Editorial |
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The two thalamic nuclei, which are considered as part of deep gray matter substance of brain, play significant roles in human central nervous system (CNS). They are involved in activation of cortex, processing and relaying sensory data to the superior cortical centers, and are crucial for normal cognition. The thalamic structures are among the heavily-affected areas of the CNS and the measurable atrophy of these deep-seated nuclei translates into significant cognitive decline which manifest in patients with multiple sclerosis (MS).
Reviews |
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Zhou Zeng, Zhiping Hu, Jie Zhang
Although knowledge of intracerebral hemorrhage (ICH) continues to evolve, it is still a major cause of morbidity and mortality worldwide. Venous thromboembolism (VTE) events, including deep vein thrombosis (DVT) and pulmonary embolism (PE), are common and preventable complications of ICH. On the basis of the current systematic review and guidelines, the optimal prophylactic treatment remains unclear.
Sara Pietracupa, Elisa Bruno, Andrea E. Cavanna, Marika Falla, Mario Zappia, Carlo Colosimo
Hyperkinetic movement disorders represent a heterogeneous group of disorders in which involuntary movements are the prevalent clinical symptoms. The five main categories of hyperkinetic disorders are tremor, dystonia, tics, myoclonus and drug-induced dyskinesia. The severity of hyperkinetic disorders is assessed by all clinicians when they examine a patient; quantifying the severity also provides a means of studying the natural history of a given disorder and the possible effect of new therapeutic interventions.
Mojtaba Farjam, Guang-Xian Zhang, Bogoljub Ciric, Abdolmohamad Rostami
Inflammatory demyelination of the central nervous system (CNS) is the hallmark of multiple sclerosis (MS), a chronic debilitating disease that affects more than 2.5 million individuals worldwide. It has been widely accepted, although not proven, that the major pathogenic mechanism of MS involves myelin-reactive T cell activation in the periphery and migration into the CNS, which subsequently triggers an inflammatory cascade that leads to demyelination and axonal damage. Virtually all MS medications now in use target the immune system and prevent tissue damage by modulating neuroinflammatory processes.
Sini Kantele, Saija Karinkanta, Harri Sievänen
This meta-analysis evaluated feasibility and efficacy of long-term whole-body vibration (WBV) training in improving mobility of multiple sclerosis (MS) patients.
Kristin M. Galetta, Don Gilden
While herpes zoster infection has been recognized since antiquity, chickenpox (varicella) was confused with smallpox until the 1800s, when both illnesses became better understood. In the 20th century, varicella zoster virus (VZV) was shown to cause varicella upon primary (first-time) infection and herpes zoster (shingles) after reactivation of latent VZV. Scientific progress over the past 50 years has rapidly advanced the understanding and prevention of disease produced by VZV. Combined imaging and virological studies continue to reveal the protean neurological, ocular and visceral disorders produced by VZV.
Original Articles |
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Phase I (PhI): assess the safety of Polyphenon E in people with multiple sclerosis (MS) and determine the futility of Polyphenon E as a neuroprotective agent. Correlate plasma levels of EGCG with neuroprotective effects. Phase II (PhII): Further assess safety and confirm the neuroprotective effects of Polyphenon E.
The European Quality of Life Questionnaire 5 level version (EQ-5D-5L) is a recently updated instrument to assess Health-Related Quality of Life (HRQoL) that has not been validated extensively. The main objective of this study was to evaluate the internal consistency and convergent validation of the EQ-5D-5L in a large sample of subjects with Parkinson's disease (PD).
Experimental evidence has indicated the benefit of simvastatin in the treatment of subarachnoid hemorrhage (SAH). Recently, acute simvastatin treatment was not shown to be beneficial in neurological outcome using modified Rankin Scale. Cognitive function is another important dimension of outcome assessment and yet had not been investigated in statin studies for aneurysmal subarachnoid hemorrhage. We therefore explored whether acute simvastatin treatment would improve cognitive outcomes.
