Two cases with postural axial tremor: Consider a genetic origin.

We present two cases with postural axial tremor predominantly involving the head, trunk, and shoulders. In the first patient, the postural tremor occurred in multiple attacks a day lasting approximately 10 min. The second patient developed a progressive tremor of his head and arms, worsened during sitting and standing. Electrophysiological supported the postural axial tremor in both patients with a varying 3-10 Hz tremor frequency between different muscles and within the same muscles at different times. Postural axial tremor is a rare and complex movement disorder. The majority of cases are caused by acquired cerebellar pathology. However, isolated cases with underlying genetic disorders are described in literature. Here, we illustrate how to differentiate paroxysmal axial tremor from other axial hyperkinetic movement disorders and extend the genetic heterogeneity of this intriguing movement disorder phenotype.

Reliability of the intracerebral hemorrhage score for predicting outcome in patients with intracerebral hemorrhage using oral anticoagulants.

BACKGROUND AND PURPOSE: The intracerebral hemorrhage (ICH) score is the most widely used and validated prognostic model for estimating 30-day mortality in ICH. However, the score was developed and validated in an ICH population probably not using oral anticoagulants (OACs). The aim of this study was to determine the performance of the ICH score for predicting the 30-day mortality rate in the full range of ICH scores in patients using OACs. METHODS: Data from admitted patients with ICH were collected retrospectively in two Dutch comprehensive stroke centers. The validity of the ICH score was evaluated by assessing both discrimination and calibration in OAC and OAC-naive patient groups. RESULTS: A total of 1752 patients were included of which 462 (26%) patients were on OAC. The 30-day mortality was 54% for the OAC cohort and 34% for the OAC-naive cohort. The 30-day mortality was higher in the OAC cohort for ICH score 1 (33% vs. 12.5%; odds ratio, 3.4; 95% confidence intervals, 1.1-10.4) and ICH score 2 (53% vs. 26%; odds ratio, 3.2; 95% confidence intervals, 1.2-8.2) compared with the predicted mortality rate of the original ICH score. Overall, the discriminative ability of the ICH score was equally good in both cohorts (area under the curve 0.83 vs. 0.87, respectively). CONCLUSIONS: The ICH score underestimated the 30-day mortality rate for lower ICH scores in OAC-ICH. When estimating the prognosis of ICH in patients using OAC, this underestimation of mortality must be taken into account.

European Academy of Neurology and European Stroke Organization consensus statement and practical guidance for pre-hospital management of stroke.

BACKGROUND AND PURPOSE: The reduction of delay between onset and hospital arrival and adequate pre-hospital care of persons with acute stroke are important for improving the chances of a favourable outcome. The objective is to recommend evidence-based practices for the management of patients with suspected stroke in the pre-hospital setting. METHODS: The GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology was used to define the key clinical questions. An expert panel then reviewed the literature, established the quality of the evidence, and made recommendations. RESULTS: Despite very low quality of evidence educational campaigns to increase the awareness of immediately calling emergency medical services are strongly recommended. Moderate quality evidence was found to support strong recommendations for the training of emergency medical personnel in recognizing the symptoms of a stroke and in implementation of a pre-hospital 'code stroke' including highest priority dispatch, pre-hospital notification and rapid transfer to the closest 'stroke-ready' centre. Insufficient evidence was found to recommend a pre-hospital stroke scale to predict large vessel occlusion. Despite the very low quality of evidence, restoring normoxia in patients with hypoxia is recommended, and blood pressure lowering drugs and treating hyperglycaemia with insulin should be avoided. There is insufficient evidence to recommend the routine use of mobile stroke units delivering intravenous thrombolysis at the scene. Because only feasibility studies have been reported, no recommendations can be provided for pre-hospital telemedicine during ambulance transport. CONCLUSIONS: These guidelines inform on the contemporary approach to patients with suspected stroke in the pre-hospital setting. Further studies, preferably randomized controlled trials, are required to examine the impact of particular interventions on quality parameters and outcome.

Adverse events associated with the use of cervical spine manipulation or mobilization and patient characteristics: A systematic review.

