Management of chronic diseases such as movement disorders can be challenging. Nurse-administered telephone follow-up programs have demonstrated clinical and cost efficacy in a variety of health care models. However, their efficacy in movement disorders has not been sufficiently addressed. This observational study fills a knowledge gap by reporting the nature of individuals utilizing a nurse-administered telephone service and the reasons for and the outcomes of calls.

Consecutive calls received by the clinic for a 12-month duration were recorded. A sample of 312 calls from 132 patient charts was analyzed. Variables for analysis and coding schema were determined a-priori and included demographic information as well as information around the reasons for and outcomes of calls. The narratives of documented calls were reviewed retrospectively and responses coded for analysis by a separate researcher. Data was analyzed using descriptive statistical methods.

Patients made the majority of calls (49%). 27% of calls related to worsening symptoms and another 35% of calls related to medication issues or renewals. The mean call duration was 15.93 minutes. The majority of calls were received mid-way between clinic visits (M = 89.24 days). The nurse resolved 84% of calls independently. The mean number of calls per patient was 2.93. Issues reported by patients were resolved (approximately 90%) without need for follow-up emergency, family, or subspecialty clinic visits.

The results underscore the complexity of medical issues in a movement disorders population. The current study provides support for a nurse-administered telephone follow-up program in movement disorders.

Reflex assessment, an essential element in the investigation of the motor system, is currently assessed through qualitative description, which lacks of normal values in the healthy population. This study quantified the amplitude and latency of patellar tendon reflex in normal subjects using motion analysis to determine the factors affecting the reflex amplitude.

100 healthy volunteers were recruited for patellar tendon reflex assessments which were recorded using a motion analysis system. Different levels of input strength were exerted during the experiments.

A linear relationship was found between reflex input and reflex amplitude (r = 0.50, P <0.001). The left knee was found to exhibit 26.3% higher reflex amplitude than the right (P <0.001). The Jendrassik manoeuvre significantly increased reflex amplitude by 34.3% (P = 0.001); the effect was especially prominent in subjects with weak reflex response. Reflex latency normality data were established, which showed a gradual reduction with increasing input strength.

The quantitative normality data and findings showed that the present method has great potential to objectively quantify deep tendon reflexes.

The allocation of limited healthcare resources poses a constant challenge for clinicians. One everyday example is the prioritization of elective neurosurgical operating room (OR) time in circumstances where cancellations may be encountered. The bioethical framework, Accountability for Reasonableness (A4R) may inform such decisions by establishing conditions that should be met for ethically-justifiable priority setting.

Here, we describe our experience in implementing A4R to guide decisions regarding elective OR prioritization.

The four primary expectations of the A4R process are: (1) relevance, namely achieved by support for the process and criteria for decisions amongst all stakeholders; (2) publicity, satisfied by the effective communication of the results of the deliberation; (3) challengeability through a fair appeals process; and (4) Oversight of the process to ensure that opportunities for its improvement are available.

A4R may be applied to inform OR time prioritization, with benefits to patients, surgeons and the institution itself. We discuss various case-, patient-, and surgeon-related factors that may be incorporated into the decision-making process. Furthermore, we explore challenges encountered in the implementation of this process, including the need for timely neurosurgical decision-making and the presence of hospital-based power imbalances.

The authors recommend the implementation of a fair, deliberative process to inform priority setting in neurosurgery, as demonstrated by the application of the A4R framework to allocate limited OR time.

Acute fulminant hepatic failure (AFHF) is common in tertiary care centres with transplant facilities. Cerebral edema frequently threatens the lives of such patients. We reviewed our cases of AFHF, noting the incidence of cerebral edema with serial CT scans and factors associated with mortality.

Patients were captured through HmRI classification of acute liver'hepatic failure. Chart review included tabulation of: demographics, INR; serum bilirubin, creatinine, albumin; in-hospital mortality. Computed tomogram (Ct) scans were re-read with blinding to clinical information and catalogued for changes in sulcal markings, ventricular size and grey-white differentiation (GWD). Inclusion criteria: age equal to or greater than 16 years, encephalopathy, hepatic failure within eight weeks of onset of liver disease, CT scans of head performed.

