Acta Neurologica Scandinavica[JOURNAL]
Sun
200 papers
Blood biomarkers for Alzheimer's disease and related disorders.
Zetterberg H, Schott JM
Acta Neurol Scand · 2022 Jul · PMID 35470421 · Publisher ↗
Alzheimer's disease (AD) is a progressive neurodegenerative disease and the single commonest cause of dementia. Many other diseases can, however, cause dementia, and differential diagnosis can be challenging, especially...
Alzheimer's disease (AD) is a progressive neurodegenerative disease and the single commonest cause of dementia. Many other diseases can, however, cause dementia, and differential diagnosis can be challenging, especially in early disease stages. For most neurodegenerative dementias, accumulation of brain pathologies starts many years before clinical onset; the ability to detect these pathologies paves the way for targeted disease-modifying prevention trials. AD is associated with β-amyloid and tau pathologies, which can be quantified using cerebrospinal fluid and imaging biomarkers and, more recently, using highly sensitive blood tests. While for the most part, specific biomarkers of non-AD neurodegenerative dementias are lacking, non-specific biomarkers of neurodegeneration are available. This review summarizes recent advances in the neurodegenerative dementia blood biomarker research and discusses the next steps required for clinical implementation.
Persistent olfactory dysfunction after COVID-19 is associated with reduced perfusion in the frontal lobe.
Yus M, Matias-Guiu JA, Gil-Martínez L … +7 more , Gómez-Ruiz N, Polidura C, Jorquera M, Delgado-Alonso C, Díez-Cirarda M, Matías-Guiu J, Arrazola J
Acta Neurol Scand · 2022 Aug · PMID 35467007 · Full text
BACKGROUND: Olfactory dysfunction is common during SARS-CoV-2 infection. The pathophysiology of the persistence of this symptom and the potential relationship with central nervous system involvement is unknown. AIM OF TH...
BACKGROUND: Olfactory dysfunction is common during SARS-CoV-2 infection. The pathophysiology of the persistence of this symptom and the potential relationship with central nervous system involvement is unknown. AIM OF THE STUDY: To evaluate the neural correlates of persistent olfactory dysfunction in a series of patients with post-COVID syndrome. METHODS: Eighty-two patients with post-COVID syndrome were assessed with the Brief Smell Identification Test and a multimodal MRI study including 3D-T1, T2-FLAIR, diffusion-tensor imaging, and arterial spin labeling. Olfactory and neuroimaging examinations were performed 11.18 ± 3.78 months after the acute infection. Voxel-based brain mapping analyses were conducted to correlate the olfactory test with brain volumes, white matter microstructure, and brain perfusion. RESULTS: Olfactory dysfunction was associated with lower tissue perfusion in the orbital and medial frontal regions in the arterial spin labeling sequence. Conversely, no statistically significant findings were detected in brain volumes and diffusion-tensor imaging. Mild changes in paranasal sinuses and nasal cavities were detected in 9.75% of cases, with no association with olfactory deficits. CONCLUSIONS: We provide new insights regarding the pathophysiology of persistent olfactory dysfunction after COVID-19, involving the main brain regions associated with the olfactory system.
Fiber selectivity of peripheral neuropathy in patients with Parkinson's disease.
Xiong YT, Liu MH, Gu HY … +7 more , Li K, Zhang JR, Cheng XY, Jin H, Chen J, Mao CJ, Liu CF
Acta Neurol Scand · 2022 Jul · PMID 35466436 · Publisher ↗
OBJECTIVE: To determine the function of each type of peripheral nerve fiber and investigate the possible role of levodopa (LD) in peripheral neuropathy (PN) in Parkinson's disease (PD) patients. METHODS: We enrolled 60 p...
