Acta Neurol Scand
· 2022 Apr · PMID 34877648
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OBJECTIVES: To evaluate the changes in prescription patterns in the treatment of idiopathic generalized epilepsy (IGE) due to updated treatment recommendations and to assess seizure outcomes of valproate compared to othe...OBJECTIVES: To evaluate the changes in prescription patterns in the treatment of idiopathic generalized epilepsy (IGE) due to updated treatment recommendations and to assess seizure outcomes of valproate compared to other antiseizure medications (ASMs), with emphasis on women with epilepsy (WWE). MATERIALS AND METHODS: Records of IGE patients treated at Tampere University Hospital between 1 January 2009 and 31 December 2018 were retrospectively inspected. Data were analysed for two subgroups based on age and sex. Seizure control with reference to the efficacy of different ASMs and their combinations was examined for each subgroup. RESULTS: The study compiled 263 subjects (166 females and 97 males). Of all patients, 72.6% remained seizure free. There was no difference in seizure control between sexes (OR 1.25, p = .48). Males used valproate more often than females while females used lamotrigine and levetiracetam more often than males. Lamotrigine and levetiracetam were used especially as monotherapy in WWE, and mostly as part of combination therapy in males. Valproate alternatives were found as effective as valproate when used in monotherapy in adults. Valproate remained the most used ASM in the paediatric subgroup. CONCLUSIONS: The use of valproate has decreased in daily clinical use with the simultaneous increased use of alternative ASMs compared to our previous study. Decreasing use of valproate in WWE did not increase the risk of seizure recurrence; therefore, valproate alternatives could be considered as first-line ASMs for WWE. Overall, IGE patients demonstrated good clinical outcomes with valproate or other broad-spectrum ASMs as monotherapy.
OBJECTIVES: Mitochondrial DNA (mtDNA)-associated Leigh syndrome (LS) is characterized by maternal inheritance, and the heteroplasmic mutant load of mtDNA pathogenic variants is known to affect clinical phenotypes. Among...OBJECTIVES: Mitochondrial DNA (mtDNA)-associated Leigh syndrome (LS) is characterized by maternal inheritance, and the heteroplasmic mutant load of mtDNA pathogenic variants is known to affect clinical phenotypes. Among mtDNA pathogenic variants, variants of the MT-ATP6 gene account for most of reported cases. In this report, we aimed to describe the clinical and genetic findings of MT-ATP6-associated LS patients diagnosed at a single tertiary institution in Korea. METHODS: Thirteen patients with genetically confirmed MT-ATP6-associated LS were selected. We reviewed each patient's clinical findings, including general characteristics, biochemical parameters, brain MR images, muscle biopsy results, and heteroplasmic mutant load over a long-term follow-up period. RESULTS: MT-ATP6-associated LS was of predominantly early onset (age <2 years), although we identified 2 late-onset (>60 months) LS patients. The heteroplasmic mutant load estimated by next-generation sequencing was 96%-100% in all nucleotide change groups. Compared with other forms of MT-ATP6-associated LS, the m.8993T>G point mutation elicited a significantly higher rate of symptom onset before 2 years of age. Brain MRI showed bilateral basal ganglia involvement in all patients, followed by cerebral atrophy, brainstem and thalamus involvement, and cerebellar atrophy. After follow-up (median 7.2 years, range 1.4 to 11.5 years), LS with m.8993T>G point mutations had a slightly more severe clinical progression compared with other forms of MT-ATP6-associated LS. CONCLUSIONS: MT-ATP6-associated LS patients presented with a broad spectrum of clinical diagnoses and had a very high heteroplasmic mutant load. This study provides valuable data on MT-ATP6-associated LS that will inform subsequent studies on LS.
