Discrimination of leptomeningeal carcinomatosis and meningeal inflammation/infection with internal acoustic canal enhancement.

PURPOSE: The purpose of this study is to investigate whether the presence and pattern of enhancement at the internal acoustic canal (IAC) could help in discriminating between leptomeningeal carcinomatosis (LCa) and meningeal inflammation/infection (MMI). METHODS: Magnetic resonance (MR) images of patients with leptomeningeal enhancement were retrospectively evaluated. MR images of the LCa group (n = 33), MMI group (n = 19) and control group (n = 33) were evaluated for the presence, type (moderate/prominent), and localization (unilateral/bilateral) of the IAC enhancement. RESULTS: The presence of IAC enhancement was significantly more common in patients with LCa (p < 0.001). In 73.7 % of patients with MMI, no contrast enhancement was observed in the IAC. In patients with contrast enhancement in the IAC, the risk of LCa in the etiology is 20 times greater than the risk of having MMI. Seventy-five percent of the IAC enhancement seen in LCa patients and 20 % of the IAC enhancements seen in MMI patients was bilateral. This difference was statistically significant (p = 0.029). CONCLUSION: Intense contrast enhancement of the IAC can be a marker for LCa.

Determinants of preventable stroke-Ankara ACROSS stroke preventability study.

BACKGROUND: Unhealthy lifestyle and inadequate control of vascular risk factors are the major contributors of stroke burden. Failure in achieving the target levels in control of these factors, not only designate missed opportunities contributing to the preventability of an incident stroke, but also set the post-stroke treatment goals in a case wise basis. In this study, we analyzed pre-event clinical features that play a role in stroke preventability, and determined the cumulative burden of risk factors that necessitate optimization following the ischemic insult. METHODS: Information about the pre-stroke optimal control of seven major modifiable risk factors (Life's Simple 7: hypertension, diabetes mellitus, hyperlipidemia, smoking, obesity, diet, and physical activity) was prospectively collected in ischemic stroke patients admitted to three tertiary academic centers in Ankara. Stroke preventability was evaluated by the overall number of factors requiring optimization with patients ≥4 risk factor conditions categorized as those suffering from a preventable stroke. Bivariate and multivariate analyzes were performed to uncover predictors of stroke preventability. RESULTS: Among 787 patients, 386 (49.0%) had ≥4 risk factor conditions requiring optimization. Preventable stroke was more common in younger patients, and patients with small artery occlusion. Multivariate analyses taking into account the pre-stroke antithrombotic treatment regimen, have highlighted age (OR: 0.99, 95%CI 0.98-1.00), female gender (1.59, 95%CI 1.17-2.16), coronary artery disease (1.54, 95%CI 1.10-2.14), small artery occlusion (1.90, 95%CI 1.13-3.18), and cardio-aortic embolism (0.53, 95%CI 0.35-0.82) as significant factors associated with preventability. CONCLUSIONS: Approximately half of the ischemic stroke patients have preventable stroke from the perspective of risk factor control. Extra care should be given to strategies directed to risk factor control and lifestyle interventions in certain high-risk groups for the prevention of future complications.

Risk Factors, Biomarkers, Etiology, Outcome and Prognosis of Ischemic Stroke in Cancer Patients

Introduction: Cerebrovascular disease is the second most common complication in individuals with tumours. The aim of this study was to investigate risk factors, biomarkers, etiology and prognosis of ischemic stroke in cancer patients (ISCPs). Methods: The medical records of 619 consecutive patients who were admitted with acute ischemic stroke from January 2012 to November 2014 were retrospectively evaluated. The patients were divided into two groups (group 1, patients with an active cancer prior to the onset of ischemic stroke; group 2, patients without an active cancer history). The demographic data, risk factors, NIHSS scores, thrombocyte count, D-dimer, fibrinogen and C reactive protein (CRP) level at admission, modified Rankin Scale (mRS) scores in the follow-up period and location of lesions on DWI were recorded. The Mann-Whitney U test, chi-squared test and logistic regression was used for analyzing data, p<0.05 being considered statistically significant. Results: A total of 46 (7.4%) ISCPs were included. Hyperlipidemia was significantly lower in the ISCP group (p=0.001). Elevated thrombocyte counts, D-dimer, fibrinogen and CRP levels at admission, acute multiple ischemic lesions, other causes, mortality in hospital and worse outcome were significantly related to ISCP (p<0.05). On logistic regression analysis, follow up mRS>3, acute multiple ischemic lesions located in more than one vascular territory (AMIMCT) and other causes were significantly associated with ISCP (p<0.001). Conclusion: In our study, other causes, AMIMCT and mRS>3 were more common in the ISCP group. We consider that CCS could be more suitable for detecting other causes than TOAST. Biomarkers could be important in the ISCP group.

