Neurological manifestation of coeliac disease with particular emphasis on gluten ataxia and immunological injury: a review article.

Coeliac disease (CD) is a gluten-induced enteropathy affecting 1% of the population and has extra intestinal manifestations. One such expression involves nervous system, and CD may present as gluten ataxia (GA), peripheral neuropathy and epileptiform disorder among others. Considerable controversy exists on the exact pathophysiological mechanism of gluten leading to ataxia. It is, however, clear that in intestinal axis tissue transglutaminase 2 (tTG2) is the primary target but in the nervous system, tTG6 may be the causative antigen although its exact role is not clear. Furthermore, it has also been postulated that anti-gangliodise antibodies may play a role in the emergence of central pathology if not the key contender. Moreover, the association of neurological injury with non-coeliac gluten sensitivity (NCGS), a related but pathologically different condition implies an independent mechanism of neuronal injury by gluten in the absence of CD. This review will touch on the salient features of CD and the nervous system and will highlight current controversies in relation to gluten and GA.

Neurological Disorders Associated With COVID-19 Hospital Admissions: Experience of a Single Tertiary Healthcare Center.

Background: Early reports have detailed a range of neurological symptoms in patients with the SARS-CoV-2 infection. However, there is a lack of detailed description and incidence of the neurological disorders amongst hospitalized COVID-19 patients. We describe a range of neurological disorders (other than non-specific neurological symptoms), including their clinical, radiological, and laboratory findings, encountered in our cohort of COVID-19 patients admitted to a large tertiary institution. Methods: We reviewed our prospectively collated database of all adult Neurology referrals, Neurology and Stroke admissions and Neurological multi-disciplinary team meetings for all hospitalized patients with suspected or proven COVID-19 from 17 March 2020 to 31 August 2020. Results: Twenty-nine of 1,243 COVID-19 inpatients (2.3%) presented with COVID-19-related neurological disorders. The mean age was 68.9 ± 13.5(SD) years, age range of 34-97 years, and there were 16 males. Twenty two patients had confirmed, five were probable and two had suspected COVID-19 infection according to the WHO case classification. Eight patients (27%) required critical care admission. Neurological symptoms at presentation included acute confusion and delirium, seizures, and new focal neurological deficits. Based on the pre-defined neurological phenotype, COVID-19 patients were grouped into four main categories. Sixteen patients had cerebrovascular events (13 with acute ischemic stroke and three had hemorrhagic features), seven patients were found to have inflammatory, non-inflammatory and autoimmune encephalopathy (including two with known Multiple Sclerosis), whilst disorders of movement and peripheral nervous system were diagnosed in three patients each. Conclusion: Although the exact prevalence and etiology remain unclear, new onset of neurological disorders, in addition to anosmia, is non-sporadic during the acute COVID-19-infection. Longitudinal follow-up of these patients is required to determine the clinical and functional outcome, treatment response and long-term effects of the SARS-CoV-2 infection.

Reduced Myelin Signal in Normal-appearing White Matter in Neuromyelitis Optica Measured by 7T Magnetic Resonance Imaging.

Whether the integrity of normal-appearing white matter (NAWM) is preserved in neuromyelitis optica spectrum disorders (NMOSD) is open to debate. To examine whether the tissue integrity of NAWM in NMOSD is compromised compared to that in healthy controls and patients with multiple sclerosis (MS), we prospectively enrolled 14 patients with NMOSD, 12 patients with MS, and 10 controls for clinical functional assessments and quantitative imaging, including T1 relaxation time (T1) and magnetization transfer ratio (MTR) at 7 Tesla. Cognitive performance on the Paced Auditory Serial Addition Test with a 3-second interstimulus interval (PASAT-3) was significantly lower in the NMOSD compared to the MS group (mean number of correct answers, 34.1 vs. 47.6; p = 0.006), but there were no differences in disease duration or disability. Histograms of T1 and MTR maps of NAWM demonstrated a decreased peak height in patients with NMOSD compared to the healthy controls, but not compared to patients with MS. Using 7T quantitative magnetic resonance imaging (MRI), this study showed that the NAWM in patients with NMOSD is abnormal, with reduced myelin signal; this was not previously observed using MRI at a lower field strength.

The UK patient experience of relapse in Multiple Sclerosis treated with first disease modifying therapies.

BACKGROUND: The fixed, progressive disability associated with late Multiple Sclerosis (MS) is known to have a major impact on patients and their families, but the impact of relapse earlier in the disease course is less well documented, particularly from the patient׳s perspective. This study aimed to understand the effects of relapse for people with MS (PwMS), focussing on the years immediately after starting disease modifying therapy (DMT) when experience of a relapse may particularly influence a patient׳s opinions of their disease and its therapy. METHODS: This was a multi-centre, retrospective, observational research study, recruiting patients from 7 UK NHS Hospital Trusts. Consenting patients with relapsing-remitting MS (RRMS), who had started a DMT more than 36 months before screening, were sent a study questionnaire. Data on MS relapses and treatments over 3 years were collected simultaneously from medical records. RESULTS: One hundred and three patients completed the questionnaires. Relapses were under-reported to health care professionals, with 28% of respondents failing to report their most recent attack and 46% declaring they had failed to report an attack in the past. During their most recent relapse, 67% of those in paid employment reported taking time off sick, 48% reduced working hours temporarily, and 41% worked reduced hours and took time off sick. Sixty-six percent required additional support to undertake routine daily tasks during their most recent relapse. A range of effects of relapse which cannot be measured in financial terms were also reported, including effects on physical abilities, mental health and family roles and relationships. CONCLUSION: This contemporary UK-based study provides an insight into the experience of relapse early in the treatment of RRMS from the patient perspective. The comparison of documented patient reported relapses reveals some deficiencies in the recording of relapses which is important to address in view of the reported impact of individual relapses, and emphasises relapse reduction as a worthy treatment aim.

Trunk movement in Parkinson's disease during rising from seated position.

Parkinson's disease (PD) is associated with particular difficulties rising from a seated position. Little is known about the mechanisms of sit-to-stand in this condition. We sought to define trunk movement during sit-to-stand in a group of patients with PD. Six patients and seven normal volunteers were studied using a six camera ELITE motion analysis system (BTS, Milan, Italy), which permitted data collection in the coronal, sagittal, and transverse planes. Retroreflective markers were positioned along the spine at C7, T3, T6, T9, T12, L3, and the sacrum. Whole-trunk kinematics and the movement at the six different trunk markers were recorded during rising. PD patients have a significantly greater degree of trunk flexion than controls, showing a significant increase in angular velocity of the trunk in the sagittal plane. The total range of movement of trunk rotation was significantly smaller in the PD group, but lateral movement in the trunk was greater than normal. These data suggest that patients with early PD compensate for their difficulties rising from a chair by generating greater trunk flexion at higher angular velocity, thus developing greater forward momentum. This process results in a decrease in the duration of the unstable transitional phase of sit-to-stand, allowing PD patients to reach the upright position as easily and safely as possible. Small rotational movements are an effective way to maintain the centre of mass within the base of support during sit-to-stand. This mechanism appears to be denied to the PD patients who may use increased movements in the coronal plane as an alternative strategy.