BACKGROUND: Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management is unknown given...BACKGROUND: Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management is unknown given the absence of randomized clinical trials. AIMS OF THE STUDY: Explore whether tocilizumab, an anti-interleukin-6 monoclonal antibody, is an effective treatment for refractory PCNSV. METHODS: Patients with PCNSV treated with tocilizumab in a single tertiary center were reviewed. RESULTS: Three patients were identified. In two of them, MRI-revealed ischemic lesions. The other presented with a subcortical hemispheric pseudotumoral lesion. Brain biopsy was inconclusive in two patients. Due to a significant number of relapses and clinical deterioration despite other immunosuppressive drugs, tocilizumab was initiated and induced a long remission period up to 44 months. Observed side effects were a fungic infection, neutropenia and thrombocytopenia (both transitory), and a pulmonary embolism in one of the cases. CONCLUSIONS: Tocilizumab might be a therapeutic option for PCNSV (Class IV evidence), given its efficacy and safety. We propose a novel pathway for diagnosis and therapeutics of PCNSV with the purpose of improving the diagnosis, monitoring, and prognosis of this heterogeneous disorder, setting the framework for future use of tocilizumab in this condition.
OBJECTIVES: Acute ischemic stroke is a common cause of mortality and morbidity worldwide. Percutaneous endovascular intervention is an important treatment method in ischemic stroke. Endovascular procedure success is asso...OBJECTIVES: Acute ischemic stroke is a common cause of mortality and morbidity worldwide. Percutaneous endovascular intervention is an important treatment method in ischemic stroke. Endovascular procedure success is associated with the clinical outcome of the patients. The CHA2DS2-VASC score is an important score used to determine the risk of ischemic stroke in patients with atrial fibrillation. In our study, we aimed to evaluate the relationship between procedure success and CHA2DS2-VASC score in patients with acute ischemic stroke who underwent endovascular intervention. MATERIALS AND METHODS: A total of 102 consecutive patients who underwent endovascular intervention with acute ischemic stroke were included in the study. The admission CHA2DS2-VASc scores of the patients were recorded. After the procedure, the relationship between the TICI score and the CHA2DS2-VASc score was evaluated. RESULTS: CHA2DS2-VASc score was significantly higher in the group that resulted in unsuccessful endovascular intervention (2.78 ± 1.44, 5.02 ± 1.77 p < .001). Receiver-operating characteristics analysis revealed the cutoff value of CHA2DS2-VASc score ≥3 as a predictor of unsuccessful intervention with 76,6% sensitivity and 83,3% specificity, positive predictive value 50%, negative predictive value 84,6% (area under the curve [AUC]: 0.827,95% CI: 0.739-0.895, p < .001). In the multivariate analysis; atrial fibrillation ([β] = 4.201; [CI]: 1.251-14.103, p = .020), CHA2DS2-VASc score ([β] = 0.053; [CI]: 0.004-0.750, p = .030) were found independent predictors for unsuccessful intervention treatment. CONCLUSIONS: In our study, we showed that the CHA2DS2-VASc score is associated with the success of endovascular intervention in patients with acute ischemic stroke who underwent percutaneous endovascular treatment.
BACKGROUND: Recently, TANK binding kinase 1 (TBK1) mutation has been reported as a causative gene for overlap frontotemporal dementia (FTD)-amyotrophic lateral sclerosis (ALS) syndrome. However, there are no reports from...BACKGROUND: Recently, TANK binding kinase 1 (TBK1) mutation has been reported as a causative gene for overlap frontotemporal dementia (FTD)-amyotrophic lateral sclerosis (ALS) syndrome. However, there are no reports from families of South Asian ethnicity. OBJECTIVE: To report a case study of a family with the proband having overlap FTD-ALS syndrome caused by a novel TBK1 variant. MATERIALS AND METHODS: Clinical, brain imaging, genetic analysis and laboratory data of the patient with FTD-ALS were performed. In addition, family-based segregation analysis of identified novel variants was also done. RESULTS: This study pertains to genetic analysis in 11 members in a family with only one member affected with overlap FTD-ALS syndrome. The whole-exome sequencing analysis in the symptomatic member showed a novel loss-of-function (LoF) variant c.1810G>T(p.E604X) in the TBK1 gene. Neuroimaging showed a pattern of asymmetric frontotemporal atrophy and hypometabolism. Segregation analysis of the variation demonstrated its presence in several family members, although none of the other members was symptomatic. Further, we observed another missense variation in the NEFH gene (p.Pro683Leu) which was seen in the symptomatic and two asymptomatic family members, the pathogenicity of which is unclear. CONCLUSION: This is the first study of a rare novel TBK1 variant associated with FTD-ALS from India. Asymptomatic family members with the variant have important clinical implications and necessitate the genetic evaluation and long-term follow-up of family members of patients detected with TBK1 mutations. Therefore, although infrequent, genetic screening for the TBK1 gene should be considered when encountering overlap FTD syndromes.
