A case of normal pressure hydrocephalus presenting as ventriculoperitoneal shunt-responsive dementia and choreodystonia
(Source: Neurological Sciences)
Source: Neurological Sciences - August 10, 2018 Category: Neurology Source Type: research

A case of recurrent transient ischemic attacks: carotid stump syndrome or posterior cerebral artery syndrome?
(Source: Neurological Sciences)
Source: Neurological Sciences - August 10, 2018 Category: Neurology Source Type: research

Moyamoya complicated by thrombotic cerebrovascular accident in a Caucasian woman with collagenous colitis
(Source: Neurological Sciences)
Source: Neurological Sciences - August 9, 2018 Category: Neurology Source Type: research

WWOX-associated encephalopathies: identification of the phenotypic spectrum and the resulting genotype-phenotype correlation
AbstractEpileptic encephalopathies are a group of disorders in which epileptiform abnormalities cause progressive deterioration in cerebral function. Genetic causes have been described in several of the epileptic encephalopathies, and many previously unknown genes have been identified. WW domain-containing oxidoreductase (WWOX) has recently been implicated in autosomal recessive spinocerebellar ataxia type 12 (SCAR12) and severe early-onset epileptic encephalopathy. With whole-exome sequencing, we identified a homozygousWWOX missense mutation, p.Leu239Arg, in a girl from a consanguineous family with psychomotor development...
Source: Neurological Sciences - August 9, 2018 Category: Neurology Source Type: research

Diastolic heart dysfunction is correlated with CTG repeat length in myotonic dystrophy type 1
In this study, clinical data for patients with DM1, including age, onset age, CTG repeat length, Medical Research Council sum score (MRCSS), and 6-min walking test (6MWT), were recorded. In addition, ECHO parameters and cardiac conduction abnormalities were evaluated. Among the cardiac parameters, the EA ratio and left ventricular end-diastolic dimension (LVEDD) were significantly correlated with the CTG repeat length (p 
Source: Neurological Sciences - August 9, 2018 Category: Neurology Source Type: research

Morvan ’s syndrome—is a pathogen behind the curtain?
AbstractMorvan ’s syndrome is a rare syndrome of likely autoimmune etiology characterized by peripheral nerve hyperexcitability, dysautonomia, insomnia, and fluctuating delirium with prominent hallucinations. Since its first mention in 1890, less than 100 cases have been described in literature. The largest exis ting review includes details of 29 cases. This case series describes 4 cases (M = 4) of Morvan’s syndrome which presented between May and November 2017 to a single tertiary care referral teaching hospital in north India. All the four patients manifested behavioral abnormalities, sleep dist...
Source: Neurological Sciences - August 9, 2018 Category: Neurology Source Type: research

Profiling of microRNAs modulating cytomegalovirus infection in astrocytoma patients
AbstractAstrocytoma is recognized as the most common neoplasm of the brain with aggressive progression. The therapeutic regime for glioblastoma, the most aggressive astrocytoma, often consists of aggressive chemo and radiotherapy. The present holistic approaches, however, have failed to influence the quality life of patients. Therefore, it is necessary to understand the underlying mechanisms of its progression for updated therapeutic evaluation. Human cytomegalovirus (HCMV) is reported to be associated with glioblastoma progression. The hypothesis still remains controversial due to the lack of concrete evidences. Here, we ...
Source: Neurological Sciences - August 8, 2018 Category: Neurology Source Type: research

Multiple sclerosis incidence in Tuscany from administrative data
ConclusionsWe found a high incidence rate, similarly to other Italian areas, especially in women, that may explain the increasing prevalence in Tuscany. To confirm this data and to calculate the possible bias caused by our inclusion method, we will validate our algorithm for incident cases. (Source: Neurological Sciences)
Source: Neurological Sciences - August 8, 2018 Category: Neurology Source Type: research

