Patient with corticobasal syndrome showing disease-associated biomarkers of dementia with Lewy Bodies: a Treviso Dementia (TREDEM) registry case report

Background: An 82-year-old right-handed man, a retired teacher, reported the occurrence, three years earlier, of difficulties in moving his left arm and foot, tremor in his left hand, and gestures of the left upper limb that appeared to be independent of the patient's will.Objective: We describe an unusual case of corticobasal syndrome (CBS) showing disease-associated biomarkers of dementia with Lewy bodies (DLB).Methods: Clinical, neuropsychological, imaging, and biomarker evaluations were conducted, including tau and amyloid-beta levels in the cerebrospinal fluid (CSF) and a RT-QuIC assay for alpha-synuclein both in the CSF and olfactory mucosa (OM), as well as a QEEG assessment.Results: The patient presented resting tremor, mild extrapyramidal hypertonus, mild bradykinesia on the left side, and severe apraxia on the left upper limb. Brain MRI showed a diffuse right hemisphere atrophy which was prominent in the posterior parietal and temporal cortices, and moderate in the frontal cortex and the precuneus area. F-18-FDG PET imaging showed reduced glucose metabolism in the right lateral parietal, temporal, and frontal cortices with involvement of the right precuneus. The putamen did not appear to be pathological at DaTQUANT. Neuropsychological tests showed memory and visual-perceptual deficits. CSF tau and amyloid measurements did not show clear pathological values. RT-QuIC for alpha-synuclein in CSF and OM samples were positive. The QEEG analysis showed a pre-alpha dominant frequency in posterior derivations, typical of early stages of DLB.Conclusion: Although in the present patient the clinical diagnosis was of probable CBS, unexpectedly positive biomarkers for DLB suggested the co-presence of multiple pathologies

Exploratory Study on Chemosensory Event-Related Potentials in Long COVID-19 and Mild Cognitive Impairment: A Common Pathway?

People affected by the Long COVID-19 (LC) syndrome often show clinical manifestations that are similar to those observed in patients with mild cognitive impairments (MCI), such as olfactory dysfunction (OD), brain fog, and cognitive and attentional diseases. This study aimed to investigate the chemosensory-evoked related potentials (CSERP) in LC and MCI to understand if there is a common pathway for the similarity of symptoms associated with these disorders. Eighteen LC patients (mean age 53; s.d. = 7), 12 patients diagnosed with MCI (mean age 67; s.d. = 6), and 10 healthy control subjects (mean age 66; s.d. = 5, 7) were recruited for this exploratory study. All of them performed a chemosensory event-related potentials (CSERP) task with the administration of trigeminal stimulations (e.g., the odorants cinnamaldehyde and eucalyptus). Study results highlighted that MCI and LC showed reduced N1 amplitude, particularly in the left frontoparietal network, involved in working memory and attentional deficits, and a reduction of P3 latency in LC. This study lays the foundations for evaluating aspects of LC as a process that could trigger long-term functional alterations, and CSERPs could be considered valid biomarkers for assessing the progress of OD and an indicator of other impairments (e.g., attentional and cognitive impairments), as they occur in MCI

Opinion, knowledge, and clinical experience with functional neurological disorders among Italian neurologists: results from an online survey

Background: Functional neurological disorders (FND) are disabling medical conditions commonly seen in neurological practice. Neurologists play an essential role in managing FND, from establishing a diagnosis to coordination of multidisciplinary team-based treatment for patients. With this study, we investigated the knowledge and the clinical experience of Italian neurologists in managing patients with FND. Methods: Members of the Italian Society of Neurology were invited via e-mail to participate in this ad hoc online survey; 492 questionnaires were returned completed. Results: The term "Functional neurological disorders" in reference to FND was used more frequently than other psychological (e.g., psychogenic or conversion), or descriptive terms (e.g., non-organic or stress-related). When speaking with patients, the respondents stated that they preferred explaining symptoms based on abnormal functioning of the nervous system than discussing mental illness and that they would refer their patient to a psychologist rather than to a psychiatrist. Few considered that physiotherapy and psychiatric interventions are useful approaches to treating FND. Some believed that patients simulate their symptoms. Conclusions: Overall, the responses suggest that knowledge about scientific advances in FND is somewhat sparse. A psychiatric-centered view of FND opens the way to an approach in which neurobiological and psychological aspects constitute essential factors of the condition. In this context, professional education could improve understanding of FND and optimize patient management

New therapies in muscular dystrophies

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Routine Shunting during Carotid Endarterectomy in Patients with Acute Watershed Stroke

Aim: To evaluate the protective role of routine shunting in patients with acute watershed stroke (WS) undergoing carotid endarterectomy (CEA). Methods: A total of 138 patients with symptomatic carotid stenosis (SCS) who underwent CEA after acute ischemic stroke from March 2008 to March 2015 were included in this study. Transient ischemic attacks were excluded. These patients were divided into 2 groups according to the topographic pattern of the stroke on magnetic resonance imaging: group 1, territorial strokes (TS) caused by emboli of carotid origin, and group 2, WS caused by a hemodynamic mechanism related to an SCS. Primary end points were 30-day mortality and postoperative neurological morbidity. The insertion of a Pruitt carotid shunt was performed systematically. Results: Ninety (65.2%) patients presented a TS of carotid origin and were included in group 1, and 48 (34.8%) of the 138 patients had a WS related to an SCS and were included in group 2. The median time between clinical onset of the cerebral ischemic event and surgery was 9 days (range: 0-89 days). Postoperative mortality was 0%. Seven (5.1%) patients had an aggravation of the neurological status during the postoperative period, of whom 2 presented a complete regression of the symptoms in less than 1 hour (definitive postoperative neurologic morbidity: 3.6%). Postoperative neurologic morbidity rate was significantly higher in the TS group (7 of 90; 7.8%) compared to the WS group (0 of 48; P =.04). No other independent predictive factor of neurologic morbidity after CEA for an SCS was found. Conclusions: Our results suggest that routine shunting should be considered in case of acute WS since it may play a protective role. Further studies are eagerly awaited to better define the timing and the best treatment option for both acute WS and TS related to an SCS in order to reduce postoperative neurologic morbidity