Miguel Viana-Baptista

Decompressive Surgery in Cerebrovenous Thrombosis A Multicenter Registry and a Systematic Review of Individual Patient Data

Background and Purpose— Herniation attributable to unilateral mass effect is the major cause of death in cerebral venous thrombosis (CVT). Decompressive surgery may be lifesaving in these patients. Methods— Retrospective registry of cases of acute CVT treated with decompressive surgery (craniectomy or hematoma evacuation) in 22 centers and systematic review of all published cases of CVT treated with decompressive surgery. The primary outcome was the score on the modified Rankin Scale (mRS) score at last follow-up, dichotomized between favorable (mRS score, 0–4) and unfavorable outcome (mRS score, 5 or death). Secondary outcomes were complete recovery (mRS score 0–1), independence (mRS score, 0–2), severe dependence (mRS score, 4–5), and death at last available follow-up. Results— Sixty-nine patients were included and 38 were from the registry. Decompressive craniectomy was performed in 45 patients, hematoma evacuation was performed in 7, and both interventions were performed in 17 patients. At last follow-up (median, 12 months) only 12 (17.4%) had un unfavorable outcome. Twenty-six (37.7%) had mRS score 0 to 1, 39 (56.5%) had mRS score 0 to 2, 4 (5.8%) were alive with mRS score 4 to 5, and 11 (15.9%) patients died. Three of the 9 patients with bilateral fixed pupils recovered completely. Comatose patients were less likely to be independent (mRS score 0–2) than noncomatose patients (45% versus 84%; P=0.003). Patients with bilateral lesions were more likely to have unfavorable outcomes (50% versus 11%; P=0.004) and to die (42% versus 11%; P=0.025). Conclusions— In CVT patients with large parenchymal lesions causing herniation, decompressive surgery was lifesaving and often resulted in good functional outcome, even in patients with severe clinical conditions.

Mitochondrial haplogroup H1 is protective for ischemic stroke in Portuguese patients

BackgroundThe genetic contribution to stroke is well established but it has proven difficult to identify the genes and the disease-associated alleles mediating this effect, possibly because only nuclear genes have been intensely investigated so far. Mitochondrial DNA (mtDNA) has been implicated in several disorders having stroke as one of its clinical manifestations. The aim of this case-control study was to assess the contribution of mtDNA polymorphisms and haplogroups to ischemic stroke risk.MethodsWe genotyped 19 mtDNA single nucleotide polymorphisms (SNPs) defining the major European haplogroups in 534 ischemic stroke patients and 499 controls collected in Portugal, and tested their allelic and haplogroup association with ischemic stroke risk.ResultsHaplogroup H1 was found to be significantly less frequent in stroke patients than in controls (OR = 0.61, 95% CI = 0.45–0.83, p = 0.001), when comparing each clade against all other haplogroups pooled together. Conversely, the pre-HV/HV and U mtDNA lineages emerge as potential genetic factors conferring risk for stroke (OR = 3.14, 95% CI = 1.41–7.01, p = 0.003, and OR = 2.87, 95% CI = 1.13–7.28, p = 0.021, respectively). SNPs m.3010G>A, m.7028C>T and m.11719G>A strongly influence ischemic stroke risk, their allelic state in haplogroup H1 corroborating its protective effect.ConclusionOur data suggests that mitochondrial haplogroup H1 has an impact on ischemic stroke risk in a Portuguese sample.

Postpartum Cerebral Angiopathy: Vasospasm, Vasculitis or Both?

Introduction Postpartum cerebral angiopathy (PCA) or Call-Fleming syndrome is a rare reversible cerebral arterial vasoconstriction syndrome occurring shortly after a normal pregnancy [1–3]. Clinical presentation includes sudden-onset headaches, seizures and focal neurological deficits. Outcome is usually good. Vasospasm is thought to be the underlying phenomenon. We report a case of severe PCA with pathologic features of small vessel vasculitis.

Cognitive function correlates with frontal white matter apparent diffusion coefficients in patients with leukoaraiosis.

