Introduction. An easily accessible and inexpensive neurophysiological technique such as conventional electroencephalography may provide an accurate and generally applicable biomarker capable of ...differentiating dementia with Lewy bodies (DLB) from Alzheimer’s disease (AD) and Parkinson’s disease-associated dementia (PDD). Method. We carried out a retrospective visual analysis of resting-state electroencephalography (EEG) recording of 22 patients with a clinical diagnosis of 19 probable and 3 possible DLB, 22 patients with probable AD and 21 with PDD, matched for age, duration, and severity of cognitive impairment. Results. By using the grand total EEG scoring method, the total score and generalized rhythmic delta activity frontally predominant (GRDAfp) alone or, even better, coupled with a slowing of frequency of background activity (FBA) and its reduced reactivity differentiated DLB from AD at an individual level with an high accuracy similar to that obtained with quantitative EEG (qEEG). GRDAfp alone could also differentiate DLB from PDD with a similar level of diagnostic accuracy. AD differed from PDD only for a slowing of FBA. The duration and severity of cognitive impairment did not differ between DLB patients with and without GRDAfp, indicating that this abnormal EEG pattern should not be regarded as a disease progression marker. Conclusions. The findings of this investigation revalorize the role of conventional EEG in the diagnostic workup of degenerative dementias suggesting the potential inclusion of GRDAfp alone or better coupled with the slowing of FBA and its reduced reactivity, in the list of supportive diagnostic biomarkers of DLB.
The official variations of status epilepticus (SE) International League Against Epilepsy (ILAE, 2015) diagnostic criteria and the non-convulsive SE (NCSE) Salzburg Consensus Criteria (2013), impose ...the collection of updated population-based epidemiological Italian data. In this study, we aimed at evaluating (a) the frequency of SE in our hospital adopting the new ILAE 2015 SE diagnostic criteria and NCSE Salzburg Consensus Criteria, (b) the frequency of adherence to current treatment guidelines for SE and their relationship with patients’ outcome, and (c) reliability of standardized prognostic scales (Status Epilepticus Severity Score—STESS—and modified STESS) for short-term outcome prediction in the setting of the newest diagnostic criteria for SE and NCSE. Detailed clinical and electrophysiological data collected in a 1-year retrospective hospital-based single-center survey on SE at Parma Hospital, Northern Italy are provided. Non-adherence to current treatment guidelines was recorded in around 50% cases, but no relation to outcome was appreciated. Mortality in our cohort increased from 30 to 50% when follow-up was extended to 30 days. STESS score was strongly correlated with short-term mortality risk (OR 18.9, 2.2–163.5, CI), and we confirm its role as easy-to-use tool for outcome evaluation also when the new ILAE diagnostic SE criteria are applied.
The timely introduction of non-invasive ventilation (NIV) is extremely relevant in the multidisciplinary management of patients affected by amyotrophic lateral sclerosis (ALS) and is based on the ...proper identification of red flags for early diaphragmatic exhaustion. Polygraphic sleep recording may provide insightful information on the ongoing respiratory impairment; in particular, atypical breathing patterns need to be recognized, as the application of current guidelines for sleep-related hypoxemia or sleep apnea may be insufficient for detecting early signs of diaphragmatic fatigue. We report the case of a 51-year-old man affected by ALS who was asymptomatic for breathing impairment, but whose nocturnal polysomnographic recording, despite not significant for obstructive sleep apnea nor for conventional hypoventilatory patterns, strongly suggested initial respiratory failure, as lately confirmed by the pulmonary follow-up. We discuss the advantages of including sleep recording in the clinical work-up of patients affected by ALS.
Few studies have focused on elderly (>80 years) amyotrophic lateral sclerosis (ALS) patients, who represent a fragile subgroup generally not included in clinical trials and often neglected because ...they are more difficult to diagnose and manage. We analyzed the clinical and genetic features of very late-onset ALS patients through a prospective, population-based study in the Emilia Romagna Region of Italy. From 2009 to 2019, 222 (13.76%) out of 1613 patients in incident cases were over 80 years old at diagnosis, with a female predominance (F:M = 1.18). Elderly ALS patients represented 12.02% of patients before 2015 and 15.91% from 2015 onwards (
= 0.024). This group presented with bulbar onset in 38.29% of cases and had worse clinical conditions at diagnosis compared to younger patients, with a lower average BMI (23.12 vs. 24.57 Kg/m
), a higher progression rate (1.43 vs. 0.95 points/month), and a shorter length of survival (a median of 20.77 vs. 36 months). For this subgroup, genetic analyses have seldom been carried out (25% vs. 39.11%) and are generally negative. Finally, elderly patients underwent less frequent nutritional- and respiratory-supporting procedures, and multidisciplinary teams were less involved at follow-up, except for specialist palliative care. The genotypic and phenotypic features of elderly ALS patients could help identify the different environmental and genetic risk factors that determine the age at which disease onset occurs. Since multidisciplinary management can improve a patient's prognosis, it should be more extensively applied to this fragile group of patients.
