Epidemiological and experimental data suggest that type 2 diabetes (DM2) and sporadic late-onset Alzheimer's disease (AD) share a common mechanism, that is able to produce accumulation of insulin and amyloid β42 (Aβ42), the major pathogenic events re
ABSTRACT Introduction The aim of this study was to demonstrate single fascicular involvement in common fibular (CF) neuropathy using high‐resolution ultrasound (US). Methods: We prospectively enrolled 40 adult patients with clinical and electrodiagnostic suspected CF neuropathy between April 2012 and December 2014. Two musculoskeletal radiologists used high‐resolution US probes to prospectively and independently evaluate the CF nerve bilaterally in these patients. The presence of single fascicular involvement (increased cross‐sectional area and loss of fascicular echotexture) was recorded. Results: US revealed involvement of only 1 fascicular component of the CF nerve in 7 patients. In all these patients, US revealed involvement of the anterior fascicles corresponding to fibers for the deep fibular nerve. Conclusions: High‐resolution US allowed identification of single fascicular involvement in CF neuropathy. Anterior fascicular involvement was present in up to 17% of patients with suspected CF neuropathy. Muscle Nerve 53 : 532–537, 2016
We evaluated 13 patients affected by myasthenia gravis (MG) who had coronavirus disease 2019 (COVID-19) before vaccination and 14 myasthenic patients who contracted severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection after vaccination to evaluate factors related to different COVID-19 outcomes. We compared the two groups' previous stability of MG and the severity of SARS-CoV-2 infection. Vaccinated and non-vaccinated patients were comparable in terms of severity of the previous MG course (mean maximum myasthenia gravis Foundation of America-MGFA-Class III) and during SARS-CoV-2 infection (mean MGFA Class II). In non-vaccinated patients, the hospitalization and severe course percentages were 61.5%, while the mortality reached 30.8%. The hospitalization, severe course, and mortality percentages in vaccinated patients were 7.1%. In deceased, non-vaccinated patients, greater myasthenia severity in the past clinical history, but not at the time of infection, was observed. Similarly, older age at MG onset and at the time of infection correlated with a more severe COVID-19 course in non-vaccinated patients (p = 0.03 and p = 0.04), but not in the group of vaccinated patients. In summary, our data support a protective role of vaccination in myasthenic patients, even if anti-CD20 therapy might be associated with a poor immune response to vaccines.
We describe the CT and MRI patterns of cerebral venous sinus thrombosis (CVST) on the basis of the venous angioarchitecture and the underlying pathophysiological mechanism. We also investigated if any radiologic data exist to establish which patients can be followed conservatively and which warrant endovascular treatment. The clinical, CT-CTA and MRI-MRA findings of 11 patients (2 men; 9 women; 24 to 69 years-old) with CVST were reviewed. The morphological patterns of CVST were divided into two major groups: Vascular signs: spontaneous sinusal hyperdensity (9); spontaneous all-sequences hyperintensity (4); venous engorgement (9); empty delta sign (4); lack of sinusal contrast-enhancement (3); delayed sinusal transit-time (11); lack of flow-related signal (3). Parenchymal signs: mass effect and cortical sulcal effacement (8), white matter edema (7), venous ischemia (6), haemorrhagic infarct (3), breakdown of the blood-brain barrier (4), hydrocephalus (2). The clinical and radiologic pictures are related to cerebral venous angioarchitecture and underlying pathophysiologic mechanism of venous thrombosis. Reversibility of clinical symptoms and parenchymal lesions is far more frequent, because vessel damage slowly and progressively develops, whereas damage to brain tissue occurs later. Consequently, a prompt CT-MRI diagnosis may allow a good prognosis. Treatment using selective sinusal instillation of urokinase is considered only when the patient clinically and radiologically does not improve within the first two weeks after heparinization.
The early-onset familial AD (EOFAD) linked to presenilin 1 ( PS1 ) mutations show a heterogeneous phenotype that is illustrated mainly by the coexistence of dementia with spastic paraparesis, described in a few kindreds.1-3⇓⇓ The altered processing of amyloid precursor protein, favoring the production of the longer amyloid β-peptide form ending at residue 42 (Aβ42), is the major pathogenic effect of PS1 mutations.4 The relationship between clinical phenotype and Aβ42 production is unclear. We report a kindred bearing a novel PS1 mutation that showed an extremely wide clinical spectrum among the three affected members.
A 45-year-old woman (I-1) showed progressive paraparesis followed after 5 years by dementia. She died at age 57, and autopsy was not performed. Her daughter (II-2) presented at 48 years with delusional thinking followed by progressive impairment of all cognitive functions. Neurologic examination at 55 years did not reveal loss of strength or pyramidal signs at the limbs. Her brother (II-3) became symptomatic at the …
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