Background and aimsDiffusion weighted imaging (DWI) displays a high sensitivity to white matter changes, even in areas where no lesions are visible. Correlation with vascular risk factors and cognitive dysfunction seems to be feasible using this technique. We aimed to test relations between age, blood pressure and cognitive function,with lesion load and average Apparent Diffusion Coefficient (ADC) values in lesioned (LWM) and in normal appearing white matter (NAWM), in patients with age related white matter lesions (ARWML).MethodsSubjects were 29 patients (mean age 72.6 ± 5.2 years) with different severity of ARWML on MRI and no (or mild) disability assessed by the Instrumental Activities of Daily Living Scale. Imaging lesion load was quantified in bilateral frontal, temporal, parieto-occipital, basal ganglia and infratentorial regions, using a simple visual rating scale; ADC was measured bilaterally in Regions of Interest in parieto-occipital and frontal NAWM, and in frontal periventricular LWM. Neuropsychological examination consisted of Raven Colored Progressive Matrices, Rey’s Complex Figure, Digit Canceling. Symbol digit Substitution, Inverse Digit Repetition and Verbal Fluency tests.ResultsVisual scales scores and ADC were significantly higher in frontal and parieto-occipital regions. Both were significantly correlated to age and blood pressure, in frontal (visual scale scores and ADC) and parieto-occipital regions (ADC). Attention skills were negatively correlated to ADC in LWM and NAWM in frontal regions and with frontal region visual scale scores.ConclusionOur findings suggest that severity of white matter ischemic changes is correlated with worse cognitive function, as well as advanced age and higher blood pressure.A higher vulnerability of frontal white matter to vascular disease seems to play an important role in executive dysfunction, mainly determined by impairment of attentional skills.DWI results suggest this could be true even for NAWM.

Stroke and Fabry disease.

Fabry disease (FD) is a rare inherited disorder of the metabolism, associated with renal, cardiac, and cerebrovascular complications. Ischemic and hemorrhagic stroke in FD present with a similar proportion to that observed in the general population, but usually at an early age. Ischemic stroke may result from cardiac embolism, large and small vessel disease, while hemorrhagic stroke is usually attributed to hypertension. Deposition of glycosphingolipids in endothelial cells results in a specific FD vasculopathy that contributes to the different vascular phenotypes. Neuroimaging features of cerebrovascular involvement in FD include white matter lesions, dolichoectasia, and the “pulvinar sign”, a T1 MRI hyperintensity of the posterior thalamus. The role of enzymatic replacement therapy in the prevention of stroke remains to be established, but its utilization should be considered in FD stroke patients, for prevention of renal and cardiac complications, together with general prevention measures. Enzymatic replacement therapy increased our awareness of FD, underlining the importance of incomplete phenotypes in specific settings such as stroke. An overview of studies on the prevalence of FD in stroke patients is presented. Available data suggest that prevalence of FD is similar to some of the rare causes of stroke usually considered, and that classic features of the disease may be absent or more subtle. Moreover, FD should be considered in both cryptogenic and all-cause stroke. The role of FD in stroke on a multifactorial basis and the identification of a putative “stroke variant” are questions that need to be further elucidated in future studies.

Orolingual Angiodema Associated with Alteplase Treatment of Acute Stroke: A Reappraisal

BACKGROUND Orolingual angioedema has been increasingly recognized as a potentially life-threatening complication associated with alteplase treatment of stroke. Concomitant treatment with an angiotension converting enzyme inhibitor (ACEi) and localization of infarction in the territory of middle cerebral artery seem to be associated with a higher risk of this complication. METHODS We report the cases of orolingual angioedema among the patients undergoing alteplase treatment in our Stroke Unit. Additionally, we reviewed the literature to evaluate the pathophysiology, clinical characteristics, and treatment options. RESULTS In our Stroke Unit, among 236 patients given alteplase for acute stroke, 8 patients (3.4%) developed angioedema. The clinical picture varied from localized labial edema to extensive lingual edema with respiratory distress but in all cases it gradually resolved with symptomatic treatment. Seven patients had a hemispheric stroke (4 with lateralized angioedema, contralateral to the ischemic lesion), whereas the other 1 patient had a right superior cerebellar artery stroke (with lateralized angioedema, ipsilateral to the ischemic lesion). The National Institutes of Health Stroke Scale score at admission ranged from 6 to 24 (median 12.5). Five patients were taking an ACEi. Our results are similar to previously published data. In the literature, it appears that orolingual angioedema occurs in .2-5.1% of all stroke patients receiving Alteplase treatment. CONCLUSIONS Orolingual angioedema is a potential complication of which treating physicians in stroke units need to be aware, even in those cases without history of ACEi treatment and without infarction in the territory of the middle cerebral artery. All patients who receive alteplase treatment should be monitored carefully.

Oral direct thrombin inhibitor as an alternative in the management of cerebral venous thrombosis: a series of 15 patients.

