Clin. Transl. Neurosci., Volume 4, Issue 1 (February 2020) – 12 articles

Background: One of the major periprocedural risks of carotid artery stenting is embolism caused either by plaque debris or by local thrombus forming. Double-layer micromesh stent design has shown to lower the chance of debris embolism but might have a slightly higher risk of local thrombus forming. Thus, we compared two different stent designs regarding safety and outcome profile in elective patients with high-grade carotid artery stenosis using a self-expanding, double-layer micromesh carotid stent system (DLCS) or a self-expanding hybrid carotid stent system (HCS). Methods: A single-center, open-label, retrospective cohort study of 67 consecutive, elective patients with high-grade symptomatic and asymptomatic carotid stenosis was executed at a comprehensive stroke center. Outcome measures were reocclusion rate, periprocedural symptomatic ischemic events, as well as other periprocedural complications, and recurrent stroke and mortality at 30 days’ follow-up. Results: Thirty-two patients (24% women, median age 75 years (interquartile range (IQR) 71–80) were treated with DLCS, and 35 patients (29% women, median age 71 years (IQR 63–76) years) with HCS. In both groups, pretreatment carotid stenosis degree was similar (median NASCET of 80%). Successful deployment was achieved in all cases without technical failure, and both groups did not differ in reocclusion rates, recurrent stroke, and mortality within 30 days. Conclusions: DCLS and HCS revealed to have similar safety and outcome profile in elective patients with high-grade symptomatic as well as asymptomatic carotid artery stenosis. Full article

Amyotrophic lateral sclerosis is a neurodegenerative illness that causes gradual loss of muscle function. Patients eventually develop bulbar impairment, requiring extensive respiratory support. Noninvasive ventilation (NIV) has gained attention as an easily accessible method with promising benefits. We conducted this systematic review to outline the therapeutic effects of NIV, add to previous publications discussing this topic by providing updates on newly completed and ongoing studies, and identify limitations that must be addressed in future trials. A search of PubMed and Cochran for relevant primary studies yielded 26 publications. Studies indicate NIV use is associated with improvements in quality of life, regardless of the severity of bulbar impairment. However, NIV’s benefits on survival were limited to patients with less bulbar impairment. In addition, our review found several limitations that undermine the efforts to establish a definitive treatment regimen. Future studies will need to address these problems in order to provide patients with better respiratory care. Full article

Dilated Virchow–Robin spaces (VRSs) are expansions of the normal perivascular spaces with a short axis greater than 2 mm or, according to some authors, greater than 3 mm. They are usually documented at the basal ganglia, at the convexity white matter (WM) and centrum semiovale, and at the mesencephalon. The anterior temporal WM is a recently described preferential location for large (≥5 mm) VRSs. The aim of our study was to evaluate the magnetic resonance imaging (MRI) features and their modifications during a long-term follow-up period (≥24 months) of the anterior temporal VRSs with a retrospective analysis among all brain MRI studies performed at our institution between January 2010 and January 2017. In our study, the presence and the stability of characteristic MRI features certainly increased our diagnostic confidence allowing us to continue conservative approach while the surrounding signal change, as reported in the literature, should not in itself prompt alternative diagnoses to be entertained. Full article

Objective: The aim of this study is to evaluate how anxiety influences the burden of disease of cluster headache. Methods: Participants completed a modified version of the EUROLIGHT questionnaire. Anxiety was measured with the anxiety subscale of the Hospital Anxiety and Depression scale. An elevated level of anxiety was assumed when eight or more points were scored. Results: The data of 1089 participants were taken for analysis. The score of the anxiety subscale of the Hospital Anxiety and Depression Scale (HADS-A) correlated weakly with the number of attacks in the last 30 days (r = 0.17). A score of eight and above in the HADS-A was associated with hurting oneself during an attack (odds ratio (OR) = 2.63), worrying about future attacks (OR = 2.95) and reporting of both failed relationships (OR = 2.81) and career problems (OR = 2.65). The odds of feeling understood by family and friends as well as colleagues and employers were lower in anxious persons (OR = 0.35 and 0.40, respectively). Conclusions: Anxiety complicates dealing with cluster headache and strongly aggravates its burden. Instead of finding help in others, anxious persons feel misunderstood and withdraw; relationships fail and difficulties at work arise. Full article

Background: Patients with visual snow syndrome (VSS) describe tiny flickering dots in the entire visual field resembling the noise of a poorly adjusted channel of analogue television with additional symptoms. Little is known about the pathophysiology and therapeutic options for this debilitating condition. Objectives: We present a case series of three patients with VSS taking mirtazapine, one of the most often prescribed antidepressants, and discuss the utility of antidepressants by reviewing our current understanding of pathophysiology and therapy. Results: Mirtazapine has no effect on VSS, neither positive nor negative. This is in line with the reports from the literature suggested only some beneficial effects from lamotrigine. Conclusions: Since the pathophysiology of VSS is not fully understood, we still rely on the reports of individual cases or patient series. This includes not only the positive, but also the negative results to avoid unnecessary treatment trials. Looking into the literature, antidepressants do not seem to be a solution for the visual symptoms. So far, best data exists for the anticonvulsant lamotrigine. Full article

Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CARASIL, Maeda syndrome) is an extremely rare autosomal-recessive genetic disorder with a serious arteriopathy causing subcortical infarcts and leukoencephalopathy. In less than 20 cases, a genetic mutation was proven. Patients suffer from alopecia, disc herniations, and spondylosis. Between the age of 30 and 40, the patients typically develop severe cerebral infarcts. Clinical symptoms, genetic study, magnetic resonance imaging (MRI), and coronary angiography of a patient with proven CARASIL are presented. The patient showed the typical phenotype with cerebral small-vessel disease, cerebral infarcts, spondylosis, and abnormal hair loss. Additionally, distinct cerebral microhemorrhage and a severe coronary artery disease (CAD) were found, which have not been reported before for CARASIL. Mutation screening revealed the presence of a homozygous c.1022G > T substitution in the HTRA1 gene. Evidence from other publications supports a pathogenetic link between the HTRA1 mutation and CAD as a new feature of CARASIL. This is the first report about CARASIL with a concomitant severe CAD. Thus, in patients with CARASIL, other vessel diseases should also be considered. Full article

Background: Transcranial Doppler (TCD) is a well-established tool for cerebrovascular assessment. Estimating the flow velocity across the intracranial arteries helps to identify children with sickle cell anaemia who are at risk for stroke. Objective: Our aim is to correlate TCD findings with clinical condition in children with sickle cell disease (SCD) to determine the value of TCD assessment as a predictive tool for stroke in SCD and to identify any association of TCD findings with disease severity, transfusion therapy and treatment administered. Methods: Eighty-five paediatric SCD patients aged from 3 years to 18 years of both genders who were followed up at the Hematology Clinic of New Children’s Hospital at Cairo University were included in this cross-sectional observational study. All our participants underwent routine laboratory investigations and TCD assessments. Results: Oof the 85 patients, two patients (2.3%) died before completing the TCD study and eventually 83 patients were included in the analysis. Seventeen (20.5%) patients had abnormal TCD findings, seven (8.4%) patients showed high-risk findings and 10 (12.1%) patients had conditional flow pattern. Logistic linear regression analysis confirmed that annual frequency of blood transfusion and hydroxyurea (HU) dose were associated with a decreased risk of abnormal TCD findings. Conclusion: The current study demonstrates that our TCD data reproduce the findings of other studies and that it is very likely the results from large trials are applicable for Egyptian children. The annual frequency of blood transfusion and HU dose were associated with a decreased frequency of abnormal TCD findings. Full article

Hemicrania continua (HC) is an indomethacin-responsive primary headache which belongs to the trigeminal autonomic cephalalgias. Although the first description of HC was 35 years ago, there are still different views regarding the clinical course, the diagnostic criteria, and the treatment. The high clinical heterogeneity of HC, missed diagnosis, and the delay to the correct diagnosis are important in patient care. Central features of HC are continuous side-locked headaches (with superimposed exacerbations) and the response to indomethacin. We are describing the case of a 29-year-old women who developed right-sided headache 3 weeks after the excision of a right-sided vestibular schwannoma. She tried different painkillers and also was started on a prophylactic treatment with oxcarbazepine, acupuncture, and physiotherapy. But nothing really helped. She was then admitted for an inpatient withdrawal program for medication overuse headache. Again the pain did not change. She has then been treated with indomethacin 50-mg tds, where after the headache improved rapidly within 3 days. This educational case presentation and review of the literature aims to consider HC as a possible differential diagnosis in chronic headache, especially when side-locked and shows that indomethacin maybe a quick therapeutic option before putting the patients on a long treatment odyssey with analgesics and other drugs. Full article

In the majority of cases, benign paroxysmal positional vertigo (BPPV) originates from the posterior or horizontal semicircular canals. If performed correctly, the maneuvers that diagnose or treat canalolithiasis or cupulolithiasis are highly effective. This article describes the provocation (i.e., diagnostic) and liberation (i.e., therapeutic) maneuvers to be applied in patients with BPPV. The step-by-step descriptions of the maneuvers are supplemented by practical recommendations. Full article

Lactate has been shown to have beneficial effect both in experimental ischemia–reperfusion models and in human acute brain injury patients. To further investigate lactate’s neuroprotective action in experimental in vivo ischemic stroke models prior to its use in clinics, we tested (1) the outcome of lactate administration on permanent ischemia and (2) its compatibility with the only currently approved drug for the treatment of acute ischemic stroke, recombinant tissue plasminogen activator (rtPA), after ischemia–reperfusion. We intravenously injected mice with 1 µmol/g sodium l-lactate 1 h or 3 h after permanent middle cerebral artery occlusion (MCAO) and looked at its effect 24 h later. We show a beneficial effect of lactate when administered 1 h after ischemia onset, reducing the lesion size and improving neurological outcome. The weaker effect observed at 3 h could be due to differences in the metabolic profiles related to damage progression. Next, we administered 0.9 mg/kg of intravenous (iv) rtPA, followed by intracerebroventricular injection of 2 µL of 100 mmol/L sodium l-lactate to treat mice subjected to 35-min transient MCAO and compared the outcome (lesion size and behavior) of the combined treatment with that of single treatments. The administration of lactate after rtPA has positive influence on the functional outcome and attenuates the deleterious effects of rtPA, although not as strongly as lactate administered alone. The present work gives a lead for patient selection in future clinical studies of treatment with inexpensive and commonly available lactate in acute ischemic stroke, namely patients not treated with rtPA but mechanical thrombectomy alone or patients without recanalization therapy. Full article

The First International Neurological Congress took place in 1931 in Berne, Switzerland, convening among >800 participants were many of the best-known neurologists and neuroscientist of that time. It had a great impact on the acknowledgement and acceptance of neurology as a specialized field in medicine and led to the formation of University Chairs and Neurological Societies in various countries. Full article