transient ischemic attack (tIA) and minor stroke have a high risk of early neurological deterioration, and patients who experience early improvement are at risk of deterioration. We generated a score for quantifying the worst reported motor and speech deficits and assessed whether this predicted outcome.

510 tIA or minor stroke (NIHSS>4) patients were included. the Historical Stroke Severity Score (HSSS) prospectively quantified the patient's description of the worst motor or speech deficits. the HSSS was rated at the time of first assessment with more severe deficits scoring higher. Motor HSSS included assessments of arm and leg motor power (score total 0-5). Speech HSSS assessed severity of dysarthria and aphasia (total 0-3). the association between motor and speech HSSS and symptom progression was assessed during the 90-day follow-up period.

the proportion of patients in each category of the motor HSSS was 0: 43% (216/510), 1: 22%(110/510), 2: 17% (89/510), 3: 7% (37/510), 4: 5% (28/510) and 5: 6% (30/510). Motor HSSS was associated with symptom progression (p=0.004) but not recurrent stroke. Speech HSSS was not associated with either progression or recurrent stroke. Motor HSSS predicted disability (p=0.002) and intracranial occlusion (p=0.012). Disability increased with increasing motor HSSS.

taking a detailed history about the severity of motor deficits, but not speech, predicted outcome in tIA and minor stroke patients. A score based on the patient's description of the severity of motor symptoms predicted symptom progression, intracranial occlusion and functional outcome, but not recurrent stroke in a tIA and minor stroke population.

the goal of this study was to identify important prognostic variables affecting placement of a percutaneous endoscopic gastrostomy (Peg) tube after acute stroke.

We retrospectively reviewed our patient database to identify acute ischemic stroke patients who placed Peg or nasogastric tube (Ngt) tube, but were free of other confounding conditions affecting swallowing. A total of 340 patients were involved in our study. We assessed the influence of age, National Institutes of Health stroke scale (NIHss) score, infarct volume, stroke subtype based on the toAst criteria, swallowing disorders, bilateral lesions in cerebrum and length of stay (los) in a logistic regression analysis.

In univariate analysis, age (p=0.048), NIHss score (p<0.0001), lesion volume (p<0.0001), los (p<0.0001), stroke location (p=0.045), and swallowing disorders (p<0.0001) were found to be the primary predictors of placing Peg. the presence of lesions in bilateral cerebral was included in the final model based on clinical considerations. After multivariate adjustment, only NIHss score (odds ratio [oR], 4.055; 95% confidence interval [CI], 2.398-6.857; p=0.0001), lesion volume (oR, 1.69; 95%CI, 1.09–4.39; p=0.014), swallowing disorders (oR, 1.151; 95% CI, 1.02-1.294; p=0.047), los (oR, 0.955; 95% CI, 0.914-0.998; p=0.0415) and bilateral lesions (oR, 2.8; 95% CI, 1.666-4.705; p=0.0001) remained significant.

our data shows that NIHss score, lesion volume, swallowing disorders, los and bilateral lesions in cerebrum can predict the requiring of Peg tube insertion in patients after stroke.

Genetic and environmental factors are important determinants of disease distribution. Several disorders associated with ataxia are known to occur more commonly in certain ethnic groups; for example, the disequilibrium syndrome in the Hutterites. The aim of this study was to determine the ethnic and geographic distribution of pediatric patients with chronic ataxia in Manitoba, Canada.

We identified 184 patients less than 17 years-of-age with chronic ataxia during 1991-2008 from multiple sources. Their diagnosis, ethnicity and place of residence were determined following a chart review.

Most patients resided in Manitoba (N=177) and the majority in Winnipeg, the provincial capital. Thirty five Aboriginal, 29 Mennonite and 11 Hutterite patients resided in Manitoba. The latter two groups were significantly overrepresented in our cohort. Ataxia telangiectasia, mitochondrial disorders, and non-progressive ataxia of unknown etiology associated with pyramidal tracts signs and developmental delay were significantly more common in Mennonite patients. Four of five patients with neuronal migration disorders associated with chronic ataxia were Aboriginal. Few isolated disorders with chronic ataxia occurred in the 11 Hutterite patients including a Joubert syndrome related disorder.

Three disorders associated with chronic ataxia were more prevalent than expected in Mennonites in Manitoba. Few rare disorders were more prevalent in the Hutterite and Aboriginal population. Further research is needed to determine the risk factors underlying these variations in prevalence within different ethnic groups. The unique risk factor profiles of each ethnic group need to be considered in health promotion endeavors.