Cervical spinal manipulation (CSM) and cervical mobilization are frequently used in patients with neck pain and headache. Pre-manipulative cervical instability and arterial integrity tests appear to be unreliable in identifying patients at risk for adverse events. It would be valuable if patients at risk could be identified by specific characteristics during the preliminary screening. Objective was to identify characteristics of 1) patients, 2) practitioners, 3) treatment process and 4) adverse events (AE) occurring after CSM or cervical mobilization. A systematic search was performed in PubMed, Embase, CINAHL, Web-of-science, AMED, and ICL (Index Chiropractic Literature) up to December 2014. Of the initial 1043 studies, 144 studies were included, containing 227 cases. 117 cases described male patients with a mean age of 45 (SD 12) and a mean age of 39 (SD 11) for females. Most patients were treated by chiropractors (66%). Manipulation was reported in 95% of the cases, and neck pain was the most frequent indication. Cervical arterial dissection (CAD) was reported in 57% (P = 0.21) of the cases and 45.8% had immediate onset symptoms. The overall distribution of gender for CAD is 55% (n = 71) for female and therefore opposite of the total AE. Patient characteristics were described poorly. No clear patient profile, related to the risk of AE after CSM, could be extracted. However, women seem more at risk for CAD. There seems to be under-reporting of cases. Further research should focus on a more uniform and complete registration of AE using standardized terminology.

Recurrence Rates After Surgical or Endovascular Treatment of Spinal Dural Arteriovenous Fistulas: A Meta-analysis.

BACKGROUND: There is an increasing tendency to treat spinal dural arteriovenous fistulas (SDAVFs) endovascularly despite the lack of clear evidence favoring embolization over surgery. OBJECTIVE: To compare the initial failure and recurrence rates of primary treatment of SDAVFs by surgery and endovascular techniques. METHODS: A meta-analysis using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) standard was performed. All the English literature from 2004 onward was evaluated. From each article that compared the 2 treatment modalities, the odds ratio (OR) was calculated. Combined ORs were calculated with Review Manager 5.3 of The Cochrane Collaboration. RESULTS: A total of 35 studies harboring 1112 patients were assessed. Initial definitive fistula occlusion was observed in 588 of 609 surgical patients (96.6%; 95% confidence interval [CI], 94.8-97.8) vs 363 of 503 endovascularly treated patients (72.2%; 95% CI, 68.1-75.9; P < .001). The combined OR from 18 studies that assessed both treatment modalities (730 patients) was 6.15 (95% CI, 3.45-11.0) in favor of surgical treatment. Late recurrence (13 studies, 480 patients) revealed an OR of 3.15 (95% CI, 1.66-5.96; P < .001) in favor of surgery. In a subgroup, recurrence was reported in 10 of 22 patients (45%) treated with Onyx vs 8 of 35 (23%) treated with n-butyle-2-cyanoacrylate (OR, 2.51; 95% CI, 0.75-8.37; P = .13). CONCLUSION: Although hampered by inclusion of poor quality studies, this meta-analysis shows a definite advantage of primary surgical treatment of SDAVF over endovascular treatment in initial failure rate and late recurrences. The often-used argument that endovascular techniques have improved and therefore outweigh surgery is not supported by this meta-analysis.

Intracranial artery dissection.

The aim of this narrative review is to evaluate the pathogenesis, clinical features, diagnosis, treatment and prognosis of intracranial artery dissection (IAD). IAD is a rare and often unrecognized cause of stroke or subarachnoid haemorrhage (SAH), especially in young adults. Two types of IAD can be identified: a subintimal or subadventitial dissection. It is suggested that a subintimal dissection results in luminal stenosis, thromboembolism and subsequently cerebral ischaemia, whilst a subadventitial IAD could result in the formation of a pseudo-aneurysm and compression on brainstem or cranial nerves. Rupture of such a dissecting aneurysm causes SAH. The exact cause of IAD remains unknown but several factors are associated with its development. Diagnosis is based on clinical presentation and specific features seen on multimodal neuroimaging. The management of IAD depends on the clinical presentation. In the case of cerebral ischaemia, anticoagulants or antiplatelet agents are used, whilst in the case of SAH endovascular treatment is primarily advocated. Prognosis depends on clinical presentation. Presentation with SAH has a worse prognosis.

The sNIHSS-4 predicts outcome in right and left anterior circulation strokes.

BACKGROUND AND PURPOSE: Stroke severity measured by the National Institutes of Health Stroke Scale (NIHSS) is a strong predictor of functional outcome. A short version, the sNIHSS-5, scoring only strength in right and left leg, gaze, visual fields and language, was developed for use in the prehospital setting. Because scoring both legs in anterior circulation strokes is not contributive, we assessed the value of a 4-item score (the sNIHSS-4), omitting the item 'strength in the unaffected leg', in predicting stroke outcome. METHODS: The study population consisted of anterior circulation ischemic stroke patients who participated in the LUB-INT-9 trial. We included all patients in whom the following data were available: NIHSS within 6h after stroke onset and daily between days 2 and 5, and the 12-week modified Rankin Scale (mRS) score. Poor outcome was defined as a mRS score>3. RESULTS: There was an excellent correlation between the NIHSS and sNIHSS-4 at all time points for both left and right-sided strokes. Scores at day 2 were a good predictor of poor outcome. Cutoff scores for NIHSS and sNIHSS-4 at day 2 were 15 and 5 in left hemispheric strokes, and 12 and 4 in right hemispheric strokes. CONCLUSION: The sNIHSS-4 is as good as the NIHSS at predicting stroke outcome in both right and left anterior circulation strokes.

