MOTOR NEURON DISEASE.pptx

motor neuron disease,als.sma,treatment of als

1. MOTOR NEU R ON DISEASE

2. INTRODUCTION Motor neuron diseases are a group of neurodegenerative disorders that affects the nerves in the spine and brain to progressively lose its function. They are a rare but serious and incurable form of progressive neurodegeneration. It is a condition that selectively affects the motor system, the cells which control voluntary muscles of the body.

3. The renowned English physicist Stephen Hawking lived with ALS for many decades until his death in March 2018. Guitar virtuoso Jason Becker is another example of someone who has been living with ALS for several years.

4. DEFINITION It is defined as a progressiv e disease that involves degeneration of the motor neurons and wasting of the muscles.

5. INCIDENCE Motor neuron disease (MND) occur in adults and children. Annual incidence is about 2/100 000, with a prevalence of about 7/100 000. Most cases are sporadic but 10% of cases are familial. It can appear at any age, but most patients are over 65 years old at diagnosis. It affects men more than women.

6. WHAT ARE MOTOR NEURONS? They are a type of nerve cell, that send messages to the body(muscle) from the brain so helps to move. Two kinds includes: UPPER MOTOR NEURONS: LOWER MOTOR NEURONS: Normally, messages from nerve cells in the brain (called upper motor neurons ) are transmitted to nerve cells in the brain stem and spinal cord (called lower motor neurons ) and from them to particular muscles.

7. CONT… As nerve cells die when pt have a motor neuron disease, electrical messages can't get from the brain to muscles. Over time, muscles waste away, known as “atrophy.” When this happens, it leads to lose control over movements. It gets harder to walk, talk, swallow, and breathe. Each kind of motor neuron disease affects different types of nerve cells or has a different cause. ALS is the most common of these diseases in adults. Here's a look at some of the types of motor neuron diseases.

8. TYPES OF MND: MNDs are classified according to whether they are inherited or sporadic(i.e-anyone can get it), and to whether degeneration affects upper motor neurons, lower motor neurons, or both. In adults, the most common MND is Amyotrophic Lateral Sclerosis (ALS) .

9. CONT… ALS, or Lou Gehrig's disease , is the most common type, affects both the UMN and LMN. It can affect the muscles of the arms, legs, mouth, and respiratory system. Mean survival time is 3 to 5 years, but some people live 10 years or more beyond diagnosis with supportive care. Progressive bulbar palsy (PBP) involves the brain stem(LMN). People with ALS often have PBP too. The condition causes frequent choking spells, difficulty speaking, eating, and swallowing.

10. CO N T… Pseudobulbar Palsy: This is similar to progressive bulbar palsy. It affects motor neurons that control the ability to talk, chew, and swallow. Pseudobulbar palsy causes people to laugh or cry with no control. Progressive muscular atrophy (PMA) : It only affects the LMN in the spinal cord. Affects slowly but progressively causes muscle wasting, especially in the arms, legs, and mouth. It may be a variation of ALS. Primary lateral sclerosis (PLS): It is a disease of UMN. It is the rare form of MND that advances more slowly than ALS. It is not fatal, but it can affect the quality of life. In children, it is known as juvenile primary lateral sclerosis.

11. CONT… Spinal muscular atrophy (SMA) is an inherited MND that affects children. There are three types, all caused by an abnormal gene known as SMA1(This gene makes a protein that protects the motor neurons. Without it, they die.). It tends to affect the trunk, legs, and arms. Long-term outlook varies according to type. The different types of MND share similar symptoms, but they progress at different speeds and vary in severity.

12. CAUSES: The exact causes are unclear. Some MNDs are inherited (10%) , but the causes of most MNDs are not known. About 5% of people with motor neurone disease have a close family relative with the condition or a related condition known as fronto temporal dementia. This is called familial motor neurone disease which can be hereditary or linked to a problem with genes (SOD) that can cause problems at a younger age. In sporadic or non inherited MNDs, environmental, toxic, viral, or genetic factors likely play a role.

