Understanding Motor Neurone Disease (Amyotrophic Lateral Sclerosis)

[Music] [Applause] [Music] hi this is Tom from zerof finals.com in this video I'm going to be going through motor neuron disease and you can find written notes on this topic at zerof finals.com mnd or in the neurology section of the second edition of the zero to finals medicine book and you can find flash cards and questions to train your knowledge on this content and help you remember the information for longer at members.of finals.com so let's jump straight in motor neuron disease is a term that incompasses a variety of specific diseases affecting the motor nerves motor neuron disease is a progressive eventually fatal condition where the motor neurons stop functioning the motor neurons are responsible for controlling muscles and muscle movement motor neuron disease has no effect on the sensory neurons meaning that sensation is normal and intact the presence of sensory symptoms suggests an alternative diagnosis amot Tropic lateral sclerosis or ALS is the most most common and well-known type of motor neuron disease Steven Hawking had amyotropic lateral sclerosis Progressive Bulba py is the second most common form of motor neuron disease and it primarily affects the muscles of talking and swallowing the Bulba muscles other types to be aware of are progressive muscular atrophy and primary lateral closis let's talk about the pathopysiology motor neuron disease involves a progressive degeneration of both the upper and lower motor neurons the exact cause is unclear although several mechanisms have been considered many genes have been linked with an increased risk of developing the condition family history is important as around five to 10% of cases are inherited there seems to be an increased risk with smoking exposure to heavy metals and certain pesticides let's talk about the presentation the typical patient is a late middle-aged for example 60 years old man possibly with an affected relative there is is an Insidious meaning slow and subtle Progressive weakness of the muscles throughout the body affecting the limbs trunk face and speech the weakness is often first noticed in the upper limbs there may be increased fatigue when exercising patients May complain about clumsiness dropping things more often or tripping over they may develop slurred speech which is called dysarthria signs on examination of disease in the lower motor neurones are muscle wasting reduced muscle tone fasiculations which are Twitches in the muscles and reduced reflexes signs on examination of disease in the upper motor neurones are increased tone or spasticity of the muscles brisk reflexes and an upgoing plantar reflex let's talk about making the diagnosis the diagnosis of motor neuron disease needs to be made very carefully it's based on the clinical presentation after excluding other conditions and it should only be made by a specialist when there's certainty the diagnosis is often delayed which causes stress finally Let's Talk About Management there are no effective treatments for halting or reversing the progression of the disease a medication called ruol can slow the progression of the disease and extend Survival by several months in patients with ALS non non invasive ventilation or NIV can be used to support breathing when the respiratory muscles weaken management of the condition involves supporting the person and their family which will include Breaking Bad News effectively and supportively when giving the diagnosis multidisciplinary team input to support and maintain their quality of life symptom control for example backen used for muscle spasticity and antimuscarinic medications for excessive saliva benzodiazapines may help breathlessness that's worsened by anxiety Advanced directives can be used to document the patient's wishes for when the disease progresses and end of Life Care research has consistently shown that testing yourself after learning a topic has a powerful effect on how long you you retain that information this is known as the testing effect studying and then testing yourself results in longer lasting and stronger recall on that information when tested at a later date even when compared with additional study sessions if you're preparing for a medical exam and you're not regularly testing your knowledge and practicing your recoil you're failing to maximize your potential the Zer to finals member site contains flash cards short answer questions multiple choice questions and extended matching questions that are purpose built to supplement the zero to finals content helping you build your internal database of knowledge and take advantage of the powerful testing effect if you like the zero to finals notes books videos and podcasts then you'll love the memb site

Channel: Zero To Finals

Views: 57,582

Keywords: medical, education, medicine, doctor

Id: dg_DntAnU8M

Length: 6min 52sec (412 seconds)

Published: Mon Jan 08 2024