甲钴胺辅助治疗急性吉兰-巴雷综合征的临床研究

目的 研究甲钴胺辅助治疗急性吉兰-巴雷综合征（GBS）的临床效果,以及相对于单纯免疫球蛋白治疗的优势.方法 采取随机对照的方法,将符合急性吉兰-巴雷综合征诊断标准的60例患者,分为2组,每组各30例.2组在丙型球蛋白治疗的基础上,治疗组注射甲钴胺,对照组给予生理盐水,观察临床症状改善时间以及疗效,然后进行统计学分析.结果 （1）在临床疗效Hughes评分方面,甲钴胺治疗组8周后与生理盐水对照组相比,症状有明显的改善;（2）在临床疗效MRC评分方面,治疗组4周与对照组患者相比,症状得到明显缓解;（3）治疗组的平均好转时间显著少于对照组.结论 与单纯的丙型球蛋白基础免疫治疗相比,甲钴胺联合治疗吉兰-巴雷综合征有较好的疗效.     

Guillain Barre Syndrome in the elderly: Experience from a tertiary-care hospital in India

Age influences incidence and prognosis of Guillain Barre Syndrome (GBS), common cause of ascending areflexic quadriparesis. Dedicated studies on elderly GBS are infrequent. This study aimed to describe clinical features and outcome at hospital-discharge in patients aged ≥ 60 years with GBS. Medical records of 70 elderly GBS over 15 years were analysed. Mean symptom-duration was 5.78 ± 4.5 days and onset-to-peak 5.14 ± 4.4 days. Antecedent events preceded GBS by 8.07 ± 9.9 days and included: fever (n = 19), respiratory infection (n = 6), and gastroenteritis (n = 5). Clinical features were weakness of facial (n = 34), bulbar (n = 13), extraocular (n = 4) and respiratory (n = 20) muscles and recurrence (n = 4). Nine had Hughes disability score (HDS) of three or less. Sensory symptoms and signs included paresthesias (n = 40), pain (n = 24), and impaired kinaesthetic sensation (n = 14). Laboratory abnormalities included albumino-cytological dissociation (n = 50), hyponatremia (n = 36) and elevated creatine kinase (n = 18). Electrophysiological subtypes were: primary demyelinating (n = 52), inexcitable (n = 3), equivocal (n = 2) and axonal (n = 1). Fifty-seven patients treated with plasmapheresis (n = 48) or intravenous immunoglobulin (n = 9) had mean HDS of 3.53 ± 0.7 at discharge. Twenty-one were ambulant (HDS ≥ 3), one had persisting respiratory weakness and one died. Striking differences between the ‘elderly’ and 100 ‘adults’ seen over 20 months were shorter symptom-duration, higher frequency of facial palsy and hyponatremia, lower frequency of pain, lower mean MRC sum score and worse HDS at study-entry and discharge (p < 0.05). Requirement for mechanical ventilation and cardiac autonomic dysfunction was higher among elderly (p:0.02). In conclusion, in this cohort of elderly GBS, there was a higher frequency severe GBS and demyelinating electrophysiology.     

Facial diplegia,a possible atypical variant of Guillain-Barré Syndrome as a rare neurological complication of SARS-CoV-2

We present a case of facial diplegia after 10 days of SARS-CoV-2 confirmed infection symptoms in a 61 year old patient without prior clinically relevant background. There are few known cases of Guillain-Barré Syndrome (GBS) related to SARS-CoV-2 infection; we propose this case as a rare variant of GBS in COVID-19 infection context, due to Its chronology, clinical manifestations and cerebrospinal fluid (CSF) findings.     

Isolated abducens nerve palsy in a patient with asymptomatic SARS-CoV-2 infection

The neuro-ophthalmological complications of SARS-CoV-2 infection are emerging but the spectrum of presentations and pathophysiological mechanism underpinning the association remains to be fully determined. We describe the case of a 44-year-old female who presented with a 12-hour history of diplopia preceded by a mild headache and found to have an isolated right abducens nerve palsy. Initial vital signs were normal but she developed a fever and nasopharyngeal swab confirmed SARS-CoV-2 infection by RT-PCR. All other investigations returned normal including blood tests, chest X-ray, MRI brain and cerebrospinal fluid analysis. She remained systemically well, and there was complete resolution of the abducens palsy and diplopia at two week follow up. In the absence of an alternative underlying cause or risk factors identified, the aetiology was presumed to be microvascular and potentially related to the viral infection. We add to the evolving literature of neuro-ophthalmological associations of SARS-CoV-2, discuss possible causal mechanisms and suggest considering asymptomatic SARS-CoV-2 infection in cases of isolated abducens palsy without clear risk factors.     