Quality of life in Parkinson ’s disease: Italian validation of the Parkinson’s Disease Questionnaire (PDQ-39-IT)
AbstractTranslation and cross-cultural adaptation of the 39-item Parkinson ’s Disease Questionnaire(PDQ-39) to the Italian culture was performed by Oxford University Innovation in 2008, but this version has never been validated. Therefore, we performed the process of validation of the Italian version of the PDQ-39 (PDQ-39-IT) following the “Consensus-Based Standards for the Selection of Health Status Measurement Instruments” checklist. The translated PDQ-39-IT was tested with 104 patients diagnosed with Parkinson’s disease (PD) who were recruited between June and October 2017. The mean age of the pa...
Source: Neurological Sciences - August 7, 2018 Category: Neurology Source Type: research

Neurosarcoidosis: clinical characteristics, diagnosis, and treatment in eight Chinese patients
AbstractNeurosarcoidosis is relatively rare and has diverse manifestations. The clinical characteristics, diagnosis, treatment, and outcome for neurosarcoidosis in China are poorly understood. We retrospectively analyzed the clinical features, laboratory and imaging results, treatment, and outcomes in patients who met the criteria for definite or probable neurosarcoidosis in Xuan Wu Hospital of Capital Medical University from 2000 to 2015. Eight patients were included in this study, accounting for 5.84% of all cases with sarcoidosis. The mean age at onset was 50.25  years, and 75% of the patients were female. Five cas...
Source: Neurological Sciences - August 7, 2018 Category: Neurology Source Type: research

The predictive power of transcranial sonography in movement disorders: a longitudinal cohort study
AbstractTranscranial sonography (TCS) is a noninvasive, easily performed, and commonly available neuroimaging technique useful for the study of brain parenchyma in movement disorders. This tool has been increasingly used in the diagnosis of Parkinson ’s disease and atypical parkinsonism. The aim of the study was to evaluate the applicability of this technique as supportive tool in the early diagnosis of movement disorders. We performed TCS on 315 individuals which were diagnosed as healthy controls or affected by idiopathic Parkinson’s disea se, monogenetic subtypes of Parkinson’s disease, atypical parkin...
Source: Neurological Sciences - August 7, 2018 Category: Neurology Source Type: research

Highlights of Issue 9, 2018
(Source: Neurological Sciences)
Source: Neurological Sciences - August 7, 2018 Category: Neurology Source Type: research

Getting the high school diploma with only one hemisphere: a case report
(Source: Neurological Sciences)
Source: Neurological Sciences - August 7, 2018 Category: Neurology Source Type: research

Incidence of orolingual angioedema after intravenous thrombolysis for stroke
DiscussionThe incidence of orolingual angioedema was very low in our series. Although OA is usually mild, anaphylactoid reactions may rarely occur, because of the variable degree of activation of the complement system and kinin cascade caused by alteplase. In such instances, admission to neurointensive care may be required. Specific bradykinin antagonists or drugs that target the kallikrein-kinin system are beginning to be used in the more severe cases. Thus, doctors and nurses caring for acute stroke patients need to be able to recognize and treat this complication. (Source: Neurological Sciences)
Source: Neurological Sciences - August 6, 2018 Category: Neurology Source Type: research

Muscle profile and cognition in patients with Alzheimer ’s disease dementia
ConclusionsIn patients with AD dementia, abundant muscle mass did not mean strong power. The simple lower-extremity muscle strength assessment is more effective in predicting cognition than a muscle mass measure in male patients. (Source: Neurological Sciences)
Source: Neurological Sciences - August 6, 2018 Category: Neurology Source Type: research

Possible clinical marker of first attack of HaNDL syndrome (stroke-like mimics) in emergency room: a case report
(Source: Neurological Sciences)
Source: Neurological Sciences - August 4, 2018 Category: Neurology Source Type: research

Therapeutic potential of combined viral transduction and CRISPR/Cas9 gene editing in treating neurodegenerative diseases
ConclusionThe CRISPR/Cas9 gene editing tool is a powerful arsenal in the field of gene therapy and molecular medicine; hence, more research should be called to focus on the ample potential this tool has to offer in the field of neurodegenerative diseases. (Source: Neurological Sciences)
Source: Neurological Sciences - August 3, 2018 Category: Neurology Source Type: research