Increased incidence rates of amyotrophic lateral sclerosis (ALS) have been recently reported across various Western countries, although geographic and temporal variations in terms of incidence, ...clinical features and genetics are not fully elucidated. This study aimed to describe demographic, clinical feature and genotype-phenotype correlations of ALS cases over the last decade in the Emilia Romagna Region (ERR). From 2009 to 2019, our prospective population-based registry of ALS in the ERR of Northern Italy recorded 1613 patients receiving a diagnosis of ALS. The age- and sex-adjusted incidence rate was 3.13/100,000 population (M/F ratio: 1.21). The mean age at onset was 67.01 years; women, bulbar and respiratory phenotypes were associated with an older age, while C9orf72-mutated patients were generally younger. After peaking at 70-75 years, incidence rates, among women only, showed a bimodal distribution with a second slight increase after reaching 90 years of age. Familial cases comprised 12%, of which one quarter could be attributed to an ALS-related mutation. More than 70% of C9orf72-expanded patients had a family history of ALS/fronto-temporal dementia (FTD); 22.58% of patients with FTD at diagnosis had C9orf72 expansion (OR 6.34,
= 0.004). In addition to a high ALS incidence suggesting exhaustiveness of case ascertainment, this study highlights interesting phenotype-genotype correlations in the ALS population of ERR.
Herpes simplex virus encephalitis (HSE) is the most common cause of letal encephalitis and its prevalence appears higher among oncologic patients who undergo brain radiotherapy (RT). We describe a ...case of 76-year-old woman with glioblastoma multiforme (GBM) who developed HSE shortly after brain RT. Cerebrospinal fluid analysis (CSF) was normal and the diagnosis was driven by brain MRI and EEG. Prompt introduction of antiviral therapy improved the clinical picture. We highlight the importance of EEG and brain MRI for the diagnosis and suggest the possibility of antiviral profilaxys in oncologic patients who undergo brain RT.
Anti-recoverin antibodies are typically found in cancer-associated retinopathy or autoimmune diseases. We present a case of anti-recoverin positive cerebellar syndrome without any signs of malignancy ...or retinopathy. The patient was treated with steroids and intravenous immunoglobulins, resulting in improvements in both cognitive and motor symptoms.
•This study supports the utility of differentiating between periodic discharges (PDs) during and after Status Epilepticus (SE)•Interictal PDs during SE are not related to outcome; PDs occurring ...24–72 h from SE resolution strongly correlate with poor prognosis.•Considering only PDs after SE cessation improves EMSE (Epidemiology-based mortality score in SE) overall accuracy.
Periodic Discharges (PDs) in Status Epilepticus (SE) are historically related to negative outcome, and the Epidemiology-based Mortality Score in SE (EMSE) identifies PDs as an EEG feature associated with unfavorable prognosis. However, supportive evidence is conflicting. This study aims to evaluate the prognostic significance of interictal PDs during and following SE.
All 2020–2023 non-hypoxic-ischemic SE patients with available EEG during SE were retrospectively assessed. Interictal PDs during SE (SE-PDs) and PDs occurring 24–72 h after SE resolution (post-SE-PDs) were examined. In-hospital death was defined as the primary outcome.
189 SE patients were finally included. SE-PDs were not related to outcome, while post-SE-PDs were related to poor prognosis confirmed after multiple regression analysis. EMSE global AUC was 0.751 (95%CI:0.680–0.823) and for EMSE-64 cutoff sensitivity was 0.85, specificity 0.52, accuracy 63%. We recalculated EMSE score including only post-SE-PDs. Modified EMSE (mEMSE) global AUC was 0.803 (95%CI:0.734–0.872) and for mEMSE-64 cutoff sensitivity was 0.84, specificity 0.68, accuracy 73%.
Interictal PDs during SE were not related to outcome whereas PDs persisting or appearing > 24 h after SE resolution were strongly associated to unfavorable prognosis. EMSE performed well in our cohort but considering only post-SE-PDs raised specificity and accuracy for mEMSE64 cutoff.
This study supports the utility of differentiating between interictal PDs during and after SE for prognostic assessment.
