Acta Neurologica Scandinavica最新文献

Background. Potassium and sodium are inextricably linked to the maintenance of cell potential and electrolyte homeostasis. Few studies have examined their joint relationship with outcomes after stroke. The objective of this study is to ascertain whether combined low nadir serum sodium and potassium levels are correlated with an unfavorable outcome in ischemic stroke. Methods. Retrospective cohort study of 2,920 consecutive patients with first-ever ischemic stroke. Serum sodium and potassium levels were measured at 10 consecutive time points over 10 days poststroke. The Youden Index was performed to identify patients with low nadir sodium (<140 mmol/L) and potassium (<3.65 mmol/L) levels, defined as a NaK score of 2. Multivariable logistic regression and Cox proportional hazard analysis were used to evaluate the independent relationship of sodium and potassium levels with clinical outcomes at three months. Results. A total of 2,920 patients with ischemic stroke were analyzed (mean age 62.14 ± 14.13 years; 60.19% male), of whom 740 (25.3%) with both low nadir sodium and potassium levels had a quintuple 3-month case fatality compared to other patients (10.6% vs. 2.1%). Multivariable analyses identified NaK = 2 as an independent predictor of 3-month death (adjusted odds ratio (OR) 2.23; 95% confidence interval (CI) 1.17-4.53; p = 0.019) and an unfavorable shift in the distribution of scores on the modified Rankin scale (adjusted OR 1.51, 95% CI 1.12-2.04; p = 0.007). Conclusions. Low sodium and potassium levels are common after ischemic stroke and are independent predictors of subsequent death.

Purpose. This retrospective study is aimed at investigating the clinical characteristics of autoimmune encephalitis (AE) and long-term prognosis of patients who initially present with seizures as well as risk factors for enduring predisposition to seizures in AE. Methods. From January 1, 2013, to October 31, 2021, a total of 343 AE patients from a single center diagnosed with autoimmune encephalitis (AE) were enrolled in this study, including 198 antibody-positive AE and 145 antibody-negative but probable AE. According to initial symptoms, AE patients were divided into two groups: onset with seizure group and onset with nonseizure group. The clinical characteristics were retrospectively reviewed. Patients were clinically evaluated at onset and at 6, 12, and 24 months of follow-up. Modified Rankin Scale (MRS) score, Clinical Assessment Scale in Autoimmune Encephalitis (CASE) score, and seizure-related information were assessed. Results. In AE, patients with seizures as the first presentation were younger, with a median-onset age of 28 years old. Compared with other types of antibody-positive AE, anti-GABABR AE more frequently began with seizures, while anti-CASPR2, anti-AMPAR, and anti-DPPX encephalitis usually began with symptoms other than seizures. The most common type of initial seizures in AE was focal to bilateral seizure (67.6%), with a significant prevalence in antibody-positive AE (P = 0.001). In addition, compared with nonseizure group, patients with seizures as an initial presentation had higher MRS and CASE scores at 24 months of follow-up. Older age at onset and focal nonmotor seizure type were independent risk factors for an enduring predisposition to seizures in AE patients. Conclusion. The younger and anti-GABABR-positive AE patients are more prone to onset with seizures. AE patients who initially presented with seizures had worse long-term neurological recovery. Onset age and seizure type should be highly appreciated when formulating the strategy for therapy at post-AE status.

Background. Although patients can present with progressive pseudobulbar palsy due to neurodegenerative diseases, detection of the precise location of radiological abnormalities can be difficult. ¹⁸F-THK5351 was initially developed as a tau positron emission tomography (PET) tracer. Later, it was found to sensitively detect astrogliosis associated with neurodegeneration. Therefore, it has been used in diagnosis of various diseases. However, its utility in progressive pseudobulbar palsy was unknown. Methods. ¹⁸F-THK5351 PET results of two patients presenting with progressive pseudobulbar palsy are reported. Results. Patient 1 was a 77-year-old man with a two-year history, and Patient 2 was a 61-year-old woman with a 1-year history. Both patients presented with gradually progressive spastic dysarthria, suggesting pseudobulbar palsy without clinical lower motor neuron signs. Facial asymmetry was detected in both patients, while left-dominant pyramidal signs in the extremities were detected only in Patient 2. Brain magnetic resonance imaging did not show signal abnormality explaining pseudobulbar palsy. However, ¹⁸F-THK5351 PET clearly visualized bilateral increased uptake in limited areas of the posterior portion of the precentral gyrus, corresponding to the midportion of the primary motor cortex. Laterality of increased ¹⁸F-THK5351 uptake corresponded to the symptom laterality and was higher on the left and right side in patients 1 and 2, respectively. After one year, Patient 1 was unable to vocalize and could only produce grunts; concomitant apraxia of speech was suspected. Conclusions. ¹⁸F-THK5351 PET is a useful method to detect bilateral primary motor cortex involvement in patients presenting with progressive pseudobulbar palsy, likely by imaging astrogliosis.