OBJECTIVE: To determine the function of each type of peripheral nerve fiber and investigate the possible role of levodopa (LD) in peripheral neuropathy (PN) in Parkinson's disease (PD) patients. METHODS: We enrolled 60 patients with idiopathic PD. All PD patients were divided into three groups: levodopa exposure >3 years (LELD), levodopa exposure ≤3 years (SELD) and de novo patients with PD (NOLD). The current perception threshold (CPT), which was measured by Neurometer at 2000, 250 and 5 Hz, the level of homocysteine, Vitamin B12 and folic acid in plasma, were compared with those of sex- and age-matched healthy controls (HCs). RESULTS: Current perception threshold was higher at 250 Hz (p < .05) and 5 Hz (p < .05) in the LELD group than the NOLD, SELD, and control group. CPT was lower at 5 Hz in the NOLD than in the HCs group (p < .05). The CPT of the more affected side of PD patients was positively correlated with H-Y stage at 5 Hz current stimulation (r = .42, p = .01). Multivariate logistic regression analysis showed that elevated homocysteine levels were the risk factor of sensory nerve injury in PD patients (p < .01). Serum homocysteine levels were positively correlated with levodopa (LD) daily dose, LD equivalent daily dose, and LD cumulative lifetime dose (p < .05). CONCLUSIONS: Peripheral neuropathy in PD patients can occur in the early stage of PD exhibiting as hyperesthesia and is fiber selectivity, especially for Aδ and C nerve fibers. PN in PD patients is related to PD itself and long-term LD exposure. Elevated plasma homocysteine is a risk factor for PN in PD patients.
Reasons and predictors of non-thrombolysis in patients with acute ischemic stroke admitted within 4.5 h.
Bergh E, Jahr SH, Rønning OM … +3 more , Askim T, Thommessen B, Kristoffersen ES
Acta Neurol Scand · 2022 Jul · PMID 35445395 · Full text
OBJECTIVES: Thrombolytic treatment in acute ischemic stroke (AIS) reduces stroke-related disability. Nearly 40% of all patients with AIS (<4.5 h) receive thrombolysis, but there is a large variation in the use between ho...
OBJECTIVES: Thrombolytic treatment in acute ischemic stroke (AIS) reduces stroke-related disability. Nearly 40% of all patients with AIS (<4.5 h) receive thrombolysis, but there is a large variation in the use between hospitals. Little is known about reasons and predictors for not giving thrombolytic treatment. Therefore, we aimed to investigate reasons for non-thrombolysis in patients admitted within 4.5 h. METHODS: All patients with AIS (<4.5 h) admitted to Akershus University Hospital, Norway, between January 2015 and December 2017 were examined. Patient characteristics and reasons for not giving thrombolysis were registered. Descriptive statistics and logistic regression analyses were performed. RESULTS: Of 535 patients admitted with AIS (<4.5 h), 250 (47%) did not receive thrombolysis and of these only 26% had an absolute contraindication to treatment. Among the 74% with relative contraindications, the most common reasons given were mild and improving symptoms. Previous stroke (OR 3.32, 95%CI 1.99-5.52), arriving between 3 h and 4.5 h after onset (OR 7.76, 95%CI 3.73-16.11) or having mild symptoms (OR 2.33, 95%CI 1.56-3.49) were all significant predictors of not receiving thrombolytic treatment in the multivariable logistic regression model. CONCLUSION: A large proportion of patients with AIS do not receive thrombolysis. This study highlights up-to-date findings that arriving late in the time window, mild symptoms, and previous stroke are strong predictors of non-treatment. It is uncertain whether there is an underuse of thrombolysis in AIS. Increasing the utility of thrombolysis in the 4.5 h time window must be weighed against possible harms.
Scattered cerebral infarction in the corona radiata predicts worse outcomes.
Kobayashi Y, Okumura G, Morizumi T … +6 more , Nagamatsu K, Shimizu Y, Sasaki T, Sato A, Sekijima Y, Hongo K
Acta Neurol Scand · 2022 Jul · PMID 35428977 · Publisher ↗
OBJECTIVES: Intracranial branch atheromatous disease often results in progressive motor deficits in the lenticulostriate arteries (LSA). In some patients with LSA infarction, magnetic resonance imaging (MRI) revealed sin...