Of our 25 cases with AFHF, acetaminophen toxicity was the most common etiology (nine cases). Twelve of the 25 patients (48%) had cerebral edema on CT, including eight of the nine (89%) with acetaminophen toxicity. Decrease in sulcal markings and ventricular size preceded conspicuous alterations in GWD. Fourteen died, including all 12 with cerebral edema, although death was due to herniation in only one patient. None of the hematological or biochemical variables correlated significantly with mortality.

Acetaminophen toxicity is a common cause of AFHF; this combination has a strong association with cerebral edema. Cerebral edema can be detected in its early stages and followed by baseline and serial CT scans. This facilitates management to prevent fatal brain herniation.

Infantile-onset saccade initiation delay (ISID), also known as congenital ocular motor apraxia, is characterized by the inability to initiate volitional horizontal saccades. Other abnormalities including developmental delay and ataxia have been reported. The frequency of these abnormalities is unknown. We performed a detailed review of the medical literature to quantify features of ISID.

We searched the English medical literature for articles related to ISID from 1952 to 2010. Whenever possible, patients were excluded if they had acquired SID, Joubert syndrome or neurodegenerative conditions. The minimum prevalence was calculated for each abnormality.

Sixty-six articles with information on 288 patients were included in the analysis. Head thrusts were reported in 84.7%. Blinks without head thrusts were used to initiate saccades in 41%. The fast phases of the optokinetic response and vestibulo-ocular reflex were impaired in 69.8% and 34.4% respectively. Smooth ocular pursuit was abnormal in 33%. Global developmental delay occurred in 41.3%, speech or language delay in 36.5%, cognitive delay in 17%, hypotonia in 35.8%, motor delay in 48.6%, and ataxia/clumsiness in 49.3% of patients. Neuroimaging was performed on 197 patients and was normal in 39.1%. Abnormalities involved the cerebellum (24.9%), cerebrum (15.7%), other infratentorial structures (11.7%), and corpus callosum (6.1%).

Infantile-onset saccade initiation delay is frequently associated with deficits in reflexive saccades and less frequently with impaired smooth ocular pursuit. Developmental delay, hypotonia, and ataxia occur frequently in ISID, suggesting more global brain impairment and not just a saccadic disorder.

Cognitive impairment in multiple sclerosis (MS) often involves attentional deficits. The Stroop colour word test, a measure of attention, lacks current normative data for an english-speaking North american MS population. Further some authors suggest the Stroop actually measures processing speed.

To generate normative data for the Stroop colour word test that can be used for a Canadian or North american MS population and to examine the relationship between processing speed tests - the Paced auditory Serial addition Test (PASAT) and Symbol Digit Modalities Test (SDMT) - and the Stroop.

Data from 146 healthy subjects aged 18-56 was collected. age was significantly although weakly correlated with general intelligence (r=0.168, p=0.043) assessed with the North american adult Reading Test (NAART), and education (r=-0.313, p<0.001). No demographic variables were associated with SDMT or PASAT. age had a low-moderate negative correlation (r=-0.403, p<0.001) with Stroop scores. The mean (±standard deviation, SD) Stroop score was 45.4(10.4). The z-score can thus be calculated as [(X-45.4)/10.4]. if adjusted for age, Xadj = [X-(-0.47)(age-37.5)] and is substituted for X. in a comparison MS population consisting of 75 randomly selected patients from the MS Cognitive clinic, Stroop and PASAT performance were not related. a relationship existed between Stroop and SDMT scores but only 12.2% of the Stroop score variance was explained by the SDMT. Therefore, the Stroop measures selective attention independently of processing speed.

This data can be used to determine impaired attention in MS patients.

Bevacizumab, a humanized recombinant anti-vascular endothelial growth factor antibody, was approved in Canada in 2010 for the treatment of high-grade glioma. We report the effectiveness and safety of bevacizumab in the treatment of patients with recurrent high-grade gliomas at a single institution.