GNE missense mutation in recessive familial amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a motor neuron disease eventually leading to death from respiratory failure. Recessive inheritance is very rare. Here, we describe the clinical findings in a consanguineous family with five men afflicted with recessive ALS and the identification of the homozygous mutation responsible for the disorder. The onset of the disease ranged from 12 to 35 years of age, with variable disease progressions. We performed clinical investigations including metabolic and paraneoplastic screening, cranial and cervical imaging, and electrophysiology. We mapped the disease gene to 9p21.1-p12 with a LOD score of 5.2 via linkage mapping using genotype data for single-nucleotide polymorphism markers and performed exome sequence analysis to identify the disease-causing gene variant. We also Sanger sequenced all coding sequences of SIGMAR1, a gene reported as responsible for juvenile ALS in a family. We did not find any mutation in SIGMAR1. Instead, we identified a novel homozygous missense mutation p.(His705Arg) in GNE which was predicted as damaging by online tools. GNE has been associated with inclusion body myopathy and is expressed in many tissues. We propose that the GNE mutation underlies the pathology in the family.

Etiologic Subtypes of Watershed Infarcts.

BACKGROUND: Two types of watershed infarcts (WI) are recognized. Internal WI are usually attributed to either severe stenosis in large arteries or acute hypotensive events, whereas external WI are thought to be caused by embolism. The aim of this study was to determine the etiologic background and prognosis of external and internal WI in our patients. METHODS: We reviewed the medical records and diffusion-weighted images of the patients who were admitted to our stroke unit with acute ischemic stroke between January 2012 and November 2014. The demographics, clinical features, radiologic investigations, and other etiologic tests of the patients with internal or external WI were recorded. We determined etiologic stroke subtypes according to the automated Causative Classification System. RESULTS: Fifty-three patients with WI were detected in our registry. Twenty-two (41.5%) of them were women. The mean age was 69 ± 12.8 (33-98) years. Twenty-one (39.6%) patients had external WI: 7 (33.3%) of them had large-artery atherosclerosis (LAA), 8 (38.1%) patients had cardioembolism, 3 (14.3%) patients had stroke due to other causes (vasculitis; n = 3), and etiologic subtype was undetermined in 3 patients (14.3%). Thirty-two (60.4%) patients had internal WI: 21 (65.6.%) of them had LAA, 5 (15.6%) patients had cardioembolism, 3 (9.4%) patients had stroke due to other causes (aneurysm; n = 1, hypercoagulability due to chronic myeloid leukemia; n = 1, vasculitis; n = 1), and etiologic subtype of 3 (9.4%) patients remained cryptogenic. LAA was significantly associated with internal WI (P = .024). Hypertension was more common in patients with internal WI (P = .035). CONCLUSIONS: In this series, cardioembolism was the most common etiologic subtype in the patients with external WI, whereas internal WI were significantly associated with LAA. Uncommon causes should also be investigated in cryptogenic patients.

Intravenous immunoglobulin for prophylaxis of acute exacerbation in Myasthenia Gravis.

Intravenous immunoglobulin (IVIg) treatment for acute exacerbations of Myasthenia Gravis (MG) was shown in several open-label studies. There are only two studies demonstrating the efficiency of regular intermittent IVIg therapy on MG patients who are not in their acute attack periods. Thirteen patients who had displayed an inadequate clinical response to immunosuppressive treatments, or who were not appropriate for immunosuppressive treatment due to the age factor and thus were given regular IVIg therapy, were retrospectively investigated. Moreover, the pre- and post-treatment attack frequencies were also evaluated. The mean number of attacks was 0.0960 attacks/year before IVIg therapy, and 0.0056 attacks/year after IVIg therapy (p = 0.002). The number and severity of the attacks were decreased in all patients. Eight patients (62 %) had used steroids; among them, steroid was completely stopped in two patients following the regular IVIg therapy, and the dose was decreased by 50 % in the other six patients. The requirement for pyridostigmine did not decrease in four patients, whereas this need decreased by 20-50 % in nine patients. IVIg can produce repeated beneficial effects in patients with MG and may be useful as an adjunct in the management of MG. IVIg has minimal adverse effects and ability to reduce corticosteroid dose. These results suggest that intravenous immunoglobulin maintenance therapy is a valid modality in patients with resistant treatment MG.

Therapeutic plasma exchange in treatment of neuroimmunologic disorders: review of 92 cases.

Therapeutic plasma exchange (TPE) is a procedure that reduces amount of circulating antibodies in patients through filtration for the treatment of neurologic diseases in which autoimmunity plays a major role. We reviewed the medical records of 92 neurologic patients who had been consecutively treated by TPE between June 2000 and April 2011 at Ankara University School of Medicine, Neurology Department and The Apaheresis Center. Neurological indications included myastehia gravis (MG, 16 patients), Guillain-Barre syndrome (GBS, 37 patients) and miscellaneous diseases (39 patients). The median TPE session number was 5 with a range of 1-8; total number of TPE procedures in all cases was 454. All MG patients improved with TPE during their hospitalization time. Regarding GBS, nearly 67% of the patients improved early, during their hospitalization time, either. In our series, 25% of GBS cases died because of dysautonomia. TPE was not effective in the treatment of the patients with Lambert-Eaton myasthenic syndrome, paraneoplastic polineuropathy, toxic polineuropathy, mononeuropathy multiplex in the case series. During the TPE procedures, 4 patients had hypotension and total number of the procedures was 21 in those patients. One patient had urticaria in only one session of total 5 TPE procedures. Two patients had septicemia; the first one had 3 and the second had 5 TPE procedures; both septic cases died. In conclusion, TPE is an effective treatment in neurologic diseases that autoimmunity plays an important role in the pathogenesis.