OBJECTIVES: Intracranial branch atheromatous disease often results in progressive motor deficits in the lenticulostriate arteries (LSA). In some patients with LSA infarction, magnetic resonance imaging (MRI) revealed single lesions at the LSA origin from the middle cerebral artery spreading in a scattered manner toward the distal area. This study aimed to elucidate the clinical characteristics of such cases. MATERIALS AND METHODS: This was a single-center, retrospective study comprising 1,840 consecutive patients admitted to the Ina Central Hospital, Japan. Two neurologists selected patients with LSA infarctions on the basis of MRI data. Patients with a single mass of infarct lesion from the origin were classified as the single group, whereas patients with infarct lesions as a single mass at LSA origin but divided and independent as the infarct area extended distally were classified as the scattered group. We compared the clinical characteristics and outcomes in these groups. RESULTS: The single and scattered groups included 119 and 35 patients, respectively. We defined worsening as an increase of one point or more on the National Institute of Health Stroke Scale. Univariate analysis demonstrated that patients in the scattered group showed significantly more worsening after hospitalization compared with those in the single group (48.6% vs. 28.6%; p < .05). Moreover, this can easily lead to increased disease severity (p < .016). In a multivariate analysis, group (odds ratio, 2.5 [95% CI, 1.11-5.74], p < .03) was an independent predictor of symptom worsening. CONCLUSIONS: Scattered infarction in the corona radiata is an aggravating factor leading to worse outcomes.
Blood biomarkers in epilepsy.
Banote RK, Akel S, Zelano J
Acta Neurol Scand · 2022 Oct · PMID 35411571 · Full text
Robust and accessible biomarkers are greatly needed in epilepsy. Diagnostic and prognostic precision in the clinic needs to improve, and there is a need for objective quantification of seizure burden. In recent years, th...
Robust and accessible biomarkers are greatly needed in epilepsy. Diagnostic and prognostic precision in the clinic needs to improve, and there is a need for objective quantification of seizure burden. In recent years, there have been advances in the development of accessible and cost-effective blood-based biomarkers in neurology, and these are increasingly studied in epilepsy. However, the field is in its infancy and specificity and sensitivity for most biomarkers in most clinical situations are not known. This review describes advancements regarding human blood biomarkers in epilepsy. Examples of biochemical markers that have been shown to have higher blood concentrations in study subjects with epilepsy include brain proteins like S100B or neuronal specific enolase, and neuroinflammatory proteins like interleukins, and tumor necrosis factor-alpha. Some of the blood biomarkers also seem to reflect seizure duration or frequency, and levels decrease in response to treatment with antiseizure medication. For most biomarkers, the literature contains seemingly conflicting results. This is to be expected in an emerging field and could reflect different study populations, sampling or analysis techniques, and epilepsy classification. More studies are needed with emphasis put on the classification of epilepsy and seizure types. More standardized reporting could perhaps decrease result heterogeneity and increase the potential for data sharing and subgroup analyses.
Pregnancy and neurologic complications of COVID-19: A scoping review.
Magalhães JE, Sampaio-Rocha-Filho PA
Acta Neurol Scand · 2022 Jul · PMID 35388457 · Full text
While neurologic complications are frequently reported among patients with COVID-19 in the general population, they are unknown in pregnant women. This paper summarizes the case reports of pregnant women with confirmed S...
While neurologic complications are frequently reported among patients with COVID-19 in the general population, they are unknown in pregnant women. This paper summarizes the case reports of pregnant women with confirmed SARS-CoV-2 infection plus a specified neurologic diagnosis. Until November 2021, 18 case reports were found. Both the central and peripheral nervous systems were equally affected: delirium (n = 1), posterior reversible encephalopathy syndrome (n = 4), cerebrovascular disease (n = 2), acute cerebral demyelinating disease (n = 1), acute necrotizing encephalopathy (n = 1), Guillain-Barré syndrome (n = 5), including one patient who also had vestibular neuritis, Bell's palsy (n = 3), and rhabdomyolysis (n = 1). The median maternal age was 32.5 (25-35) years, the median gestational age was 34 (30-36.5) weeks, and 38.9% presented previous medical conditions. Respiratory symptoms were reported in 76.5%, and 76.5% received immunotherapies to treat the COVID-19 or the neurologic complications. Half the women required admission to ICU and, more often, were those with central nervous system involvement (77.8% vs. 22.2%; Chi-square test, p = .018). For 64.7% of women, the most common method of delivery was surgical, although just one case was due to the neurologic complication. There were reports of one spontaneous abortion, two fetal deaths, and no maternal deaths. Only one case presented a poor neurologic outcome. It is possible that our findings are underestimated, considering that there are thousands of reports regarding neurologic complications in the general population with COVID-19.
Prehospital recognition of stroke is associated with a lower risk of death.