Peripheral neuropathy is a cardinal manifestation of the autosomal recessive spastic ataxia of Charlevoix- Saguenay (ARSACS), although its type of neuromuscular involvement has not been definitely established, and magnetic resonance imaging (MRI) plays an important role in the assessment of muscle and nerve diseases. The objective of this work has been to define the patterns of muscle weakness and of abnormal muscular MRI in ARSACS.

Five patients with a molecular diagnosis of ARSACS, aged 39 to 59 years, whose electrophysiological findings were consistent with an axonal neuropathy of distal distribution superimposed on a developmental defect of myelinization, underwent neurological and MRI lower-limb examinations. Conventional FSE T1-weighted and STIR sequences were performed, looking for fatty infiltration and oedema in the musculature of the thighs, legs and feet, together with their distribution along the longitudinal axis of the muscle bellies.

On clinical examination, paralysis was apparent in foot muscles; moderate weakness, in leg musculature; and normal strength, in thigh muscles. MRI demonstrated massive fat deposition in the foot muscles and medial gastrocnemii in every case, distal fat infiltration and oedema in every leg muscle group, and preservation of thigh muscles, albeit with diffuse minimal non-specific fat infiltration. An inverse correlation between strength and degree of fat infiltration in lower-limb muscles became apparent.

The preponderance of weakness and MRI abnormalities in distal muscle groups was concordant with the presence of a length-dependent axonopathy, as described in ARSACS.

We review clinical, neuroimaging, and genetic information on six individuals with isolated sulfite oxidase deficiency (ISOD).

All patients were examined, and clinical records, biochemistry, neuroimaging, and sulfite oxidase gene (SUOX) sequencing were reviewed.

Data was available on six individuals from four nuclear families affected by ISOD. Each individual began to seize within the first week of life. neurologic development was arrested at brainstem reflexes, and severe microcephaly developed rapidly. neuroimaging within days of birth revealed hypoplasia of the cerebellum and corpus callosum and damage to the supratentorial brain looking like severe hypoxic-ischemic injury that evolved into cystic hemispheric white matter changes. Affected individuals all had elevated urinary S-sulfocysteine and normal urinary xanthine and hypoxanthine levels diagnostic of ISOD. Genetic studies confirmed SUOX mutations in four patients.

ISOD impairs systemic sulfite metabolism, and yet this genetic disease affects only the brain with damage that is commonly confused with the clinical and radiologic features of severe hypoxic-ischemic encephalopathy.

Incidental findings arising from imaging research have important implications for patient safety. Magnetic resonance imaging is widespread in multiple sclerosis (MS) studies and care, yet the prevalence rate of incidental findings in MS is poorly defined. The absence of such reports in the MS literature suggests that such findings may be deemed inappropriate for documentation in research publications, or possibly, not fully reported at all.

We sought to document incidental findings from a study designed to detect features of chronic cerebrospinal venous insufficiency (CCSVI) in MS patients and control subjects.

Magnetic resonance images were obtained as part of a prospective study conducted between October 2010 and September 2012. Patients with MS (relapsing-remitting, primary progressive, secondary progressive), clinically isolated syndromes, and neuromyelitis optica and age/sex-matched healthy controls were included. All images were reviewed by neuro-radiologists for quality-control purposes.

Magnetic resonance imaging was successfully obtained in 166 participants (110 patients, 56 controls). Incidental abnormalities (n = 33) were detected in 15% of patients (n = 17) and 27% of controls (n = 15), comprising 19% overall (n = 32).

The prevalence of incidental findings from the MS population was not significantly different from the control population. However, the overall prevalence was high and warrants a careful management strategy for future imaging studies.

There is currently no definite neuroimaging test to detect amyotrophic lateral sclerosis (ALS), which leads to significant delay in diagnosis, particularly if one takes into account the rapidity of disease evolution. Hyperintensity of the corticospinal tracts (CST) on T2 or fluid-attenuated inversion recovery (FLAIR) weighted magnetic resonance imaging (MRI) has been well described, but data on sensitivity and specificity in larger series is lacking to help guide its application to clinical care.

We analyzed clinical and MRI data from 64 patients with a definite retrospective diagnosis of ALS. In this case-control study, two experienced blinded neuroradiologists systematically assessed defined rostrocaudal segments of the intracranial course of the CST.