Serum magnesium but not calcium was associated with hemorrhagic transformation in stroke overall and stroke subtypes: a case-control study in China
AbstractAssociation between serum calcium and magnesium versus hemorrhagic transformation (HT) remains to be identified. A total of 1212 non-thrombolysis patients with serum calcium and magnesium collected within 24  h from stroke onset were enrolled. Backward stepwise multivariate logistic regression analysis was conducted to investigate association between calcium and magnesium versus HT. Calcium and magnesium were entered into logistic regression analysis in two models, separately: model 1, as continuous va riable (per 1-mmol/L increase), and model 2, as four-categorized variable (being collapsed into quartiles). H...
Source: Neurological Sciences - July 19, 2018 Category: Neurology Source Type: research

Analysis of the clinical features of early Parkinson ’s disease with comparatively integrated intestinal function
ConclusionsThese results suggest that PD without constipation in early stage may represent a unique clinical phenotype, which may be more benign than PD with constipation. (Source: Neurological Sciences)
Source: Neurological Sciences - July 17, 2018 Category: Neurology Source Type: research

The ancient history of dementia
ConclusionsThe study of the ancient texts proves that dementia was distinguished from senile dementia early in history. This differentiation was manifested even with a shift in the use of specific terminology. Finally, the attempt to correlate modern medical entities with dementia-related issues results in interesting hypotheses. (Source: Neurological Sciences)
Source: Neurological Sciences - July 17, 2018 Category: Neurology Source Type: research

National Institutes of Health Stroke Scale in patients with primary intracerebral hemorrhage
The objective of our study was to investigate whether NIHSS is a reliable instrument of clinical monitoring and correlates with mortality and functional outcome in ICH.MethodsOne hundred fifty-six consecutive subjects with primary ICH were included. We evaluated NIHSS at admission. The functional state after a 30-day and a 3-month-long follow-up was assessed by the modified Rankin Scale (mRS). Spearman ’s rank correlation coefficient analysis was used for statistics. Sensitivity, specificity, positive predictive value, negative predictive value, global accuracy, and ROC curve were computed using the median score 7 as...
Source: Neurological Sciences - July 16, 2018 Category: Neurology Source Type: research

The serum level of inflammatory markers in chronic and episodic migraine: a case-control study
AbstractThe exact mechanism of the migraine pathophysiology remained unclear. Although there are some reports showing low-grade inflammation in migraineurs, further studies are needed in this field. Thus, we designed a study to evaluate the serum levels of two main proinflammatory markers in migraine patients. In this case-control research, 43 migraine patients (23 chronic and 20 episodic migraineurs) and 40 age-sex-matched headache-free controls were studied. Demographic, dietary, and anthropometric data, headache characteristics, and serum C-reactive proteins (CRP) and tumor necrosis factor-alpha (TNF- α) assessmen...
Source: Neurological Sciences - July 15, 2018 Category: Neurology Source Type: research

Lyme borreliosis as a trigger for NMDA receptor encephalitis?
(Source: Neurological Sciences)
Source: Neurological Sciences - July 14, 2018 Category: Neurology Source Type: research

Highlights of Issue 8, 2018
(Source: Neurological Sciences)
Source: Neurological Sciences - July 14, 2018 Category: Neurology Source Type: research

Allele-specific methylation contributed by CpG-SNP is associated with regulation of ALOX5AP gene expression in ischemic stroke
AbstractPrevious studies have shown that CpG-SNPs might have influence on gene function via allele-specific DNA methylation (ASM). However, association study between DNA methylation and the promoter CpG-SNPs inALOX5AP gene with IS has not been reported. The present study aims to explore the relationship among CpG-SNPs, methylation levels, and messenger RNA (mRNA) expression levels ofALOX5AP gene. Firstly, we made a two-stage association study to identify a potential associated CpG-SNP (rs4073259) by SNaPshot genotyping approach (P = 0.015,OR = 0.672, 95%CI 0.487 –0.927;P = 0....
Source: Neurological Sciences - July 12, 2018 Category: Neurology Source Type: research

Wake-up stroke and CT perfusion: effectiveness and safety of reperfusion therapy
AbstractObjectiveIschemic stroke is a neuroemergency condition highly treatable with thrombolysis and thrombectomy. Recently, observational studies have brought insights into clinical and imaging characteristics of wake-up stroke, which interested up to 25% of ischemic stroke patients. In clinical practice, wake-up strokes are usually not considered for reperfusion therapy. The aim of this study was to investigate the use CT perfusion imaging in patients with wake-up stroke and to assess the effect of neuroimaging information provided by CT perfusion maps on the efficacy and safety of thrombolysis and thrombectomy.Patients...
Source: Neurological Sciences - July 10, 2018 Category: Neurology Source Type: research