Magnusson C, Herlitz J, Sunnerhagen KS … +3 more , Hansson PO, Andersson JO, Jood K
Acta Neurol Scand · 2022 Aug · PMID 35385136 · Full text
OBJECTIVE: Among patients assessed by the emergency medical service (EMS) and hospitalized with a final diagnosis of stroke, to describe delays, patient characteristics, actions taken and outcome in relation to the early...
OBJECTIVE: Among patients assessed by the emergency medical service (EMS) and hospitalized with a final diagnosis of stroke, to describe delays, patient characteristics, actions taken and outcome in relation to the early recognition of stroke by the EMS clinician. METHODS: Patients admitted to any of six stroke units in Region Västra Götaland, Sweden, with a final diagnosis of stroke from 1 January 2013 to 31 December 2015 were included. Data on follow-up were retrieved from the Swedish Stroke Register. RESULTS: In all, 5467 patients were included. Stroke was recognized by the EMS clinician in 4396 cases (80.4%). The mean difference in the time from dialling 112 until arrival at the stroke unit was 556 min shorter when stroke was recognized, while the mean difference in the time from dialling 112 until a preliminary report from a computed tomography (CT) scan was 219 min shorter as compared with the patients in whom stroke was not recognized. After adjustment for age, sex, neurological deficits and coma, a lack of suspicion of stroke on EMS arrival was associated with an increased risk of death during three months of follow-up (odds ratio 1.66; 95% confidence interval 1.19-2.32; p = .003). CONCLUSION: Among patients with a stroke, more than 80% were recognized by the EMS clinician. Early recognition of stroke was associated with a markedly shorter time until arrival at the stroke unit and until the preliminary report of a CT scan. A lack of early stroke recognition was associated with an increased risk of death.
Blood-based biomarkers and traumatic brain injury-A clinical perspective.
Posti JP, Tenovuo O
Acta Neurol Scand · 2022 Oct · PMID 35383879 · Publisher ↗
Blood-based biomarkers are promising tools to complement clinical variables and imaging findings in the diagnosis, monitoring and outcome prediction of traumatic brain injury (TBI). Several promising biomarker candidates...
Blood-based biomarkers are promising tools to complement clinical variables and imaging findings in the diagnosis, monitoring and outcome prediction of traumatic brain injury (TBI). Several promising biomarker candidates have been found for various clinical questions, but the translation of TBI biomarkers into clinical applications has been negligible. Measured biomarker levels are influenced by patient-related variables such as age, blood-brain barrier integrity and renal and liver function. It is not yet fully understood how biomarkers enter the bloodstream from the interstitial fluid of the brain. In addition, the diagnostic performance of TBI biomarkers is affected by sampling timing and analytical methods. In this focused review, the clinical aspects of glial fibrillary acidic protein, neurofilament light, S100 calcium-binding protein B, tau and ubiquitin C-terminal hydrolase-L1 are examined. Current findings and clinical caveats are addressed.
Predicting the functional outcomes of anti-LGI1 encephalitis using a random forest model.
Li G, Liu X, Wang M … +3 more , Yu T, Ren J, Wang Q
Acta Neurol Scand · 2022 Aug · PMID 35373330 · Publisher ↗
OBJECTIVES: To establish a model in order to predict the functional outcomes of patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis and identify significant predictive factors using a random forest a...
OBJECTIVES: To establish a model in order to predict the functional outcomes of patients with anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis and identify significant predictive factors using a random forest algorithm. METHODS: Seventy-nine patients with confirmed LGI1 antibodies were retrospectively reviewed between January 2015 and July 2020. Clinical information was obtained from medical records and functional outcomes were followed up in interviews with patients or their relatives. Neurological functional outcome was assessed using a modified Rankin Scale (mRS), the cutoff of which was 2. The prognostic model was established using the random forest algorithm, which was subsequently compared with logistic regression analysis, Naive Bayes and Support vector machine (SVM) metrics based on the area under the curve (AUC) and the accuracy. RESULTS: A total of 79 patients were included in the final analysis. After a median follow-up of 24 months (range, 8-60 months), 20 patients (25%) experienced poor functional outcomes. A random forest model consisting of 16 variables used to predict the poor functional outcomes of anti-LGI1 encephalitis was successfully constructed with an accuracy of 83% and an F1 score of 60%. In addition, the random forest algorithm demonstrated a more precise predictive performance for poor functional outcomes in patients with anti-LGI1 encephalitis compared with three other models (AUC, 0.90 vs 0.80 vs 0.70 vs 0.64). CONCLUSIONS: The random forest model can predict poor functional outcomes of patients with anti-LGI1 encephalitis. This model was more accurate and reliable than the logistic regression, Naive Bayes, and SVM algorithm.