Twenty-seven consecutive patients with high-grade glioma (anaplastic glioma and glioblastoma) at first or subsequent relapse were treated with bevacizumab alone or in combination with chemotherapy. The primary endpoint was progression-free survival (PFS) and secondary endpoints were objective response rate, six month PFS, overall survival (OS), and safety profile.

The clinical benefit rate (complete and partial responses plus stable disease) was 59%. Median PFS was 4.3 (95% CI, 3.0-10.9) months, with a six month PFS rate of 43%. Median OS after current relapse was 8.9 (95% CI, 5.8-not reached) months. Ten episodes of grade 3/4 adverse events were observed in nine patients, including fatigue (n = 3), thrombocytopenia (n = 4), and myelotoxicity, febrile neutropenia, and pulmonary embolism (each n = 1).

We consider the efficacy and safety profile of bevacizumab is comparable to other cohorts of patients treated for recurrent high-grade glioma at other international institutions.

The role of diffusion tensor tractography (DTT) has become increasingly important in the preoperative mapping of brain white matter. Recently, functional magnetic resonance imaging (fMRI) driven DTT has provided the ability to evaluate the spatial relationship between the corticospinal tract (CST) and motor resection tumor boundaries. The main objective of this study was improvement of the preoperative assessment of the CST in patients with gliomas involving the motor cortical areas.

Seventeen patients with gliomas involving motor cortical areas underwent 3 dimensions (3D) T1-weighted imaging for anatomical referencing, using both fMRI and diffusion tensor imaging (DTI). We used the fast-marching tractography (FMT) algorithm to define the 3D connectivity maps within the whole brain using seed points selected in the white matter adjacent to the location of fMRI activation. The target region of interest (ROI) was placed in the cerebral peduncle. Karnofsky performance status (KPS) scores were evaluated for each patient before and after surgery.

The CST of a total seventeen patients were successfully tracked by choosing seed and target ROI on the path of the fibers. What is more, DTT can indicate preoperatively the possibility for total glioma removal or the maximum extent of surgical resection. The postoperative average KPS score for the seventeen patients enrolled increased by more than 10 points.

Incorporation of fMRI driven DTT showed a maximum benefit in surgical treatment of gliomas. Our study of the assessment precision should enhance the accuracy of glioma operations with a resulting improvement in postoperative patient outcome.

To determine the safety and tolerability of home-based intravenous immunoglobulin (IVIG) (Gamunex) as maintenance treatment in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in Canada.

We enrolled ten subjects with CIDP who had previously received IVIG in the hospital setting to receive the comparable IVIG dose (1-2 g/kg/month) in the home for six months. The patients were evaluated in the clinic at three months and at six months to evaluate their clinical status as well as the safety and tolerability of IVIG.

All subjects tolerated home-based IVIG treatment as maintenance treatment of CIDP. There were no serious adverse events related to IVIG. Subjects did experience “anticipated” IVIG events post-infusion such as headache and fatigue, which were managed with analgesics and supportive counseling. One subject withdrew consent at end of study due to hospitalization. This event was not related to the IVIG. Another subject experienced a “flare” of CIDP symptoms near the end of the study, however, completed all visits as per protocol. All subjects expressed excellent satisfaction with the individualized therapy, and almost all (nine out of ten) patients preferred home-infusion to hospital-infusion.

Intravenous immunoglobulin can be delivered safely and is well tolerated outside the hospital setting in Canada in patients with chronic, stable neuromuscular conditions such as CIDP who have previously tolerated IVIG in the hospital medical day Care Unit.

The autonomic reflex screen (ARS) is a composite of well-defined tests of various autonomic domains and is an essential part of the diagnosis of autonomic disorders. Institutional and regional differences exist and necessitate the ongoing development of control values for the ARS. Here we present data obtained from healthy participants from Southwestern Ontario.

A total of 121 healthy individuals underwent quantitative sudomotor axon reflex testing (QSART), heart rate response to deep breathing (HRDB), and Valsalva maneuver using standard protocols as part of the ARS.