Radiographic and microsurgical characteristics of proximal (A1) segment aneurysms of the anterior cerebral artery
ConclusionCompared to ICBIF aneurysms, proximal A1 aneurysms were smaller and directed posteriorly, with incorporating perforators. Because of these characteristics, it may be difficult to perform clipping with 360 ° view in microsurgical field. Therefore, when planning to treat proximal A1 aneurysms, different treatment strategies may be necessary, relative to those used for ICBIF aneurysms. (Source: Neurological Sciences)
Source: Neurological Sciences - July 10, 2018 Category: Neurology Source Type: research

Acute-on-chronic subdural hematoma: the death of the famous XIX century soprano Maria Malibran —a study of the sources
AbstractMaria Malibran (1808 –1836) is one of the most famous sopranos of the nineteenth century. In 1825, along with her father, the renowned tenor Manuel Garcia, she introduced the Italian opera in America for the first time. The European debut in Paris (1828) definitively crowned her as a star. Thus, she was requested by t he most famous European theaters. In July 1836, during an equestrian excursion in London, she fell from her horse dashing her head against the ground, resulting in astate of insensibility. Since that accident, she had suffered fromcontinual headache and nervous attacks, but she continued to work...
Source: Neurological Sciences - July 10, 2018 Category: Neurology Source Type: research

B álint syndrome caused by bilateral medial occipital infarcts
(Source: Neurological Sciences)
Source: Neurological Sciences - July 10, 2018 Category: Neurology Source Type: research

Amyloid-positive late-onset semantic variant primary progressive aphasia
(Source: Neurological Sciences)
Source: Neurological Sciences - July 10, 2018 Category: Neurology Source Type: research

Symptomatic intracranial atherosclerotic disease: an ultrasound 2-year follow-up pilot study
The objective of this single-center pilot study was to assess if symptomatic intracranial atherosclerotic disease (ICAD) ultrasound features change through the 2  years after acute ischemic stroke or TIA, being ICAD a relevant cause of acute ischemic stroke or TIA, linked to high rates of recurrent stroke.MethodsWe consecutively enrolled 48 patients with acute ischemic stroke or TIA with symptomatic ICAD detected by transcranial color-coded duplex sonography (TCCS) and confirmed by MR-angiography and/or CT-angiography. We set a neurosonological and clinical follow-up at 3, 6, 12, and 24  months (T0, T1, T2, T3, a...
Source: Neurological Sciences - July 9, 2018 Category: Neurology Source Type: research

The comorbidity of acute ischemic stroke and splenic infarction resulting from essential thrombocythemia
(Source: Neurological Sciences)
Source: Neurological Sciences - July 8, 2018 Category: Neurology Source Type: research

A novel compound heterozygous mutation of the L2HGDH gene in a Chinese boy with L-2-hydroxyglutaric aciduria: case report and literature review
ConclusionThe results suggested that NGS could provide clues for identifying patients with abnormal neuroradiological findings in the subcortical white matter. (Source: Neurological Sciences)
Source: Neurological Sciences - July 6, 2018 Category: Neurology Source Type: research

Brain volume loss is present in Japanese multiple sclerosis patients with no evidence of disease activity
AbstractNo evidence of disease activity-3 (NEDA-3), defined as absence of clinical relapse, disability progression, and brain magnetic resonance imaging (MRI) activity, has emerged as the therapeutic target of disease-modifying therapy for multiple sclerosis (MS). However, recent studies have revealed that NEDA-3 might not be sufficient to prevent cognitive deterioration and predict long-term disability. In addition to NEDA-3, brain atrophy has recently been recognized as a pivotal biomarker that is closely associated to disability in patients with MS. This retrospective observational study included 22 Japanese MS patients...
Source: Neurological Sciences - July 4, 2018 Category: Neurology Source Type: research