Pathophysiology of transient neurological deficit in patients with chronic subdural hematoma: A systematic review.
Blaauw J, Zundert JMV, den Hertog HM … +7 more , van der Gaag NA, Jellema K, Dammers R, Groen RJM, Lingsma HF, van der Naalt J, Jacobs B
Acta Neurol Scand · 2022 Jun · PMID 35355247 · Publisher ↗
Patients with chronic subdural hematoma (CSDH) can have transient neurological deficits deficit (TND) mimicking transient ischemic attacks. The prevalence of TNDs in CSDH varies between 1%-24%, depending on TND definitio...
Patients with chronic subdural hematoma (CSDH) can have transient neurological deficits deficit (TND) mimicking transient ischemic attacks. The prevalence of TNDs in CSDH varies between 1%-24%, depending on TND definition. Despite this high prevalence the pathophysiology of TND in CSDH is not clear in many cases. In this systematic review, we aim to unravel the responsible mechanism. Pubmed and Embase were searched for all articles concerning the pathophysiology of TND as a presenting symptom in patients with CSDH. There were no publication date restrictions for the articles in the search. Two reviewers independently selected studies for inclusion and subsequently extracted the necessary data. Out of 316 identified references, 15 met the inclusion criteria. Several articles mentioned multiple pathophysiological mechanisms. One of the proposed etiologies of TND was epileptic activity, stated by three articles. In contrast, three different studies stated that seizures are unlikely to cause TND. Five papers suggested that obstruction of blood flow, caused by the hematoma or subsequent swelling, might be the cause. Six articles made no definite statement on the responsible pathophysiological mechanism of TND. Different mechanisms have been proposed to be the cause of TNDs in patients with CSDH. Based on this review, the exact pathophysiology of TND remains unclear. We suggest that future studies on this topic should incorporate MRI of the brain (with diffusion-weighted imaging) and EEG, to provide better insight into TND pathophysiology. The knowledge resulting from future studies might contribute to better understanding of TND and optimal treatment in CSDH.
Sports and trauma as risk factors for Motor Neurone Disease: New Zealand case-control study.
Chen GX, Douwes J, van den Berg LH … +3 more , Glass B, McLean D, 't Mannetje AM
Acta Neurol Scand · 2022 Jun · PMID 35355246 · Full text
OBJECTIVES: To assess whether sports, physical trauma and emotional trauma are associated with motor neurone disease (MND) in a New Zealand case-control study (2013-2016). METHODS: In total, 321 MND cases and 605 populat...
OBJECTIVES: To assess whether sports, physical trauma and emotional trauma are associated with motor neurone disease (MND) in a New Zealand case-control study (2013-2016). METHODS: In total, 321 MND cases and 605 population controls were interviewed collecting information on lifetime histories of playing sports, physical trauma (head injury with concussion, spine injury) and emotional trauma (14 categories). ORs were estimated using logistic regression adjusting for age, sex, ethnicity, socioeconomic status, education, smoking status, alcohol consumption and mutually adjusting for all other exposures. RESULTS: Head injury with concussion ≥3 years before diagnosis was associated with MND (OR 1.51, 95% CI: 1.09-2.09), with strongest associations for two (OR 4.01, 95% CI: 1.82-8.86), and three or more (OR 2.34, 95% CI: 1.00-5.45) head injuries. Spine injury was not associated with MND (OR 0.81, 95% CI: 0.48-1.36). Compared to never playing sports, engaging in sports throughout childhood and adulthood increased MND risk (OR 1.81, 95% CI: 1.01-3.25), as was more than 12 years playing football/soccer (OR 2.35, 95% CI: 1.19-4.65). Reporting emotionally traumatic events in more than three categories was associated with MND (OR 1.88, 95% CI: 1.17-3.03), with physical childhood abuse the only specific emotional trauma associated with MND (OR 1.82, 95% CI: 1.14-2.90), particularly for those reporting longer abuse duration (OR 2.26, 95% CI: 1.14-4.49; OR 3.01, 95% CI: 1.18-7.70). For females, having witnessed another person being killed, seriously injured or assaulted also increased MND risk (OR 2.68, 95% CI: 1.06-6.76). CONCLUSIONS: This study adds to the evidence that repeated head injury with concussion, playing sports in general, and playing football (soccer) in particular, are associated with an increased risk of MND. Emotional trauma, that is physical abuse in childhood, may also play a role.