Sillanpää M, Hermann B, Rinne JO
… +7 more, Parkkola R, Saarinen MM, Karrasch M, Saunavaara J, Rissanen E, Joutsa J, Shinnar S
Acta Neurol Scand
· 2022 Mar · PMID 34837220
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PURPOSE: To determine the impact of childhood-onset uncomplicated epilepsy (COE) on brain aging over 50-year prospective follow-up. METHODS: A population-based cohort of 41 aging subjects with COE and their 46 matched co...PURPOSE: To determine the impact of childhood-onset uncomplicated epilepsy (COE) on brain aging over 50-year prospective follow-up. METHODS: A population-based cohort of 41 aging subjects with COE and their 46 matched controls participated in a detailed in-person prospective assessment in 2012 and 2017 to characterize ongoing changes in the aging brain. RESULTS: The mean age of the COE participants was 63.2 years (SD 4.14, median 63.2, range 55.8-70.6) and 63.0 years (mean, SD 4.13, median 63.3, range 56.0-69.9) years for controls. Neurologic signs were significantly more common in COE participants not in remission (p = .015), and the most frequent abnormalities were cerebellar signs (p < .001). Neurologic signs in general (p = .008) and cerebellar signs in particular (p = .018) were significantly more common in focal than in generalized epilepsies. MRI white matter abnormalities were significantly associated with absence of vocational education (p = .011), and MRI hippocampal atrophy in COE subjects was associated with arterial hypertension versus normal blood pressure (p = .017). In the combined study cohort of COE subjects and controls, presenting neurologic signs increased both in the subjects and in the controls from the 2012 to 2017 study. CONCLUSIONS: At ultra-long-term follow-up, clinical and neuroimaging findings show tendencies to brain aging that is more accelerated in COE participants with active adult childhood-onset epilepsy, and particularly in focal epilepsy.
OBJECTIVE: To perform a meta-analysis of all-cause, cause-specific and gender-specific standardized mortality ratio and crude mortality rate for people with multiple sclerosis. We also examined the temporal trends in thi...OBJECTIVE: To perform a meta-analysis of all-cause, cause-specific and gender-specific standardized mortality ratio and crude mortality rate for people with multiple sclerosis. We also examined the temporal trends in this data. METHODS: Medline, Cochrane Library and Scopus were searched. Keywords were "multiple sclerosis" and "standardized mortality ratio" or "Standardized Mortality Ratio". We included longitudinal studies with available data on the number of deaths, follow-up period, person years and reports of standardized mortality ratio (SMR). Crude mortality ratio (CMR) was calculated and SMR was extracted. CMRs and log-SMR were pooled by the method of inverse variance. Meta-regression models were used to investigate temporal trends. RESULTS: Fifty-seven articles were screened. Fifteen studies were included covering a period 1949-2013 (160,000 patients; 21,225 deaths). The all-cause SMR for people with MS was 2.61 (95% CI 2.58 to 2.65). For men this was 2.47 (95% CI 2.42 to 2.52) and for women 2.57 (95% CI 2.53 to 2.61). The CMR was 13.45/1000 person years. Cause-specific SMR was 1.74 (1.67 to 1.81) for CVD, 4.70 (4.45 to 4.87) for respiratory disease and infection, 1.81 (1.64 to 2.0) for accident and suicide and 0.99 (0.93 to 1.06) for cancer. Meta-regression analysis of the SMR compared to midpoint follow-up year revealed no relationship (co-efficient 0.001, p = .98). CONCLUSIONS: People with multiple sclerosis (MS) have reduced overall survival and increased risk of death from cardiovascular, respiratory and infectious disease as well as accidents and suicide. This does not appear to have changed over the last 65 years.