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) at onset manifests an early seizure (ES) usually lasting more than 30 min. Following ES, some patients exhibit almost clear consciousness with no neurological symptoms, and no MRI abnormality for a few days, which may lead to an initial misdiagnosis of prolonged febrile seizures (PFS). To allow an early diagnosis of AESD, we retrospectively analyzed clinical manifestations, laboratory data, and radiologic and EEG findings in patients with AESD (n = 62) having ES of over 30 min, and ones with PFS (n = 54), using logistic regression analyses.
In amyotrophic lateral sclerosis (ALS), not only impairment of motor neurons but also impairment of the autonomic nervous system has been demonstrated by previous physiological studies. Several investigators have reported a correlation between autonomic dysfunction and respiratory dysfunction in ALS. This study analyzed the relation between parameters of respiratory function and muscle sympathetic nerve activity (MSNA) in a large number of ALS patients.
In a recent phase 2 clinical trial in spinocerebellar ataxia type 3/Machado Joseph disease (SCA3/MJD), a neurogenetic disorder without specific therapy, benefits of lithium carbonate were found only on secondary efficacy outcomes, all related to ataxic features. In order to help designing future studies, we further analyzed the trial data searching for treatment response modifiers and metric properties of spinocerebellar ataxia (SCA) scales.
Many patients with dementia live in the community and depend on a family member for assistance. Taking care of non-self-sufficient people such as those with dementia causes distress. This study concerns factors contributing to feelings of burden and consequent coping strategies adopted by family caregivers of patients with Alzheimer's disease living in the community.
PPARδ belongs to a receptor family of ligand-activated transcription factors involved in the regulation of inflammation, cellular glucose uptake, protection against atherosclerosis and endothelial cell function. Through these effects, they might be involved with the ischemic stroke (IS). We recruited 200 subjects (100 IS patients diagnosed by CTs or/and magnetic resonance imaging (MRI) and 100 normal healthy controls from Chinese Uyghur population) to assess the nature of the functional polymorphisms of PPARδ +294T/C and any links with IS in this unique population.
Amyotrophic lateral sclerosis (ALS) patients can suffer episodes of lower respiratory tract infections (LRTI) leading to an acute respiratory failure (ARF) requiring noninvasive ventilation (NIV).
People with multiple sclerosis (PwMS) frequently experience walking and balance impairments. In our previous report, we demonstrated that spatio-temporal gait parameters, collected by the Zebris FDM-T instrumented treadmill (Zebris Medical GmbH, Germany), serve as valid markers of neurological impairment in the MS population. In the current study, we focused on a unique outcome statistic of the instrumented treadmill, the "butterfly" diagram which reflects the variability of the center of pressure trajectory during walking.
Myasthenia gravis (MG) is the autoimmune disorder in which the thymus is considered the pathogenic organ. Thymectomy (TE) is a therapeutic option for MG and often ameliorates clinical symptoms.
We are developing a novel intradural spinal cord (SC) stimulator designed to improve the treatment of intractable pain and the sequelae of SC injury. In-vivo ovine models of neuropathic pain and moderate SC injury are being implemented for pre-clinical evaluations of this device, to be carried out via gait analysis before and after induction of the relevant condition. We extend previous studies on other quadrupeds to extract the three-dimensional kinematics of the limbs over the gait cycle of sheep walking on a treadmill.
About three quarters of stroke deaths occur in developing countries including those in sub-Saharan African. Short and long-term stroke fatality data are needed for health service and policy formulation.
Tumefactive demyelinating lesions (TDLs) are associated with a variety of demyelinating diseases in the central nervous system (CNS). However, there are no current guidelines describing how to classify and treat patients with this rare phenotype. Thus, the present study aimed to determine the long-term evolution and disease course of patients initially presenting with TDLs and to describe their clinical and radiographic characteristics.
Impaired emotional facial expressiveness is an important feature in Parkinson's disease (PD). Although there is evidence of a possible relationship between reduced facial expressiveness and altered emotion recognition or imagery in PD, it is unknown whether other aspects of the emotional processing, such as subjective emotional experience (alexithymia), might influence hypomimia in this condition. In this study wee aimed to investigate possible relationship between reduced facial expressiveness and altered emotion processing (including facial recognition and alexithymia) in patients with PD.
