Motor neuropathies and lower motor neuron syndromes.

Motor neuropathies and lower motor neuron syndromes. Rev Neurol (Paris). 2017 Apr 20;: Authors: Verschueren A Abstract Motor or motor-predominant neuropathies may arise from disease processes affecting the motor axon and/or its surrounding myelin. Lower motor neuron syndrome (LMNS) arises from a disease process affecting the spinal motor neuron itself. The term LMNS is more generally used, rather than motor neuronopathy, although both entities are clinically similar. Common features are muscle weakness (distal or proximal) with atrophy and hyporeflexia, but no sensory involvement. They can be acquired or hereditary. Immune-mediated neuropathies (multifocal motor neuropathy, motor-predominant chronic inflammatory demyelinating polyneuropathy) are important to identify, as effective treatments are available. Other acquired neuropathies, such as infectious, paraneoplastic and radiation-induced neuropathies are also well known. Focal LMNS is an amyotrophic lateral sclerosis (ALS)-mimicking syndrome especially affecting young adults. The main hereditary LMNSs in adulthood are Kennedy's disease, late-onset spinal muscular atrophy and distal hereditary motor neuropathies. Motor neuropathies and LMNS are all clinical entities that should be better known, despite being rare diseases. They can sometimes be difficult to differentially diagnose from other diseases, particularly from the more frequent ALS in its pure LMN form. Nevertheless, correct identification of t...
Source: Revue Neurologique - Category: Neurology Tags: Rev Neurol (Paris) Source Type: research

Related Links:

CONCLUSIONS: The quality of life of several Colombian patients diagnosed with multiple sclerosis was very good. This positive result was also observed in caregivers as evidenced by the results of the CareQol questionnaire. We also observed and indicated an inversely proportional correlation between the Expanded Disability Status Scale and the quality of life indexes. PMID: 32220169 [PubMed - as supplied by publisher]
Source: Biomedica : Revista del Instituto Nacional de Salud - Category: International Medicine & Public Health Tags: Biomedica Source Type: research
CONCLUSIONS: One session of active tDCS did not have an immediate effect on quadriceps activity and subjective scores of pain and symptoms. To determine if tDCS is a valid modality for this patient population, a larger scale investigation with multiple treatments of active tDCS is warranted. PMID: 32221043 [PubMed - as supplied by publisher]
Source: Journal of Sport Rehabilitation - Category: Sports Medicine Tags: J Sport Rehabil Source Type: research
Condition:   Amyotrophic Lateral Sclerosis Interventions:   Drug: Trametinib (0.5 mg);   Drug: Trametinib (1 mg);   Drug: Trametinib (2 mg);   Drug: Riluzole (100 mg) Sponsor:   Genuv Inc. Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Authors: Laforgia M, Calabrò C, Scattone A, Laface C, Porcelli M, Gadaleta CD, Nardulli P, Ranieri G Abstract Introduction: Mast cell leukemia (MCL) is one of the most aggressive forms of Systemic Mastocytosis (SM), a complex family of rare diseases, for which standard therapies are very few. MCL represents only
Source: Expert Opinion on Pharmacotherapy - Category: Drugs & Pharmacology Tags: Expert Opin Pharmacother Source Type: research
Authors: Quaglino F, Manfrino L, Cestino L, Giusti M, Mazza E, Piovesan A, Palestini N, Lauro C, Castellano E Abstract Parathyroid carcinoma (PC) is a rare disease responsible for about 1% of primary hyperparathyroidism (PHPT) cases. PC usually has an indolent course, tough to differentiate from the benign causes of PHPT, and the only certain diagnosis is histologic. The gold standard surgical treatment is the en bloc resection associated with the homolateral thyroid loboistmectomy. The aim of this study was to underline the main differences between PC and benign PHPT, along with gathering epidemiological knowledge...
Source: International Journal of Endocrinology - Category: Endocrinology Tags: Int J Endocrinol Source Type: research
Abstract Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited tumor syndrome. Clear cell chondrosarcoma (CCCS) is a rare variant of chondrosarcoma. Here, we report a case of CCCS in the talus occurring in a patient with VHL disease. A 32-year-old man presented with ankle pain for 2 years. MRI revealed a 4.2 cm mass in the talus, and needle biopsy was performed. The patient underwent curettage but the final diagnosis was CCCS. Adjuvant radiation therapy was performed without additional talectomy and the patient was alive without recurrence for a follow-up period of 2 years. To our knowledge, this is t...
Source: International Journal of Clinical and Experimental Pathology - Category: Pathology Authors: Tags: Int J Clin Exp Pathol Source Type: research
Zolgensma is a gene therapy that addresses the genetic root cause of spinal muscular atrophy by replacing the defective or missing SMN1 gene to halt disease progression.International Approvals
Source: Medscape Neurology and Neurosurgery Headlines - Category: Neurology Tags: Neurology & Neurosurgery News Alert Source Type: news
Publication date: Available online 27 March 2020Source: Journal of Clinical NeuroscienceAuthor(s): Antonio Canosa, Maurizio Grassano, Marco Barberis, Maura Brunetti, Umberto Manera, Rosario Vasta, Stefania Cammarosano, Giovanni De Marco, Andrea Calvo, Adriano Chiò, Cristina Moglia
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
Swiss drugmaker Novartis on Friday won a key European recommendation for its gene therapy Zolgensma against spinal muscular atrophy (SMA), clearing a hurdle for $2.1 million per patient treatment for approval in Europe within months.
Source: Reuters: Health - Category: Consumer Health News Tags: healthNews Source Type: news
FRIDAY, March 27, 2020 -- Kids and teens spend as much as five hours a day helping care for relatives with amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, a new study finds. Although the young people often help with...
Source: Drugs.com - Daily MedNews - Category: General Medicine Source Type: news
More News: ALS | Brain | CIDP | Motor Neurone Disease | Neurology | Paraneoplastic Syndrome | Rare Diseases | Spinal Muscular Atrophy