OBJECTIVES: Minor hallucinations (MH) are psychotic symptoms that can occur anywhere between prodromal to early Parkinson's disease and after onset of motor problems. MH include visual illusions, presence hallucinations,...OBJECTIVES: Minor hallucinations (MH) are psychotic symptoms that can occur anywhere between prodromal to early Parkinson's disease and after onset of motor problems. MH include visual illusions, presence hallucinations, and passage hallucinations. Isolated rapid eye movement sleep behavior disorder (RBD) is a harbinger of neurodegenerative diseases. We conducted a retrospective cohort study to investigate the clinical characteristics of isolated RBD with MH and the risk of phenoconversion. MATERIALS AND METHODS: We retrospectively analyzed the data of 36 patients with isolated RBD (four females; median age, 75.0 years). We defined cases reporting at least one minor hallucination as RBD with MH. Demographic data and cognitive function were compared between patients with and without MH, and Cox proportional hazards models estimated the risk of phenoconversion. RESULTS: We included 10 (27.8%) patients with MH and 26 (72.2%) without MH. Patients with MH were older, had less dopamine transporter accumulation, more severe autonomic dysfunction, more depressive symptoms, and lower verbal fluency and symbol coding test scores than patients without MH. After follow-up (median, 2.50 years), 13.9% (5/36) of all patients developed phenoconversion (Parkinson's disease, two patients; dementia with Lewy bodies, three patients). The rate of phenoconversion was significantly higher in patients with MH (40.0% vs. 3.8%, p = .005). The Cox proportional hazards model adjusted for age, sex, and disease duration revealed that MH was a significant risk factor for phenoconversion (hazard ratio, 14.72; 95% confidence interval, 1.35-160.5). CONCLUSIONS: Minor hallucinations may be utilized as early clinical markers for prodromal estimation of neurodegenerative diseases.
BACKGROUND: Few MRA-based studies have systematically evaluated the prevalence and laterality of a fetal configuration of the posterior cerebral artery (FTP) in ischemic stroke populations versus other populations. This...BACKGROUND: Few MRA-based studies have systematically evaluated the prevalence and laterality of a fetal configuration of the posterior cerebral artery (FTP) in ischemic stroke populations versus other populations. This common variant is important in the setting of acute stroke and secondary prevention decisions. OBJECTIVE: To determine the prevalence and laterality of FTP configurations in MRI-DWI verified acute ischemic stroke patients investigated with MRA, and compare the findings with an unselected hospital population investigated with computed tomography angiography (CTA). We also evaluated the association of FTP with posterior cerebral artery (PCA) territory infarctions. METHODS: We reviewed the MRAs of 1407 ischemic stroke patients with acute lesions on MRI-DWI sequences and 546 consecutive CTAs of patients investigated on any indication in a tertiary hospital. The MRA and CTA assessments were made by neuroradiologists blinded to original reports on stroke location and vessel anatomy. RESULTS: The prevalence of any FTP was similar in ischemic stroke patients (31%) and unselected patients (32%). Unilateral FTP was significantly more frequent on the right than on the left side in both groups (15% right vs. 8% left). The presence of FTP ipsilateral to stroke side was not associated with involvement of the PCA territory versus no FTP on the stroke side. CONCLUSIONS: FTP is present in approximately 30% of ischemic stroke patients and unselected hospital populations and was detected significantly more frequently on the right versus left side in both groups. PCA territory infarction was not associated with the presence of ipsilateral FTP.
