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BACKGROUND AND PURPOSE: Cavitary plaques have been reported as a manifestation of otospongiosis. They have been related to third window manifestations, complications during cochlear implantation, and sensorineural hearing loss. However, their etiology and clinical implications are not entirely understood. Our purpose was to determine the prevalence, imaging findings, and clinical implications of cavitary plaques in otospongiosis. MATERIALS AND METHODS: We identified patients with otospongiosis at a tertiary care academic medical center from January 2012 to April 2017. Cross-sectional CT images and clinical records of 47 patients (89 temporal bones) were evaluated for the presence, location, and imaging features of cavitary and noncavitary otospongiotic plaques, as well as clinical symptoms and complications in those who underwent cochlear implantation. RESULTS: Noncavitary otospongiotic plaques were present in 86 (97%) temporal bones and cavitary plaques in 30 (35%). Cavitary plaques predominated with increasing age (mean age, 59 years; P = .058), mostly involving the anteroinferior wall of the internal auditory canal (P = .003), and their presence was not associated with a higher grade of otospongiosis by imaging (P = .664) or with a specific type of hearing loss (P = .365). No patients with cavitary plaques had third window manifestations, and those with a history of cochlear implantation (n = 6) did not have complications during the procedure. CONCLUSIONS: Cavitary plaques occurred in one-third of patients with otospongiosis. Typically, they occurred in the anteroinferior wall of the internal auditory canal. There was no correlation with the degree of otospongiosis, type of hearing loss, or surgical complications. Cavitary plaques tended to present in older patients.

We present two further cases of the pharyngeal-cervical-brachial (PCB) form of GBS, with unfavourable outcome, showing dramatic dissociation between upper and lower body Symptoms. Both patients showed rapidly progressive motor denervation with disappearance of Compound Muscle Action Potentials (CMAPs) in upper limbs muscles. Sensory Nerve Action Potentials (SNAPs) were instead normal. Normal reflexes, F waves and action potentials were elicited in lower limbs. Despite i.v. Immunoglobulin treatment no recovery was observed and both patients died within a year from onset of symptoms.

Parkinson Disease (PD), Dementia with Lewy Bodies (DLB) and Multiple System Atrophy (MSA) are characterized by deposition of Lewy Bodies (LB), consisting of eosinophilic intracellular (intraneural in PD and DLB and intraglial in MSA) inclusions of ┙-synuclein and allowing the three disorders to be categorized as synucleinopathies. The identification in the last two decades of specific clinical features of synucleinopathies has been a major breakthrough in Neurology, as it prompted a reconsideration of diagnostic and therapeutic approaches to dementia and parkinsonism. The recognition of synuclein and ubiquitin markers in dementia resulted in the reclassification of patients previously considered as affected by Alzheimer Disease (AD) and Vascular Dementia (VaD), and there is now agreement that DLB is the second most common cause of dementia in elderly population: its prevalence is reported to be in the 25-43% range in different studies. DLB is clinically characterized by the presence of prominently dysexecutive dementia (frontal lobe or subcortical dementia according to earlier classification [1]), cognitive fluctuations, consisting of remitting-relapsing episodes of blunted conscience reaching levels of stupor, by the occurrence of visual hallucinations and delusions, by parkinsonian motor signs and hypersensitivity to neuroleptic treatment, ranging from worsening of parkinsonism to the possible lethal neuroleptic-malignant syndromes [2]. Yet, variances of presentation and overlapping symptoms with AD and Fronto-Temporal lobe Degeneration (FTD), could be misleading in the process of addressing a clinical diagnosis with consequent therapeutic risks(e.g. introducing neuroleptic treatments in patient with DLB), or economic costs (e.g. addressing patients with DLB to treatment protocols dedicated to AD patients, based on vaccines or antibodies against ┚ amyloid proteins, a neuropathological feature of AD). It is evident the stringent need for improvement of reliable diagnostic tools (u.e. biomarkers) to differentiate the diseases associated with dementia. In 2010 the National Institute of Health, NIH, held a symposium focused on the development of possible biomarkers for DLB. Among the different suggested biomarkers, neurophysiological assessments were reconsidered, as a large amount of neurophysiological studies had been devoted to AD and PD, whose characteristics are shared by DLB.

Summary Aims of the study Earlier P300 studies were conducted when the prevalence of dementia with Lewy Bodies (DLB) was unknown. Our study aims to examine whether P300 abnormalities are present in DLB and to evidence possible differences between DLB and Alzheimer's disease (AD). A second aim of this study is to look for correlations between P300 recordings and EEG, as abnormal EEG variability has been described in DLB. Patients and methods Auditory P300 responses were recorded by a classic oddball paradigm in 50 controls, in 36 DLB patients, and in 40 AD patients with MMSE > 20. Results Reliable auditory P300 responses were obtained in 26 DLB (72%), 33 AD (82.5%), and 46 controls (92%). P300 was more delayed and had lower amplitude in DLB compared to AD groups. P300 topography was also different as the anterior-to-posterior scalp amplitude gradient was reversed in DLB. P300 latency correlated with neuropsychological test scores and with EEG variables. Gradient inversion and delayed P300 responses in frontal derivations evidenced differences between DLB and AD patients with a sensitivity of 70% and a specificity of 97%. Conclusions P300 recordings are abnormal in DLB and can be useful to distinguish DLB from AD.