Nervous system: Neurodegenerative diseases
Definitions & Key takeaways
Neurodegenerative diseases are conditions that cause the progressive loss of function in the nervous system, including the brain and spinal cord. These diseases can cause a wide range of symptoms, depending on the specific condition and the areas of the nervous system affected. Some common neurodegenerative diseases include: Parkinson's disease: a brain disorder that causes tremors, stiffness, and difficulty with movement. It results from the loss of certain neurons that produce dopamine. Multiple sclerosis: a disease that affects the protective covering of nerve cells in the brain and spinal cord, causing problems with movement, sensation, and other functions. Amyotrophic lateral sclerosis (ALS): a progressive neurodegenerative disease that affects the nerve cells responsible for controlling muscle movement, leading to muscle weakness and wasting. Huntington disease: an inherited neurodegenerative disease that causes the progressive loss of brain cells, leading to changes in movement, cognition, and behavior.
Intro0:00–0:32
Neurodegenerative diseases are a group of disorders characterized by the degeneration, or loss, of neurons, which are the main cells of the nervous system.
Some of the most famous neurodegenerative diseases include Parkinson disease; Huntington disease; multiple sclerosis; and amyotrophic lateral sclerosis, formerly called Lou Gehrig’s disease.Okay, let’s start with Parkinson disease.
This is a slowly progressive disorder that primarily affects individuals over 50 years old. It is caused by the degeneration of certain neurons in the brain that produce dopamine.
Parkinson Disease0:32–3:32
Dopamine is a neurotransmitter, or a chemical used to relay messages between neurons within the brain, and normally, one of its main roles is to promote movement.
In Parkinson disease, a decrease in dopamine results in difficulty initiating or controlling movement. This is typically manifested with four main symptoms, which can be remembered with the mnemonic “TRAP.” “T” stands for tremor, which is classically described as a resting, pill-rolling tremor because it looks like someone is rolling a pill between their thumb and index finger.
“R” stands for rigidity, which is caused by tensed up muscles that resist movement. When someone tries to flex the client’s limbs, they could move in a series of stops and jerks, which is called “cogwheel rigidity.” If it remains rigid throughout the movement, it’s called “lead pipe rigidity.” “A” stands for akinesia, which is the absence of movement and is a severe form of the more common finding: bradykinesia, which is the slowness of movement.
This can manifest as a slow, shuffling gait or a decrease in facial expressions almost to the point where the individual’s face looks like they’re wearing a mask.
“P” stands for postural instability, which causes a stooped posture, problems with balance, and an increased frequency of falls.
If the muscles involved in speech are also affected, the client might either slur words or talk too quickly to the point that it’s hard to understand them.
Likewise, dysfunction of the muscles used for chewing or swallowing might cause difficulties with eating and drinking. They are more likely to drool, and there’s an increased risk of choking.
In fact, one of the most common complications of Parkinson disease is aspiration where food, fluid, or saliva enter the airway instead of the esophagus.
These can carry harmful microbes into the lungs, leading to aspiration pneumonia. In the later stages of the disease, nonmotor brain functions can be affected as well, leading to other common symptoms that include depression, dementia, sleep disturbances, and difficulty smelling.
Interestingly, seborrheic dermatitis, an oily skin rash that appears on the scalp, face, chest, and axilla, has also been associated with Parkinson disease.
Treatment primarily includes medications that increase brain dopamine signaling. If medications aren’t effective at controlling symptoms, then a surgical treatment called deep brain stimulation can be done.
In deep brain stimulation, an electrical lead is placed in the brain region controlling muscle movement. In any case, clients with Parkinson disease may also need physical therapy to improve their posture, balance, and range of motion and speech therapy to limit speech and swallowing difficulties.
Okay, next is multiple sclerosis. This is an autoimmune demyelinating disease, meaning the immune system inappropriately attacks and destroys the myelin.
Multiple Sclerosis3:32–5:18
Myelin is the protective sheath that surrounds the axons of neurons, allowing them to quickly send electrical impulses. So, without myelin, the communication between neurons breaks down, ultimately leading to all sorts of sensory, motor, and cognitive problems.
Now, symptoms of multiple sclerosis usually manifest when the client’s between the ages of 20 and 40. These typically occur in bouts, meaning the symptoms will undergo remission for weeks, months, or even years before a relapse where they return.
