Neurology and neurorehabilitation

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Research outputs from the Neurology department at the RD&E.

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    Decline in Cognition from Mid-Life Improves Specificity of Mini-Mental State Examination: Diagnostic Test Accuracy in Caerphilly Prospective Study (CaPs)
    (IOS Press, 2022-08-18) Creavin, S.; Fish, M.; Bayer, A.; Gallacher, J.; Ben-Shlomo, Y.
    BACKGROUND: The merit of using baseline cognitive assessments in mid-life to help interpret cross-sectional cognitive tests scores in later life is uncertain. OBJECTIVE: Evaluate how accuracy for diagnosing dementia is enhanced by comparing cross-sectional results to a midlife measure. METHODS: Cohort study of 2,512 men with repeated measures of Mini-Mental State Examination (MMSE) over approximately 10 years. Index test MMSE at threshold of 24 indicating normal, as a cross-sectional measure and in combination with decline in MMSE score from mid-life. Reference standard consensus clinical diagnosis of dementia by two clinicians according to Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV). RESULTS: 1,150 men participated at phase 4 of whom 75 had dementia. A cross-sectional MMSE alone produced a sensitivity of 60% (50% to 70% ) and specificity 95% (94% to 97% ) with a threshold of≥24 points indicating normal. For lower-scoring men in late life, with cross sectional scores of <  22, combining cross-sectional AND a three-point or more decline over time had a sensitivity of 52% (39% to 64% ) and specificity 99% (99% to 100% ). For higher-scoring men in later life, with cross sectional scores <  26 combining cross-sectional OR decline of at least three points had a sensitivity of 98% (92% to 100% ) and specificity 38% (32% to 44% ). CONCLUSION: It may be helpful in practice to formally evaluate cognition in mid-life as a baseline to compare with if problems develop in future, as this may enhance diagnostic accuracy and classification of people in later life.
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    Bickerstaff brainstem encephalitis: clinical, neurophysiological, laboratory and postmortem findings of a case presenting as encephalomyelitis
    (BMJ, 2022-02-02) Imam, I.; Sarrigiannis, P. G.; Shivane, A. G.
    Our patient was admitted to hospital with a 1-week history of an upper respiratory tract infection and a rapidly progressive encephalopathy dominated by brainstem features and widespread areflexia. Her antiganglioside antibodies and electroencephalography were consistent with Bickerstaff brainstem encephalitis (BBE), and her postmortem examination revealed a predominantly florid brainstem encephalitis and myelitis. Her sputum and throat swabs isolated Haemophilus influenzae and Fusobacterium, respectively, the former being the most probable trigger of BBE. Our patient's death, despite the otherwise good prognosis of the disorder, may reflect the severity of the pathological changes at postmortem or the association of comorbid disorders such as sepsis-associated encephalopathy. Her poor outcome may also be an indication to treat rapidly progressive cases of BBE with more than one immune modulating drug.
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    A multiple sclerosis nurse specialist narrative, focusing on supporting people to manage cognitive changes
    (Mark Allen Group, 2022-01-01) Jarrett, Louise
    Background: Cognitive changes are common in people with multiple sclerosis (MS). Interventions from health professionals can focus on assessment, measurement and advice on management strategies. Such interventions are often delivered in a discreet number of planned therapy sessions. MS nurse specialists plan appointments and education sessions, but also provide access for a person with MS to seek unplanned support for example via MS nurse advice lines or digital services. Aim: This article explores my role as an MS specialist nurse. Centring on reflective practice as a tool to access my nursing experience. Specialist nurse interventions to support people with MS to manage their cognitive function are highlighted. Method: Narrative methodology was used to reflect on my practice over a 10-month period. Findings: Insights into my practice are presented as a narrative text. A fundamental thread emerged of cultivating trust and a therapeutic relationship. Strategies included: being with people on their MS journey, informing, educating, confirming and legitimising their experiences; completing ongoing assessments and monitoring; making timely interventions and assessing their impact; acknowledging and working with an individual's decisions and recognising opportunities to suggest change; discussing research opportunities and considering the impact of medicines and comorbidities. Conclusion: This narrative is unique to my nursing practice and aims to resonate with the practice of other nurses. It demonstrates that an MS specialist nurse can provide timely advice, reinforcement of management strategies, and space for a person with MS to explore how their cognition may be affecting them. MS nurses have the opportunity to play a vital role in supporting people with MS to manage cognitive changes throughout the disease trajectory.
