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A literature search found no clinical trials or guidelines addressing the management of spontaneous intracranial hypotension (SIH). Based on the available literature and expert opinion, we have developed recommendations for the diagnosis and management of SIH. For typical cases, we recommend brain magnetic resonance (MR) imaging with gadolinium to confirm the diagnosis, and conservative measures for up to two weeks. If the patient remains symptomatic, up to three non-directed lumbar epidural blood patches (EBPs) should be considered. If these are unsuccessful, non-invasive MR myelography, radionuclide cisternography, MR myelography with intrathecal gadolinium, or computed tomography with myelography should be used to localize the leak. If the leak is localized, directed EPBs should be considered, followed by fibrin sealant or neurosurgery if necessary. Clinically atypical cases with normal brain MR imaging should be investigated to localize the leak. Directed EBPs can be used if the leak is localized; non-directed EBPs should be used only if there are indirect signs of SIH.
About one third of patients with epilepsy are pharmacoresistent. For a subgroup of this population, the ketogenic diet can be highly efficacious and should be considered early. This review discusses the different types of ketogenic diet, proposed mechanism of actions and its evidence for use in children and adults with both generalized and focal epilepsies where surgery is not feasible. In addition we discuss a practical approach to diet initiation, maintenance and monitoring for side effects. We also summarize the emerging evidence for the use of ketogenic diet in a broad range of neurological disorders.
A systematic review/meta-analysis of literature addressing a possible association between traumatic injury and onset of multiple sclerosis was conducted. Medline, Embase, Cochrane DSR, Ovid HealthStar, CINAHL, ISI Web of Science and Scopus were searched for analytical studies from 1950 to 2011. Two investigators independently reviewed articles for inclusion, assessing their quality using the Newcastle-Ottawa Scale. Of the 13 case-control studies included, 8 were moderate quality and 5 low; of the 3 cohort studies 2 were high and 1 moderate. Meta-analysis including moderate and low quality case-control studies produced a modest but significant odds ratio: 1.41 (95% confidence interval: 1.03, 1.93). However, when low quality studies were excluded, the resulting odds ratio was non-significant. Cohort studies produced a non-significant standardized incidence ratio of 1.00 (95% confidence interval: 0.86, 1.16). These findings support the conclusion that there is no association between traumatic injury and multiple sclerosis onset; more high quality cohort studies would help to confirm this observation.
The risk of stroke in patients with asymptomatic carotid stenosis (ACS) is now so low that it is important to have methods to identify those patients most likely to benefit from intervention, or who may require special consideration in choice of medical therapy. We studied the prediction of stroke, death or transient ischemic attacks (stroke/death/TIA) in patients with ACS by intracranial arterial stenosis, and microemboli on transcranial Doppler (TCD), and the effect of diabetes mellitus on microemboli, intracranial stenosis and risk of events.
Patients with ACS > 60% by Doppler ultrasound were recruited from the Stroke Prevention Clinic of University Hospital, London, Canada. All 339 participants underwent TCD for detection of intracranial stenosis and detection of microemboli, and carotid ultrasound to measure extracranial stenosis and total carotid plaque area. Participants were followed for three years, to determine the risk of stroke/death/TIA.
Stroke/death/TIA occurred in 38% of patients with microemboli versus 10% without (p=0.0001), and in 18% of patients with intracranial stenosis, versus 10% without (p=0.042). Diabetics were significantly more likely to have intracranial stenosis (45% vs. 29%, p =0.014), microemboli (38% vs. 10%, p <0.0001), and had significantly higher risk of stroke/death/TIA over three years (21% vs. 11% without; p=0.024). Survival free of stroke, TIA or death was significantly better without microemboli or intracranial stenosis (p<0.0001).
Diabetes, microemboli and intracranial stenosis predicted higher risk of stroke, death or TIA than did extracranial stenosis or total plaque area; diabetics may need more intensive therapy.
Stereotactic radiosurgery (SRS) is known to safely result in a high obliteration rate for small and medium sized arteriovenous malformations (AVM).
To evaluate the long-term outcome of patients treated with SRS, with special emphasis given to obliteration and toxicity rates.
We performed a review of 43 cerebral AVM patients, treated from 1998 to 2008 with a single SRS dose ranging from 21-25 Gy. Of these, 37 had a minimal follow-up of one year. Medical files were reviewed to assess patient and AVM characteristics, the SRS treatment, therapy prior to SRS, the obliteration rate and toxicities. Whenever necessary, outcome data was supplemented by telephone interviews with the patient or treating physician.
AVM size was ≥3cm in diameter in 21% of patients. Five patients (11.6%) underwent surgery prior to SRS and 31 patients (72.1%) received one or more embolizations prior to SRS. Of the patients followed with angiography ≥1 year post-SRS, 89% (33/37) had a complete obliteration of the nidus, after a median time of 24.7 months post-treatment. Embolization prior to SRS was not predictive of outcome. One patient suffered a non-fatal haemorrhage between treatment and obliteration. The rate of symptomatic radiation-induced radiological changes was 8.1%.
