Cerebellar ataxia with sensory ganglionopathy; does autoimmunity have a role to play?

Background and purpose: Cerebellar ataxia with sensory ganglionopathy (SG) is a disabling combination of neurological dysfunction usually seen as part of some hereditary ataxias. However, patients may present with this combination without a genetic cause. Methods: We reviewed records of all patients that have been referred to the Sheffield Ataxia Centre who had neurophysiological and imaging data suggestive of SG and cerebellar ataxia respectively. We excluded patients with Friedreich's ataxia, a common cause of this combination. All patients were screened for genetic causes and underwent extensive investigations. Results: We identified 40 patients (45% males, mean age at symptom onset 53.7 ± 14.7 years) with combined cerebellar ataxia and SG. The majority of patients (40%) were initially diagnosed with cerebellar dysfunction and 30% were initially diagnosed with SG. For 30% the two diagnoses were made at the same time. The mean latency between the two diagnoses was 6.5 ± 8.9 years (range 0-44). The commonest initial manifestation was unsteadiness (77.5%) followed by patchy sensory loss (17.5%) and peripheral neuropathic pain (5%).Nineteen patients (47.5%) had gluten sensitivity, of whom 3 patients (7.5%) had biopsy proven coeliac disease. Other abnormal immunological tests were present in another 15 patients. Six patients had malignancy, which was diagnosed within 5 years of the neurological symptoms. Only 3 patients (7.5%) were classified as having a truly idiopathic combination of cerebellar ataxia with SG. Conclusion: Our case series highlights that amongst patients with the unusual combination of cerebellar ataxia and SG, immune pathogenesis plays a significant role

Psychiatric manifestations of coeliac disease, a systematic review and meta-analysis

Background: Coeliac disease (CD) is increasingly prevalent and is associated with both gastrointestinal (GI) and extra-intestinal manifestations. Psychiatric disorders are amongst extra-intestinal manifestations proposed. The relationship between CD and such psychiatric disorders is not well recognised or understood. Aim: The aim of this systematic review and meta-analysis was to provide a greater understanding of the existing evidence and theories surrounding psychiatric manifestations of CD. Methodology: An online literature search using PubMed was conducted, the prevalence data for both CD and psychiatric disorders was extracted from eligible articles. Meta analyses on odds ratios were also performed. Results: A total of 37 articles were included in this review. A significant increase in risk was detected for autistic spectrum disorder (OR 1.53, 95% CI 1.24–1.88, p < 0.0001), attention deficit hyperactivity disorder (OR 1.39, 95% CI 1.18–1.63, p < 0.0001), depression (OR 2.17, 95% CI 2.17–11.15, p < 0.0001), anxiety (OR 6.03, 95% CI 2.22–16.35, p < 0.0001), and eating disorders (OR 1.62, 95% CI 1.37–1.91, p < 0.00001) amongst the CD population compared to healthy controls. No significant differences were found for bipolar disorder (OR 2.35, 95% CI 2.29–19.21, p = 0.43) or schizophrenia (OR 0.46, 95% CI 0.02–10.18, p = 0.62). Conclusion: CD is associated with an increased risk of depression, anxiety, eating disorders as well as ASD and ADHD. More research is required to investigate specific biological explanations as well as any effect of gluten free diet

Alcohol-induced autonomic dysfunction: a systematic review

Purpose Autonomic dysfunction is a known consequence of chronic and excessive alcohol consumption. The aim of this systematic review was to characterise this phenomenon, describe the frequency at which it occurs and to explore the best management strategies. Methods A systematic, computer-based search was conducted using the PubMed database. All studies identified by the search were evaluated independently by at least three authors. For inclusion, studies had to report human subjects consuming ethanol in excess. Case reports and non-original studies were excluded from this review. Results A total of 55 studies were included in this review. According to cardiovascular reflex tests, 16–73% of chronic alcohol abusers suffer from autonomic dysfunction. The most commonly occurring symptom is erectile dysfunction, whilst other features such as postural dizziness are rare. The most important risk factor for this condition is total lifetime dose of ethanol, although there is mixed evidence supporting the role of other risk factors. The only management strategy currently explored in the literature is abstinence, which appears to lead to significant improvement in autonomic investigations. Conclusion Current literature includes studies of highly heterogeneous populations, consuming differing volumes of alcohol over variable periods of time and utilising a number of different autonomic test batteries and criteria to diagnose autonomic dysfunction. Therefore, further research using homogeneous methods for measuring autonomic dysfunction in the field is needed. Despite this limitation, our review demonstrated that autonomic dysfunction is very common among alcohol abusers

