BMC Neurology

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ISSN / EISSN : 1471-2377 / 1471-2377
Current Publisher: Springer Science and Business Media LLC (10.1186)
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Nazibrola Botchorishvili, Nino Shiukashvili, Nina Mikeladze, Ann Dzagnidze, Nino Mikava, Maia Tighashvili, Marina Janelidze
BMC Neurology, Volume 21, pp 1-6; doi:10.1186/s12883-021-02249-x

Abstract:
Background Cognitive impairment is one of the common features of multiple sclerosis (MS). Despite high prevalence, cognitive decline is often overlooked by neurologists. The Brief International Cognitive Assessment for MS (BICAMS) was therefore introduced by the international expert committee as a brief and effective tool for the assessment and monitoring of cognitive functions in patients with MS. The validity and reliability of BICAMS have been demonstrated in many countries. Our aim was to validate the BICAMS in Georgian patients with MS. Methods A total of 68 patients with MS and 68 matched controls were assessed by the Georgian-language BICAMS. All healthy controls and seven patients were re-evaluated with identical tests to assess retest reliability. Results In comparison to healthy controls, patients with MS performed significantly worse on all tests in the assessment battery. Test–retest reliability measures were good for all tests. The prevalence of cognitive impairment in patients with MS was 43%. Conclusion The Georgian-language BICAMS is a reliable and valid battery for the assessment of cognitive function in patients with MS.
Zheng Wang, Jiangyong Miao, Lina Wang, Ying Liu, Hui Ji, Xiangjian Zhang, Lili Cui
BMC Neurology, Volume 21, pp 1-6; doi:10.1186/s12883-021-02236-2

Abstract:
Background Presentation with massive systemic embolization as the initial manifestation of occult malignancy is infrequent. The standard management of cancer-related arterial thromboembolism has not yet been established. Case presentation We described a case of Trousseau’s syndrome resulting in acute ischemic stroke concomitant with multiple embolizations in the spleen and kidney during oral administration of dabigatran for pulmonary embolism preceding the diagnosis of a malignant tumor. A cancer-related hypercoagulable state was suspected because the patient was admitted to the neurology department due to acute ischemic stroke with three territory infarcts on diffusion-weighted imaging (DWI) in the absence of identifiable conventional risk factors and brain vessel narrowing. The patient was subsequently diagnosed with epidermal growth factor receptor (EGFR) mutation–positive non-small-cell lung cancer (NSCLC) (stage IV) with pleural metastasis. Administration of low-molecular-weight heparin followed by long-term dabigatran under effective cancer therapy comprising gefitinib and subsequent chemotherapy did not cause stroke relapse during the 1-year follow-up. Conclusions This case suggests that cancer-related hypercoagulability should be considered an important etiology for stroke patients who develop unexplained disseminated acute cerebral infarction without conventional stroke risk factors, especially concomitant with multiple organ embolization. Novel oral anticoagulants may be an alternative therapy for the long-term management of cancer-related arterial thromboembolism under effective cancer therapy.
Chaohua Cui, Yanbo Li, Jiajia Bao, Shuju Dong, Lijie Gao, Li He
BMC Neurology, Volume 21, pp 1-9; doi:10.1186/s12883-021-02259-9