Microglial activation is thought to be a key pathophysiological mechanism underlying disease activity in all forms of MS. Hydroxychloroquine (HCQ) is an antimalarial drug with immunomodulatory properties that is widely used in the treatment of rheumatological diseases. In this series of experiments, we explore the effect of HCQ on human microglial activation in vitro and on the development of experimental autoimmune encephalitis (EAE) in vivo.
The large-conductance, voltage- and Ca2+-activated K+ channels (termed BK) are associated with age-related dysfunctions or diseases. Previously, with our colleagues, we reported that the rβ2-associated inactivating BK (BKi) channels play an essential role in rat dorsal root ganglion (DRG) neurons. However, the age-dependent changes in BKi channels are still elusive. Here, we identify three types of BK channels in small DRG neurons, the single exponential BKi, the double exponential BKi and the non-inactivating BK.
As the pathophysiology of tremor in Parkinson disease (PD) involves a complex interaction between central and peripheral mechanisms, we propose that modulation of peripheral reflex mechanism by electrical muscle stimulation (EMS) may improve tremor temporarily.
Charcot–Marie–Tooth 2A (CMT2A) is caused by mutations in mitochondrial fusion protein mitofusin 2 (MFN2). CMT2A had a large variety of clinical symptoms and several cohort studies were published recently. This study is to summarized the clinical, electrophysiological, pathological and genetic features in Han Chinese CMT2A.
The hippocampus exhibits regional differences in vulnerability to ischemia, wherein pyramidal cells in the CA1 region are vulnerable to ischemia while pyramidal cells in the CA3 region and granule cells in the dentate gyrus (DG) region are relatively ischemia resistant. However, pyramidal cells in the CA1 region reportedly exhibit ischemic tolerance following exposure to a brief non-lethal period of ischemia known as ischemic preconditioning. In this study, we used proteomic analysis to examine the difference in protein expression between naïve rat CA1 and CA3/DG regions, as well as the altered protein expression in the CA1 region after 3 min of ischemic preconditioning.
The majority of studies of Parkinson's disease (PD) focused on basal ganglia initially; however, accumulating evidence suggests cerebellar involvement in pathophysiology. We aimed to investigate the effects of tremor onset on middle cerebellar peduncle (MCP) width of PD patients and of disease duration on differential diagnosis. We measured MCP width of 81 PD, 34 multiple system atrophy (MSA) and 16 normal controls, using MRI. A meta-analysis was performed including two previous and the present studies.
Muscles are small in spinal muscular atrophy (SMA). It is not known if muscle size changes over time in SMA type 1. We quantified changes over time in muscle size and echointensity during two repeated ultrasound examinations of unilateral proximal (biceps brachii/brachialis and quadriceps) and distal (anterior forearm flexors and tibialis anterior) muscles in three children with SMA type 1. We compared muscle thickness (MT) to body weight-dependent normal reference values. Children were 1, 6, and 11 months old at baseline and had 2, 2 and 4 months between ultrasound examinations, respectively.
A correct diagnosis and a proper treatment may yield a rapid and simple cure for benign paroxysmal positional vertigo (BPPV). Although the Gufoni maneuver is widely used to treat apogeotropic horizontal-canal BPPV (HC-BPPV), few studies have clarified the relationship between the speed and intensity of maneuver execution and successful canalith reposition. To evaluate the effect of accelerated execution of the Gufoni maneuver, a prospective randomized controlled study was conducted with HC-BPPV patients in a single dizziness clinic.
We evaluated effectiveness of using copyrighted computer-based stimulation programs in the correction of cognitive function in patients with coronary heart disease after coronary bypass surgery. A total of 74 patients were examined, all the patients underwent a course of drug therapy, 37 patients underwent a course of rehabilitation in addition to medical therapy using computer-based stimulation programs (1 time per day for 20 min within 10 days). A course of rehabilitation using computer-based stimulation programs in patients with coronary heart disease after coronary bypass surgery was proved to be an effective way of correcting cognitive function.