Aggressive immunosuppression in Susac ’s syndrome: 10 years of follow-up
(Source: Neurological Sciences)
Source: Neurological Sciences - July 4, 2018 Category: Neurology Source Type: research

Neurological sciences impact factor 2017 is increased to 2.285: a comment from the Editor in Chief
(Source: Neurological Sciences)
Source: Neurological Sciences - July 3, 2018 Category: Neurology Source Type: research

Highlights of Issue 7, 2018
(Source: Neurological Sciences)
Source: Neurological Sciences - July 2, 2018 Category: Neurology Source Type: research

Hyperhomocysteinemia influenced malnutrition in Parkinson ’s disease patients
AbstractIntroductionParkinson ’s disease (PD) is a neurodegenerative disease with many motor and non-motor symptoms. Hyperhomocysteinemia is reported in many PD patients. Homocysteine (Hcy) is reported to be a risk factor for some PD non-motor symptoms.AimThe aim was to analyze Hcy level and its correlation with physical activity and motor and some non-motor symptoms (depression and cognition) in PD patients.Patients and methodsPatients were surveyed for physical activity and demographic data. Blood samples were obtained for Hcy, vitamin B12, and folic acid determination. The Mini Nutritional Assessment (MNA), Unifie...
Source: Neurological Sciences - June 30, 2018 Category: Neurology Source Type: research

Sleep disorders in Cimarosa ’s “The Secret Marriage”
(Source: Neurological Sciences)
Source: Neurological Sciences - June 28, 2018 Category: Neurology Source Type: research

The mind-body problem in the philosophy of mind and cognitive neuroscience: a physicalist naturalist solution
AbstractUsing an analysis of a voluntary action caused by a visual perception, I suggest that the three fundamental characteristics of this perception (being conscious, self-conscious, and provided with a content) are neurologically implemented by three distinct higher order properties of brain dynamics. This hypothesis allows me to sketch out a physicalist naturalist solution to the mind-body problem. According to this solution, primary phenomenal consciousness is neither a non-physical substance, nor a non-physical property but simply the “format” that the brain gives to a part of its dynamics in order to obt...
Source: Neurological Sciences - June 27, 2018 Category: Neurology Source Type: research

Ocular flutter, generalized myoclonus, and ataxia associated with anti-GM1, GD1a, and GD1b antibodies in a 6-year-old child
(Source: Neurological Sciences)
Source: Neurological Sciences - June 27, 2018 Category: Neurology Source Type: research

Relation between lipoprotein-associated phospholipase A 2 mass and incident ischemic stroke severity
This study evaluated the association of lipoprotein-associated phospholipase A2 (Lp-PLA2) mass and initial severity.MethodsWe employed a retrospective analysis on our hospital-based registry and recruited 488 first-onset ischemic stroke patients admitted within 24  h after onset and with Lp-PLA2 mass measured. Stroke severities evaluated by National Institutes of Health Stroke Scale (NIHSS) were compared between Lp-PLA2 categories dichotomized by median. Multivariate logistic regression was used to detect the independent risk factors of severe stroke (NIHSS ≥ 7) and receiver operator curve (ROC) was construc...
Source: Neurological Sciences - June 25, 2018 Category: Neurology Source Type: research

Investigating the effect of STN-DBS stimulation and different frequency settings on the acoustic-articulatory features of vowels
ConclusionOur results indicated that STN-DBS stimulation at 130  Hz had a significant positive effect on articulation of [a] compared to off-stimulation. Although there is not any statistical significant stimulation at 60 and 230 Hz may also have an effect on the articulation of [e], [i], and [o] but this effect needs to be investigated in future studies with higher numbers of participants. (Source: Neurological Sciences)
Source: Neurological Sciences - June 25, 2018 Category: Neurology Source Type: research

The effects and potential mechanisms of folic acid on cognitive function: a comprehensive review
AbstractCognitive impairment and dementia are major neurocognitive disorders that cause a noticeable decline in cognitive abilities, including memory and thinking skills. The effectiveness of using folic acid as an adjuvant for management of neurocognitive disorders is not well-known. This paper explains the role of folic acid, including its usefulness, effectiveness, adverse side effects, and active mechanisms on cognitive functions of the elderly. In the current study, we reviewed clinical and experimental studies investigating the use of folic acid prior to November 2017, using PubMed, ISI Web of Knowledge, Science Dire...
Source: Neurological Sciences - June 23, 2018 Category: Neurology Source Type: research