Abstract:
Background For acute ischaemic stroke patients, it is uncertain whether intravenous thrombolysis combined with statins might increase the therapeutic effect. Additionally, using high-intensity statins after thrombolysis may increase the risk of bleeding in patients. Asian stroke patients often take low-dose statins. It is speculated that reducing the dose of statins may improve the risk of bleeding. Methods Data from consecutive acute ischaemic stroke patients with intravenous thrombolysis were prospectively collected. Efficacy outcomes included NIHSS (National Institutes of Health Stroke Scale) score improvement at 7 days after admission and mRS (Modified Rankin Scale) improvement at 90 days. Safety outcomes included haemorrhage events (intracerebral haemorrhage and gastrointestinal haemorrhage) in the hospital and death events within 2 years. Results The study finally included 215 patients. The statin group had a higher percentage of NIHSS improvement at 7 days (p < 0.001) and a higher percentage of a favourable functional outcome (FFO, mRS < = 2) (p < 0.001) at 90 days. The statin group had a lower percentage of intracerebral haemorrhage (p < 0.001) and gastrointestinal haemorrhage (p = 0.003) in the hospital and a lower percentage of death events (p < 0.001) within 2 years. Logistic regression indicated that statin use was significantly related to NIHSS improvement (OR = 4.697, p < 0.001), a lower percentage of intracerebral haemorrhage (OR = 0.372, p = 0.049) and gastrointestinal haemorrhage (OR = 0.023, p = 0.016), and a lower percentage of death events (OR = 0.072, p < 0.001). Conclusion For acute ischaemic stroke patients after intravenous thrombolysis, the use of low-dose statins was related to NIHSS improvement at 7 days and inversely related to haemorrhage events in the hospital and death events within 2 years, especially for moderate stroke or noncardioembolic stroke patients.
Yuying Yan, Shuai Jiang, Ruilin Wang, Xiang Wang, Peng Li,
BMC Neurology, Volume 21, pp 1-5; doi:10.1186/s12883-021-02250-4

Abstract:
Background Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is an adult-onset rare monogenic microvasculopathy. Its typical neuroimaging features are punctate white matter lesions or pseudotumor alterations. RVCL-S is often under-recognized and misdiagnosed because of its rarity and similar imaging manifestations to multiple sclerosis or brain malignant mass. Case presentation Here we report a case of a 36-year-old Chinese man who developed multiple tumefactive brain lesions spanning over two years leading to motor aphasia, cognitive decline, and limb weakness. He also presented with slight vision loss, and fundus fluorescein angiography indicated retinal vasculopathy. He underwent brain biopsies twice and showed no evidence of malignancy. Given the family history that his father died of a brain mass of unclear etiology, RVCL-S was suspected, and genetic analysis confirmed the diagnosis with a heterozygous insertion mutation in the three-prime repair exonuclease 1 gene. He was given courses of corticosteroids and cyclophosphamide but received little response. Conclusions The present case is one of the few published reports of RVCL-S with two-year detailed imaging data. Serial magnetic resonance images showed the progression pattern of the lesions. Our experience emphasizes that a better understanding of RVCL-S and considering it as a differential diagnosis in patients with tumefactive brain lesions may help avoid unnecessary invasive examinations and make an earlier diagnosis.
Makoto Shibata, Reona Hoshino, Chisato Shimizu, Masayuki Sato, Natsumi Furuta,
BMC Neurology, Volume 21, pp 1-5; doi:10.1186/s12883-021-02253-1

Abstract:
Background Lacosamide (LCM) is the antiepileptic drug approved by the U.S. Food and Drug Administration in 2008 that facilitates slow activation of the voltage-gated sodium channels. Neutropenia and cardiac events including sinus node dysfunction (SND) and atrioventricular block have been previously reported as adverse effects of LCM. To date, there have been no reports of severe agranulocytosis resulting in death associated with LCM. Additionally, there have been no reports of concomitant SND and agranulocytosis after LCM administration. Herein we report the first case of LCM-induced severe SND followed by agranulocytosis. Case presentation The patient with focal epilepsy was initiated on LCM 100 mg/day and the dose was increased to 200 mg/day on the 9th hospital day. Severe SND developed on the 10th hospital day and LCM was discontinued. Thereafter agranulocytosis appeared on the 11th hospital day, and the patient died from septic shock on the 15th hospital day. Conclusions This case illustrates the need for careful follow-up of the electrocardiogram and the complete blood cell counts when initiating LCM. Moreover, it should be noticed that various side effects may occur simultaneously in the early period of LCM use, even for a short time and at low dosages.
Benjamin Ilse, Bernd Alt-Epping, Albrecht Günther, Jan Liman,
BMC Neurology, Volume 21, pp 1-7; doi:10.1186/s12883-021-02244-2