Objective. Nocturnal seizures are usually underestimated and represent a major problem in adult patients with epilepsy. Our aim was to study the effectiveness of perampanel for the treatment of nocturnal seizures in adult patients with epilepsy. Methods. Observational study of a prospectively acquired sample of adult patients with focal and generalized epilepsy in which perampanel was started from January to October 2021 in a specialized epilepsy unit in a tertiary hospital. Demographic and clinical characteristics were recorded. All patients completed a follow-up period of at least 3 months. Seizure frequency during the 6-month period before the patient started treatment was obtained from medical records. Retention and responder rates (considered as a nocturnal seizure frequency reduction of ≥50%) and improvement of subjective sleep disturbances were analyzed as outcome measures. Results. Forty-eight patients were included (mean age 39.8 ± 17.4; 60.4% men), and 38 of them had a 6-month follow-up. Focal epilepsy was the most common diagnosis (81.3%), and most patients had a structural etiology (56.3%). Thirty-four (70.8%) patients had drug-resistant epilepsy. The mean nocturnal seizure frequency per month at baseline was 13.2 ± 35.9. Fifteen (31.3%) patients had subjective sleep disturbances at baseline, of which insomnia was the most frequent complaint (16.7%). Perampanel was started at a median dose of 4 mg/day (range = 2-14). At 3-month follow-up, the retention rate was 74.6%; 64.6% were considered responders (54.2% were seizure-free). Monthly nocturnal seizures decreased significantly at 3 months (8.2 ± 26.7 vs. 13.2 ± 35.9 seizures/month; p = 0.044) and 6 months (5.3 ± 18.2 vs. 13.2 ± 35.9 seizures/month; p = 0.006). Subjective sleep disturbances improved at 3-month follow-up (10.4% vs. 31.3%; p = 0.002) and 6-month follow-up (10.5% vs. 31.3%; p = 0.022). Significance. Perampanel can be a suitable treatment option in adult patients with both focal and generalized epilepsy with nocturnal seizures and can reduce the presence of sleep complaints.

Background. Acute sensorineural hearing loss (SNHL) is a rare development in the central nervous system (CNS) demyelinating diseases such as aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods. We retrospectively reviewed consecutive patients with seropositive NMOSD or MOGAD in the CNS Inflammatory and Demyelinating Disease Registry at Samsung Medical Center from January 2015 to December 2020. After the medical chart review, the demographic data and the results of brain magnetic resonance imaging (MRI) and audiometry of patients with hearing loss were collected. Results. Five patients (NMOSD, n = 3; MOGAD, n = 2) were diagnosed with SNHL, two developed SNHL before the first core clinical symptom, and another two patients who underwent brain MRI at the timing of hearing loss showed lesions. Only three patients received high-dose steroids; however, hearing loss did not improve in any patients. Conclusion. SNHL was observed in a small number of patients with seropositive NMOSD and MOGAD; however, it could be underrecognized. Further large cohort prospective studies are helpful to elucidate the clinical implication of SNHL in NMOSD and MOGAD.

The outcome of spontaneous intracranial hypotension (SIH) years after onset is largely unknown. The objective of our study was to describe our clinical experience and long-term outcomes in a case series of patients with SIH. From March 2007 to March 2022, demographic variables, clinical symptoms, neuroimaging findings, and response to treatment were retrospectively analyzed in patients with confirmed SIH and in a subgroup of patients with clinical symptoms but not confirmed by MR or LP, probable SIH (pSIH). We have included 37 SIH and 13 pSIH patients. The average age at onset was 44 years, and 59% (pSIH 46%) were women. All patients presented with a new-onset orthostatic headache. In the SIH group, brain MRI showed signs of intracranial hypotension in all patients, spinal MR was performed in 70%, and pathological findings were identified in 73%. The range of EBP was 1-8 (average 2.2). Good outcome after single or 2 EBPs had 42% (pSIH 46%) of patients. At follow-up, 81% (pSIH 54%) of patients had a favorable outcome. Relapse occurred in 16% of patients in the SIH group and none in the pSIH group. The mean follow-up time was 60 months. EBP is an effective and minimally invasive treatment, and efficacy seems independent of disease duration. The long-term prognosis is favorable in 80% of SIH patients and in half of pSIH patients. Despite the lack of MRI signs of low intracranial pressure on neuroimaging, pSIH patients should also be offered EBP, and more awareness of SIH is needed.