Quality of life and resource utilization-Swedish data from the Care of Late-Stage Parkinsonism (CLaSP) study.
M Norlin J, Hjalte F, Kruse C … +3 more , Dodel R, Rosqvist K, Odin P
Acta Neurol Scand · 2022 Jun · PMID 35312034 · Publisher ↗
BACKGROUND: Few studies have investigated the health-related quality of life (HRQoL), resource use and costs in patients with late-stage Parkinson's disease (PD), and data from the Swedish setting are scarce. OBJECTIVES:...
BACKGROUND: Few studies have investigated the health-related quality of life (HRQoL), resource use and costs in patients with late-stage Parkinson's disease (PD), and data from the Swedish setting are scarce. OBJECTIVES: First, we analyse the HRQoL in late-stage PD in Sweden. Second, we analyse the resource use and costs per severity level. Third, we analyse the relationship between costs and physician- and patient reported-outcome measures. MATERIALS AND METHODS: The study was based on Swedish data from the Care of Late-Stage Parkinsonism (CLaSP) study. The costs of healthcare contacts, drugs, formal and informal care, and productivity loss were collected over three months. Assessments at baseline were used for outcomes (EQ-5D, Hoehn and Yahr (H&Y), Schwab and England Scale, Unified Parkinson's Disease Rating Scale subscales (UPDRS) and Non-Motor Symptoms Scale (NMSS)). Costs were estimated in € 2016. RESULTS: In total, 106 patients were included. The mean EQ-5D score in the total group was 0.24 (±0.33). The mean total cost excluding informal care per patient in the three-month period was approximately €14,097 (BCa 95% CI €12,007 and €16,039). Professional care accounted for the largest share (75 percent) of the total costs. The EQ-5D, H&Y, Schwab and England Scale, and NMSS were statistically significant predicting factors for total costs. CONCLUSION: Patients with late-stage PD are a vulnerable patient group that is costly to society and the impairment in patients' HRQoL is immense. Thus, healthcare decision-makers should optimize the organization and provision of healthcare for these patients.
Genetic analysis reveals novel variants for vascular cognitive impairment.
Mönkäre S, Kuuluvainen L, Schleutker J … +5 more , Bras J, Roine S, Pöyhönen M, Guerreiro R, Myllykangas L
Acta Neurol Scand · 2022 Jul · PMID 35307828 · Full text
OBJECTIVES: The genetic background of vascular cognitive impairment (VCI) is poorly understood compared to other dementia disorders. The aim of the study was to investigate the genetic background of VCI in a well-charact...
OBJECTIVES: The genetic background of vascular cognitive impairment (VCI) is poorly understood compared to other dementia disorders. The aim of the study was to investigate the genetic background of VCI in a well-characterized Finnish cohort. MATERIALS & METHODS: Whole-exome sequencing (WES) was applied in 45 Finnish VCI patients. Copy-number variant (CNV) analysis using a SNP array was performed in 80 VCI patients. This study also examined the prevalence of variants at the miR-29 binding site of COL4A1 in 73 Finnish VCI patients. RESULTS: In 40% (18/45) of the cases, WES detected possibly causative variants in genes associated with cerebral small vessel disease (CSVD) or other neurological or stroke-related disorders. These variants included HTRA1:c.847G>A p.(Gly283Arg), TREX1:c.1079A>G, p.(Tyr360Cys), COLGALT1:c.1411C>T, p.(Arg471Trp), PRNP: c.713C>T, p.(Pro238Leu), and MTHFR:c.1061G>C, p.(Gly354Ala). Additionally, screening of variants in the 3'UTR of COL4A1 gene in a sub-cohort of 73 VCI patients identified a novel variant c.*36T>A. CNV analysis showed that pathogenic CNVs are uncommon in VCI. CONCLUSIONS: These data support pathogenic roles of variants in HTRA1, TREX1 and in the 3'UTR of COL4A1 in CSVD and VCI, and suggest that vascular pathogenic mechanisms are linked to neurodegeneration, expanding the understanding of the genetic background of VCI.