The overall sensitivity and specificity of conventional MRI for the diagnosis of ALS were 48% and 76% respectively. Highest specificities for CST hyperintensity were noted for the subcortical white matter (92%), centrum semiovale (88%) and medullary pyramids (92%). The lowest specificities were found for the cerebral peduncle (36%) and internal capsule (32%). We did not find a correlation with the rate of clinical progression, age of onset or the presence of upper motor neuron signs on examination.

Conventional MRI was not found to be a reliable diagnostic tool for ALS and it did not help predict clinical characteristics such as speed of evolution or prominence of upper motor neuron signs. Its main role in the setting of ALS should remain to help exclude alternative diagnostic considerations. A multimodal approach relying on newer functional and structural MRI techniques still needs to be developed and validated.

To investigate whether repeat saccadic reaction time (SRT) measurements using a portable saccadometer is useful to monitor patients with mild traumatic brain injury (mTBI).

Seven patients with newly-diagnosed mTBI and five agematched controls were prospectively recruited from an emergency Department. Saccadic eye movements, symptom self-reporting and neuropsychological tests were performed within one week of injury and again at follow-up three weeks post-injury. Control patients underwent saccade recordings at similar intervals.

Median saccade reaction times were significantly prolonged within one week post-injury in mTBI compared to controls. At follow-up assessment there was no significant between-groups difference. Changes in median SRT between the two assessments were not statistically significant. Four of the seven mTBI patients showed significantly increased SRT at follow-up; three of the mTBI patients and all controls showed no significant change. Among the three mTBI patients with persistent decreased SRT, two experienced loss of consciousness and reported the greatest symptoms, while the third was the only subject with significant decrease in neuropsychological testing scores at both assessments.

In three of seven mTBI patients, saccadic eye movements remained delayed within three weeks post-injury. These three patients also showed persistent symptoms or no improvement on neuropsychological testing. This pilot study using a portable saccadometer suggests that comparing SRT from three weeks post-injury to that within one week of injury may be useful for early detection of a subpopulation at risk of persistent disability from mTBI. This finding suggests that further investigation in a large study population is warranted.

Patients confronted with the daunting prospect of a potentially life-altering procedure with uncertain outcome demonstrate high levels of anxiety and need for information. Regardless, many patients are left unsatisfied by the amount of information received from physicians. This study sought to examine the information-seeking patterns of patients and suggest ways to optimize the communication of medical information, specifically within the context of neurosurgery.

Semi-structured interviews were conducted with 31 neurosurgical patients operated on for benign or malignant brain tumors. Interviews were transcribed and subjected to thematic analysis in NVivo10.

Three major themes relating to information-seeking by neurosurgical patients were identified: 1) almost all patients searched for information on the Internet; 2) in addition to characterizing the tumor as benign or malignant, patients sought additional information such as the location of the tumor in the brain; and 3) patients with malignant tumors were less likely to seek information online and more likely to consider alternative therapies. To improve the provision of information to neurosurgical patients, physicians can 1) offer to review imaging results with patients; 2) promote an environment open to questions; 3) provide information in a forthright manner, avoiding the use of medical jargon; and 4) consider guiding patients to reliable Internet sites and facilitating written records of consultations.

There are many ways that physicians can improve the provision of information to patients, including providing written information and physician recommended online resources, and being mindful of patient perceived time constraints and barriers to effective communication.

To report an unusual case of malignant degeneration in a ganglioneuroma of the jugular foramen post stereotactic radiosurgery as well as to systematically review the pertinent literature involving radiosurgery associated malignancies.

Ganglioneuromas are uncommon lesions of the peripheral nervous system and infrequently reported in the head and neck region. There have been no previously described cases of ganglioneuromas occurring in the jugular foramen. Malignant degeneration of these lesions is rarely described, especially in the context of radiosurgery. Radiosurgery associated malignancies overall are exceptionally rare and only a limited number of cases have been reported.

We describe a case of a ganglioneuroma occurring in the jugular foramen and mimicking the appearance of a jugular foramen schwannoma. This lesion underwent stereotactic radiosurgery and developed a delayed degeneration into a malignant nerve sheath tumor. The occurrence of ganglioneuroma in this region has not been previously described and presumed malignant degeneration is even rarer. This case highlights a previously understated risk associated with stereotactic radiation treatments. To date, 33 previous cases of stereotactic radiosurgery associated malignancies have been reported. We review the pertinent details of these cases. We caution that as follow up periods and awareness increases, reports such as this may become increasingly common.