degenerative brain disease Flashcards
degenerative brain diseases
slowly progressive diseases which stop proper neurological function
speed varies, but decline inevitable
e.g.
- multiple sclerosis
- motor neurone disease
- parkinson’s disease
most common CNS disorder of young
multiple sclerosis
80:100000
multiple sclerosis
CNS lesions only
patchy distribution
progressive functional loss – variations
- Any part of body where neurological function needed - cognitive, motor, sensory, autonomic
women with 4th decade onset most severe
lesions in MS
DEMYLINATION OF AXONS
Changes in myelin sheath connecting different parts of brain
- Due to inflammatory change – red parts on MRISame pt years apart – see white patchy plaques stand out as abnormal and red areas happening, next scan red inflammatory changes are in different areas
typical of MS, areas affected change from time to time but permanent changes in CNS so permanent neurological deficit and loss of function
- Any part of body where neurological function needed - cognitive, motor, sensory, autonomic
aetiology of MS
- unknown
- infective, environmental, genetic
- susceptibility acquired during childhood
- ? altered host reaction to a infective agent
- background genetic/immune factors
- more common in identical twins
- have different levels of disease – so genetics only part of issue
- more common amongst immediate family members
- more common in identical twins
MS symptoms
- Muscle weakness
- numbess
- stiffness, spasms
- Visual disturbance
- blurred
- temporary blindness
- dark spots
- Paraesthesia
- Changes in sensation – loss of facial sensation - risk
- Autonomic dysfunction
- frequent urination
- incontinence
- constipation or diarrhea
- sexual and hormonal problems
- dizziness/ vertigo
- Dysarthria
- Issues in speech
- Pain
- Protracted pain with no contributing damage
- Balance/hearing loss
- fatigue
MS signs
- muscle weakness
- Spasticity
- upper motor neuron lesion, damage to the nerve is not from spinal cord to muscle but in cord itself
- Altered reflexes
- Tremor (intention)
- Optic Atrophy
- Light shone in eye – take a finite amount of time to each occipital lobe
- Inflammatory MS changes in optic nerve (common), conduction slowed delayed receiving signal/ in pupil constriction?
- Light shone in eye – take a finite amount of time to each occipital lobe
- Proprioceptive loss/ spatial awareness
- Loss of touch
impact of MS being degenerative on monitoring
- Get degenerative changes in brain which are permanent*
- Can measure them to see progression*
- e.g. optic nerve speed of conduction*
MS Investigations
- History & examination
- Magnetic Resonance Imaging
- Can see plaques in brain and sites of previous damage
- CSF analysis
- reduced lymphocytes
- increased IgG protein
- Visual Evoked Potentials
- ALWAYS reduced after optic neuritis
symptoms
pt complaints
sigsn
clinician notes
MS outcome
2 types
relapsing and remitting type
primary progressive type
relapsing and remitting MS
acute exacerbations and periods of respite
Damage builds up with each episode
- Eventually disabled due to loss of function due to collective damage in CNS
Many will eventually develop progressive form (“secondary progressive”)
primary progressive MS
slow steady progressive deterioration no exacerbations and remission
Cumulative neurological damage
symptomatic management of MS at time of acute attack
antibiotics
antispasmodics
analgesia
steroids
syptomatic management of MS to maintain function
phsyiotherapy and occupational therapy
MS therapy
Physiotherapy and Occupational therapy have role with function loss
- Disease modifying therapies – may also slow some progressive forms
- cladibine
- spinomomod
- oclrelizumab
- Stem Cell Transplant – ‘reboot’ the immune system
disease modifying therapies for MS
- Slow done rate of new lesions occurring*
- Don’t reverse existing damage*
- cladibine
- spinomomod
- oclrelizumab
stem cell transplant therapy for MS
Esp if cause is an unhealthy immune response to environmental trigger
- Risky procedure
- Any existing disability not reversed
MS dental aspects (5)
limited mobility
- access
- OH
psychological disorders
- due to degenerative changes in brain lead to mood difficulties and problems with oral care
treat under LA
- suggestion GA hastens the onset of damage in brain
orofacial motor & sensory disturbance
- present with sudden loss of sensory or motor function get MRI scan for changes/plaques
- esp suspect in younger patients, pt reports motor/sensory changes inn other parts of body that has resolved
Chronic orofacial pain possible
- Enhanced TRIGEMINAL NEURALGIA risk
- suspect in younger patients!
