CNS CONDITIONS Flashcards
MULTIPLE SCLEROSIS (MS) - DEFINITION
- MS is an autoimmune disease
- This is when the immune system mistakenly attacks healthy cells
- In people with MS the immune system attacks cells in the myelin, which is the protective sheath that surrounds nerves in the brain and spinal cord
- Damage to the myelin sheath interrupts nerve signals from the brain to other parts of the body
- The damage can lead to symptoms affecting the brain, spinal cord and eyes
MULTIPLE SCLEROSIS (MS) - TYPES
- 4 types of MS
- Clinically isolated syndrome (CIS)
o When someone has a first episode of MS symptoms, it is often categorised as CIS. Not everyone who has CIS goes on to develop MS - Relapsing-remitting MS (RRMS)
o Most common form
o People with RRMS have flare ups of new or worsening symptoms
o Periods of remission follow the flare-ups - Primary progressive MS (PPMS)
o Symptoms slowly and gradually worsen without any periods of relapse or remission - Secondary progressive MS (SPMS)
o In many cases people originally diagnosed with RRMS eventually progress to SPMS
o With this nerve damage is accumulated and symptoms progressively worsen
o There may still be flare-ups but without the periods of remission after
MULTIPLE SCLEROSIS (MS) - CAUSE
- No definite cause is known
- Factors that may trigger MS
o Exposure to certain viruses or bacteria
o Where you live – areas further from the equator have higher rates of MS
o How the immune system functions
o Gene mutations
MULTIPLE SCLEROSIS (MS) - POPULATION AFFECTED
Female, white adults aged between 20 and 40
MULTIPLE SCLEROSIS (MS) - RISK FACTORS
- Age – 20 – 40
- Sex – women are more likely by 2 to 3 times
- Family history
- Certain infections
- Race – white people
- Climate – cold climate more likely
- Vitamin D
- Genes
- Obesity
- Certain autoimmune diseases
- Smoking
MULTIPLE SCLEROSIS (MS) - CLINICAL PRESENTATION
- Signs and symptoms may differ greatly from person and over the course of the disease depending on the location of the affected nerve fibres
- Numbness or weakness in one or more limbs that typically occurs on one side of the body at a time
- Tingling
- Electric-shock sensations that occur with certain neck movements
- Lack of coordination
- Unsteady gait or inability to walk
- Partial or complete loss of vision, usually in one eye at a time, often with pain during eye movement
- Prolonged double vision
- Blurry vision
- Vertigo
- Problems with sexual, bowel and bladder dysfunction
- Fatigue
- Slurred speech
- Cognitive problems
- Mood disturbances
MULTIPLE SCLEROSIS (MS) - DIAGNOSIS
- Multiple tests are used to diagnose MS
- Blood tests
- MRI
o MRI looks for evidence of lesions in the brain or spinal cord that indicate MS - Lumbar puncture
- Evoked potentials test
MULTIPLE SCLEROSIS (MS) - TREATMENT
- Currently no cure for MS
- Treatment focuses on managing symptoms, reducing relapses and slowing the diseases progression
- DMTs
- Relapse management medications
o High dose of corticosteroids for a severe attack - Physical rehabilitation
- Mental health counselling
MULTIPLE SCLEROSIS (MS) - PREVENTION
- Eating a healthy diet
- Getting regular exercise
- Managing stress
- Not smoking and limiting alcohol intake
MULTIPLE SCLEROSIS (MS) - PROGNOSIS
- In some cases, MS does lead to disability and loss of some physical or mental function
- Most people with MS will continue to lead full, active and productive lives
- Taking steps to manage health and lifestyle can help improve the long-term outcome
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - DEFINITION
- ALS is a type of motor neuron disease
- It is also known as Lou Gehrig’s disease
- ALS affects motor neurons – nerves that control the voluntary muscles
- Voluntary muscles are the ones used for actions like chewing, talking and moving the arms and legs
- It is a neuromuscular disorder that causes muscle weakness
- It affects both the upper and lower motor neurons
- In ALS the muscles begin to atrophy, making it difficult to walk, talk, swallow and eventually breathe
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - TYPES OF ALS
- Classical ALS
o Most common type, affects more than 2/3 of people with ALS
o Both the upper and lower motor neurons decline - Progressive bulbar palsy (PBP)
o Affects about 25% of people with ALS
o It starts with difficulty speaking, chewing and swallowing caused by a breakdown of the upper and lower motor neurons to the mouth and throat
