Microscopic Polyangiitis With Selective Involvement of Central and Peripheral Nervous System : A Case Report

Background: Microscopic polyangiitis (MPA) is a necrotizing vasculitis that affects predominantly small-sized vessels in many organ systems. The disease generally causes glomerulonephritis, pulmonary damage, arthritis, and neuropathy. An exclusive involvement of both central nervous system (CNS) and peripheral nervous system (PNS) is extremely rare. Case Presentation: A 62-year-old woman was admitted to our hospital with a 3 months history of right foot drop, recently complicated by intense myalgia, arthralgia, and allodynia to tactile, vibratory, and pressure stimuli. Since blood tests revealed elevated inflammatory indexes, we suspected either infectious or immune-mediated disorders. Chest radiograph, blood culture series, and echocardiogram revealed normal findings, while urinalysis showed a bacterial infection that was successfully treated. The neurophysiological findings were compatible with multiple mononeuritis, and a brain MRI evidenced ischemic lesions of both basal ganglia and thalamus. A wide-spectrum autoantibody assay revealed the presence of high-titer perinuclear anti-neutrophil cytoplasmic antibodies specific for myeloperoxidase (MPO-ANCA). According to these findings, the diagnosis of MPA was made, and the patient was successfully treated with intravenous (IV) methylprednisolone, followed by two doses of rituximab. Conclusions: An assessment of both CNS and PNS should be included in the diagnostic evaluation of MPA. The involvement of the PNS may raise the risk of a relapsing course and treatment failure, therefore it should be considered in the choice of induction and maintenance therapy

Clinical reasoning: a 75-year-old man with parkinsonism, mood depression, and weight loss

A 75-year-old man presented to the emergency department with a 1-year history of 66-pound weight loss and alternating bowel habits. He was admitted to the hospital, where he underwent several examinations to investigate the presence of a malignancy. A colonoscopy, a gastroscopy, an ultrasound of the abdomen, and a contrast-enhanced CT scan of thorax and abdomen did not detect any neoplasia. The only findings consisted of a prostatic hypertrophy and a basal pleural-parenchymal hyperdensity in the left lung, which was described as the result of an infective process. Neoplastic markers CA19.9, carcinoembryonic antigen, neuron-specific enolase, and \u3b1-fetoprotein were also negative. Wide-spectrum blood tests were unremarkable, except for hypogammaglobulinemia and elevated \u3b22 microglobulin

Degenerative amnesia for past public events: an attempt to measure storage and retrieval.

A still unsettled issue of amnesia concerns the differential contributions to recall impairment of the underlying retrieval and storage abilities. The aim of the present study was to disentangle and to measure such roles in the recall of past public events comparing patients with degenerative amnesia and healthy elderly. The experiment included 44 healthy elderly and two groups of participants with degenerative amnesia, namely 17 patients with amnestic mild cognitive impairment and 22 mild Alzheimer's disease patients. Recall of famous past public events was assessed by means of a 52-item questionnaire standardized for the Italian population. A latent-variable approach was adopted in order to infer the contributions of retrieval and storage to the recall performances. A stochastic model was adopted, which in a previous study of recall of recent and remote past public events in healthy elderly succeeded to prove reduced retrieval efficiency for more recent events. The results of the present study suggest that retrieval is more fragile than storage in all three experimental groups. A storage impairment turned out only in the Alzheimer's disease group, where it was limited to more recent memories. In view of the combined roles of the hippocampus and cortex in past memory processing, our results are consistent with the hypothesis that the degenerative process primarily impairs the strategic memory search. However, the sufficiency criterion of the adopted Markov model fell short of significance. Due to this statistical shortcoming, our conclusions, though consistent with the clinical predictions, are to be taken as provisional

Mild Cognitive Impairment does entail retrograde amnesia for pubblic events

In this study memory for public events was evaluated in 15 amnesic mild cognitive impairment (aMCI) patients, whose clinical diagnosis was refined through a stringent selection procedure. A total of 9 patients were longitudinally reassessed over an 18-month period. About half of the participants were impaired at baseline and nearly 80% at the end of the 18-month follow-up. Moreover, retrograde memory declined significantly over time. Evidence of a pathological Ribot-type temporal gradient was found in about half of the aMCI patients. This is the first report of a remote memory deficit in aMCI. It highlights amnesia for public events as a frequent accompaniment of this condition. The findings tie in with the hypothesized role of the hippocampal complex in long-term memory

Whose face is this? Italian norms of naming celebrities

Naming celebrities from visual input (i.e., face presentation) was examined in 98 healthy participants. Normative data are provided both for a 12-and a 63-item test. The first one is a brief screening tool, while the latter is to be administered whenever a pathological performance turns out in the former. Age adversely affected both performances. In both tests the most frequent errors were no responses, followed by semantic errors. The study makes explicit reference to current serial models of familiar person processing and naming. Findings allow to complement previously reported testing procedures, with the specific aim of qualifying proper name anomia by identifying the cognitive locus of lesion

Mirror asymmetry of category and letter fluency in traumatic brain injury and Alzheimer's patients

In this study we contrasted the Category fluency and Letter fluency performance of 198 normal subjects, 57 Alzheimer's patients and 57 patients affected by traumatic brain injury (TBI). The aim was to check whether, besides the prevalence of Category fluency deficit often reported among Alzheimer's patients, the TBI group presented the opposite dissociation. According to some recent claims, in fact, the deficit of TBI would be equally severe for both fluency types. The inquiry followed different approaches for data analysis, including the evaluation of a unique index (Fluency Type Index or FTI), independent of the overall fluency and aimed at expressing at individual subject level the relationship between Category and Letter fluency. The results confirmed that Alzheimer's patients are more defective on Category than Letter fluency, and also clearly indicated that an opposite pattern applies to TBI patients. TBI seems to cause a relatively more severe impairment of Letter than Category fluency, probably due to its impact on the frontal lobe structures. We discuss whether, on the basis of the statistical distribution of our data, it is worth considering as homogeneous populations broadly defined groups as Alzheimer's or TBI patients