OBJECTIVE: To examine the Multiple Sclerosis Impairment Scale (MSIS) in secondary progressive MS (SPMS) in relation to the Expanded Disability Status Scale (EDSS), magnetic resonance imaging (MRI) outcomes, and mobility....OBJECTIVE: To examine the Multiple Sclerosis Impairment Scale (MSIS) in secondary progressive MS (SPMS) in relation to the Expanded Disability Status Scale (EDSS), magnetic resonance imaging (MRI) outcomes, and mobility. METHODS: In this observational single-center study, 68 secondary progressive multiple sclerosis (SPMS) patients were examined by MSIS, EDSS, functional mobility tests of upper/lower extremities, and multimodal MRI. Participants had EDSS ≥3.5, a decline in daily activities over the last year unrelated to relapses, and/or 6-month confirmed disability progression. RESULTS: Mean disease duration was 23.1 ± 8.3 years and mean age 54.4 ± 8.1 years. MSIS, EDSS, and their corresponding motor, cerebellar, and sensory subscores correlated (p < .0001). Motor subscores of MSIS correlated stronger with Timed-25-Foot-Walk (T25FW) than pyramidal functional system score (FSS) (p = .03), but EDSS had a stronger correlation to T25FW than the total MSIS score (p = .01). MSIS cerebellar subscore correlated stronger with 9-Hole Peg Test (9-HPT) than cerebellar FSS (p = .04). The sensory MSIS subscore also showed correlation with 9-HPT in contrast to sensory FSS (p = .006). MSIS subscores had stronger correlations with MRI volumetry measures than FSS scores (lesion volume and putamen, thalamus, corpus callosum volumetry, p = .0001-0.0017). CONCLUSION: In patients with SPMS, MSIS correlated with functional motor tests. MSIS showed stronger correlations with atrophy of central nervous system areas, and may be more sensitive to scale cerebellar and sensory function than EDSS.
The presence of a "central vein sign" (CVS) has been introduced as a biomarker for the diagnosis of multiple sclerosis (MS) and shown to have the ability to accurately differentiate MS from other white matter diseases (M...The presence of a "central vein sign" (CVS) has been introduced as a biomarker for the diagnosis of multiple sclerosis (MS) and shown to have the ability to accurately differentiate MS from other white matter diseases (MS mimics). Following the development of susceptibility-based magnetic resonance venography that allowed the in vivo detection of CVS, a standard CVS definition was established by introducing the "40% rule" that assesses the number of MS lesions with CVS as a fraction of the total number of lesions to differentiate MS lesions from other types of lesions. The "50% rule," the "three-lesion criteria," and the "six-lesion criteria" were later introduced and defined. Each of these rules had high levels of sensitivity, specificity, and accuracy in differentiating MS from other diseases, which has been recognized by the Magnetic Resonance Imaging in MS (MAGNIMS) group and the Consortium of MS Centers task force. The North American Imaging in Multiple Sclerosis Cooperative even provided statements and recommendations aiming to refine, standardize and evaluate the CVS in MS. Herein, we review the existing literature on CVS and evaluate its added value in the diagnosis of MS and usefulness in differentiating it from other vasculopathies. We also review the histopathology of CVS and identify available automated CVS assessment methods as well as define the role of vascular comorbidities in the diagnosis of MS.
OBJECTIVE: To investigate the temporal course of medication response and associated prognostic factors in a cohort of juvenile myoclonic epilepsy (JME) patients over a long-term follow-up. MATERIALS AND METHODS: Data fro...OBJECTIVE: To investigate the temporal course of medication response and associated prognostic factors in a cohort of juvenile myoclonic epilepsy (JME) patients over a long-term follow-up. MATERIALS AND METHODS: Data from 113 JME patients diagnosed according to recently proposed class II criteria were retrospectively reviewed. Early sustained remission was defined as 4-year seizure remission starting within 2 years from the first antiseizure medication (ASM) intake, as opposed to delayed sustained remission. Spontaneous relapse rate (ie, not related to ASM withdrawal) was also investigated, along with factors associated with seizure relapse. RESULTS: Four-year seizure remission was obtained by 76/113 (67.3%) subjects. Early sustained remission was achieved by 45/76 (59.2%) patients. Absence seizures were significantly associated with no-remission at multivariable multinomial logistic regression analysis. Catamenial seizures and earlier age at epilepsy onset significantly predicted delayed sustained remission. Spontaneous seizure relapse after 4-year remission occurred in 15.7% of patients with early sustained remission and in 35.5% of those with delayed sustained remission (p = 0.045). The most common concomitant factors for a spontaneous relapse were irregular lifestyle habits and pregnancy-related switch from valproate to another ASM. Patients with a history of catamenial seizures were more likely to experience a spontaneous generalized tonic-clonic seizure relapse after 4-year remission at univariable analysis. SIGNIFICANCE: Our data support the prognostic relevance of early medication response in JME patients. Furthermore, the prognostic significance of catamenial seizures and the impact of valproate switch on seizure relapse after a prolonged remission account for the challenging therapeutic management of women with childbearing potential.