Spinal angiolipoma in a pregnant woman presenting with acute epidural hemorrhage

A 26-year-old woman in week 31 of pregnancy presented to the emergency room with acute onset of paraplegia. Her medical history was unremarkable. Neurological examination revealed complete paraplegia, total sensory loss below the T7 dermatome, and significant vesicorectal dysfunction. MRI revealed an intraspinal mass from T3 to T4, which was hyperintense on both T1-weighted and T2-weighted images. Blood examination found no abnormality. She underwent emergent hemilaminectomy and removal of the hematoma. Intraoperatively, unusually ectatic venous vessels were found adhered to the lower surface of the epidural clot. No concurrent vascular malformations were identified and the dura mater was intact. The histological diagnosis was angiolipoma. Postoperatively her neurological deficits showed remarkable improvement, and she gave birth to a healthy baby. Spinal angiolipoma in a pregnant woman may be complicated with acute epidural hemorrhage. Emergent surgical evacuation can be performed safely with a good functional prognosis.     

Bell’s palsy in a pediatric patient with hyper IgM syndrome and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)

Bell’s palsy is an acute facial paralysis with known association to viral infections. We describe a medically complex 6-year-old male with hyper IgM syndrome who presented with unilateral facial droop and positive SARS-CoV-2 RT-PCR. This is the first reported pediatric case of Bell’s palsy in the setting of SARS-CoV-2 infection.     

Central pontine and extra-pontine myelinolysis: a complication of lithium toxicity in a pregnant woman

Osmotic demyelinating syndromes consisting of central pontine and extra-pontine demyelination are very uncommon disorders characterized by non-inflammatory lesions involving the pons and sometimes spreading to other areas. Rapid changes in serum sodium concentration are usually regarded as the main pathophysiological mechanism. We report herein the case of a 23-year-old woman in the 24th week of pregnancy, who demonstrated both central pontine and extra-pontine demyelination occurring at the time of a recently introduced treatment with lithium. The disorder was related to the rapid correction of pregnancy-related hyponatremia, as a consequence of lithium-induced diabetes insipidus. Hence, lithium toxicity is a rare cause of osmotic demyelinating syndromes and appears to correlate with disturbances in sodium homeostasia.     

妊娠合并中枢神经细胞瘤足月顺产一例

患者 女性,22岁,宫内妊娠22周,因入院前2d夜间睡眠时癫痫全面强直一阵挛发作于2005年6月1日入院.     

Neurological manifestations associated with SARS-CoV-2 and other coronaviruses: A narrative review for clinicians

IntroductionThe past two decades have been marked by three epidemics linked to emerging coronaviruses. The COVID-19 pandemic highlighted the existence of neurological manifestations associated with SARS-CoV-2 infection and raised the question of the neuropathogenicity of coronaviruses. The aim of this review was to summarize the current data about neurological manifestations and diseases linked to human coronaviruses.Material and methodsArticles have been identified by searches of PubMed and Google scholar up to September 25, 2020, using a combination of coronavirus and neurology search terms and adding relevant references in the articles.ResultsWe found five cohorts providing prevalence data of neurological symptoms among a total of 2533 hospitalized COVID-19 patients, and articles focusing on COVID-19 patients with neurological manifestations including a total of 580 patients. Neurological symptoms involved up to 73% of COVID-19 hospitalized patients, and were mostly headache, myalgias and impaired consciousness. Central nervous system (CNS) manifestations reported in COVID-19 were mostly non-specific encephalopathies that represented between 13% and 40% of all neurological manifestations; post-infectious syndromes including acute demyelinating encephalomyelitis (ADEM, n = 13), acute necrotizing encephalopathy (ANE, n = 4), Bickerstaff's encephalitis (n = 5), generalized myoclonus (n = 3) and acute transverse myelitis (n = 7); other encephalitis including limbic encephalitis (n = 9) and miscellaneous encephalitis with variable radiologic findings (n = 26); acute cerebrovascular diseases including ischemic strokes (between 1.3% and 4.7% of COVID-19 patients), hemorrhagic strokes (n = 17), cerebral venous thrombosis (n = 8) and posterior reversible encephalopathy (n = 5). Peripheral nervous system (PNS) manifestations reported in COVID-19 were the following: Guillain–Barré syndrome (n = 31) and variants including Miller Fisher syndrome (n = 3), polyneuritis cranialis (n = 2) and facial diplegia (n = 2); isolated oculomotor neuropathy (n = 6); critical illness myopathy (n = 6). Neuropathological studies in COVID-19 patients demonstrated different patterns of CNS damage, mostly ischemic and hemorrhagic changes with few cases of inflammatory injuries. Only one case suggested SARS-CoV-2 infiltration in endothelial and neural cells. We found 10 case reports or case series describing 22 patients with neurological manifestations associated with other human coronaviruses. Among them we found four MERS patients with ADEM or Bickerstaff's encephalitis, two SARS patients with encephalitis who had a positive SARS-CoV PCR in cerebrospinal fluid, five patients with ischemic strokes associated with SARS, eight MERS patients with critical illness neuromyopathy and one MERS patient with Guillain–Barré Syndrome. An autopsy study on SARS-CoV patients demonstrated the presence of the virus in the brain of eight patients.ConclusionThe wide range of neurological manifestations and diseases associated with SARS-CoV-2 is consistent with multiple pathogenic pathways including post-infectious mechanisms, septic-associated encephalopathies, coagulopathy or endothelitis. There was no definite evidence to support direct neuropathogenicity of SARS-CoV-2.     

