Neurology - Degenerative Brain Disease Flashcards
What are degenerative brain diseases?
- These are slowly progressing diseases which stop proper neurological function
- This can be quicker in some and slower in others but inevitably decline will occur
What is the most common CNS disorder of the young?
- Multiple sclerosis
What is multiple sclerosis and why does it happen?
- Happens because of changes in the myelin sheath around the nerve axons connecting different parts of the brain
- Classified as a demyelinating condition
The demyelination occurs due to inflammatory change - Can affect any part of the body function where nerves are involved from cognitive to motor to sensory and to autonomic
- It has a patchy distribution
- And it a progressive functional loss
Who is likely to be more severely affected by multiple sclerosis?
- Womein with 4th decade onset most severe
What is the aetiology of multiple sclerosis?
- Cause is uncertain
- Susceptibility acquired during childhood
- ? altered host reaction to an infective agent
- Background genetic/imune factors
When is MS more common? (2)
- More common in identical twins
- More common amongst immediate familt members
Symptoms of MS can vary from one person to the other. What are some of the more common symptoms seen? (16)
- Fatigue
- Cognitive changes
- depression
- Dizziness and vertigo
- Hormonal effects
- frequent urination
- Difficulty walking
- Blurred vision
- Temporary blindness
- Seeing dark spots
- Numbness
- Pain
- Sexual problems
- Muscle weakness, stiffness and spasms
What are common symptoms of MS? (7)
- Muscle weakness
- Visual disturbance
- Paraesthesia (change in sensation)
- Autonomic dysfunction
- Dysarthia
- Pain
- Balance/hearing loss
What is dysarthia?
- Difficulty speaking
What are common signs of MS? (7)
- Muscle weakness
- Spasticity (this happens when there is an upper motor neuron lesion)
- Altered reflexes
- Tremor (intention - when the patient is trying to complete a task)
- Optic atrophy
- Proprioceptive loss
- Loss of touch - thic can cause issues in trying to control the position and the use of limbs
Importantly the patient with MS will often get degenerative changes in the nerves which are permanent and one of these which can easily be measured are changes in the optic nerves speed of conduction. How would we test this?
- If a light is shone in the eye it will take a finite amount of time for that stimulation to reach the occipital lobe in the brain
- If the patient has had inflammatory MS changes in the optic nerve (which is quite common) this conduction will be slowed and therefore there will be a delay in receiving the signal
- This can be measures and is a common test when assessing MS
What investigations might we use to determine if a patient has MS? (4)
- History & examination
- Magnetic resonance imaging (MRI remains a very useful test and can show clealry areas of plaques in the brain where there has been previous damage)
- CSF analysis (reduced lymphocytes, increased IgG protein)
- Visual evoked potentials (ALWAYS reduced after optic neuritis)
What are the 2 types of MS outcome?
- relapsing and remitting type
- Primary progressive type
What is the relapsing and remitting type of MS? (3)
- Acute exacerbations and periods of restbite
- damage builds up with each episode
- Many will eventually develop progressive form (secondary progressive)
What is the primary progressive type of MS? (2)
- Slow steadt progressive deterioration
- Cumulative neurological damage
What is the symptomatic management of MS? (6)
- Antibiotics, antispasmodics, analgesia, steroids
- Physiotherapy & occupational theraphy (to try to maintain function)
What might we use as a therapy for the relapsing and remitting type of MS? (4)
- Disease modifying therapies - may also slow some progressive forms
- (they do not reverse the damage that has already happened but they do seem effective in slowing down the rate at which new lesionsw ill occur)
- Cladribine, Siponomod, Ocrelizumab
How can stem cell transplant be a possible therapy for MS?
- ‘reboot’ the immune system
- These are not without thier own risk - patient needs to determine whether it is worth it
What are the dental aspects of MS? (5)
- Limited mobility & psychological disorders
- Treat under LA (as there is a suggestion that GA increases the onset of damage within the brain)
- Orofacial motor & sensory disturbance ( - These are key things for the dentist to be aware of
A patient presenting with sudden loss of motor or sensory function which is unexplained should undergo an MRI to look for any changes or plaque changes particularly if the patient reports previous sudden onset in motor or sensory change in any other part of their body which subsequently resolved) - Chronic orofacial pain possible
- Enhanced trigeminal neuralgia risk - suspect in younger patients
What is motor neurone disease?
- Degeneration in the spinal cord (corticospinal tracts/anterior horns)
- This has degeneration of the motor nerves in the anterior forns of the corticospinal tracts in the spinal cord
- Can also affect the motor nuclei in the brain stem (bulbar motor nuclei)
What age group are usually affected by MND?
- Patients aged 30-60yrs
How long is the life-expectancy of someone once they have been diagnosed with MND?
- Death with 3 years of diagnosis
How common is MND in males compared to females?