o This will sometimes worsen to affect the rest of the body, in which case the condition is called bulbar-onset ALS - Progressive muscular atrophy (PMA)
o Affects only the lower motor neurons - Primary lateral sclerosis
o Rarest form of ALS
o Only the upper motor neurons decline
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - CATEGORIES OF ALS
- Sporadic ALS
o About 90% to 95% of ALS cases are sporadic
o The disease happens randomly
o It is not tied to any clear factor or family history of the disease - Familial ALS
o Affects 5% to 10% of people with ALS
o It happens when you inherit the disease from one or both of your parents
o The disease runs in families and gets passed down through genetic mutations or changes
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - CAUSE
- Its not known what causes ALS
- It is believed to be a combo of factors
- Genetics
o Mutations or changes in certain genes may lead to motor neuron breakdown - Environment
o Getting exposed to certain toxic substances, viruses or physical trauma may cause ALS
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - POPULATION AFFECTED
White men aged between 55 and 75
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - RISK FACTORS
- Age – between 55 and 75
- Race and ethnicity – white people and non-Hispanics
- Sex – earlier in life, men are at a higher risk than men but as age increases, the risk is about the same
- Military veterans
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - CLINICAL PRESENTATION
- At first, you may notice muscle weakness or stiffness
- Limb onset ALS is when the symptoms start in the legs or arms
- Bulbar onset is when the symptoms start with your speech or swallowing
- No matter where the ALS symptoms begin, they soon spread to other parts of the body
- Initial symptoms include
o Muscle cramps and twitching, especially in the hands and feet
o Fasciculations
o Difficulty using arms and legs
o Thick speech and difficulty projecting the voice
o Weakness and fatigue
o Weight loss - As ALS gets more severe symptoms include
o Shortness of breath
o Difficulty breathing, chewing and swallowing
o Inability to stand or walk independently
o Weight loss, since people with ALS burn calories at a faster rate
o Depression and anxiety as people become aware of what is happening to them
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - CLINICAL PRESENTATION - BREATHING PROBLEMS
o Shortness of breath, even during rest
o Weak cough
o Difficulty clearing throat or lungs
o Extra saliva
o Inability to lie flat in bed
o Repeated chest infections and pneumonia
o Respiratory failure
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - DIAGNOSIS
- Blood test
- Urine test
- A neurological examination – to test reflexes
- Electromyogram (EMG)
- A nerve conduction study
- MRI
- Other tests cant diagnose ALS but can rule out other diseases
o A spinal fluid test
o A muscle and/or nerve biopsy
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - TREATMENT
- No cure
- Treatment can slow progression of the disease
- Medications
o To relieve muscle cramps, extra saliva and other symptoms
o Riluzole – may help reduce damage to the motor neurons, it cannot reverse damage that has already occurred
o Edaravone – can slow the decline in someone’s functioning - Physical therapy – to stay mobile
- Nutritional counselling
- Speech therapy
- Assistive devices
- Special equipment
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - PREVENTION
- No proven way to prevent ALS
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - PROGNOSIS
- A persons prognosis depends on how quickly the symptoms progress
- People with ALS typically live for 3 to 5 years after diagnosis
EPILEPSY - DEFINITION
- Epilepsy is a chronic disease that causes repeated seizures due to abnormal electrical signals produced by damaged brain cells
- A burst of uncontrolled electrical activity within brain cells causes a seizure
- Seizures can include changes to the patients awareness, muscle control, sensations, emotions and behaviour
- Epilepsy is also called a seizure disorder
EPILEPSY - CAUSE
- In up to 70% of cases the cause of seizures is unknown
- Known causes include
o Genetics
o Mesial temporal sclerosis
o Head injuries
o Brain infections
Meningitis
Encephalitis
o Immune disorders
o Developmental disorders
Focal cortical dysplasia
Polymicrogyria
Tuberous sclerosis
o Metabolic disorders
o Brain conditions and brain vessel abnormalities
Brain tumours
Strokes
Dementia