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Riva N.,San Raffaele Scientific Institute | Agosta F.,San Raffaele Scientific Institute | Lunetta C.,NEuroMuscular Omnicentre NEMO | Filippi M.,San Raffaele Scientific Institute | Quattrini A.,San Raffaele Scientific Institute
Journal of Neurology | Year: 2016

ALS is a relentlessly progressive and fatal disease, with no curative therapies available to date. Symptomatic and palliative care, provided in a multidisciplinary context, still remains the cornerstone of ALS management. However, our understanding of the molecular mechanisms underlying the disease has advanced greatly over the past years, giving new hope for the development of novel diagnostic and therapeutic approaches. Here, we have reviewed the most recent studies that have contributed to improving both clinical management and our understanding of ALS pathogenesis. © 2016, The Author(s).

Tremolizzo L.,Neurology Unit | Tremolizzo L.,University of Milan Bicocca | Susani E.,Neurology Unit | Susani E.,University of Milan Bicocca | And 6 more authors.
Journal of Neurology | Year: 2014

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. © 2014 Springer-Verlag Berlin Heidelberg.

Pagnini F.,University of Milan | Simmons Z.,Pennsylvania State University | Corbo M.,NEuroMuscular Omnicentre NEMO | Molinari E.,University of Milan | Molinari E.,Psychology Research Laboratory
Amyotrophic Lateral Sclerosis | Year: 2012

The literature on psychological aspects of amyotrophic lateral sclerosis (ALS) has explored quality of life, depression, anxiety, spirituality, hopelessness, and other constructs in an attempt to understand the patient's grief and other psychological responses to the disease. However, there is a lack of research on the efficacy of psychological interventions. We believe it is important to develop 'best practices' for the improvement of quality of life and the reduction of psychological distress related to ALS. © 2012 Informa Healthcare.

Saresella M.,Don C. Gnocchi Foundation | Piancone F.,Don C. Gnocchi Foundation | Tortorella P.,Don C. Gnocchi Foundation | Marventano I.,Don C. Gnocchi Foundation | And 7 more authors.
Clinical Immunology | Year: 2013

MS (multiple sclerosis) and ALS (amyotrophic lateral sclerosis) differ in important respects, but common pathogenic features seem to be shared in these two diseases. To shed light on such features, immunophenotypic and functional analysis were performed in peripheral monocytes and T lymphocytes of ALS and primary progressive (PP) MS patients and healthy controls (HC). Results showed that TH1-, TH17-, and IL-6-driven inflammation characterize both diseases; this is unsuccessfully hampered by TH2 activation and, possibly, BDNF secretion. Results herein clarify the pathogenic similarities between ALS and PP-MS and could be helpful for the design of novel diagnostic and therapeutic approaches to ALS. © 2013 Elsevier Inc.

Cerami C.,Vita-Salute San Raffaele University | Cerami C.,San Raffaele Scientific Institute | Dodich A.,Vita-Salute San Raffaele University | Canessa N.,Vita-Salute San Raffaele University | And 11 more authors.
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration | Year: 2014

Amyotrophic lateral sclerosis (ALS) is a multisystem condition, in which executive and/or behavioural symptoms can occur. Deficits of social cognition, including defective cognitive and emotional empathy, have been recently reported in ALS subjects. The neurostructural correlates of these disorders in ALS are still unknown. The aims of this study were to evaluate two components of empathy in non-demented ALS subjects, and to associate performance with regional grey-matter density using voxel-based morphometry (VBM). Twenty non-demented sporadic probable or definite ALS patients and 56 matched healthy controls (HC) participated in a non-verbal task requiring the attribution of emotional versus cognitive states to identify the correct ending of comic strips, compared with a control condition requiring identifying causal relationships devoid of social components. A subgroup of 14 ALS and 20 HC joined the VBM study. Results demonstrated that, compared with controls, ALS patients showed defective emotional empathy attribution, related with reduced grey-matter density in the anterior cingulate cortex and right inferior frontal gyrus. Our study provided evidence of a specific impairment of emotional empathy in ALS patients, reflecting neural damage in a limbic prefrontal network involved in emotional processing. Social cognition disorders may represent a marker of cognitive dysfunction in ALS. © 2014 Informa Healthcare.

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