OBJECTIVES: The prevalence of dementia is growing rapidly worldwide. The early identification and treatment of cognitive decline could reduce the burden on the health care system. Our objective was to investigate whether...OBJECTIVES: The prevalence of dementia is growing rapidly worldwide. The early identification and treatment of cognitive decline could reduce the burden on the health care system. Our objective was to investigate whether factors measured at an examination at age 50 predict cognitive impairment (CI) 23 years later. MATERIALS & METHODS: In 1993 we enrolled a randomly selected sample of 798 men, 50 years of age, from the general population. They all underwent a physical examination, provided blood samples and filled out questionnaires addressing lifestyle and psychosocial factors. Cognitive testing was offered to all participants still alive in 2016, at age 73. RESULTS: A total of 333 men participated in the cognitive study, of which 80 (24.0%) performed at a level corresponding to mild cognitive impairment, and four (1.2%) at a level consistent with severe cognitive impairment. After the first step in the multivariable analysis, hypertension, heavy smoking, high intake of alcohol, financial stress, difficulty falling asleep, and cogwheel rigidity were associated with cognitive impairment. After further adjustment, only wide waist circumference measured in cm (OR 1.04, 95% CI 1.00-1.08, p = .04), leg pendulousness (OR 41.97, 95% CI 3.27-538.62, p = .004) and self-assessed hidden irritability (OR 2.18, 95% CI 1.10-4.32, p = .03) at baseline, remained as being associated with cognitive impairment 23 years later. CONCLUSIONS: Extrapyramidal symptoms such as leg pendulousness, at the age of 50, may be an indicator for very early identification of future cognitive decline.
Microglia are a type of glial cells that play a key role in the repair of damage to the central nervous system (CNS). In the pathological condition of Alzheimer's disease (AD), β-amyloid peptide and a variety of pro-infl...Microglia are a type of glial cells that play a key role in the repair of damage to the central nervous system (CNS). In the pathological condition of Alzheimer's disease (AD), β-amyloid peptide and a variety of pro-inflammatory factors can activate microglia, resulting in the secretion of a variety of inflammatory factors and neurotoxins. This leads to neuronal damage and even apoptosis, thus triggering AD. In contrast, microglia can protect the CNS by phagocytizing Aβ to slow down AD development. In this review, the roles of microglia in AD neuroinflammation and the scope of immunotherapy for AD are summarized to provide a theoretical basis for AD prevention and treatment.
BACKGROUND: Ischemic strokes in orally anticoagulated patients pose challenges for acute management and secondary prevention but the characteristics of these strokes are poorly understood. We examined the clinical and im...BACKGROUND: Ischemic strokes in orally anticoagulated patients pose challenges for acute management and secondary prevention but the characteristics of these strokes are poorly understood. We examined the clinical and imaging features, the presumed underlying etiology and the subsequent antithrombotic management. METHODS: We analyzed a consecutive series of patients enrolled into the EIDASAF study, a single center, observational study of ischemic stroke patients with a diagnosis atrial fibrillation (AF) prior to the index event who had been admitted to the Hyperacute Stroke Unit of Imperial College London between 2010 and 2017. We compared patients with oral anticoagulation therapy prior admission (OAC ) with those without anticoagulation (OAC ). Brain imaging was analyzed centrally. RESULTS: 763 patients were included in the analysis. 481 (63%) were OAC while 282 (37%) were OAC . Patients with OAC were younger, more often had a previous history of stroke or transient ischemic attack (TIA), and more often suffered from hypertension and diabetes. In OAC patients, large and deep middle cerebral artery infarcts occurred more often than in OAC patients. The groups differed significantly in the distribution of competing etiologies underlying their stroke. At discharge, OAC more frequently were (re)-anticoagulated compared to OAC patients. Within the OAC group, patients with recurrent strokes did not differ from those with a first stroke regarding clinical characteristics and pattern of cerebral infarction but they were less frequently anticoagulated. CONCLUSIONS: Ischemic strokes on OAC represent a significant proportion of AF-related strokes. There is an unmet need to better understand the causes underlying these strokes and to optimize the medical management.