Our study shows both obliteration and complication rates in the upper limit of those reported in the literature. SRS seems an attractive treatment option for small AVMs. Unlike other reports, the prior use of embolization did not impact negatively on obliteration rates.
We describe the internal cerebral vein (ICV) sign, which is a hypo-opacification of the ICV on computed tomogram angiography (CTA) as a new marker of increased cerebral blood transit-time in ipsilateral internal carotid artery occlusions (ICAO).
A retrospective analysis of 153 patients with acute unilateral M1 middle cerebral artery (MCA) occlusions ± ICAOs was performed. The degree of contrast opacification of the ICV on the ipsilesional side was compared to that of the unaffected side.
Of 153 patients in our study, 135 had M1 MCA occlusions ± intra-cranial ICAO (M1±iICAO) and 18 had isolated extracranial ICAO (eICAO). In the patients with proximal M1±iICAO, 57/65 (87.1%) showed the ICV sign. Of the 8 patients without the ICV sign in this group, 6 had prominent lenticulostriate arteries arising from the non-occluded M1 segment, 1 had a recurrent artery of Huebner, and 1 had filling of distal ICA/M1 segment through prominent Circle of Willis collaterals. For the 70 patients with isolated distal M1±iICAO, 7/70 (10%) showed the ICV sign, with all 7 showing occluded lenticulostriate arteries. Of the patients with eICAO, 8/18 showed the ICV sign, all 8 with the ICV sign had poor Circle of Willis collaterals.
The ICV sign correlates well with presence of proximal M1±iICAO in patients with either occluded lenticulostriate arteries or poor Circle of Willis collaterals. In patients with eICAO, the sign correlates with reduced Circle of Willis collaterals and may be a marker of increased ipsilateral cerebral blood transit time.
Screening for cognitive impairment is recommended in patients with cerebrovascular disease. We sought to establish the incidence of cognitive impairment using the Montreal Cognitive Assessment (MoCA) in a cohort of consecutive patients attending our stroke prevention clinic (SPC), and to determine whether a subset of the MoCA could be derived for use in this busy clinical setting.
The MoCA was administered to 102 patients. Incidence of cognitive impairment was compared to presenting complaint and final diagnosis. extent of cerebral white matter changes (WMC) was rated using the Age Related White Matter Changes (ARWMC) scale in 80 patients who underwent neuroimaging. A subset of the three most predictive test elements of the MoCA was derived using regression analysis.
63.7% of patients scored <26/30 on the MoCA, in keeping with cognitive impairment. This was unrelated to the final diagnosis or extent of WMC, although a trend for lower MoCA scores was observed in older patients. A mini-MoCA subscore combining the clock drawing test, five-word delayed recall, and abstraction was highly correlated with the final MoCA score (R=0.901). A score of <7/10 using this 10-point mini-MoCA identified cognitive impairment as defined by the MoCA with a sensitivity of 98.5%, and a specificity of 77.6%.
Two-thirds of SPC patients demonstrated evidence for cognitive impairment, irrespective of their final diagnosis or the presence of WMC. A mini-MoCA comprised of the clock drawing test, five-word delayed recall, and abstraction represents a potential alternative to the full MoCA in this population.
To evaluate the prevalence of nonlesional focal epilepsy in an adult epilepsy clinic and its refractoriness to antiepileptic drug therapy.
Focal epilepsy is frequently, but not always, associated with structural epileptogenic lesions identifiable on magnetic resonance imaging (MRI).
We analyzed the data from all patients evaluated at an adult epilepsy clinic from January 2002 to December 2011. Clinical and paraclinical findings were used to diagnose focal epilepsy. Magnetic resonance imaging were reviewed and classified as normal, with an epileptogenic lesion, or with a lesion of unclear epileptogenicity. Epileptogenic lesions were further categorized as tumours, vascular malformations, gliosis (including hippocampal atrophy/sclerosis), and malformations of cortical development. Our study group included patients with no lesions on MRI. Pharmacoresistance of patients with nonlesional focal epilepsy was assessed using the ILAE and Perucca's criterias.
Out of 1521 patients evaluated (mean age 44 years; range 14-93 years), 843 had focal epilepsy. Magnetic resonance imaging data, available for 806 (96%) subjects, showed epileptogenic lesions in 65%, no obvious epileptogenic lesions in 31% and lesions of unclear epileptogenicity in 4%. Magnetic resonance imaging-identified lesions included gliosis due to an acquired insult (52% including 17% of hippocampal atrophy or sclerosis), tumours (29%), vascular malformations (16%) and malformations of cortical development (10%). Fifty-two percent of nonlesional focal epileptic patients were drug-refractory.
In a tertiary epilepsy clinic, close to a third of patients with focal epilepsy were found to be nonlesional, half of which were drug-resistant.
