CLINICAL CHARACTERISTICS OF MOTOR NEURON DISEASE : A SINGLE CENTRE DATA FROM GUJARAT STATE.

2021 ◽  
pp. 9-11
Author(s):  
Joshi Pranav B ◽  
Shah Shalin D ◽  
Sikariya Kusum K ◽  
Chaudhary Jay ◽  
Patel Mayank A
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Clinical Characteristics ◽  
Age Of Onset ◽  
Western India ◽  
Amyotropic Lateral Sclerosis ◽  
Heterogenous Group ◽  
One Year

Motor Neuron Disease (MND) is a heterogenous group of disorders with degeneration of upper and/or lower motor neurons. Limited data is available for clinical characteristics of MND from western India. Methods: We retrospectively observed all cases of MND, evaluated at our centre. Those with conrmed diagnosis, exclusion of secondary causes and with one year of minimal follow up were included, for the anaysis. RESULTS: Out of 51 patients of MND, 36 were diagnosed as Amyotropic Lateral Sclerosis (ALS) and 15 patients were having pure Lower Motor Neuron(LMN) type of MND. Male: female ration was 2.8:1 in ALS group , with mean age of 50 years. Out of 10 bulbar onset MND patients, 5 died in the follow-up period. In the LMN subgroup, younger onset monomelic amyotrophy, of upper limb onset (Hirayama) was commonest subtype. No patients with isolated Upper motor Neuron type of MND was found. SUMMARY: ALS subgroups of patients had younger age of onset in western Indian population, with signicant male rd preponderance. Hirayama disease was commonest LMN type of MND, with onset in 3 decade and more commonly seen in males

scholarly journals ALS-Like Disorder in Three HIV-Positive Patients: Case Series

2021 ◽  
pp. 59-64
Author(s):  
Zachary Aaron Satin ◽  
Elham Bayat
Keyword(s):  
Antiretroviral Therapy ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Case Series ◽  
Retrospective Chart Review ◽  
Effective Control ◽  
Hiv Positive ◽  
Disease Course ◽  
Retroviral Infection

There appears to be a relationship between retroviruses such as HIV and the development of an ALS-like syndrome. Few cases have been reported; however, there exists evidence of a higher frequency of motor neuron disease in HIV-infected patients, as well as potential slowing and reversibility of disease course with combination antiretroviral therapy. We conducted a retrospective chart review of patients presenting to the George Washington University ALS Clinic from September 2006 to June 2018 to identify patients with HIV receiving HAART who were subsequently diagnosed with ALS or an ALS-like disorder. Our goals were to describe our patients’ disease course and compare them to general characteristics of ALS. We report three cases of HIV-positive individuals, all male, who were subsequently diagnosed with ALS. Each presented with symptoms of limb onset ALS with involvement of upper and lower motor neurons and whose disease originated at the cervical level. All three had been diagnosed with HIV prior to presentation and were presumably compliant with antiretroviral therapy throughout. Our patients demonstrated effective control of their HIV infection. Each experienced relatively slow progression of motor impairment compared to general ALS characteristics. Our study offers a distinct profile of HIV-positive patients compliant with HAART subsequently diagnosed with an ALS-like disorder. Further study should aim to uncover pathophysiological similarities between motor neuron disease both in the presence and absence of retroviral infection and to develop effective medical therapy for each.

scholarly journals Amyotrophic lateral sclerosis: one or multiple causes?

2011 ◽  
Vol 3 (1) ◽  
pp. 4 ◽  
Author(s):  
Aline Furtado Bastos ◽  
Marco Orsini ◽  
Dionis Machado ◽  
Mariana Pimentel Mello ◽  
Sergio Nader ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Viral Infections ◽  
Vigorous Physical Activity ◽  
Disease Etiology ◽  
New Research ◽  
Lateral Sclerosis

The Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in the adulthood, and it is characterized by rapid and progressive compromise of the upper and lower motor neurons. The majority of the cases of ALS are classified as sporadic and, until now, a specific cause for these cases still is unknown. To present the different hypotheses on the etiology of ALS. It was carried out a search in the databases: Bireme, Scielo and Pubmed, in the period of 1987 to 2011, using the following keywords: Amyotrophic lateral sclerosis, motor neuron disease, etiology, causes and epidemiology and its similar in Portuguese and Spanish. It did not have consensus as regards the etiology of ALS. Researches demonstrates evidences as regards intoxication by heavy metals, environmental and occupational causes, genetic mutations (superoxide dismutase 1), certain viral infections and the accomplishment of vigorous physical activity for the development of the disease. There is still no consensus regarding the involved factors in the etiology of ALS. In this way, new research about these etiologies are necessary, for a better approach of the patients, promoting preventive programs for the disease and improving the quality of life of the patients.