Male 2.5:1 female
Is MND genetic?
- No good family history - most are sporadic
Why does MND occur?
- There has been no progress in uncerstanding why this condition occurs or in how to manage it
MND causes a progrssive loss of motor function. Where in the body does this occur? (4)
- Limbs
- Intercostal
- Diaphragm
- Motor cranial nerves VII-XII (these involve swallowing and facial expression)
How can MND cause death? (2)
Death due to:
- Ventilation failure
- Aspiration pneumonia (swallowing/cough)
What might a patient notice if they have MND? (6)
- Weakness in the ankle or leg (leading to tripping, or find it harder to climb stairs)
- Slurred speech, which may develop into difficulty swallowing some foods
- A weak grip - dropping things, or finding it hard to open jars or do buttons up
- Muscle cramp and twitches
- Weight loss - arms or leg muscles may become thinner over time
- Emotional lability - crying or laughing in inappropriate situations
What treatment do we have for MND? (4)
- NONE effective
- Physiotherapy & occupational therapy (these aim to maintain function for as long as possible but progression is inevitalbe)
- Riluzole (some get 6-9 months life extension)
- Aspiration prevention (PEG tube feed or reduce salivation)
What are the dental aspecrs of MND? (3)
- Difficulty in acceptance of dental care - muscle weakness of head and neck (This will affect OH)
- Realistic treatment planning (Patients life expectancy is short)
- Drooling and swallowing difficulties (patients often find this embarresing - can help either though medications (such as anticholinergics) or injecting botox into the salivary glands
Is Parkinson’s disease common?
- Quite common
Parkinsons disease is disabling and progressive, what is the cause of it?
- It is due to a lack of neurotransmitter dopamine in the substanciaenigra of the brain
Who is often affected by Parkinson’s disease?
- Usually older people (over age of 50 years)
- However, some variations of the condition can be found in younger people
How is parkinson’s caused? (3)
- Degeneration of dopaminergic neurones in the basal ganglia of the brain (substantia nigra)
- Shortage of dopamine results in difficulty of message passaging from ‘thinking’ to ‘doing’ brain (this can lead to a delay in the patient doing things or understanding things)
- The underlying reason for this is unclear
What are the clinical signs of Parkinson’s disease? (4)
- Bradykinesia (slow movement, and slow initiation of movement)
- Rigidity (increased muscle tone)
- Tremor (slow amplitude) (this occurs at rest - when the patient makes an intentional movement the tremor will go away)
- Can progress to on/off movement disorder - often after treatment
What is a key feature of Parkinson’s disease?
- The rest tremor
What are some possible clinical observations of Parkinson’s disease? (4)
Manifestation:
- Impaired gait and falls
- Impaired use of upper limbs
- Mask-like face (very flat looking appearance)
- Swallowing problems (due to the effect on the voluntary muscles of the upper pharynx)
What is possible physical support treatment we can give someone with Parkinson’s? (2)
- Physiotherapy and occupational therapy
- These work to maintain function at as high a level for as long as possible
What is possible medical treatment we can give someone with Parkinson’s? (2)
- Dopamine
- Dopamine analogues
Give an example of dopamine treatment for Parkinson’s disease and explain if this is effective or not?
- Levadopa
- This is a very effective treatment at the beginning but the dose needs to be continually increased to keep that benefit and therefore the consequences of side effects of the medicine eventually become intolerable
What forms of dopamine analogues can be given to treat Parkinson’s disease? (3)
- Tablets
- Injection
- Infusion
What are dopamine analogues?
- They mimic the effect of dopamine
Give 2 examples of dopamine analogues in tablet form?
- Promipexole
- Selegiline
Give an example of dopamine analogues in injection form?
- Apomorphine - subcutaneous
Give an example of dopamine analogues in infusion form?
- Duodopa - directly into the gut
What can be a problem with some of the medications used to treat Parkinson’s disease?
- Unfortunately, some of the medications used to treat Parkinson’s disease can lead to compulsions and can lead to issues with gambling - it is important that the patient and relatives are aware of some of the issues in case problems develop
What is possible surgical treatment of Parkinson’s disease? (2)
- Stereotactic surgery (deep brain stimulation - This means using 3 dimensional techniques to locate electrodes in the part of the brain and this deep brain stimulation can help some patients with Parkinson’s return to a much more normal function)
- Stem cell transplant (transferring stem cells that produce dopamine into the substancianigra - this is not yet a major therapy)
What are the dental aspects of Parkinson’s disease? (4)
Difficulty accepting treatment:
- Tremor at rest of body
- Often facial tremor reduces on purposful movements e.g. mouth opening
- Dry mouth (anticholinergic effects of drugs)
- Drug interactions possible
- Dentist needs to be aware that the patient may take some time to comply with the request and being patient