BACKGROUND: Multimorbidity is an emerging challenge in older myasthenia gravis patients, which can have even greater impact on quality of life and outcome than symptoms of myasthenia. AIMS OF THE STUDY: We aimed to inves...BACKGROUND: Multimorbidity is an emerging challenge in older myasthenia gravis patients, which can have even greater impact on quality of life and outcome than symptoms of myasthenia. AIMS OF THE STUDY: We aimed to investigate comorbidities in older population and compare early-onset (EOMG) and late-onset (LOMG) myasthenia patients. METHODS: We investigated clinical information of patients from Oxford Myasthenia Centre age 50 or older. Data on 60 chronic disorders were extracted. RESULTS: We included 327 myasthenia patients (30.9% EOMG and 69.1% LOMG) with a median age of 70 years. Comorbidities were present in 94.5% of patients and accumulated with age. Hypertension (58.4% vs. 31.7%), hypercholesterolemia (41.2% vs. 23.8%), diabetes (24.8% vs. 11.9%), cataract (15.5% vs. 5.0%) and prostate disorders (15.0% vs. 2.0%) were more common in LOMG than EOMG, but there were no differences between 70 EOMG and 70 LOMG patients matched according to age and sex. CONCLUSIONS: Comorbidities in older patients with myasthenia are very common, increase with age, and do not differ between early- and late-onset disease.
Acta Neurol Scand
· 2022 Jan · PMID 34750810
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SARS-CoV-2 and adverse reactions to SARS-CoV-2 vaccinations show a tropism for neuronal structures and tissues. This narrative review was conducted to collect and discuss published data about neurological side effects of...SARS-CoV-2 and adverse reactions to SARS-CoV-2 vaccinations show a tropism for neuronal structures and tissues. This narrative review was conducted to collect and discuss published data about neurological side effects of SARS-CoV-2 vaccines in order to discover type, frequency, treatment, and outcome of these side effects. The most frequent neurological side effects of SARS-CoV-2 vaccines are headache, Guillain-Barre syndrome (GBS), venous sinus thrombosis (VST), and transverse myelitis. Other neurological side effects occur in a much lower frequency. Neurological side effects occur with any of the approved vaccines but VST particularly occurs after vaccination with vector-based vaccines. Treatment of these side effects is not at variance from similar conditions due to other causes. The worst outcome of these side effects is associated with VST, why it should not be missed and treated appropriately in due time. In conclusion, safety concerns against SARS-CoV-2 vaccines are backed by an increasing number of studies reporting neurological side effects. The most frequent of them are headache, GBS, VST, and transverse myelitis. Healthcare professionals, particularly neurologists involved in the management of patients having undergone SARS-CoV-2 vaccinations, should be aware of these side effects and should stay vigilant to recognize them early and treat them adequately.
Manea MM, Dragoș D, Enache I
… +2 more, Sirbu AG, Tuta S
Acta Neurol Scand
· 2022 Feb · PMID 34725821
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BACKGROUND: The novel COVID-19 vaccines have side effects that require efficient and close monitoring. AIMS OF THE STUDY: To examine whether the Pfizer-BioNTech vaccine is associated with multiple cranial neuropathy. MET...BACKGROUND: The novel COVID-19 vaccines have side effects that require efficient and close monitoring. AIMS OF THE STUDY: To examine whether the Pfizer-BioNTech vaccine is associated with multiple cranial neuropathy. METHODS: We report the case of a 29-year-old male patient with no notable history who presented with left oculomotor, abducens, trigeminal and facial palsies 6 days after receiving the first dose of the Pfizer-BioNTech COVID-19 vaccine. RESULTS: Gadolinium-enhanced MRI of the brain revealed enhancement in the left facial, trigeminal and oculomotor nerves, which persisted upon repeated examination. The cerebrospinal fluid analysis showed no sign of inflammation, both initially and after 1 month from the start of the patient's symptoms. Other causes were excluded by laboratory tests. The patient received high doses of corticosteroids, with improvement of symptoms. CONCLUSIONS: In our case, the most probable etiology of the patient's multiple cranial neuropathy is the Pfizer-BioNTech vaccine, which highlights the need for prolonged surveillance of COVID-19 vaccine neurological complications.