scholarly journals Multidisciplinary Interventions in Motor Neuron Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-10 ◽  
Author(s):  
U. E. Williams ◽  
E. E. Philip-Ephraim ◽  
S. K. Oparah
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Neurons ◽  
Neuronal Cell ◽  
Cell Loss ◽  
Cellular Processes ◽  
Team Care ◽  
Respiratory Management ◽  
Care Approach ◽  
Management Of Dysphagia

Motor neuron disease is a neurodegenerative disease characterized by loss of upper motor neuron in the motor cortex and lower motor neurons in the brain stem and spinal cord. Death occurs 2–4 years after the onset of the disease. A complex interplay of cellular processes such as mitochondrial dysfunction, oxidative stress, excitotoxicity, and impaired axonal transport are proposed pathogenetic processes underlying neuronal cell loss. Currently evidence exists for the use of riluzole as a disease modifying drug; multidisciplinary team care approach to patient management; noninvasive ventilation for respiratory management; botulinum toxin B for sialorrhoea treatment; palliative care throughout the course of the disease; and Modafinil use for fatigue treatment. Further research is needed in management of dysphagia, bronchial secretion, pseudobulbar affect, spasticity, cramps, insomnia, cognitive impairment, and communication in motor neuron disease.

scholarly journals Far beyond the motor neuron: the role of glial cells in amyotrophic lateral sclerosis

2016 ◽  
Vol 74 (10) ◽  
pp. 849-854
Author(s):  
Paulo Victor Sgobbi de Souza ◽  
Wladimir Bocca Vieira de Rezende Pinto ◽  
Flávio Moura Rezende Filho ◽  
Acary Souza Bulle Oliveira
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Glial Cell ◽  
Glial Cells ◽  
Motor Neurons ◽  
Cell Interaction ◽  
Cell Functions ◽  
Glial Cell Interactions ◽  
Lateral Sclerosis

ABSTRACT Motor neuron disease is one of the major groups of neurodegenerative diseases, mainly represented by amyotrophic lateral sclerosis. Despite wide genetic and biochemical data regarding its pathophysiological mechanisms, motor neuron disease develops under a complex network of mechanisms not restricted to the unique functions of the alpha motor neurons but which actually involve diverse functions of glial cell interaction. This review aims to expose some of the leading roles of glial cells in the physiological mechanisms of neuron-glial cell interactions and the mechanisms related to motor neuron survival linked to glial cell functions.

scholarly journals Chromosome 10 Abnormality Predicts Prognosis of Neuroblastoma Patients With Bone Marrow Metastasischromosome 10 Abnormality Predicts Prognosis of Neuroblastoma Patients With Bone Marrow Metastasis

2021 ◽  
Author(s):  
Chiyi Jiang ◽  
Xiao Xu ◽  
Binglin Jian ◽  
Xue Zhang ◽  
Zhixia Yue ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Clinical Characteristics ◽  
Chromosomal Abnormalities ◽  
Abnormal Chromosome ◽  
Mycn Amplification ◽  
Chromosome 10 ◽  
Orbital Metastases ◽  
One Year ◽  
The Relationship

Abstract Background Neuroblastoma (NB) is the most common extracranial solid tumor in children with high heterogeneity and concealed onset. The mechanism for its occurrence and development has not been revealed. The purpose of this study was to summarize the clinical characteristics of children with NB and abnormal chromosome 10. To investigate the relationship between the number and structure of chromosome 10 abnormality and NB prognosis.MethodsWe used chromosome G-banding in the first diagnosis to evaluate the genetics of chromosomes in patients with NB, and follow up their clinical characteristics and prognosis. All participants were diagnosed with NB in Hematology Oncology Center, Beijing Children’s Hospital from May 2015 to December 2018, and were followed up for at least one year. ResultsOf all 150 patients with bone marrow metastases, 42 were clearly diagnosed with chromosomal abnormalities. There were 13 patients with chromosome 10 abnormalities definitely, and the loss of chromosome 10 was the most common decrease in the number of chromosomes. These 13 patient had higher LDH, lower OS and EFS than that of children in abnormal group without chromosome 10 abnormality. Eight patients both had MYCN amplification and 1p36 deletion. Two of them had optic nerve damage and no vision, and 1 had left supraorbital metastases five months after treatment. Among the 16 children with suspected chromosome 10 abnormalities, 3 also had orbital metastases. ConclusionsThe above results showed that chromosome 10 might be a new prognostic marker. MYCN amplification and 1p36 deletion may be related with chromosome 10 abnormalities in NB. And NB patients with abnormal chromosome 10 were prone to have orbital metastases.