Double-hemorrhage rat models of subarachnoid hemorrhages (SAH) are most effective at simulating delayed cerebral vasospasms (CVS). The present study modified the models to minimize additional trauma and investigated injury of the corticospinal tract (CST) using diffusion tensor imaging (DTI).
Natalizumab therapy reduces inflammation and degeneration of the CNS in relapsing-remitting multiple sclerosis (RRMS). In cerebrospinal fluid (CSF) the concentration of 24S-hydroxycholesterol (24OHC) reflect neurodegeneration, whereas 27-hydroxycholesterol (27OHC) is dependent on the integrity of the blood-brain barrier (BBB).
To review the clinical features of optic neuritis (ON) combined with chronic hepatitis B (CHB) retrospectively.
Charcot Marie Tooth disease type 1A (CMT1A) is the most commonly inherited demyelinating polyneuropathy with variable phenotypes, affected by several comorbidities, especially diabetes mellitus (DM). Previous studies showed that DM exacerbates the clinical manifestations of CMT1A.
While imaging is useful in confirming the diagnosis of ischemic stroke, negative diffusion weighted imaging (DWI) is reported in up to 25% of patients. Our aim was to identify predictors of MRI-positive stroke from the itemized NIHSS.
Oxidative stress is considered a major factor in various neuronal diseases including ischemia-reperfusion injury. Proviral Integration Moloney 2 (PIM2) proteins, one of the families of PIM kinases, play crucial roles in cell survival. However, the functions of PIM2 protein against ischemia are not understood. Therefore, the protective effects of PIM2 against oxidative stress-induced hippocampal HT22 cell death and brain ischemic injury were evaluated using Tat-PIM2, a cell permeable fusion protein.
Cognitive impairment is an important predictor of quality of life at all stages of MS. Magnetic Resonance Imaging (MRI) markers have been used to associate tissue damage with cognitive dysfunction.
The clinical spectrum of West Nile Virus (WNV) infection ranges from a flu-like febrile condition to a more severe neuro-invasive disease that can cause death. The exact mechanism of neurodegeneration in neuro-invasive form of WNV infection has not been elucidated; however, a destructive role played by glial cells in promoting WNV mediated neurotoxicity has widely been speculated. The clinical studies revealed that the astroglial protein S100B is significantly elevated in the blood and CSF of patients with WNV infection, even in the absence of neuro-invasive disease.
An appropriate biomarker for spinocerebellar degeneration (SCD) has not been identified. Here, we performed gait analysis on patients with pure cerebellar type SCD and assessed whether the obtained data could be used as a neurophysiological biomarker for cerebellar ataxia. We analyzed 25 SCD patients, 25 patients with Parkinson's disease as a disease control, and 25 healthy control individuals. Acceleration signals during 6 min of walking and 1 min of standing were measured by two sets of triaxial accelerometers that were secured with a fixation vest to the middle of the lower and upper back of each subject.
Cryptococcal meningitis is a severe infection among immunosuppressed individuals, with a high mortality rate. Although amphotericin B is the first-choice drug for treatment, its use is restricted when adverse effects are clinically problematic. The usefulness of intraventricular infusion of antifungal drugs through Ommaya reservoirs for cryptococcal meningitis treatment has been unconfirmed. We evaluated the efficacy of intraventricular infusion of amphotericin B through Ommaya reservoirs.
The Global Health Delivery (GHD) framework (Farmer, Kim, and Porter, Lancet 2013;382:1060–69) allows for the analysis of health care delivery systems along four axes: a care delivery value chain that incorporates prevention, diagnosis, and treatment of a medical condition; shared delivery infrastructure that integrates care within existing healthcare delivery systems; alignment of care delivery with local context; and generation of economic growth and social development through the health care delivery system.