Background Cerebral vein thrombosis is a rare cause of stroke with significant risk of death and long-term dependency. Anticoagulation has been associated with better long-term prognosis, and vitamin K antagonists are usually prescribed in this setting. Aim The aim of this study was to present a series of 15 cerebral vein thrombosis patients treated with dabigatran. Methods Retrospective study of clinical, imaging, and follow-up characterization of all patients admitted with cerebral vein thrombosis and treated with dabigatran in a tertiary neurology department between June 2011 and December 2013 was conducted. Complications and adverse effects were recorded. Modified Rankin Scale was used to assess clinical severity; excellent outcome was defined as modified Rankin Scale at six-months of 0 to 1. Recanalization was assessed with an angiographic method (computer tomography, magnetic resonance imaging, or digital subtraction angiography). Results Eighteen patients were admitted for cerebral vein thrombosis. Dabigatran was started in 11 patients, and warfarin was started in 7. Four patients on warfarin were switched to dabigatran because of adverse effects at 0·5, 1, 3·5, and 4 months. A total of 15 patients were treated with dabigatran with median follow-up time of 19 months. Excellent outcome was observed in 87% of patients and recanalization in 80%. Conclusions We report the largest series of cerebral vein thrombosis patients treated with dabigatran. Clinical outcome was excellent in most patients and not different from other studies. Dabigatran could possibly be considered an alternative to warfarin; nevertheless, further prospective assessment with randomized controlled studies is warranted.

Motor dysfunction correlates with frontal white matter ischemic changes in patients with leukoaraiosis.

Objectives. To test the relation between white matter lesions (WML) location and physical performance, in aged patients. Methods. Subjects: 29 patients (17 males), aged >65 (mean age 72.6 ± 5.2), with leukoaraiosis. WML was quantified with a visual scale; Apparent Diffusion Coefficient (ADC) was measured bilaterally in frontal periventricular lesioned white matter and frontal and parieto-occipital normal appearing white matter (NAWM). Motor performance was studied using the Short Physical Performance Battery (SPPB), single leg stand time, finger tapping and grooved pegboard tests (GPT). Results. There were significant correlations between the frontal region visual scale scores and SPPB chair stands (r = −0.379; P = .039) and Grooved Pegboard (r = 0.393; P = .032); frontal NAWM ADC values and SPPB standing balance (r = −0.450; P = .014) and SPPB 4 meter walk (r = −0.379; P = .043). Conclusion. Frontal WML are negatively related to motor performance in patients with leukoaraiosis. DWI results suggest that this may be true even for NAWM.

Embolic stroke of unknown source (ESUS) in young patients

The Cryptogenic Stroke International Working Group defined a new subgroup of cryptogenic stroke – embolic stroke of undetermined source (ESUS), based on clinical and imaging findings. Minor cardioembolic and atheroembolic sources were putatively implicated, and it was hypothesized that anticoagulant therapy might be warranted (1). We aimed to assess the role of minor embolic sources in ESUS in young stroke patients by comparing the prevalence of minor embolic sources in patients with ESUS and in patients with stroke of determined origin. Etiology was defined according to the TOAST criteria. ESUS and minor cardioembolic/atheroembolic stroke were defined according to the proposed criteria (1). We studied 100 consecutive ischemic stroke patients (see Table 1) aged between 18 and 55 years admitted in a Neurology department between January 2010 and August 2014 (mean age 46 years, range 22–55; 56% male; mean NIHSS at admission 4). The overall prevalence of minor cardioembolic sources was 53%, and of atheroembolic sources was 32%. Cryptogenic stroke was diagnosed in 51 patients. Among these, 42 patients were ESUS (mean age 45 years, range 26–55; 50% male; mean NIHSS at admission 4). The ESUS group neither have a higher prevalence of minor cardioembolic, 50% (21/42) vs. 50% (27/54), nor a higher prevalence of minor atheroembolic, 38·1% (16/42) vs. 30·8% (15/47), P = 0·641, sources, when compared with all strokes with a determined cause other than cardioembolic and other than large vessel disease, respectively. Although most young patients with cryptogenic stroke fulfill ESUS criteria, minor embolic sources do not seem to be more prevalent in ESUS than in strokes of determined cause; therefore, further explanations should be sought in this group.

Predictors of Cognitive Decline in the Early Stages of Parkinson's Disease: A Brief Cognitive Assessment Longitudinal Study

Our objectives were to perform a longitudinal assessment of mental status in early stage Parkinsons disease (PD) patients, with brief neuropsychological tests, in order to find predictive factors for cognitive decline. Sixty-one, early stage, and nondemented patients were assessed twice, over a 2-year interval, with a global cognitive test (mini-mental state examination (MMSE)) and a frontal function test (frontal assessment battery (FAB)) and motor function scales. Dementia and hallucinations were diagnosed according to the DSM-IV criteria. Cognitive function scores did not decrease significantly, except for FAB lexical fluency score. Four patients presented with dementia at followup. The MMSE score below cut-off, worse gait dysfunction, the nontremor motor subtype, and hallucinations were significantly related to dementia. Rigidity and speech dysfunction were related to dementia and a decrease in FAB scores. We can conclude that decline in the MMSE and FAB scores is small and heterogeneous in the early stages of PD. Scores below cut-off in the MMSE could be helpful to predict dementia. Nontremor motor deficits could be predictive factors for frontal cognitive decline and dementia.