scholarly journals Motor neuron disease presenting with fetal akinesia

2018 ◽  
Vol 45 (S1) ◽  
pp. S4-S4
Author(s):  
P. Shannon ◽  
D. Chitayat ◽  
K. Chong ◽  
C. Dunham ◽  
C. Fallet-Bianco
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Globus Pallidus ◽  
Motor Neurons ◽  
Muscular Atrophy ◽  
Neuronal Loss ◽  
Ischemic Injury ◽  
Neuroaxonal Dystrophy ◽  
Lysosomal Storage ◽  
Whole Exome

By contrast to infantile spinal muscular atrophy, which usually links to deletions in the SMN genes, fetal onset motor neuron disease is poorly reported. We collected a series of twelve cases of fetal arthrogryposis (16-31 weeks gestational age) with fetal motor neuron disease and excluded infectious diseases, lysosomal storage disease and neuroaxonal dystrophy. Of these twelve, 3 were thought to be ischemic in nature with microvascular alterations and systemic or central nervous system ischemic injury. The remaining 9 all displayed marked reduction in anterior horn motor neurons. Of these 9, four demonstrated mineralised neurons, four demonstrated either neuronal loss or cavitation in the globus pallidus, and in two, degenerating neurons were detectable in the brainstem or globus pallidus. Specific sequencing of SMN1 was performed in 6 of 9 and was reported as normal. Whole exome sequencing was performed in 4 without definitive diagnosis. We conclude that fetal motor neuron disease can be distinguished from ischemic injury, is morphologically heterogeneous, may affect the globus pallidus and is rarely linked to SMN1 mutations.

Clinical analysis of first-ever acute ischemic stroke involving the territory of paramedian mesencephalic arteries

Open Medicine ◽  
2007 ◽  
Vol 2 (1) ◽  
pp. 37-46 ◽  
Author(s):  
Peterus Thajeb ◽  
Teguh Thajeb ◽  
Dao-Fu Dai
Keyword(s):  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Age Of Onset ◽  
Clinical Analysis ◽  
Clinical Triad ◽  
One Year ◽  
Associated Risk Factors ◽  
A Prospective Study ◽  
Bilateral Lesions

AbstractTo determine one-year clinical outcome of patients with first-ever acute ischemic stroke involving the territory of paramedian mesencephalic arteries (PMAS), we conducted a prospective study evaluating the cognitive functions of 28 patients with PMAS. Neuropsychological tests were performed during the first month of stroke onset and at the 12th month of follow-up. There were 12 women and 16 men. Mean age of onset for women and men was 70 years and 65 years, respectively. Progressing strokes occurred in 62% of patients and 96% developed a full-blown picture of the clinical triad of akinetic mutism, hypersomnolence, and bilateral blepharoptosis and ophthalmoparesis. Involuntary movements occurred in 6, and focal myoclonus in 4 patients. The top four associated risk factors were hypertension (68%), hyperlipidemia (57%), diabetes mellitus (46%), and atrial fibrillation (36%). Unilateral midbrain infarctions occurred in 12 patients and bilateral lesions in 16. Thalamic infarctions were unilateral in 10 and bilateral in 13 cases. Three of the 28 (11%) patients died of recurrent cerebral infarctions within 1 year of the onset of PMAS. The recurrent infarctions involved the basilar artery territory in two cases and the carotid system in another. One patient died of acute myocardial infarction. Of the 24 patients who had survived the stroke by 1 year, 20 (71%) developed dementia. We conclude that first-ever ischemic stroke with PMAS is not a benign syndrome. Most patients developed dementia by 1 year after the stroke.

Lower Motor Neuron Disease with Accumulation of Neurofilaments in a Cat

1976 ◽  
Vol 13 (6) ◽  
pp. 428-435 ◽  
Author(s):  
M. Vandevelde ◽  
C. E. Greene ◽  
E. J. Hoff
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Histological Examination ◽  
Muscle Atrophy ◽  
Motor Neurons ◽  
Nerve Cells ◽  
Lower Motor Neuron ◽  
Motor Neuron Diseases ◽  
Lower Motor Neuron Disease

A young cat had signs of tetraparesis that progressed to tetraplegia within a few weeks. Clinically, there was lower motor neuron disease with areflexia and muscle atrophy in all limbs. Degeneration of the motor neurons in the spinal cord was seen on histological examination. Ultrastructurally, the degeneration of nerve cells was characterized by abnormal proliferation of neurofilaments. These findings were compared to other motor neuron diseases and neurofibrillary accumulations in man and animals.

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