It is well known that neurons in the dentate gyrus (DG) of the hippocampus are resistant to short period of ischemia. Hyperthermia is a proven risk factor for cerebral ischemia and can produce more extensive brain damage related with mortality rates. The aim of this study was to examine the effect of hyperthermic conditioning (H) on neuronal death, gliosis and expressions of SODs as anti-oxidative enzymes in the gerbil DG following 5 min-transient cerebral ischemia. The animals were randomly assigned to 4 groups: 1) (N + sham)-group was given sham-operation with normothermia (N); 2) (N + ischemia)-group was given 5 min-transient ischemia with N; 3) (H + sham)-group was given sham-operation with H; and 4) (H + ischemia)-group was given 5 min-transient cerebral ischemia with H.
Aim was to analyze predictors of burden among primary caregivers (CGs) of Indian Parkinson's disease (PD) patients. 150 PD patients were administered using Unified Parkinson's Disease Rating Scale (UPDRS), Hoehn and Yahr Scale (H&Y), Montgomery Asberg Depression Rating Score (MADRS) and Mini Mental State Examination (MMSE) in this cross-sectional evaluation study. CG burden was assessed by Caregiver's Burden Scale (CBS), Hospital Anxiety and Depression Scale (HADS), SF-36 and 20-item Burden Assessment Schedule (BAS).
Background. Amyotrophic Lateral sclerosis (ALS) is associated with a significant distress, being linked to changes in hypothalamic–pituitary–adrenal axis activity. A loss of cortisol circadian rhythmicity in ALS patients was suggested, while more recently an increased plasma cortisol level in the disease has been reported.Objective. To assay the circadian plasma cortisol level in ALS and to study its relationship with the clinical phenotype and the rate of disease progression.Patients and methods.
To evaluate utricular and saccular function during the acute and resolved phases of BPPV, ocular and cervical vestibular evoked myogenic potentials (VEMPs) were studied in 112 patients with BPPV and 50 normal controls in a referral-based University Hospital. Ocular (oVEMPs) and cervical VEMPs (cVEMPs) were induced using air-conducted sound (1000 Hz tone burst, 100 dB normal hearing level) at the time of initial diagnosis and 2 months after successful repositioning in patients with BPPV, and the results were compared with those of the controls.
Machado–Joseph disease is defined as an autosomal dominant ataxic disorder caused by degeneration of the cerebellum and its connections and is associated with a broad range of clinical symptoms. The involvement of the vestibular system is responsible for several symptoms and signs observed in the individuals affected by the disease. We measured cervical and ocular vestibular evoked myogenic potentials in a sample of Machado–Joseph disease patients in order to assess functional pathways involved.
The goal of the present study was to clarify the clinical characteristics and laboratory results of parkinsonian symptoms among patients with and without camptocormia.
Background and purpose: Multiple sclerosis (MS) is a neuroinflammatory and neurodegenerative disease that progresses to axonal loss and demyelinization. Olfactory dysfunction in patients with MS has been reported frequently. We were interested in the associations of olfactory bulb (OB) and olfactory sulcus depth (OSD) with disease duration and attack frequency. Methods: We included 25 patients with MS and 30 age- and sex-matched controls in this study. The Expanded Disability Status Scale, Beck Depression Inventory, and Mini Mental State Examination were applied.
Cerebrospinal fluid (CSF) biomarkers have been increasingly studied for dementia diagnosis, however the accuracy to distinguish between different forms of dementia is still unsatisfactory. In this study, the added value of another CSF Aβ-peptide (Aβ40), along with the core CSF markers t-Tau, p-Tau, and Aβ42, in the discrimination between two large dementia groups of Frontotemporal Lobar Degeneration (FTLD; n = 107), Alzheimer's Disease (AD; n = 107) and non-demented subjects (n = 33) was evaluated. In FTLD, t-Tau and p-Tau were significantly increased in relation to controls, but lower than in AD, while Aβ42 was similar in FTLD and controls, but higher than in AD.
Sexual dysfunction (SD) is a common but often overlooked and undertreated symptom in multiple sclerosis (MS). The purpose of our longitudinal study was to explore the changes in the level of sexual functioning in MS cohort after a period of 3 and 6 years of follow-up, as well as to investigate the predictors of changes in SD during the period of observation.