During the remission periods, the client could have little to no symptoms. Common symptoms of multiple sclerosis include speech and swallowing difficulties as well as vision problems, like double vision, blurring in one or both eyes, involuntary rapid eye movements, or even vision loss.
The bladder and bowel function might also be disturbed, leading to constipation and urinary retention or bowel and urinary incontinence.
Motor symptoms may include muscle weakness; muscle spasms; ataxia, or loss of balance and coordination; and tremor, twitching, or jerking movements that intensify as the client moves the affected limb.
In the late stages, multiple sclerosis can also lead to paralysis. Finally, other functions of the brain can also be affected, causing poor concentration and critical thinking as well as depression and anxiety.
There is no cure for multiple sclerosis, but there are medications that can lessen the severity and frequency of the relapses.
Moving on to amyotrophic lateral sclerosis, or ALS. This is caused by the death of neurons controlling muscle movement.
Amyotrophic Lateral Sclerosis5:18–6:09
ALS typically manifests between age 40 to 60 with muscle weakness, frequent falls, and speech or swallowing difficulties.
Now, ALS is a purely motor disorder, so through all this, there are no sensory or cognitive symptoms, but due to the debilitating nature of the disease, the client will often experience depression or other psychological problems.
While there is no cure, physical and speech therapy typically play an important role in the client’s care. Huntington disease is a relatively rare disorder that involves a genetic mutation that results in the death of neurons in a specific area of the brain.
Huntington Disease 6:09–6:56
This leads to abnormal movement, like purposeless, dance-like jerking movements, or slower, writhing, “snake-like” movements that mainly affect the hands.
Other motor problems include abnormal eye movements; poor coordination; and swallowing difficulties, which put the client at high risk for aspiration.
Huntington disease can also lead to cognitive problems, like dementia, as well as mental problems, like depression. Now, there is no cure, but several medications along with physical and speech therapy have the potential to improve the clients' symptoms.
Common Care Tips6:56–8:57
When caring for clients with neurodegenerative disorders, there are some common care tips you need to remember. For clients with Parkinson disease, keep an eye out for the on/off phenomenon and report it to the nurse so that the type and dose of the prescribed medication can get adjusted accordingly.
It might be also helpful to take advantage of the client’s “on” times to arrange personal hygiene and other activities. It’s also important to encourage these clients to stay as physically active as possible, while taking the appropriate safety precautions to reduce the risk of falls or other injuries.
These may include avoiding slippery floors, getting rid of potential obstacles on the floor, covering hard surfaces with padded mats, and making sure that there’s adequate lighting.
Remember to check the client’s care plan for instructions regarding their optimal positioning. If the client is lying in bed or sitting for a long period of time, keep an eye out for signs of pressure ulcers, like pain and redness that doesn’t go away after pressure is relieved and inform the nurse right away.
To prevent these from developing, practice proper skin care and assist them to change positions to keep pressure off. Make sure to also attend to your client’s special needs regarding elimination, personal hygiene, and communication, as well as food and fluid ingestion.
To prevent aspiration, make sure that the client remains upright during the meal as well as for at least 1 hour afterward.
Also, make sure to assist them during mealtimes if needed. For ALS, in the later stages, a feeding tube as well as respiratory support, like mechanical ventilation and suctioning, might be also required.
Finally, in all cases, it’s important to provide support and encouragement for clients with these difficult disorders.All right, as a quick recap...
Recap8:57–10:23
Parkinson disease is caused by the degeneration of dopamine-producing neurons in certain parts of the brain, which leads to tremor, rigidity, problems initiating movement, and postural instability.
In multiple sclerosis, the immune system attacks and destroys the myelin sheaths of neurons, leading to a variety of sensory, motor, and cognitive problems, which typically occur in bouts or relapses followed by periods of remission with little or no symptoms.
Amyotrophic lateral sclerosis, or ALS, is caused by the death of neurons responsible for muscle movement, resulting in motor symptoms without sensory or cognitive symptoms and with preserved bowel and bladder function.
Huntington disease is caused by a genetic mutation leading to neuron death and presents with abnormal dance-like jerking or “snake-like” movements as well as cognitive and mental symptoms.
When caring for clients with neurodegenerative disorders, make sure to attend to their special needs and assist them with movement, self-care, personal hygiene, communication, and food or fluid ingestion.
Reduce the risk of falls and injuries by practicing safety measures. Also, prevent pressure ulcers with proper and regular skin care and assisting with position changes.
Avoid aspiration by making sure they remain upright during and
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