, Neurobehavioral Rating Scale score, the Functional Independence Measure cognitive score and the Glasgow Outcome Scale score.

The clinical profile of the population revealed a mean age of 40.2 (±18.7) and a mean of 11.5 (±3.6) years of education. Most patients presented with frontal (57.6%) and temporal (40%) lesions. Sixty-two percent had post-traumatic amnesia of less than 24 hours. Seventy percent presented with mild TBI, 14% with moderate and 15% with severe TBI. The cognitive deficits most frequently observed on the Neurobehavioral Rating Scale were in the areas of attention, memory and mental flexibility as well as slowness and mental fatigability. Most patients had good cognitive outcome on the Functional Independence Measure and scores of 2 and 3 were frequent on the GOS. Forty-five percent of the patients returned home after discharge, 51.7% were referred to in or out patient rehabilitation and 3.2% were transferred to long-term care facilities.

Because of the specialized mandate of acute care institutions, the information provided here concerning characteristics of our TBI population is essential for more efficient decision-making and planning/programming with regards to care and service delivery.

Deep brain stimulation (DBS) is used increasingly worldwide for the treatment of Parkinson's disease, dystonia, tremor and pain. As with any implanted system, however, DBS introduces a new series of problems related to its hardware. Infection, malfunction and lead migration or fracture may increase patient morbidity and should be considered when evaluating the risk/benefit ratio of this therapy. This work highlights several factors felt to increase DBS hardware complications.

The authors undertook a prospective analysis of their patients receiving this therapy in two Canadian centres, over a four-year period.

One hundred and forty-four patients received 204 permanent electrode implants. The average follow-up duration was 24 months. Complications related to the DBS hardware were seen in 11 patients (7.6%). There were two lead fractures (1.4%) and nine infections (6.2%) including two erosions (1.4%). There was a significantly greater risk of infection in patients who underwent staged procedures with externalization. In patients with straight scalp incisions, the rate of infection was higher than that seen with curved incisions.

Hardware complications were not common. A period of externalization of the electrodes for a stimulation trial was associated with an increased infection rate. It is also possible that a straight scalp incision instead of curvilinear incision may lead to an increase in the rate of infection. With a clear understanding of the accepted DBS device indications and their potential complications, patients may make a truly informed decision about DBS technology.

Human immunodeficiency virus (HIV) infection frequently results in neurological complications but the impact of different neurological syndromes on patients' quality of life remains unknown.

We investigated health-related quality of life (HRQoL) parameters among HIV/Acquired Immune Deficiency Syndrome (AIDS) patients with and without neurological disease, including 11 dimensions of HRQoL within the Medical Outcomes Short-form Health Survey-HIV.

Comparisons of sociodemographic and systemic clinical variables did not differ between HIV/AIDS patients with (n=94) and without (n=75) neurological disease. However, patients with neurological diseases exhibited significantly lower HRQoL scores compared to matched controls, which was most evident among HIV/AIDS patients with cognitive impairment and sensory neuropathy. Prospective analysis revealed diminishing HRQoL scores prior to neurological diagnosis followed by a progressive and sustained improvement in HRQoL scores after intervention over a 96-week period.

These studies indicate that while HIV-related neurological diseases are associated with reduced HRQoL scores, enhanced neurological care has a positive impact on HIV/AIDS patients' overall well-being.

Changes in brain lesion loads assessed with magnetic resonance imaging obtained at 1.5 Telsa (T) are used as a measure of disease evolution in natural history studies and treatment trials of multiple sclerosis.

A comparison was made between the total lesion volume and individual lesions observed on 1.5 T images and on high-resolution 4 T images. Lesions were quantified using a computer-assisted segmentation tool.

There was a 46% increase in the total number of lesions detected with 4 T versus 1.5 T imaging (p<0.005). The 4 T also showed a 60% increase in total lesion volume when compared with the 1.5 T (p<0.005). In several instances, the 1.5 T scans showed individual lesions that coalesced into larger areas of abnormality in the 4 T scans. The relationship between individual lesion volumes was linear (slope 1.231) showing that the lesion volume observed at 4 T increased with the size of the lesion detected at 1.5 T. The 4 T voxels were less than one quarter the size of those used at 1.5 T and there were no consistent differences between their signal-to-noise ratios.

The increase in signal strength that accompanied the increase in field strength compensated for the loss in signal amplitude produced by the use of smaller voxels. This enabled the acquisition of images with improved resolution, resulting in increased lesion detection at 4 T and larger lesion volumes.