Pre-stroke use of beta-blockers does not affect ischaemic stroke severity and outcome.

BACKGROUND AND PURPOSE: It is unclear whether pre-stroke beta-blockers use may influence stroke outcome. This study evaluates the independent effect of pre-stroke use of beta-blockers on ischaemic stroke severity and 3 months functional outcome. METHODS: Pre-stroke use of beta-blockers was investigated in 1375 ischaemic stroke patients who had been included in two placebo-controlled trials with lubeluzole. Stroke severity was assessed by either the National Institute of Health Stroke Scale (NIHSS) or the European Stroke Scale (ESS). A modified Rankin scale (mRS) score of >3 at 3 months was used as measure for the poor functional outcome. RESULTS: Two hundred and sixty four patients were on beta-blockers prior to stroke onset, and 105 patients continued treatment after their stroke. Pretreatment with beta-blockers did not influence baseline stroke severity. There was no difference in stroke severity between nonusers and those on either a selective beta(1)-blocker or a non-selective beta-blocker. The likelihood of a poor outcome at 3 months was not influenced by pre-stroke beta-blocker use or beta-blocker use before and continued after stroke onset. CONCLUSIONS: Pre-stroke use of beta-blockers does not appear to influence stroke severity and functional outcome at 3 months.

Treatment of central nervous system tuberculosis infections and neurological complications of tuberculosis treatment.

Tuberculosis (TB) with central nervous system (CNS) manifestation is a form of TB with a high mortality and morbidity. Tuberculous meningitis (TM) is the most common form of CNS-TB. Although diagnosis of CNS-TB can be challenging, early treatment of CNS-TB is related to a better outcome. If CNS-TB is suspected, even though the clinical picture is not specific, it should be immediately treated. For the treatment of CNS-TB, knowledge of the penetration across the blood-brain barrier of the various antituberculosis agents used in TB treatment is important. These will be described here in order to serve as a guide in choosing a treatment for CNS-TB. Corticosteroids have an evidence-based value in the treatment of TM and so are recommended. As for thalidomide use in CNS-TB, sound evidence is still lacking. We will also include a description of the adverse neurotoxic effects of the various other agents including their psychiatric, ototoxic and ophthalmic adverse effects.

Effect of selective serotonin re-uptake inhibitors (SSRIs) on functional outcome in patients with acute ischemic stroke treated with tPA.

BACKGROUND: Selective serotonin re-uptake inhibitors (SSRIs) may have therapeutic potential in the treatment of ischemic stroke by effects on neuronal cell survival and the plasticity of brain processes. In the present study, we investigated whether prior treatment with a SSRI is associated with more favorable functional outcome in a cohort of patients with acute ischemic stroke treated with tissue plasminogen activator (tPA). METHODS: In a prospective observational cohort study of 476 acute ischemic stroke patients treated with tPA we investigated the relationship between prior SSRI treatment and functional outcome at 3 months. Ischemic stroke subtypes were defined according to the Oxfordshire Community Stroke Project Classification. Favorable outcome was defined as a modified Rankin Scale score

tPA treatment for acute ischaemic stroke in patients with leukoaraiosis.

BACKGROUND AND PURPOSE: Whether leukoaraiosis on baseline CT is associated with an increased risk of symptomatic intracerebral haemorrhage (sICH) or poor outcome following tissue plasminogen activator (tPA) treatment for acute ischaemic stroke is still a matter of debate. OBJECTIVE: To investigate the relationship between the presence and severity of leukoaraiosis on baseline CT and the risk of sICH and functional outcome after tPA treatment for acute ischaemic stroke. METHODS: A single-center observational cohort study with a retrospective analysis on consecutive patients with ischaemic stroke treated with tPA in the period 2002-2008. Outcome measures were the occurrence of sICH and functional outcome at 3 months. RESULTS: Of the 400 patients, 24% had leukoaraiosis on their baseline CT. Eleven patients (11%) with leukoaraiosis versus thirteen (4%) patients without leukoaraiosis had a sICH [odds ratio (OR) 2.85 95%-CI 1.23-6.60, P = 0.02]. Multivariate analysis showed a non-significant trend towards an association of leukoaraiosis and sICH (OR 1.9, 95%-CI 0.78-4.68, P = 0.16). Leukoaraiosis was independently associated with poor functional outcome (OR 2.39, 95%-CI 1.21-4.72, P = 0.01). No difference was observed in the outcome measures amongst patients with moderate or severe leukoaraiosis. CONCLUSION: Our study demonstrates that patients treated with tPA and leukoaraiosis on their baseline CT are at greater risk of sICH and have a worse functional outcome compared to patients without leukoaraiosis. It is important to note that these results should not lead to exclusion of patients with leukoaraiosis for tPA treatment.