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    The impact of smoking cessation on multiple sclerosis disease progression
    (Oxford University Press, 2021-10-08) Rodgers, J.; Friede, T.; Vonberg, F. W.; Constantinescu, C. S.; Coles, A.; Chataway, J.; Duddy, M.; Emsley, H.; Ford, H.; Fisniku, L.; Galea, I.; Harrower, T.; Hobart, J.; Huseyin, H.; Kipps, C. M.; Marta, M.; McDonnell, G. V.; McLean, B.; Pearson, O. R.; Rog, D.; Schmierer, K.; Sharrack, B.; Straukiene, A.; Wilson, H. C.; Ford, D. V.; Middleton, R. M.; Nicholas, R.
    The negative impact of smoking in MS is well established, however, there is much less evidence as to whether smoking cessation is beneficial to progression in MS. Adults with MS registered on the United Kingdom MS Register (2011-2020) formed this retrospective and prospective cohort study. Primary outcomes were changes in 3 patient reported outcomes (PROs): normalised MS Physical Impact Scale (MSIS-29-Phys), normalised MS Walking Scale (MSWS-12) and the Hospital Anxiety and Depression Scale (HADS-Anxiety and HADS-Depression). Time to event outcomes were clinically significant increases in the PROs. 7983 participants were included, 4130 (51.7%) of these had ever smoked; of whom 1315 (16.5%) were current smokers and 2815/4130 (68.2%) were former smokers. For all PROs, current smokers at the time of completing their first questionnaire had higher PRO scores indicating higher disability compared to those who had never smoked (∼10 points difference in MSIS-29-Phys and MSWS-12; 1.5-1.8 point for HADS-anxiety and HADS-depression). There was no improvement in PRO scores with increasing time since quitting in former smokers. 923 participants formed the prospective parallel group, which demonstrated that MSIS-29-phy 5.03, [3.71, 6.34], MSWS-12 5.28, [3.62, 6.94] and HADS-depression 0.71, [0.47, 0.96] worsened over a period of 4 years, whereas HADS-anxiety remained stable. Smoking status was significant at year 4; current smokers had higher MSIS-29-Phys and HADS-Anxiety scores (3.05 [0.22, 5.88], 1.14 [0.52,1.76]) while former smokers had a lower MSIS-29 score of -2.91[-5.03, -0.79]. 4642 participants comprised the time to event analysis. Still smoking was associated with a shorter time to worsening event in all PROs (MSIS-29-Phys: n = 4436, p = 0.0013; MSWS-12: n = 3902, p = 0.0061; HADS-anxiety: n = 4511, p = 0.0017; HADS-depression: n = 4511, p < 0.0001). Worsening in motor disability (MSIS-29-Phys and MSWS-12) was independent of baseline HADS-anxiety and HADS-depression scores. There was no statistically significant difference in the rate of worsening between never and former smokers. When smokers quit, there is a slowing in the rate of motor disability deterioration so that it matches the rate of motor decline in those who have never smoked. This suggests that smoking cessation is beneficial for people with MS.
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    Sudomotor dysfunction in patients with gluten neuropathy
    (Springer, 2021-11-01) Zis, P.; Shafique, F.; Sarrigiannis, P. G.; Artemiadis, A.; Rao, D. G.; Sanders, D. S.; Hadjivassiliou, M.
    BACKGROUND AND AIM: Gluten neuropathy (GN) is a common neurological manifestation of gluten sensitivity (GS), characterized by serological evidence of GS, while other risk factors for developing neuropathy are absent. The degree of small fiber dysfunction in GN has not been studied in depth to date. Small fiber involvement may lead to pain, thermal perception abnormalities, and sweat gland dysfunction. Sudomotor innervation refers to the cholinergic innervation of the sympathetic nervous system through small fibers in the sweat glands. The aim of our study was to assess the sudomotor function of GN patients. METHODS: Patients with GN were recruited. Clinical and neurophysiological data were obtained. HLA-DQ genotyping was performed. The skin electrochemical conductance (ESC) was measured with SUDOSCAN(TM). RESULTS: Thirty-two patients (25 males, mean age 69.5±10.2 years) were recruited. Thirteen patients (40.6%) had abnormal sudomotor function of the hands. Sixteen patients (50%) had abnormal sudomotor function of the feet. Twenty-one patients (65.6%) had abnormal sudomotor function of either the hands or feet. Sudomotor dysfunction did not correlate with the type of neuropathy (length-dependent neuropathy or sensory ganglionopathy), gluten-free diet adherence, severity of neuropathy, and duration of disease or HLA-DQ genotype. No differences in the ESC were found between patients with painful and patients with painless GN. CONCLUSION: Sudomotor dysfunction affects two-thirds of patients with GN. The lack of correlation between pain and sudomotor dysfunction suggests different patterns of small fiber involvement in patients with GN.