Abstract:
Background The concept of clinical ethics consultation (CECs) was implemented to provide support in ethical controversies in clinical settings and are offered in at least every second hospital in Germany. Neurological disorders often require complex decision-making. The aims of this study were to determine which situations lead to CEC in neurology and to investigate the influence of the individual patient’s wishes on the recommendation. Methods Standardised CEC protocols in the years 2011 to 2017 at the University Hospitals of Goettingen and Jena were retrospectively surveyed. The contents were categorised along existing protocol templates of CEC scenarios and subsequently paraphrased and reduced to significant meanings. Results 27 CEC scenarios which were facilitated by various professional disciplines were reviewed. Stroke was the most frequent underlying condition. Nearly all patients were not able to consent. Mostly, the relatives acted as representatives or health advocates. In 67 % of cases, a sense of conflict triggered a CEC; in 33 % a sense of uncertainty was the reason for the CEC request. In 21 CEC scenarios, a recommendation was reached in consensus with all parties involved. In 59 % of cases, a decision was made to continue medical therapy. In seven cases, the patient’s wishes led to a limitation of therapy, while in just two cases this decision was made primarily relying on the patient’s best interest. In only 13 % of cases, a valid advance directive led to respective therapeutic consequences. Conclusions CEC is feasible for consensus-finding not only in conflicts, but also in situations of therapeutic uncertainty in neurology. There is a special importance of the patient’s wishes in decision-making in neurology. However, only in a few cases were advance directives precise and specific enough to have sufficient and decisive weight in therapeutic decision-making.
Ja Yoon Kim, Yong Bae Kim,
BMC Neurology, Volume 21, pp 1-6; doi:10.1186/s12883-021-02254-0

Abstract:
Background Chronic inflamed tissue in nasal cavity is a rare complication of transsphenoidal approach (TSA). Inflamed tissue is rich in blood vessels, which can lead to frequent nosebleeds. In addition, chronic inflammation can cause pseudoaneurysm, whose rupture results in massive epistaxis. There have been few reported cases of pseudoaneurysm of ICA occurring more than 10 years after TSA surgery. Case presentation We report a case of a patient who had recurrent epistaxis for over a decade after TSA surgery, and analyzed the causes of the nosebleeds. The aspect of occurrence of the nosebleeds and the result of biopsy and imaging tests suggest that the nosebleeds were due to chronic inflamed tissue and an associated pseudoaneurysm. The rupture of pseudoaneurysm recurred after treatment with stent placement, and brain abscess was developed. After removing the inflamed tissue by endoscopic resection, the patient no longer had recurrence of ruptured pseudoaneurysm or nosebleeds. Conclusions In patients with recurrent nosebleeds, the possibility of intranasal inflammation and subsequent pseudoaneurysm should be considered. Therefore, people who consistently have epistaxis after TSA, even if the bleeding is not in large amount, should be actively screened and treated for nasal chronic inflammation.
, Masahiro Sonoo
BMC Neurology, Volume 21, pp 1-6; doi:10.1186/s12883-021-02247-z

Abstract:
Background The course of the corticobulbar tract (CBT) to the facial nucleus has been investigated by some previous studies. However, there are some unclear points of the course of the CBT to the facial nucleus. This study aimed to elucidate the detailed course of the CBT to the facial nucleus through the analysis of lateral medullary infarction (LMI) cases. Methods The neurological characteristics and magnetic resonance imaging findings of 33 consecutive patients with LMI were evaluated. The location of the lesions was classified rostro-caudally (upper, middle, or lower) and horizontally. Further, we compared the neurological characteristics between the groups with and without central facial paresis (FP). Results Eight (24%) patients with central FP ipsilateral to the lesion were identified. Dysphagia and hiccups were more frequently observed in the group with central FP than in the group without central FP. In patients with central FP, middle medullary lesions and those including the ventral part of the dorsolateral medulla were more frequently observed. Contrastingly, patients with lesions restricted to the lateral and dorsal regions of the dorsolateral medulla did not present with central FP. Conclusion The results of this study indicate that the CBT to the facial nucleus descends with the corticospinal tract at least to the middle portion of the medulla, and then ascends to the facial nucleus through the medial and ventral areas of the dorsolateral medulla after decussation.
Bing Wang, Wentao Jiang, Weiqiang Yan, Jianhong Tian, Jianxing Xu, Yulin Li, Yanzhen Zhao, Yi Dai, , Gangqiang Hou
BMC Neurology, Volume 21, pp 1-9; doi:10.1186/s12883-021-02242-4