Statin use and functional outcome after tissue plasminogen activator treatment in acute ischaemic stroke.

BACKGROUND: Preliminary findings suggest that statins may have a neuroprotective effect in patients with acute ischaemic stroke. This study investigated whether patients on prior statin therapy and treated with tissue plasminogen activator (tPA) for acute ischaemic stroke have a better functional outcome than statin-naïve patients. METHODS: In a prospective observational cohort study of 476 acute ischaemic stroke patients treated with tPA we investigated the relationship between prior statin use and functional outcome at 3 months, the occurrence of symptomatic intracerebral haemorrhage (SICH) and early in-hospital mortality. Ischaemic stroke subtypes were defined according to the TOAST classification. Favourable outcome was defined as a modified Rankin Scale score < or =2. RESULTS: Of the 476 patients included, 98 (20.6%) used a statin at stroke presentation. In the entire cohort, 45.6% of patients had a favourable outcome with no difference between patients with or without statin therapy (45.9 vs. 45.5%, p = 0.94). In the multivariable analysis, statin use was not associated with favourable outcome (OR = 1.1, 95% CI = 0.6-1.9, p = 0.87). In none of the different stroke subtype groups was statin use associated with favourable outcome. Finally, statin use was not an independent risk factor of SICH or of early in-hospital mortality. CONCLUSION: Prior statin therapy in patients with acute ischaemic stroke treated with tPA is not associated with a more favourable outcome, and this is independent of stroke subtype.

Thrombolysis for acute ischemic stroke.

In the last decennium, thrombolytic therapy has changed the management of acute ischemic stroke. Randomized clinical studies have demonstrated that intravenous thrombolysis with tissue plasminogen activator improves functional outcomes. Recently the time window for intravenous thrombolysis has been extended from 3 to 4.5 hours after stroke onset, which will allow more stroke patients to benefit from this treatment. Currently several studies are investigating how to improve recanalization rates of thrombolytic therapy. In this review several aspects of intravenous and intra-arterial thrombolysis are discussed.

Pharmacokinetics of moxifloxacin in cerebrospinal fluid and plasma in patients with tuberculous meningitis.

Moxifloxacin cerebrospinal fluid (CSF) penetration was evaluated by obtaining full plasma and CSF time concentration curves for 4 patients with tuberculous meningitis. The geometric mean ratio of the areas under the curve for CSF to plasma were 0.82 (range, 0.70-0.94) at 400 mg once per day and 0.71 (0.58-0.84) at 800 mg once per day.

Hyperdense middle cerebral artery sign and outcome after intravenous thrombolysis for acute ischemic stroke.

BACKGROUND: The presence of a hyperdense middle cerebral artery sign (HMCAS) on baseline brain CT is associated with poor clinical outcome in stroke patients treated with intravenous recombinant tissue plasminogen activator (tPA). It remains uncertain whether the presence of HMCAS is associated with acute neurological deterioration after tPA treatment. OBJECTIVE: To evaluate the effect of HMCAS in routinely intravenous tPA-treated patients with anterior circulation stroke on acute neurological deterioration, the 3-month functional outcome and the occurrence of symptomatic ICH. METHODS: We analyzed data from a single stroke unit registry of 384 consecutive patients with anterior circulation infarction, treated with intravenous tPA. Logistic regression models were used to assess if HMCAS was independently associated with predefined outcome definitions. RESULTS: We found a HMCAS in 104 patients (27%). The HMCAS was related to the risk of early neurological deterioration (p=0.04) and poor functional outcome (p<0.001) on univariate analysis. The incidence of symptomatic ICH was not significantly different between patients with and without HMCAS (7% versus 6%, p=0.81). In the multivariable analysis, the presence of HMCAS was significantly associated with a poor outcome (p=0.004). CONCLUSIONS: The HMCAS is associated with early neurological deterioration and poor functional outcome, but not with symptomatic ICH.