BMJ Neurology Open

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EISSN : 2632-6140
Published by: BMJ (10.1136)
Total articles ≅ 70
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Mohamad Shadi Alkarrash, , Mohammad Nour Kitaz, Roaa Rhayim, Mahmoud Mohamad Alhasan, Maya Alassadi, Ahmad Aldakhil, Mohamad Alkhamis, Mohanad Ajam, Mohamad Douba, et al.
Published: 13 October 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000211

Abstract:
Introduction Headache disorders are among the most common 10 causes of disability worldwide according to the global burden of disease survey 2010. Headache is also wildly common among universities students when compared with other populations. The purpose of this study is to assess headache prevalence among Aleppo University medical, dental and pharmaceutical undergraduate students. Methods A questionnaire-based cross-sectional study was conducted among medical, dental and pharmaceutical students at Aleppo University, Syria. We determined the type of headache according to the International Classification of Headache Disorder-III. The total number of participants was 2068. A χ2 test was used to evaluate the association between the categorical outcomes. P<0.05 was considered significant. Results Out of 2068 participants, 1604 (77.6%) were medical students, 205 (9.9%) were dental students and 259 (12.5%) were pharmaceutical students. The effect on daily activities was higher in chronic tension headache (96.7%) and migraine without aura (94.6%) than migraine with aura (91.3) and episodic tension headache (85.1%). Out of 1191 who had a headache, only 188 (15.9%) had a medical consultation. Conclusions There was no a statistically significant difference in prevalence of tension headache and migraine according to faculties. There was a statistically significant difference in patients with migraine according to academic year, living with family and smoking. The effect on daily activities was higher in chronic tension-type headache and migraine without aura. There is a significant lack of medical consultation among students and most of them took over the counter analgesics depending on personal choice.
Saiumaeswar Yogakanthi, Christine Wools, Susan Mathers
Published: 11 October 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000205

Abstract:
Background Bulbar involvement is a recognised feature of motor neuron disease/amyotrophic lateral sclerosis (MND/ALS), both as a presenting complaint and as a consequence of advancing disease. Hoarseness and dysphonia have been associated with vocal cord abductor weakness. This is usually bilateral and has also been reported as the presenting clinical feature in a handful of patients with superoxide dismutase 1 (SOD1) gene mutations. Presentation with an isolated, unilateral vocal cord adductor weakness, however, is atypical and rare. Case In this report, we detail the case of a 38-year-old woman with dysphonia and a family history of an SOD1 mutation. Neurological features remained confined to the territory of the left vagus nerve for the next 12 months, before a more rapid rate of disease dissemination and progression. Conclusions This case highlights the importance of recognition of vocal cord palsy as an early manifestation of MND/ALS and the critical need for monitoring to recognise potential disease progression.
Alexander Bryson
Published: 27 September 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000193

Abstract:
Background Hemicrania continua is an uncommon subtype of trigeminal autonomic cephalgia that exhibits dramatic therapeutic response to indomethacin. Unfortunately, indomethacin is associated with a range of adverse effects, including neuropsychiatric complications, which limits its use in many patients. Although no other effective pharmacologic agents exist, there is emerging evidence for interventional treatments such as occipital nerve and vagus nerve stimulation, which may act by modulating neural activity within the trigeminovascular system. Case We present a 30-year-old woman with long-standing refractory hemicrania continua who suffered adverse effects to indomethacin. She experienced temporary, but near-complete, symptom resolution following piercing of the crus of the ear helix ipsilateral to her headache, whereas contralateral piercing produced no benefit. Conclusions To our knowledge, this case is the first to describe a therapeutic benefit following ear piercing in a patient with trigeminal autonomic cephalgia. We argue that symptom relief was obtained through a similar mechanism to occipital or vagus nerve stimulation.
Ivo Bekavac, Matea Matejic, Riley J Woods, John I Halloran
Published: 6 September 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000174