Painful peripheral neuropathy and cancer

Peripheral neuropathy (PN) is very prevalent in cancer patients and a leading cause of pain related to cancer. However, the underlying pathophysiological mechanisms vary significantly. Peripheral neuropathy can be a direct or an indirect complication of cancer or cancer-related treatment, or a pre-existing comorbidity not related to cancer. PN might also occur as a paraneoplastic neurological syndrome. Such syndromes are immune-mediated manifestations that usually precede the diagnosis of cancer or cancer's relapse. Pain is very prevalent in paraneoplastic peripheral neuropathies and, therefore, merits attention

COVID-19-related burning eye syndrome and burning mouth syndrome: a systematic review and meta-analysis

Background: Previous research highlights burning eye syndrome (BES) and burning mouth syndrome (BMS) as chronic complications of COVID-19 infection. The aim of this systematic review and meta-analysis is to establish the prevalence of COVID-19-related BES and COVID-19-related BMS and describe their phenomenology. Methodology: A literature search in the PubMed database was performed, and seven papers (five on BES and two on BMS) were eligible to be included in this systematic review and meta-analysis. Results: The pooled prevalence of COVID-19-related BES was 9.9% (95% CI 3.4–25.4%). The frequency of COVID-19-related BMS is only reported in isolated cases and ranges from 4% in mild-to-moderate cases to 15% in severe, hospitalized cases, with female patients being mostly affected. COVID-19 severity is a potential risk factor for both BES and BMS. Neither syndrome occurs in isolation. COVID-19-related BES usually appears within the first week post-infection, persisting up to 9 weeks later. COVID-19-related BMS occurs during and after initial infection, and may also persist as a chronic disease. Conclusions: Both BES and BMS are neuropathic COVID-19 infection complications, still under-studied and under-investigated, despite the fact that both are prevalent. Both COVID-19-related BES and COVID-19-related BMS could potentially be initial long COVID syndrome manifestations, and further research should be carried out in this field

B12 as a treatment for peripheral neuropathic pain : a systematic review

Neuropathic pain describes a range of unpleasant sensations caused by a lesion or disease of the somatosensory nervous system. The sensations caused by neuropathic pain are debilitating and improved treatment regimens are sought in order to improve the quality of life of patients. One proposed treatment for neuropathic pain is vitamin B12, which is thought to alleviate pain by a number of mechanisms including promoting myelination, increasing nerve regeneration and decreasing ectopic nerve firing. In this paper, the evidence for B12 as a drug treatment for neuropathic pain is reviewed. Twenty four published articles were eligible for inclusion in this systematic review in which a range of treatment regimens were evaluated including both B12 monotherapy and B12 in combination with other vitamins or conventional treatments, such as gabapentinoids. Overall, this systematic review demonstrates that there is currently some evidence for the therapeutic effect of B12 in the treatment of post-herpetic neuralgia (level II evidence) and the treatment of painful peripheral neuropathy (level III evidence)

Subspecialty preferences among Neurologists of the future.