SARS-CoV-2-associated first episode of acute mania with psychotic features

Despite neuropsychiatric outcomes of SARS-CoV-2 infection are now under close scrutiny, psychoneuroimmunological characteristics of COVID-19 and precise pathophysiology of neuropsychiatric manifestations of the infection are still obscure. Moreover, there still exists a shortfall in demonstrating specific clinical manifestations of the brain involvement of the virus. Here, we presented a 33-year-old female patient with COVID-19, reporting acute-onset paranoid delusions symptoms, insomnia and irritability. Cranial MRI showed an hyperintense signal in the splenium of the corpus callosum with decreased apparent diffusion coefficient, which might possibly indicate the presence of cytotoxic edema related to the brain involvement of the infection. Following the completion of SARS-CoV-2 treatment, both cytotoxic edema and psychiatric symptoms resolved. In light of this report, we suggest that either heightened immune response and direct viral infection that SARS-CoV-2 may lead to such psychiatric manifestations and neuropsychiatric monitoring should be performed in patients with COVID-19. Prompt recognition of psychiatric consequences of COVID-19 may help clinicians provide guidance for differential diagnosis and manage them accordingly.     

The impact of hybrid immunity on immune responses after SARS-CoV-2 vaccination in persons with multiple sclerosis treated with disease-modifying therapies

Background and purpose

Hybrid immunity to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) develops from a combination of natural infection and vaccine-generated immunity. Multiple sclerosis (MS) disease-modifying therapies (DMTs) have the potential to impact humoral and cellular immunity induced by SARS-CoV-2 vaccination and infection. The aims were to compare antibody and T-cell responses after SARS-CoV-2 mRNA vaccination in persons with MS (pwMS) treated with different DMTs and to assess differences between naïvely vaccinated pwMS and pwMS with hybrid immunity vaccinated following a previous SARS-CoV-2 infection.

Methods

Antibody and T-cell responses were determined in pwMS at baseline and 4 and 12 weeks after the second dose of SARS-CoV-2 vaccination in 143 pwMS with or without previous SARS-CoV-2 infection and 40 healthy controls (HCs). The MS cohort comprised natalizumab (n = 22), dimethylfumarate (n = 23), fingolimod (n = 38), cladribine (n = 30), alemtuzumab (n = 17) and teriflunomide (n = 13) treated pwMS. Immunoglobulin G antibody responses to SARS-CoV-2 antigens were measured using a multiplex bead assay and FluoroSpot was used to assess T-cell responses (interferon γ and interleukin 13).

Results

Humoral and T-cell responses to vaccination were comparable between naïvely vaccinated HCs and pwMS treated with natalizumab, dimethylfumarate, cladribine, alemtuzumab and teriflunomide, but were suppressed in fingolimod-treated pwMS. Both fingolimod-treated pwMS and HCs vaccinated following a previous SARS-CoV-2 infection had higher antibody levels 4 weeks after vaccination compared to naïvely vaccinated individuals. Antibody and interferon γ levels 12 weeks after vaccination were positively correlated with time from last treatment course of cladribine.

Conclusion

These findings are of relevance for infection risk mitigation and for vaccination strategies amongst pwMS undergoing DMT.     

Supra and infratentorial massive strokes in previously healthy young patients with SARS-CoV-2. The role of neurosurgery

The coronavirus disease 2019 (COVID-19) has amazed by its distinct forms of presentation and severity.COVID-19 patients can develop large-scale ischemic strokes in previously healthy patients without risk factors, especially in patients who develop an acute respiratory distress syndrome (SARS-CoV-2).We hypothesize that ischemic events are usually the result of the combined process of a pro-inflammatory and pro-coagulant state plus vascular endothelial dysfunction probably potentiated by hypoxia, hemodynamic instability, and immobilization, as reported in other cases.To the best of our knowledge, we report the first case of partial obstruction of a vertebral artery in a patient with COVID-19.Decompressive surgery remains a life-saving maneuver in these patients (as in other non-COVID-19 strokes) and requires further investigation.