Abstract:
Objective To present a case of two identical twins presenting concurrently with symptoms and subsequent initial diagnosis of neuromyelitis optica spectrum disorder (NMOSD). Methods Clinical, laboratory and MRI findings for both twins were reviewed and presented here. Results Twin A presented with right eye pain and subsequent blurred vision in right eye. MRI of the brain and spine demonstrated pre-chiasmal right optic nerve enhancement and T2 hyperintense lesions in the spinal cord at T7 and T9 levels. Cerebrospinal fluid (CSF) analysis was remarkable for NMO/aquaporin-4 (AQP4) fluorescence-activated cell sorting (FACS) titre of 1:32 and a serum NMO/AQP4-IgG positive titre of 1:10 000. Twin B presented with diplopia. MRI of the brain and spine demonstrated T2 hyperintense lesions in the periventricular cerebral white matter, in the periaqueductal white matter of the pons, in the midbrain and the cervical spinal cord. Neurological examination findings revealed incomplete right trochlear palsy, rotatory nystagmus, an incomplete left internuclear ophthalmoplegia and hyper-reflexia. CSF analysis was remarkable for NMO/AQP4 FACS titre of 1:256 and a serum NMO-IgG positive titre of 1:10 000. Both twins responded well to intravenous steroid therapy. There was no adverse environmental exposure present. Conclusion We present an interesting and rare case of identical twins presenting concurrently and for the first time with NMOSD.
Bridget J Chen, Nicholas O Daneshvari,
Published: 17 August 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000156

Abstract:
Background Intravenous tissue plasminogen activator (rtPA) and arterial endovascular therapy (ET) rapidly restore cerebral perfusion in eligible patients who had an acute ischaemic stroke (AIS). It is unknown whether patients who had an AIS with premorbid cardiac disease respond differently to reperfusion therapies than those without. These patients may have risk factors that worsen outcomes or may represent those who would most benefit from reperfusion therapy. Objective To determine whether patients who had an AIS with the most frequently encountered pre-existing cardiac conditions, atrial fibrillation (AF), heart failure (HF), left ventricular assist devices (LVADs), or taking anticoagulation for cardiac indications, are at increased risk for poor outcome, such as symptomatic intracranial haemorrhage (sICH), after reperfusion therapy. Results Although AF is an independent risk factor for poor poststroke outcomes, intravenous rtPA is not associated with increased risk of sICH for those not on anticoagulants. Likewise, HF is independently associated with mortality post stroke, yet these patients benefit from reperfusion therapies without increased rates of sICH. Patients with LVADs or who are on anticoagulation should not be given IV rtPA; however, ET remains a viable option in those who meet criteria, even patients with LVAD. Conclusion There is no evidence of an increased risk for sICH after intravenous rtPA or ET for those with AF or HF. Intravenous rtPA should not be given to patients on anticoagulation or with LVADs, but ET should be offered to them when eligible. Whenever possible, future AIS reperfusion research should include patients with premorbid cardiac disease as they are frequently excluded, representing a gap in evidence.
, Steven Severyn, Songzhu Zhao, David Kline, Matthew Linsenmayer, Kristina Kelly, Marco Tellez, Amy Bartlett, Sarah Heintzman, Jerry Reynolds, et al.
Published: 12 August 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000164

Abstract:
Objective Spinal muscular atrophy (SMA) is a motor neuron disease caused by low levels of survival motor neuron (SMN) protein. Prior work in models and patients has demonstrated electrophysiological and morphological defects at the neuromuscular junction (NMJ). Therapeutic development has resulted in clinically available therapies to increase SMN protein levels in patients and improve muscle function. Here we aimed to investigate the effect of SMN restoration (via nusinersen) on NMJ transmission in adults with SMA. Methods Participants undergoing nusinersen treatment underwent 3 Hz repetitive nerve stimulation (RNS) of the spinal accessory nerve to assess compound muscle action potential amplitude decrement. Maximum voluntary isometric contraction (MVICT), Revised Upper Limb Module (RULM), and 6 min walk test (6MWT) were assessed for correlations with decrement. Results Data from 13 ambulatory (7 men/6 women, mean age 40±11 years) and 11 non-ambulatory (3 men/8 women, mean age 38±12 years) participants were analysed. Cross-sectional analyses of RNS decrement were similar at 14 months of nusinersen (−14.2%±11.5%, n=17) vs baseline (−11.9%±8.3%, n=15) (unpaired t-test, p=0.5202). Longitudinal comparison of decrement in eight participants showed no change at 14 months (−13.9%±6.7%) vs baseline (−16.9%±13.4%) (paired t-test, p=0.5863). Decrement showed strong correlations with measures of MVICT, RULM and 6MWT but not age or disease duration. Conclusion Adults with SMA had significant NMJ transmission defects that were not corrected with 14 months of nusinersen treatment. NMJ defects were negatively associated with physical function, and thus may represent a promising target for additive or combinatorial treatments.
Mariarosaria Valente, , Daniele Bagatto, Riccardo Garbo, Christian Lettieri, Andrea Bernardini, Annacarmen Nilo, Maria Rosaria Peri, Davide Pecori, Gian Luigi Gigli
Published: 23 July 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000176

