Amyotrophic lateral sclerosis : Multisystem degeneration.

CONCLUSIONS: Nonmotor symptoms are regularly seen in ALS, although they usually do not dominate the clinical picture. Recent neuropathological findings offer new perspectives for diagnostics and therapy in ALS. PMID: 26646612 [PubMed - as supplied by publisher]
Source: Der Nervenarzt - Category: Neurology Authors: Tags: Nervenarzt Source Type: research

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ConclusionsThese results show the importance of expressing quality of life in a conceptually appropriate way, as failing to take account of the multidimensional nature of QoL can result in important nuances being overlooked. Clinicians must be aware that pain, depression and anxiety all worsen QoL across their ranges, and not just when pain is severe or when anxiety or depression reach case level.
Source: Journal of Neurology - Category: Neurology Source Type: research
Conclusion: Patient-reported prevalence of NMS in adult SMA was low, which does not argue for a clinically relevant multisystemic disorder in SMAII/III. Importantly, adult SMA patients do not seem to suffer more frequently from symptoms of depression or adaptive disorders compared to controls. Our results yield novel information on previously underreported symptoms and will help to improve the medical guidance of these patients.
Source: Frontiers in Neurology - Category: Neurology Source Type: research
ConclusionsComputational brainstem imaging captures the degeneration of both white and grey matter components in ALS. Our longitudinal data indicate progressive brainstem atrophy over time, underlining the biomarker potential of quantitative brainstem measures in ALS. At a time when a multitude of clinical trials are underway worldwide, there is an unprecedented need for accurate biomarkers to monitor disease progression and detect response to therapy. Brainstem imaging is a promising addition to candidate biomarkers of ALS and PLS.
Source: NeuroImage: Clinical - Category: Radiology Source Type: research
In this study, we have investigated the major molecular factors that mainly contribute to SOD1 destabilization, intrinsic disorder, and misfolding using sequence and structural information. We have analysed 153 ALS causing SOD1 point mutants for aggregation tendency using four different aggregation prediction tools, viz., Aggrescan3D (A3D), CamSol, GAP and Zyggregator. Our results suggest that 74-79 mutants are susceptible to aggregation, due to distorted native interactions originated at the mutation site. Majority of the aggregation prone mutants are located in the buried regions of SOD1 molecule. Further, the mutations ...
Source: International Journal of Biological Macromolecules - Category: Biochemistry Authors: Tags: Int J Biol Macromol Source Type: research
ConclusionThis review shows that CAM may be useful for ALS treatment, but more evidence regarding the efficacy and molecular mechanisms is required to establish CAM as a good therapy for the treatment of ALS patients.
Source: Integrative Medicine Research - Category: Complementary Medicine Source Type: research
Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Conclusions This study demonstrates that cerebral degeneration in ALS is more pronounced in the motor than prefrontal cortex, that multicenter MRS studies are feasible, and that motor tNAA/Ino shows promise as a potential biomarker.
Source: Neurology Clinical Practice - Category: Neurology Authors: Tags: MRI, MRS, Amyotrophic lateral sclerosis Research Source Type: research
Authors: Valori CF, Guidotti G, Brambilla L, Rossi D Abstract Motor neuron disorders are highly debilitating and mostly fatal conditions for which only limited therapeutic options are available. To overcome this limitation and develop more effective therapeutic strategies, it is critical to discover the pathogenic mechanisms that trigger and sustain motor neuron degeneration with the greatest accuracy and detail. In the case of Amyotrophic Lateral Sclerosis (ALS), several genes have been associated with familial forms of the disease, whilst the vast majority of cases develop sporadically and no defined cause can be...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
People living with amyotrophic lateral sclerosis (ALS) and their families were provided with equitable open access to a new dedicated psychology service. An integrative psychotherapeutic model of support was offered, incorporating existential therapy. The therapeutic potential of existential therapy, which was evident here, has not been explored previously in ALS. Cognitive behavioural interventions were used in a minority of cases. One third of all patients attending the Bristol motor neurone (MND) centre elected to use this service.
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
Amyotrophic Lateral Sclerosis (ALS) is an incurable motor neuron (MN) disorder, characterized by degeneration of MNs leading to progressive paralysis and death within two to five years after diagnosis. Gene therapy is emerging as promising treatment option for patients affected by familial forms of ALS (fALS), representing 10% of all ALS cases. Taking advantage of viral vectors derived from Adeno-Associated Virus serotype 10 (AAV10) and the small nuclear RNA U7, we developed a gene therapy for SOD1-linked ALS (20% of fALS).
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
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