It is estimated that some hundreds of Canadian patients with multiple sclerosis (MS) have journeyed abroad to avail themselves of ‘liberation therapy’ (venoplasty) following the initial report by Zamboni et al in 2009. That study also led to public pressure upon Departments of Health in Canadian Provinces to fund the procedure. The present study was done in order to advise the Government of Newfoundland and Labrador as to whether or not it should do so.
We conducted an observational study of 30 MS subjects who had submitted to venoplasty, using objective, semi-objective and subjective measures.
Significant subjective improvement was reported by half of the subjects at three months, although the degree of perceived improvement was less at 12 months. The objective and semi-objective tests employed did not indicate improvement in any area over the one-year follow-up period. Seven of the 29 subjects in whom CT venography was performed at the end of the study year were found to have uni- or bilateral occlusion or >50% stenosis of at least one cervical draining vein, but they showed no deterioration in their clinical status compared to those in whom no venous occlusion nor stenosis was found.
No objective improvement was found at one year in thirty MS subjects who had undergone venoplasty, although many reported a degree of subjective benefit.
To compare neurologist and patient perceptions of multiple sclerosis (MS)-related health status.
MS patients (n=99) were recruited from six sites in Canada. Following a consultation with their neurologist, patients estimated their relapse frequency, rated their general health and quality of life (QoL), reviewed descriptions of eight health domains and selected the three most important, and completed a utility assessment using the standard gamble (SG). Concurrently, neurologists independently used the same instruments to rate their patients' health status. Assessments were compared on the basis of paired mean values of both groups and the degree of exact agreement quantified by intraclass coefficient (ICC) and kappa analyses, which yield values of 1.0 with 100% agreement.
There were significant differences (p<0.001) between patient and neurologist ratings for relapses in the last year (0.86 vs. 0.4, respectively), QoL (61.2 vs. 69.7 (maximum score = 100) and utility (0.864 vs. 0.971); ICC analysis revealed moderate to poor levels of agreement (0.56 for QoL to 0.03 for SG). There was little concordance in identification of important health domain and the only significant associations were in bodily pain and social functioning (kappa statistic = 0.24, p = 0.026 for both). Neurologists identified physical functioning domains as important, while patients placed more emphasis on mental health domains.
Discrepancies between neurologist and patient perceptions of MS were observed. The study identifies a need to educate neurologists on the recognition of MS health domains that are important in the definition of patient QoL.
Electromyography (EMG) for suspected cervical or lumbosacral root compression is often negative, producing expense and physical discomfort that could have been avoided. To improve patient selection for testing, we sought to identify clinical features that would accurately predict presence of radiculopathy on EMG.
Adult patients consecutively evaluated for suspected cervical or lumbosacral root compression at an academic clinical neurophysiology laboratory were prospectively enrolled. Presence of clinical features suggesting root disease (neck or back pain, dermatomal pain or numbness, myotomal weakness, segmental reflex loss, and straight leg-raising) was recorded prior to testing. EMG examination to confirm root compression was conducted per standard protocols. Analysis was based on computation of sensitivity, specificity, predictive values, and accuracy.
A total of 200 patients (55% male; mean age 46.4 years; 38% suspected of cervical and 62% of lumbosacral disease) were included. EMG evidence of root disease was detected in 31% of cervical and 62% of lumbosacral referrals. Dermatomal pain was the most sensitive, and segmental reflex loss and myotomal weakness the most specific individual predictors of root disease. Combined presence of dermatomal pain or numbness with segmental reflex loss and myotomal weakness approached specificities of 78% (lumbosacral disease) and 99% (cervical disease). In all cases, myotomal weakness was the most accurate predictor of root disease.
The diverse symptoms and signs of cervical and lumbosacral root compression predict a positive electrodiagnosis of radiculopathy with varying degrees of accuracy, and may be used to guide patient selection for EMG testing.
Ultrasonic assessment of optic nerve sheath diameter (ONSD) as a non-invasive measure of intracranial pressure (ICP) has been evaluated in the literature as a potential valid technique for rapid ICP estimation in the absence of invasive intracranial monitoring. The technique can be challenging to perform and little literature exists surrounding intra-operator variability.
In this study we describe the creation of a novel model of ONSD to be utilized in ultrasound training of this technique. We demonstrate the realistic ultrasonographic images created utilizing this novel model.
We designed ocular models composed of gelatin spheres and variable three dimensional printed cylinders, which simulate the globe of the eye and variable ONSD's respectively. These models were suspended in a gelatin background and ultrasound of the ONSD was conducted using standard techniques described in the literature.
This model produces clear and accurate representation of ONSD that closely mimics in vivo images. It is affordable and easy to produce in large quantities, portending its use in an educational environment.
Utilizing the standard linear array ultrasound probe for ONSD measurements in our model provided realistic images comparable to in vivo. This provides an affordable and exciting means to test intra- and inter- operator variability in a standardized environment. Knowing this, we can further apply this novel model of ONSD to ultrasound teaching and training courses with confidence in its ability and the technique's ability to produce consistent results.
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.
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.