Abstract:
Background Subacute sclerosing panencephalitis is a progressive neurodegenerative disorder caused by a latent and mutant measles virus which is extremely rare in developed countries. The lack of effective treatments leads to the research of other anti-inflammatory and neuroprotective treatments. Case Here we present a case of a 17-year-old patient affected by subacute sclerosing panencephalitis who manifest a dramatic improvement in neurological and general clinical conditions, as well as an arrest in the progression of demyelinating process in the central nervous system, after the beginning of a high ratio ketogenic diet. Conclusions Given its anti-inflammatory, antioxidant and metabolic effects, we believe that ketogenic diet utilisation could be a rational approach, can be considered a safe add-on therapy, carrying on with only a minimal risk of adverse effects or interactions.
Michele De Sciscio, Paul De Sciscio, Wilson Vallat, Timothy Kleinig
Published: 13 July 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2021-000166

Abstract:
Background and aims Having anecdotally noted a high frequency of lobar-restricted cerebral microbleeds (CMBs) mimicking cerebral amyloid angiopathy (CAA) in patients with previous cardiac surgery (especially valve replacement) presenting to our transient ischaemic attack (TIA) clinic, we set out to objectively determine the frequency and distribution of microbleeds in this population. Methods We performed a retrospective comparative cohort study in consecutive patients presenting to two TIA clinics with either: (1) previous coronary artery bypass grafting (CABG) (n=41); (2) previous valve replacement (n=41) or (3) probable CAA (n=41), as per the Modified Boston Criteria, without prior cardiac surgery. Microbleed number and distribution was determined and compared. Results At least one lobar-restricted microbleed was found in the majority of cardiac surgery patients (65%) and 32/82 (39%) met diagnostic criteria for CAA. Valve replacement patients had a higher microbleed prevalence (90 vs 51%, p<0.01) and lobar-restricted microbleed count (2.6±2.7 vs 1.0±1.4, p<0.01) than post-CABG patients; lobar-restricted microbleed count in both groups was substantially less than in CAA patients (15.5±20.4, p<0.01). In postcardiac surgery patients, subcortical white matter (SWM) microbleeds were proportionally more frequent compared with CAA patients. Receiver operator curve analysis of a ‘location-based’ ratio (calculated as SWM/SWM+strictly-cortical CMBs), revealed an optimal ratio of 0.45 in distinguishing cardiac surgery-associated microbleeds from CAA (sensitivity 0.56, specificity 0.93, area under the curve 0.71). Conclusion Lobar-restricted microbleeds are common in patients with past cardiac surgery, however a higher proportion of these CMBs involve the SWM than in patients with CAA.
, Chiayi Chen, Tamela Stuchiner, Lindsay Lucas, Lois Grote, Stanley Cohan
Published: 7 July 2021
by BMJ
BMJ Neurology Open, Volume 3; https://doi.org/10.1136/bmjno-2020-000108

Abstract:
Background To monitor long-term outcomes of ocrelizumab treatment. Objective To evaluate safety and treatment outcomes of ocrelizumab in a community-based multiple sclerosis (MS) population. Methods Adult patients with MS prescribed ocrelizumab were eligible. Chart reviews were conducted at the start of ocrelizumab treatment and every 6 months thereafter. Results Of the 355 patients enrolled, 71.9% were female; mean (SD) age was 51.8 (12.5) years; 78.3% had relapsing MS (RMS). Median baseline Expanded Disability Status Scale (EDSS) (IQR) was 3.0 (2.0–4.0) for RMS, 6.5 (6.0–7.5) for secondary progressive MS, and 6.5 (6.0–7.0) for primary progressive MS. Respiratory infections occurred in 40.1% and urinary tract infections in 33.1% of patients. There was no difference in the percentage of infections among patients <55 (68.5%, n=122), and those ≥55 of age (67.5%, n=104) (p=0.94). Twenty-five hospitalisations were due to infections; 69.2% of these patients were ≥55 with a mean EDSS of 5.7 (±1.86). Four patients have died. Serum IgM and IgG levels did not predict infection risk. Annualised relapse rate was 0.34 for the patients with RMS in the preceding 2 years and 0.09 in patients who received ≥2 ocrelizumab 600 mg courses. The first on-treatment MRI was stable in 262 (90.0%) patients, 6.9% had new T2 lesions, 2.7% had enlarging T2 lesions and 1.4% had gadolinium-enhancing lesions. Median EDSS at 12 months was unchanged. Conclusion Ocrelizumab effectively controlled relapse risk and disability worsening. Although only 12.1% of patients have discontinued ocrelizumab, infections resulting in hospitalisation are a concern, especially in older and disabled patients.
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