INTRODUCTION: In the era of neurological subspecialization, most neurologists will have a field of specialist interest. The aim of this cross-sectional multi-national study was to identify the key areas of interest among trainees or junior specialists, assess the potential influence of an interest in research, and consider the results in light of population needs. METHODS: A total of 300 residents and junior neurologists who received a bursary to attend the European Academy of Neurology conference were invited to participate in this study. Demographic and work-related characteristics, as well as main subspecialty of choice were examined via an anonymous electronic questionnaire. Participants holding a higher degree (PhD/MD) or working in research posts were considered research oriented. RESULTS: In total, 191 Neurologists in training or junior specialists responded (response rate 63.7%). Full data were available for 187 participants (59.4% females). The study sample had a mean age of 30.5±3.4 years (range 25 - 45). The most popular subspecialty was movement disorders (18.2%), followed by multiple sclerosis (11.2%) and epilepsy (10.2%). This did not differ significantly between the participants who were or were not research-oriented. CONCLUSIONS: There is a potential mismatch between the interests of trainees, and the future needs of the populations they serve, which it is important to identify for workforce planning

Behavioral therapy approaches for the management of low back pain: An up-to-date systematic review

Low back pain is one of the most common causes for seeking medical treatment and it is estimated that one in two people will experience low back pain at some point during their lifetimes. Management of low back pain includes pharmacological and non-pharmacological approaches. Non-pharmaceutical treatments include interventions such as acupuncture, spinal manipulation, and psychotherapy. The latter is especially important as patients who suffer from low back pain often have impaired quality of life and also suffer from depression. Depressive symptoms can appear because back pain limits patients' ability to work and engage in their usual social activities. The aim of this systematic review was to overview the behavioral approaches that can be used in the management of patients with low back pain. Approaches such as electromyography (EMG) biofeedback, cognitive behavioral therapy, and mindfulness-based stress reduction are discussed as non-pharmacological options in the management of low back pain

Headache associated with coeliac disease: a systematic review and meta-analysis

OBJECTIVE: The aim of this systematic review was to explore the relationship between coeliac disease (CD) and headache. The objectives were to establish the prevalence of each entity amongst the other, to explore the role of gluten free diet (GFD), and to describe the imaging findings in those affected by headaches associated with CD. METHODOLOGY: A systematic computer-based literature search was conducted on the PubMed database. Information regarding study type, population size, the age group included, prevalence of CD amongst those with headache and vice versa, imaging results, the nature of headache, and response to GFD. RESULTS: In total, 40 articles published between 1987 and 2017 qualified for inclusion in this review. The mean pooled prevalence of headache amongst those with CD was 26% (95% CI 19.5⁻33.9%) in adult populations and 18.3% (95% CI 10.4⁻30.2%) in paediatric populations. The headaches are most often migraine-like. In children with idiopathic headache, the prevalence of CD is 2.4% (95% CI 1.5⁻3.7%), whereas data for adult populations is presently unavailable. Brain imaging can be normal, although, cerebral calcifications on CT, white matter abnormalities on MRI and deranged regional cerebral blood flow on SPECT can be present. GFD appears to be an effective management for headache in the context of CD, leading to total resolution of headaches in up to 75% of patients. CONCLUSIONS: There is an increased prevalence of CD amongst idiopathic headache and vice versa. Therefore, patients with headache of unknown origin should be screened for CD, as such patients may symptomatically benefit from a GFD

Increased oxidative stress as a risk factor in chronic idiopathic axonal polyneuropathy

Chronic idiopathic axonal polyneuropathy (CIAP) is a disorder with insidious onset and slow progression, where no etiology is identified despite appropriate investigations. We aimed to investigate the role of oxidative stress as a risk factor for the pathogenesis of CIAP. Sera of patients with CIAP were tested for protein carbonyl (PC) and 8-hydroxydeoxyguanosine (8H). As a control group, we recruited patients with gluten neuropathy. Twenty-one patients with CIAP and 21 controls were recruited. The two groups did not differ significantly regarding demographics or clinical characteristics (i.e., neuropathy type or disease severity). After adjusting for gender, having CIAP was positively correlated with both the 8H titer (standardized beta coefficient 0.349, p = 0.013) and the PC titer (standardized beta coefficient 0.469, p = 0.001). Oxidative stress appears to be increased in CIAP and might have a role in the pathogenesis of the disease