Comment on comorbidities in older patients with myasthenia gravis.
Galassi G
Acta Neurol Scand · 2022 Jun · PMID 35307817 · Publisher ↗
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Dopamine transporter SPECT imaging in corticobasal syndrome: A peak into the underlying pathology?
Constantinides VC, Souvatzoglou M, Paraskevas GP … +4 more , Chalioti M, Boufidou F, Stefanis L, Kapaki E
Acta Neurol Scand · 2022 Jun · PMID 35307816 · Publisher ↗
BACKGROUND: Multiple pathologies may underlie corticobasal syndrome (CBS), including Alzheimer's disease (AD). Dopamine transporter density imaging with Ioflupane I SPECT (DaTscan) may be normal in CBS. No studies to da...
BACKGROUND: Multiple pathologies may underlie corticobasal syndrome (CBS), including Alzheimer's disease (AD). Dopamine transporter density imaging with Ioflupane I SPECT (DaTscan) may be normal in CBS. No studies to date have examined the relationship between DaTscan status and underlying pathology in CBS. OBJECTIVES: The main objective of the study was to test whether a normal DaTscan in CBS patients is indicative of an underlying AD pathology, as determined by cerebrospinal fluid (CSF) biomarkers. METHODS: Eighteen CBS patients were included. They were divided into patients with an AD and a non-AD disease pathology, based on their cerebrospinal fluid biochemical profile. A typical AD CSF profile was defined as an increase in total and phosphorylated at threonine 181 tau protein in addition to a decrease in amyloid-beta with 42 amino acids. DaTscan data were compared in these two groups. RESULTS: Eight of the 18 CBS patients (44%) had a normal DaTscan. Seven of the 18 CBS patients (39%) had an AD cerebrospinal fluid biochemical profile. Two of seven CBS patients with AD biomarker profile had abnormal DaTscans. Three of 11 CBS patients with a non-AD biomarker profile had normal DaTscans. A normal DaTscan was indicative of AD pathology with suboptimal (~70%) sensitivity and specificity. Semi-quantitative DaTscan analysis did not differentiate between AD from non-AD CSF biomarker profile in CBS. CONCLUSION: A normal DaTscan is indicative of AD in CBS, but the sensitivity and specificity of DaTscan as an in vivo marker of AD pathology is suboptimal.
Bone health, intellectual disability and epilepsy: An observational community-based study.
Winterhalder R, McCabe J, Young C … +5 more , Lamb K, Sawhney I, Jory C, O'Dwyer M, Shankar R
Acta Neurol Scand · 2022 Jun · PMID 35297524 · Full text
OBJECTIVES: Intellectual disability (ID) and epilepsy are independent risk factors for osteoporosis. Diverse predisposing factors influence this, for example in ID, genetics and poor nutrition and in epilepsy, anti-seizu...
OBJECTIVES: Intellectual disability (ID) and epilepsy are independent risk factors for osteoporosis. Diverse predisposing factors influence this, for example in ID, genetics and poor nutrition and in epilepsy, anti-seizure medication (ASM). Around 25% people with ID have epilepsy, majority treatment resistant. ASMs polypharmacy is common. However, little is known about the bone-related characteristics of this vulnerable group. A prospective observational cohort study of bone profile across a community ID Epilepsy service was undertaken to understand this. MATERIALS & METHODS: Participants were on minimum 2 years of ASMs. Baseline demographics, epilepsy data, bone metabolism biomarkers, bone mineral density (BMD) and vitamin D levels were collected. Doses needed to correct vitamin D insufficiency/deficiency were calculated. RESULTS: At baseline, of 104 participants, 92 (90.2%) were vitamin D insufficient/deficient. Seventy-six (73.1%) had a DEXA scan, 50 of whom-in the osteopaenic/osteoporotic range. DEXA scores between ambulant and non-ambulant patients were significantly different (p = .05) but not for ID severity. A high alkaline phosphatase (ALP) predicted lower vitamin D levels. Borderline significance (p = .06) in calcium levels between normal and high ALP was identified. There were no significant associations between parathyroid hormone, inorganic phosphate and magnesium levels, with vitamin D status or DEXA hip T-scores. Normalizing vitamin D levels (mean 101.4 nmol/L) required an average of 1951IU cholecalciferol daily. CONCLUSIONS: Vitamin D deficiency is highly prevalent in people with ID and epilepsy treated with ASMs impacting likely on their bone health. Screening with vitamin D levels, ALP and DEXA in this group should be pro-actively and routinely considered.