Amyloid deposition and CBF patterns predict conversion of mild cognitive impairment to dementia
AbstractMild cognitive impairment (MCI) can include the transition from a normal state to dementia. To explore biomarkers for the development of dementia, we performed an 18-month follow-up study in 28 patients with amnestic MCI. Amyloid deposition was examined using PiB PET, and cerebral blood flow (CBF) was examined using SPECT. Cognitive function was periodically assessed. The rate of conversion to dementia was higher in the PiB-positive/equivocal group (74%) than in the PiB-negative group (33%) (p = 0.041). Perfusion SPECT was performed in 16 patients. MCI patients with an AD-characteristic pattern of red...
Source: Neurological Sciences - June 23, 2018 Category: Neurology Source Type: research

Pantothenate kinase-associated neurodegeneration mimicking Tourette syndrome: a case report and review of the literature
(Source: Neurological Sciences)
Source: Neurological Sciences - June 21, 2018 Category: Neurology Source Type: research

Chronic obstructive pulmonary disease may complicate Alzheimer ’s disease: a comorbidity problem
DiscussionCOPD is known to be associated with the development of cognitive deficits, in particular, regarding for executive functions and attention, memory and logical reasoning. In this context, MMSE has a low diagnostic accuracy to underline effective cognitive impairment in AD-COPD. Our study shows a higher frequency of frontal deficits and behavioral disturbances in patients with AD and COPD than patients with AD-only. COPD could complicate the management of AD patients, thus necessitating a closer and multidisciplinary monitoring. (Source: Neurological Sciences)
Source: Neurological Sciences - June 21, 2018 Category: Neurology Source Type: research

Blood-brain barrier permeability assessed by perfusion computed tomography predicts hemorrhagic transformation in acute reperfusion therapy
AbstractHemorrhagic transformation (HT) is one of the most feared complications of acute recanalization therapies. The aim of this study was to evaluate whether blood-brain barrier permeability (BBBP) imaging can predict HT in the setting of acute recanalization therapy and to determine the sensitivity and specificity of BBBP for the prediction of HT according to the type of reperfusion therapy. We assessed a total of 46 patients who received recanalization therapy (intravenous (IV) recombinant tissue plasminogen activator (tPA), mechanical thrombectomy with a stent retriever or both) for acute ischemic stroke within the i...
Source: Neurological Sciences - June 16, 2018 Category: Neurology Source Type: research

Three novel mutations in 20 patients with hereditary spastic paraparesis
This study was conducted to elucidate the genetic etiology of patients with the pure type AD-HSP diagnosis. The patient group consisted of 23 individuals from 6 families in Turkey. In the first step of work, Sanger sequencing (SS) was performed inATL1,SPAST, andREEP1 genes and the second phase whole-exome sequencing (WES) was performed following SS analysis for the patients with no detected mutations in these genes. The results of this study revealed that inATL1, 6 patients have previously reported c.776C  >  A mutation and 6 patients have novel c.470 T >  C mutation. InSPAST, 3 patie...
Source: Neurological Sciences - June 16, 2018 Category: Neurology Source Type: research

A new tool to assess responsiveness in disorders of consciousness (DoC): a preliminary study on the Brief Post-Coma Scale (BPCS)
AbstractIntroductionThe Brief Post-Coma Scale (BPCS) is an easy diagnostic tool for individuals with disorders of consciousness (DoC), in a reduced version from a previously Post-Coma Scale, that could distinguish patients in the minimally conscious state (MCS) from those in unresponsive wakefulness syndrome (UWS), formerly defined as vegetative state (VS).ObjectiveAim of the study was to assess the diagnostic validity of the BPCS in comparison with the Coma Recovery Scale-Revised (CRS-R), in its Italian validated version, the Disability Rating Scale (DRS), the Level of Cognitive Functioning (LCF), and the Glasgow Outcome ...
Source: Neurological Sciences - June 15, 2018 Category: Neurology Source Type: research