Sweat volumes obtained during QSART are presented by site (forearm, proximal leg, distal leg and foot) and by gender. Data is expressed as the mean sweat volume per site with the associated 2.5th, 5th and 95th percentiles. Data for males and females is also stratified by age group (14-25, 26-40 and 41-76 years). Measurements of cardiovagal parasympathetic function including HRDB and Valsalva ratio are stratified by age group (14-25, 26- 40 and 41-76 years). Data is expressed as the mean with associated percentiles (2.5, 5, 95 and 97.5 percentiles).

The current manuscript provides control data for the various components of the ARS to aid in the diagnosis of autonomic disorders.

To study different radiological signs and sequences including apparent diffusion coefficient (ADC) and gradient echo (GRE) to differentiate degenerative parkinsonian syndromes.

Multiple system atrophy (MSA), Parkinson's disease (PD), progressive supranuclear palsy (PSP) and corticobasal degeneration (CbD) differ in the pattern of neurodegeneration and cellular damage. Measuring the ADC, GRE sequences for paramagnetic substances and simple anatomical assessments have been reported individually to assist in separating some of these disorders, but have not been compared.

brain MRIs from May 2002 to February 2008 were retrospectively evaluated by raters blinded to the clinical diagnosis for predefined MRI signs on T1, T2 and GRE sequences. ADC values were quantitatively measured. Medical records were objectively analyzed using standard clinical criteria for different parkinsonian syndromes.

195 cases comprising of 61 PD, 15 MSA-P, 7 MSA-C, 21 PSP, 6 Corticobasal syndrome, 21 not fitting criteria and 64 controls were evaluated. 73% of patients with MSA-P had hypointensity of the putamen (compared to the pallidum) on GRE. The specificity of this sign to diagnose MSA-P was 90% versus PD and 76% versus PSP. When GRE hypointensity was combined with atrophy of the putamen the specificity improved to 98% (versus PD) and 95% (versus PSP) without altering the sensitivity. The ADC values were significantly higher in the middle cerebellar peduncle in cases with MSA-C versus controls, PD and PSP (p<0.001).

The combination of hypointensity and atrophy of the putamen on GRE is useful in differentiating MSA-P from other parkinsonian syndromes.

vascular dementia (VaD) and mixed Alzheimer's disease (AD/VaD) are common. How best to monitor treatment is not clear. Our objective was to compare responsiveness and construct validity of change scores, following donepezil treatment, of the standardized Mini-Mental State Examination (sMMSE) and other measures potentially usable in primary care.

A six-month, outcome measurement study. The Disability Assessment for Dementia (DAD), CLOX-1 and 2, Phonetic Fluency, a short Neuropsychiatric Inventory, (the NPI-Q), Clinical Global Impression (CGI) and the SymptomGuide™ (SG) were measured. Construct validity was tested by correlating change scores, and responsiveness by calculating standardized response means (SRMs).

Of 148 treated patients, 116 completed. The mean sMMSE increased by 0.7 (95% Confidence Interval (CI) = -0.005, 1.41; p=0.06; SRM= 0.15). There was no statistically significant difference in the DAD. The NPI-Q (-1.4; 95% CI = -2.08, -0.72; p<0.01; SRM=0.24), ClOX-1 (0.9; 95% CI = 0.19, 1.61; p<0.01; SRM=0.21), ClOX-2 (0.9; 95% CI = 0.17, 1.63; p=0.03; SRM=0.26), Phonetic Fluency (0.9; 95% CI = 0.19, 1.61; p=0.02; SRM=0.21) and SG (0.35; 95% CI = 0.20,0.51; p<0.01; SRM=0.28) each detected significant improvement. The CGI suggested improvement in 74 completers (64%) - mostly “minimal” (44/116, 38%) - while 21/116 (18%) were worse. Change scores at 24 weeks were at best modestly correlated with each other (range -0.22 to 0.30).

Different measures showed different responsiveness, in a setting in which the mean treatment effect seems to have been small, but clinically detectable. Patient-centered and executive function measures might be useful in vascular and mixed dementia.