motor neurone disease
degeneration at 2 sites:
- motor nerves in the spinal cord
- anterior horns in the corticospinal tracts
- motor nuclei in the brainstem
- bulbar motor nuclei in CNs
unremitting, progressive
- death with 3 years of diagnosis
no progress in aetiology of management currently – unknown
MND affects
- patients aged 30-60yrs
- male 2.5:1 females
- No good family history – most are sporadic
MND causes
progressive loss of motor function
- limbs
- intercostal
- diaphragm
- impair ventilation -> hypoxia
- motor cranial nerves VII-XII
- swallowing, facial expression
MND deaths due to (2)
- ventilation failure and type 2 respiratory failure
- aspiration pneumonia (swallowing/cough)
- Unable to use protective reflexes in larynx to keep food out of lungs when eating
pt symptoms in MNS
6 aspects
- 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 up buttons
- Muscle cramps and twitches
- Weight loss
- arms or leg muscles may become thinner over time (muscle bulk lost)
- Emotional lability
- crying or laughing in inappropriate situations
MND treatment
NO effective
- Physiotherapy & occupational therapy
- Aim to maintain function for as long as possible
- Progression inevitable
- Aim to maintain function for as long as possible
- Riluzole
- some get 6-9 months life extension
- Aspiration prevention
- Prevent taking food by mouth (direct into stomach)
- PEG tube feed
- reduce salivation
- dentist help possible
- saliva not aspirated carrying oral bacteria to lungs
- dentist help possible
- Prevent taking food by mouth (direct into stomach)
MND dental aspects
difficulty in acceptance of dental care
- muscle weakness of head & neck
- tipping forward of head and drooling - distressing
- OH – power and control of muscles gone
realistic treatment planning
- pt life expectancy short
- try and improve self-image
- complicated treatments for maintenance for long term – little place
- consult if in doubt
drooling & swallowing difficulties
Parkinson’s disease
- Quite common
- Disabling
- Progressive
- Usually, older people
- Some versions in younger
Lack of neurotransmitter – dopamine – in substantia nigra
cause of Parkinson’s disease
unknown
Degeneration of dopaminergic neurones in the basal ganglia of the brain (substantia nigra)
- Shortage of Dopamine results in difficulty of message passage from cortex (‘thinking’) to cerebellum and brainstem (‘doing’)
- Delays in doing or understanding things
Underlying reason for this is unclear
3 clinical signs of parkinson’s disease
BRADYKINESIA
- Slow movement and slow initiation of movement
RIGIDITY
- Increased muscle tone
- E.g. bend and unbend the elbow – stiffer, move in jerks
TREMOR arm, mandible
- slow amplitude
- When pt makes an intentional movement the tremor will go away – rest tremor
- Can progress to on/off movement disorder – often after treatment
- E.g. can move (perhaps badly) to being unable to move at all
bradykinesia
Slow movement and slow initiation of movement
rigidity
increased muscle tone
e.g. bend and unbend elbow - stiffer, move in jerks
tremor in parkinson’s
arm, mandible
slow amplitude
When pt makes an intentional movement the tremor will go away
- rest tremor
Can progress to on/off movement disorder – often after treatment
- E.g. can move (perhaps badly) to being unable to move at all
effect of Parkinson’s disease
- Movement disorder
- Posture
- Communication
- cognition
- effects that are non motor – can be distressing for pt*
- E.g. loss of cognitive functions
clinical observations
management of parkinson’s disease
- Impaired gait and falls
- Impaired use of upper limbs – not swing naturally when walk
- Mask-like face – basal, flat appearance
- Swallowing problems – effect on voluntary muscles of upper pharynx
3 treatment classes for parkinsons
physical support
medical
surgical
physical support treatment for parkinsons
- Physiotherapy and Occupational therapy
- work to maintain function at as high a level for as long as possible
medications for parkinsons
Dopamine
- Levadopa
- Effective at beginning, dose need continually increased to keep effectiveness – side effects become intolerable
Dopamine analogues
- Tablets – Promipexole, Selegiline
- Injection – apomorphine - subcutaneous
- Infusion – duodopa – directly into the
- Individuals need to try many medications to find what is helpful for them*
- Drugs used to treat Parkinson’s can cause abnormal compulsions and lead to gambling – make family*
surgical treatment for parkinsons
Stereotactic surgery
- 3D technique to locate electrodes deep into brain
- Deep brain stimulation
- Return to a much more normal function – greater effect than medicines
- Deep brain stimulation
- Stem cell transplant?*
- Into substantia nigra – not yet used
dental aspects of parkinsons
Difficulty accepting treatment
- Mobility
- Tremor at rest of body
- Esp mandible – may make dentist wary of carrying out operative care
- Often facial tremor reduces on purposeful movements e.g. mouth opening
Dry mouth
- Anticholinergic effects of the drugs
- Water and saliva substitutions
Drug interactions
- Check BNF that what you are prescribing is compatible