Pulmonary and respiratory muscle function impairment are common in patients with Parkinson's disease (PD). Inspiratory muscle training may improve strength, dyspnea and functional capacity in healthy subjects and in those with chronic obstructive pulmonary disease. This study investigated the effect of specific inspiratory muscle training (SIMT) on pulmonary functions, inspiratory muscle performance, dyspnea and quality of life, in patients with PD.

Twenty patients with PD (stage II and III Hoehn and Yahr scale) were recruited for the study and were divided into two groups: a) ten patients who received SIMT and b) ten patients who received sham training, for three months. Pulmonary functions, the respiratory muscle strength and endurance, the perception of dyspnea (POD) and the quality of life were studied before and within one week after the training period. All subjects trained daily, six times a week, each session consisting of 1/2 hour, for 12 weeks.

Following the training period, there was a significant improvement, in the training group but not in the control group, in the following parameters: inspiratory muscle strength, (PImax, increased from 62.0±8.2 to 78.0±7.5 cm of H2O (p<0.05), inspiratory muscle endurance (increased from 20.0±2.8 to 29.0±3.0 cm of H2O (p<0.05), and the POD (decreased from 17.9±3.2 to 14.0±2.4 units (p<0.05). There was a close correlation between the increase in the inspiratory muscle performance and the decrease in the POD.

The inspiratory muscle performance may be improved by SIMT in patients with PD. This improvement is associated with a significant decrease in their POD.

To determine the impact of check size and interstimulus interval (ISI) on neuromagnetic visual cortical responses.

We recorded visual evoked fields to pattern-reversal stimulation with central occlusion in ten subjects. The ~100 ms magnetic activation (P100m) was analyzed by single dipole modeling.

With 1 s ISI, P100m strengths increased as check size increased from 15' up to 120' of visual arc, and larger checks elicited less P100m activation. With 120' checks, we found no P100m attenuation as ISI decreased from 4 s to 0.16 s. P100m sources around the calcarine sulcus did not vary with check size or ISI.

The magnitude of cortical activation during visual contrast processing is check size-dependent and the 120' checks are optimum for future studies on neuromagnetic visual cortical functions using central-occluded stimulation. The corresponding neuronal activation demonstrated a short refractory period less than 0.16 s. We also found significantly overlapping cortical representation areas for different check sizes or ISIs.

To date, few pediatric series of neurofibromatosis type 1 (NF-1) have been described in the literature even though it is the most frequently encountered phakomatosis.

We reviewed 987 charts of pediatric patients with a presumptive diagnosis of NF-1 who were evaluated at Ste-Justine hospital from January 1, 1991 to July 31, 2002. Patients who presented with two or more cardinal criteria were diagnosed with NF-1. Clinical and laboratory data were retrospectively collected, including: demographics, neuroimaging and presence or absence of associated symptoms or signs of NF-1.

A total of 279 patients were diagnosed with NF-1. The mean age at diagnosis was 3.4 years. Ninety-nine percent of the patients had café au lait spots and 47% had a first degree relative with NF-1. Almost 60 percent (59.6%) of those seen by an ophthalmologist had Lisch nodules. Optic glioma was found in in 14.7%, cutaneous neurofibromas in 38.4%, plexiform neurofibromas in 24.7%, neurofibrosarcoma in 1.8%, learning disabilities in 39%, attention deficit disorder in 40.5%, osseous dysplasias in 7.2%, pseudoarthrosis in 3.6%, precocious puberty in 3.2% and short stature in 17.9%. Magnetic resonance, when performed, showed hyperintense T2 lesions in 87.1% of cases. The mean period of follow-up was 7.4 years.

Neurofibromatosis type 1 is a multisystemic disorder associated with increased risk of malignancy. It can be diagnosed at a very young age and clinical follow-up is advised. To our knowledge, this is the largest single center study of NF-1 in a pediatric population.

An estimated 20-25% of all strokes occur during sleep and these patients wake up with their deficits. This study evaluated outcomes among patients who woke up with stroke compared to those who were awake at stroke onset.

Using data from the Registry of the Canadian Stroke Network Phases 1 and 2, we compared demographics, clinical data and six-month outcomes between patients with stroke-on-awakening versus stroke-while-awake. Strokes of all types (ischemic stroke, transient ischemic attack, intracerebral hemorrhage and subarachnoid hemorrhage) were included. Standard descriptive statistics, multivariable logistic regression and general linear modeling were applied to the data to compare variables.