13. RISK F AC T ORS: HEREDITARY. AGE. SEX. Some experts have linked military experience to a higher chance of developing the disease. Studies have found that professional footballers are more likely to die from ALS, Alzheimer's disease, and other neurodegenerative diseases, compared with other people. This implies a possible link with recurrent head trauma and neurological disease.

14. SYMPTOMS: MND can be divided into three stages; early, middle , and advanced. Early stage signs and symptoms: Symptoms develop slowly and can be confused with symptoms of some other unrelated neurological conditions. Early symptoms depend upon which body system is affected first. Typical symptoms begin in one of three areas : the arms and legs, the mouth (bulbar), or the respiratory system.

15. They include: a weakening grip, making it hard to pick up and hold things Fatigue, difficulty swallowing muscle pains, cramps, and twitches slurred and sometimes garbled speech weakness in the arms and legs increased clumsiness and stumbling trouble breathing or shortness of breath

16. Middle stage signs and symptoms: As the condition progresses, symptoms become more severe. Muscle pain and weakness increase, and spasms and twinges worsen. Limbs become progressively weaker. Limb muscles start to shrink. Movement in affected limbs becomes more difficult. Limb muscles may become abnormally stiff. Joint pain grows. Eating, drinking, and swallowing become harder.

17. Drooling occurs, due to problems controlling saliva. Yawning occurs, sometimes in uncontrollable bouts Jaw pain may result from excessive yawning. Speech problems worsen, as muscles in the throat and mouth become weaker. The person may show changes in personality and emotional state, with bouts of uncontrollable crying or laughing. Secondary symptoms include insomnia, anxiety, and depression.

18. Advanced stage signs and symptoms Eventually, the patient will be unable to move, eat, or breathe without assistance. Without supportive care, an individual will pass away. Despite the best of care currently available, complications of the respiratory system are the most common causes of death.

19. DIAGNOSIS: In the early stage, MND can be hard to diagnose, because the signs and symptoms are common to other conditions, such as multiple sclerosis (MS), an inflamed nerve, or Parkinson's disease. Blood test. Urine test. Mri. Emg( electromyography ). Nerve conduction study. Lumbar puncture or spinal tap. Muscle biopsy. Transcranial magnetic stimulation .

20. After tests, a doctor will normally monitor the patient for some time before confirming that they have MND. Criteria known as El Escorial criteria can help a doctor check for distinctive neurological signs, that may aid in the diagnosis of ALS. These include: muscle shrinking, weakness or twitching muscle stiffness or abnormal reflexes symptoms spreading into new muscle groups having no other factors that explain the symptoms

21. TREATMENT: There is no cure for MND, so treatments focus on relieving the symptoms, slowing the progression and maximizing patient independence and comfort. This can include the use of breathing, feeding, mobility and communication appliances and devices. Rehabilitation therapy may include physical, occupational and speech therapy.

22. MEDICAL MGT: Two drugs are currently approved by the U.S. Food and Drug Administration (FDA) for ALS. Riluzole or Rilutek lowers the amount of glutamate in the body. It appears most effective in the early stages of ALS and in older individuals. It has been given to improve survival. In early 2017, the drug Radicava ( Edaravone ) was approved by the FDA for the treatment of ALS. How it works is not well understood, but it may delay disease progression by working against tissue damage. Scientists are currently exploring a possible role for stem cells in the treatment of ALS.

23. Muscle cramps and stiffness can be treated with physical therapy and medications, such botulinum toxin (BTA) injections. BTA blocks the signals from the brain to the stiff muscles for about 3 months. Scopolamine, a drug for motion sickness, may help control symptoms of drooling. It is worn as a patch behind the ear. Antidepressants, called serotonin reuptake inhibitors (SSRIs), may help with episodes of uncontrollable laughter or crying, known as emotional lability. Advice from a speech and language therapist. Advice from a dietitian about diet and eating.

24. PROGNOSIS: Prognosis varies depending on the type of MND and the age of onset. Some MNDs, such as PLS are not fatal and progress slowly. People with SMA may appear to be stable for long periods, but improvement should not be expected. Some MNDs, such as ALS and some forms of SMA, are fatal.