Abstract:
Background DDMS is a rare disease diagnosed by clinical and radiological characteristics. But the complexity of radiological and clinical manifestations of DDMS has become a challenge diagnostically. To date, the reported cases with DDMS had highly varied clinical manifestations including seizures, contralateral hemiplegia/hemiparesis, facial asymmetry, mental retardation, etc. In addition to typical clinical findings, some new characteristics have been recently added to the spectrum of DDMS. However, few cases have been reported to be associated with neuropsychiatric symptoms according to the literature. This study aimed to investigate the neuropsychiatric manifestations associated with Dyke-Davidoff-Masson syndrome (DDMS) and related imaging findings. Methods This study included 7 patients diagnosed with DDMS between 2014 and 2020. The clinical characteristics, neuropsychiatric manifestations, and radiological results were retrospectively evaluated. Results Seven patients (five males and two females) with a mean age of 28.0 ± 9.73 (range 15.0–41.0) years were included. Five patients were admitted to the psychiatric unit due to psychological and behavioral disorders. Two patients were referred to the neurology unit mainly due to epilepsy. Six patients had epileptic seizures, 4 had hemiplegia, 3 had mental retardation, 2 patients had external ear deformities, and 2 had facial asymmetry. Neuropsychiatric symptoms were presented in 6 (85.7 %) cases. Cases 2–6 developed affective disorders. Deficits in verbal communication, impairment of social interaction, lack of insight, adulia and hypobulia appeared in cases 1–4. Schizophrenia with apathy, and epileptic schizoid psychosis were observed in cases 4 and 5 respectively. Case 6 had behavioral disorders, hyperactivity, tic disorder, mental retardation, anxiety, catatonic symptoms and suicidal tendency. Case 7 had seizures and mental retardation, and no psychiatric symptoms were presented. Radiological examinations showed unilateral cerebral atrophy, enlarged lateral ventricles, and various compensatory hypertrophy of the skull in all cases. The midline structure has shifted to the affected side in 5(71.4 %) cases. Atrophy of the basal ganglia or brain stem was observed in 4(57.1 %) cases. Conclusions The hallmark imaging manifestations of DDMS facilitated the diagnosis in most cases. This study illustrated that a variety of psychoneurotic disorders and ear abnormalities were correlated with DDMS.
Tao Liang, Zhiwei Zhou, Xiaolin Hu, Zhong Luo
BMC Neurology, Volume 21, pp 1-5; doi:10.1186/s12883-021-02245-1

Abstract:
Background Graves’ disease and anti-GQ1b antibody syndrome are both autoimmune diseases, and there have been few reports on whether there is a correlation between the two. In this study, we present the case of a woman who was diagnosed with Graves’ disease and anti-GQ1b antibody syndrome in succession. Case presentation The chief complaints of this patient were limb weakness and blurred vision. Graves’ disease was diagnosed by examination of thyroid function and thyroid autoantibodies, but the clinical symptoms were not relieved after antihyperthyroidism treatment. Finally, it was found that Graves’ disease was complicated by anti-GQ1b antibody syndrome, and the symptoms were relieved after treatment with glucocorticoids and intravenous immunoglobulin. We also explored the possible mechanism of these diseases through a literature review. Conclusions We report a rare case of the cooccurrence of Graves’ disease and anti-GQ1b antibody syndrome. Immune dysregulation might be the pathogenesis of the association, but there is no precise supporting evidence, and more research is needed.
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