An atypical case of mononeuritis multiplex in primary Sjogren's syndrome

We present an atypical case of peripheral nervous system (PNS) involvement in Sjogren's syndrome in a 63 year-old woman. Symptoms of an entrapment neuropathy were the first manifestation of the systemic disease and they were subsequently coupled to those of a mononeuritis multiplex. Clinical and laboratory signs for the diagnosis of Sjogren's syndrome became subsequently overt. The mononeuritis multiplex remained clinically limited to the upper limbs and characterized by unusually severe motor symptoms which progressed up to the development of a final complete deplegia. By contrast, sensory symptoms at the upper limbs remained mild over the entire course of the disease and the lower limbs revealed a subclinical sensory-motor damage only during the late stage.     

Peripheral cytokine release in Alzheimer patients: correlation with disease severity

Various studies suggested that inflammation is involved in the pathogenesis of Alzheimer's disease (AD). We investigated cytokine release from LPS-stimulated blood cells of 32 AD patients, with different disease severity, compared to 16 age-related controls. A significant decrease of IL-1beta and IL-6 secretion was observed in severely demented patients; TNF-alpha release was also decreased, but not significantly. By contrast, mild and moderate patients showed a cytokine release similar to controls. IL-1beta, IL-6 and TNF-alpha secretion was negatively correlated with the severity of dementia, quantified by the MMSE. Our data suggest that alterations of the immune profile are associated with AD progression.     

Validity and metric of MiniMental Parkinson and MiniMental State Examination in Parkinson’s disease

The MiniMental Parkinson (MMP) has been derived from the MiniMental State Examination (MMSE) for the screening of cognitive impairment in Parkinson’s disease by adding subtests that were focused on executive and visuo-spatial impairment more than on memory or language deficits. In this multicenter study, the psychometric and validity properties of the MMP have been evaluated in 69 cognitively intact and 52 cognitively impaired patients with Parkinson’s disease, classified according to their performance at the Dementia Rating Scale. The MMP showed better metrics and convergent validity, and higher screening ability. However, its performance was not fully satisfying in terms of data distribution, coefficient of variation and specificity, and Receiver Operating Characteristic curves did not show clear cut superiority of either scale at their best sensitivity–specificity trade off. The MMP seems to be slightly preferable to the MMSE only at a cut off that favours sensitivity with respect to specificity, for screening purposes. The test is simple and quick, but has limitations in terms of validity.     

The applause sign in cortical and cortical-subcortical dementia

The “applause sign” is a motor perseveration described in focal and neurodegenerative disorders and characterized by fronto-subcortical dysfunction. Most previous formal investigations focused on Parkinson’s disease or progressive supranuclear palsy. We assessed the prevalence of the applause sign in patients affected by Alzheimer’s disease (AD), Lewy body dementia (LBD), corticobasal syndrome (CBS), and posterior cortical atrophy (PCA), with the aim to verify its contribution to the differential diagnosis. We enrolled 20 patients with AD, 20 with LBD, 16 with CBS, and ten with PCA, and 30 healthy controls. The three clap test (TCT) was used to elicit the applause sign, and was scored by raters blinded to the diagnosis. Correlation with motor (extrapyramidal) and cognitive measures was also performed. A maximum 40 % prevalence of a positive applause sign was found in the two parkinsonian syndromes, which could be discriminated from the two cortical groups with a positive predictive value of 82 % and a negative predictive value of 55 %. According to our findings, a diagnosis of LBD or CBS, rather than of AD or PCA, is highly probable in the presence of an abnormal TCP, but cannot be ruled out based on a negative result. No relevant correlates emerged that could clarify the origin and nature of the applause sign.     

ALS Cognitive Behavioral Screen-Phone Version (ALS-CBS™-PhV): norms,psychometrics, and diagnostics in an Italian population sample

Neurological Sciences - Up to 50% of motor neuron disease (MND) patients show neuropsychological deficits which negatively affect prognosis and care. However, disability-related logistical issues...     

Psychometrics and diagnostics of Italian cognitive screening tests: a systematic review

Neurological Sciences - Cognitive screening tests (CSTs) are crucial to neuropsychological diagnostics, and thus need to be featured by robust psychometric and diagnostic properties. However, CSTs...     

Brand new norms for a good old test: Northern Italy normative study of MiniMental State Examination

Neurological Sciences - Mini-Mental State Examination (MMSE) is one of the most used tests for the screening of global cognition in patients with neurological and medical disorders. Norms for the...     

Qualitatively different forms of pure alexia

In this study we investigated two patients with pure alexia, F.C. and L.D.S., in order to make inferences about how processes and levels involved in the early stage of visual word recognition are organized and how they can be selectively damaged. Moreover, we investigated whether pure alexia can be caused by different functional deficits. F.C. and L.D.S. were presented with tasks of letter processing and tasks of orthographic integration. There was a clear double dissociation between the pattern of performance of F.C. and L.D.S. F.C. was able to process single letters rapidly and accurately, but was unable to group together the letters that he had correctly identified. By contrast, L.D.S. was slower and more impaired at letter identification, but she could use letter groups to assist reading. Thus, two different forms of pure alexia emerged: F.C. has a higher level deficit in integrating letters, whereas L.D.S. has a lower level deficit in letter processing. The results support the assumption of a functional organization of the reading process that involves a series of orthographic units (i.e., single letters, sublexical letter groups, and the lexical unit), which can be selectively damaged. Finally, our data present difficulties for models of pure alexia that assume all patients to have a low-level processing deficit.     

Primitive reflexes in amyotrophic lateral sclerosis: prevalence and correlates

Identifying frontal impairment in ALS is an important goal albeit disease-dedicated tools are still scarce. For this reason, we decided to consider primitive reflexes (PRs), variably regarded as correlates of frontal release and/or of upper motor neuron (UMN) impairment, often in the setting of dementias. Specifically, the aims of this work consisted in assessing the exact prevalence of the combination of seven PRs in ALS, trying to clarify their role as putative proxies of cognitive impairment or of UMN dysfunction. In this cross-sectional study, 50 consecutive ALS outpatients were evaluated for the presence of: palmomental (PM), corneomandibular (CM), glabella tap (MY), rooting, sucking, snout, and grasping reflexes. Cognitive screening was performed by the Frontal Assessment Battery (FAB) and the Weigl’s Sorting test (WST); UMN dysfunction was concomitantly evaluated. PM, CM and MY were more frequently detected (62, 52, and 44 % of the ALS sample, respectively), while the other reflexes were under-represented. Patients displaying three or more PRs had significantly lower FAB and WST scores. On the other hand, UMN dysfunction was only moderately associated to PRs. In conclusion, PRs’ assessment is a promising complementary tool for screening cognitive impairment in ALS; however, further work will be necessary to establish its added value with respect to already existing ALS-dedicated screening tools for cognition.