Among 2,585 stroke patients, 349 (13.5%) woke up with stroke and 2,236 (86.5%) did not. Patients with stroke-on-awakening were more likely to have higher blood pressure and to suffer ischemic stroke, but stroke severity, measured by level of consciousness, did not differ. Mortality both at discharge and at six-month follow-up did not differ between the two cohorts. However, patients with stroke-on-awakening were less likely to return home, and their median Stroke Impact Scale-16 scores were 7.0 points lower compared to those with stroke-while-awake.

There are minor demographic and clinical differences between patients with stroke-on-awakening and stroke-while-awake. Functional outcomes are slightly worse among patients with stroke-on-awakening, an effect which was driven by poor outcomes among patients with subarachnoid hemorrhage.

Cysts of the ligamentum flavum are rare and unusual causes of spinal compression.

We report our experience of four cases of ligamentum flavum cysts occurring in the lumbar spine and discuss some of the possible etiologies and pathophysiologic mechanisms according to the available literature.

This entity is clearly different from the synovial facet-joints or ganglion cysts.

Sympathomimetic-related intracerebral hemorrhage is well-documented. Most cases are associated with phenylpropanolamine use.

We report a case of intracerebral hemorrhage occurring in a middle-aged man who suffered from chronic sinusitis and had been ingesting pseudoephedrine daily for one year. This patient was previously well with no known cardiovascular risk factors. Clinical examination revealed no evidence of vasculitis nor coagulopathy and initial neuroimaging (i.e., computed tomography, angiography, magnetic resonance imaging) demonstrated no features consistent with aneurysm, arteriovenous malformation (AVM), cavernoma, nor cerebral metastases. A follow-up cerebral angiogram demonstrated a small AVM arising off a branch of the pericallosal artery and a small arteriovenous fistula arising off the costal marginal branch. The AVM was embolized without incident, however, the AVF was not accessible.

Sympathomimetics have long been associated with intracerebral hemorrhage. Since 1979, over 30 published case reports have documented the relationship between phenylpropanolamine and stroke. Only one report links phenylpropanolamine consumption to an intracerebral hemorrhage in a patient with an AVM. There is a paucity of literature etiologically inculpating other ephedra alkaloids in the causation of intracerebral hemorrhage. This is a case of pseudoephedrine-induced intracerebral hemorrhage in a patient with an underlying vascular malformation.

Hyaline body myopathy (HBM) is a rare chronic nonprogressive congenital myopathy, with variable patterns of inheritance.

We describe a patient with congenital HBM with progression of weakness and increasing muscle pain in adulthood. Three muscle biopsies, done at various times in her life, are reported.

Symptoms started during childhood; however, as an adult, following a period of stability with no progression of the disease, the patient became symptomatic with worsening proximal limb weakness, severe aching pain and hypertrophy of calves. Moderate elevations of serum creatine kinase and myopathic features were noted on electrophysiologic testing. Muscle pathology showed significant fatty infiltration of skeletal muscle and increased number of fibers with internal nuclei. Histology demonstrated the presence of subsarcolemmal, well-delineated hyaline areas, which on histochemical studies was shown to be limited to type1 fibers. The hyaline bodies were dark with pH 4.2 ATPase and with immunohistochemical studies reacted only with myosin heavy chain slow. Electron microscopy showed the hyaline bodies to be composed of nonmembrane bound, fairly even sized granular material, which merged with the adjacent myofibrils. Earlier muscle biopsies, done during childhood, also revealed presence of similar subsarcolemmal hyaline deposits.

There appears to be a pattern of presentation with adulthood progression in HBM, which has not been described before. Further case studies are required to understand the clinical progression in HBM.

Cervical internal carotid artery (ICA) occlusion associated with middle cerebral artery (MCA) embolic occlusion requires prompt revascularization to prevent devastating stroke. With the advent of endovascular techniques for chemical and mechanical thrombolysis, the clinical outcome of patients with major arterial occlusions will improve. Finding the most expedient pathway to the site of end organ occlusion for thrombolysis is important.

We present two cases of acute stroke secondary to thrombotic occlusion of the cervical ICA associated with MCA embolic occlusion treated with intra-arterial thrombolysis via catheter navigation through the posterior communicating artery to the site of MCA arterial occlusion. No attempt was made to transverse the occluded ICA.

Near complete restoration of flow was achieved in one patient and minimal vessel reopening was observed in the other patient. Both patients had good outcomes.

Intraarterial thrombolysis via Circle of Willis collaterals such as the posterior communicating artery for the treatment of acute thrombotic occlusion of the cervical internal carotid artery associated with embolic occlusion of the middle cerebral artery is a therapeutic option. This treatment option avoids the potential complications of navigating through an occluded proximal internal carotid artery and may expedite reopening of the MCA.