Introduction. Spinal muscular atrophy (SMA) is an autosomal recessive disorder causing lower motor neuron degeneration leading to weakness and muscle atrophy due to reduction of survival motor neuron (SMN) protein. Although SMA was considered an exclusively motor neuron disease, few reports indicate the involvement of nonmotor neurons. The aim of this study is to investigate autonomous nervous system (ANS) involvement in SMA. Materials and Methods. We investigated 9 SMA adult patients and 36 age- and sex-matched controls. ANS was evaluated by sympathetic skin response (SSR). Results. SSR was not elicited in 28% of measurements in cases and in 0% of measurements in controls (p < 0.001). Both palmar (p < 0.001) and plantar (p < 0.001) SSR latencies were significantly longer in cases than controls. Palmar SSR amplitudes were smaller (p = 0.036) in patients compared to controls. Conclusions. This study provides new evidence of ANS dysfunction in SMA patients.

Background. Migraine is a neurological disorder that results in disability accumulation, and there are no blood-based markers for indicating migraine susceptibility. Here, we aimed to evaluate the possible biomarker role of neuropeptides, vasoactive intestinal peptide (VIP), and pituitary adenylate cyclase-activating polypeptide (PACAP) in chronic (CM) and episodic migraine (EM). Method. PubMed, medRxiv, and Google Scholar databases were searched up to the 7^th of September 2022 using the search syntax to define all relevant articles addressing the plasma and serum levels of VIP and PACAP in migraineurs and controls. Concerning bias assessment, the risk of bias in nonrandomized studies-I (ROBINS-I) was assessed. Also, the standardized mean difference (SMD) was measured with a random effects model. Results. Five case-control studies with 503 migraine cases were included. CM patients had elevated VIP levels compared to controls (SMD = 0.44, 95% CI 0.25-0.62, p < 0.00001). In contrast, PACAP levels were lower in EM patients (SMD = −0.30, 95% CI -0.48 to -0.11, p = 0.002). Overall, migraine cases had higher VIP levels (SMD = 0.25, 95% CI 0.11-0.39, p = 0.0006) but lower PACAP levels (SMD = −0.16, 95% CI -0.30 to -0.03, p = 0.020) than controls. Conclusion. The results support the role of VIP and PACAP neuropeptides in migraine pathophysiology. CM patients have significantly higher serum VIP levels, while EM patients have lower serum PACAP levels compared to controls. Further studies should confirm these findings.

Background. Nowadays, there is a lack of studies reporting techniques for the selective monitoring of the primary somatosensory cortex and the adjacent areas of the superior and inferior parietal lobules. We hypothesized a more specific and targeted test for awake surgery for monitoring the sensory area during resection of tumors involving it. Materials and Methods. We collected patients suffering from tumors involving the parietal areas and undergoing awake surgery for the resection. Intraoperative standard neurophysiological monitoring was performed, and we added a new intraoperative test. It consisted of a series of different objects with standard 3D conformations. The patient was asked to recognize the object shape using only the tactile sensibility, without seeing the object itself; in some cases, he was also asked to put the object in the corresponding hole, according to the shape. Results. We collected 6 patients. One patient with a right parieto-occipital lesion, at the stimulation of the anterior cortical margin of the surgical field, showed problems in naming the objects and collocating them in the corresponding spaces, while he was touching them with the left hand. Therefore, the areas of proprioception and perception of the objects were mapped and numbered. This deficit got better in the postoperative days with a total remission of the ideomotor apraxia and the psychomotor slowdown. The other 5 patients did not show an impairment with the new test. Conclusions. This is a preliminary study with the aim of enhancing the specificity of the neuropsychological test performed during awake surgery to allow the surgeon to monitor the neurological functions of the parietal cortex. More cases are needed to validate it.