Ketamine in adult super-refractory status epilepticus: Efficacy analysis on a prospective registry.
Caranzano L, Novy J, Rossetti AO
Acta Neurol Scand · 2022 Jun · PMID 35274736 · Full text
BACKGROUND: Status epilepticus (SE) persisting despite two anti-seizures medications (ASM) and anesthetics is labeled super refractory (SRSE), correlating with important morbidity and mortality. Its treatment relies on e...
BACKGROUND: Status epilepticus (SE) persisting despite two anti-seizures medications (ASM) and anesthetics is labeled super refractory (SRSE), correlating with important morbidity and mortality. Its treatment relies on expert opinions. Due to its pharmacological properties, ketamine (KET) has received increasing attention, but data are essentially retrospective. AIMS: To describe an unselected cohort of adults receiving KET for SRSE. METHODS: Analysis of a prospective registry of consecutive SE episodes, identifying SRSE patients receiving ketamine (KET). Comparison with recent adult series including more than 10 patients. RESULTS: Eleven patients received KET after a median of 4 days (range: 2-20); median dose was 5 mg/kg/h (range: 2.5-15). KET provided permanent SE control in three (27%). Previous series, using KET administration delays and doses similar to our cohort, report KET efficacy in 28-96% of cases. CONCLUSIONS: We found a lower SE control rate than existing literature, whose data are, however, often retrospective, potentially selecting patients with less severe SE forms or responding to KET. This might explain outcome differences, as KET administration modalities were comparable with our cohort. Since randomized controlled studies are lacking on this subject, the analysis of this prospective, unselected cohort, if confirmed, suggests a current overestimation of KET efficacy in SRSE.
Epileptic seizure control during and after pregnancy in Australian women.
Vajda FJE, O'Brien TJ, Graham JE … +4 more , Hitchcock AA, Perucca P, Lander CM, Eadie MJ
Acta Neurol Scand · 2022 Jun · PMID 35257362 · Publisher ↗
OBJECTIVES: To study factors that affected previous epileptic seizure control throughout pregnancy, during labour, and in the post-natal weeks. MATERIALS & METHODS: Analysis of data concerning seizure freedom that was av...
OBJECTIVES: To study factors that affected previous epileptic seizure control throughout pregnancy, during labour, and in the post-natal weeks. MATERIALS & METHODS: Analysis of data concerning seizure freedom that was available at various stages of 2337 pregnancies in the Raoul Wallenberg Australian Pregnancy Register of Antiepileptic Drugs, mainly employing multiple variable logistic regression techniques. RESULTS: Based on data available at the outset of pregnancy, the risk of seizure-affected that is, not seizure-free pregnancy was statistically significantly (p < .05) higher in pregnancies where there was previously uncontrolled epilepsy (78.1% vs. 20.8%) and focal epilepsy (51.3% vs. 39.7%), and decreased with later onset-age epilepsy (41.8% vs. 52.2% with onset before age 13 years), The risk did not differ between initially antiseizure medication (ASM)-treated or untreated pregnancies. For epilepsy receiving ASM therapy, 90.6% of 160 pregnancies of women with uncontrolled focal epilepsy that began before the age of 13 were seizure-affected. None of the above factors influenced the risk of seizures during labour, though having seizures during pregnancy increased the hazard (3.93 vs. 0.6%). Either ASM-treated pregnancy or labour being seizure-affected increased the risk of post-partum period seizures (33.0% vs. 6.67% for both stages being seizure-free). Use of particular ASMs had no statistically significant effect on the seizure control situation at any of the pregnancy stages studied. CONCLUSIONS: Obtaining full seizure control before pregnancy appeared to be the main factor in maintaining seizure freedom during pregnancy, labour and the post-natal weeks.
Stroke after COVID-19 vaccination.
Famularo G
Acta Neurol Scand · 2022 Jun · PMID 35257361 · Full text
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