Degenerative Brain Disease Flashcards
Give 3 examples of degenerative brain diseases
- multiple sclerosis
- motor neurone disease
- Parkinson’s disease
What is the definition of a degenerative brain disease?
- slowly progressive diseases which stop neurological function
- variable rates of progression
What is multiple sclerosis?
- disease of the central nervous system involving the demyelination of axons
- most common CNS disease of the young
- progressive functional loss
What causes multiple sclerosis?
- inflammatory changes resulting in demyelination
How does multiple sclerosis present on an MRI?
- white patchy plaques with red areas of inflammatory processes
- areas experiencing inflammation change over time, affecting different parts of the body
What is the impact of multiple sclerosis?
- permanent neurological deficit and loss of function
- can occur anywhere containing neurones
- motor, cognitive, sensory, autonomic
What is the aetiology of multiple sclerosis?
- Unknown but thought to have a genetic propensity
- more common in identical twins
- twins can have different disease levels
- more common in immediate family members
- susceptibility is potentially acquired during childhood
- potentially a host reaction to an infective agent
- potentially related to vitamin D
- incidence increases further from the equator
What are the signs of multiple sclerosis?
- muscle weakness
- visual disturbance
- paraesthesia
- burning/prickling sensation
- autonomic dysfunction
- dysarthria
- difficulty speaking
- pain
- balance and hearing loss
What are the symptoms of multiple sclerosis?
- muscle weakness
- spasticity
- indicative of an UMN lesion
- altered reflexes
- tremor
- intention
- when trying to complete a task
- optic atrophy
- reduced optic nerve conduction speed
- longer to conduct to occipital lobe
- proprioceptive loss
- issues with limb positioning
- loss of touch
- pain felt but no cause
- sudden sensation loss
How can multiple sclerosis be investigated?
- history and examination
- MRI
- shows location of plaques
- shows previous inflammation
- CSF analysis
- reduced lymphocytes
- increased IgG protein
- visual evoked potentials
- reduced after optic neuritis
What are the two types of multiple sclerosis?
- relapsing and remitting
- primary progressive
Describe relapsing and remitting type multiple sclerosis
- acute exacerbations and periods of respite
- damage builds up with each episode
- stable between episodes
- often become secondary progressive
Describe primary progressive type multiple sclerosis
- slow and steady progressive deterioration
- cumulative neurological damage
- no exacerbation and remission periods
How is multiple sclerosis managed?
- symptomatic management
- management during acute attack
- symptoms must be present
- antibiotics, antispasmodics, analgesia, steroids
- slow the episodic nature of relapsing and remitting type
- do not reverse damage
- disease modifying therapies
- may slow some progressive forms
- cladribine
- siponomod
- ocrelizumab
- physiotherapy and occupational therapy
- stem cell transplant
- to reboot the immune system
- immune system recognition changed
- risky procedure
- existing damage not reversed
What are the dental considerations of multiple sclerosis?
- limited mobility and psychological disorders
- difficult accessing dental care
- unable to perform oral hygiene adequately
- mental health problems common
- treatment under local anaesthetic
- general anaesthetic may hasten damage
- orofacial motor and sensory disturbance
- suspicious in younger patients
- unexplained loss of motion or sensation
- refer for MRI immediately
- chronic orofacial pain possible
- may be no cause of pain
- enhanced trigeminal neuralgia risk
- suspicious in younger patients
What is motor neurone disease?
- degeneration of the motor neurone in the anterior horns of the corticospinal tracts in the spinal cord
- can affect bulbar motor nuclei (brain stem)
- progressive disease
- short lifespan
Who is most likely to be diagnosed with motor neurone disease and what is the prognosis?
- 30-60 year olds
- more common in males
- yeah within 3 years of diagnosis
What are the signs of motor neurone disease?
- progressive loss of motor function
How does motor neurone disease result in death?
- ventilation failure
- impaired ventilation
- hypoxia
- aspiration pneumonia
- caused by coughing and swallowing
- protective reflexes of pharynx lost
How is motor neurone disease treated?
- no effective treatments
What body parts are affected by motor neurone disease?
- limbs
- intercostal muscles
- impaired ventilation
- diaphragm
- impaired ventilation
- motor cranial nerves (CNVII-CNXII)
- swallowing difficulties
- muscles of facial expression altered
What are the main symptoms the patient may notice?
- weakness in ankle or leg
- tripping
- hard to climb stairs
- slurred speech
- progresses to difficulty swallowing
- weak grip
- dropping things
- hard to open jars/do up buttons etc.
- muscle cramps and twitches
- weight loss
- limbs become thinner over time
- muscle bulk is lost
- emotional lability
- crying or laughing at inappropriate situations
How is motor neurone disease managed?
- physiotherapy and occupational therapy
- maintain function for as long as possible
- riluzole
- benzothiazole
- 6-9 month life extension
- aspiration prevention
- PEG tube feed (reduced aspiration risk)
- reduce salivation (oral bacteria not in lungs)
What are the dental aspects of motor neurone disease?
- difficulty accepting dental care
- muscle weakness in head and neck
- tipping forward of head and drooling
- oral hygiene ability reduced
- realistic treatment planning
- must think of quality of life
- complex and long lasting treatments are not necessary
- drooling and swallowing difficulties
- medications used to dry up saliva
- anticholinergics
- botox injection into salivary gland
What is Parkinson’s disease?
- a degenerative disease due to a lack of dopamine in the substantia nigra
What are the features of Parkinson’s and who does it affect?
- relatively common
- disabling
- progressive
- as disease progresses, dopamine reduced
- on scans show dopamine marker lost
- usually older people
- some versions found in younger patients
What causes Parkinson’s disease?
- degeneration of dopaminergic neurones in the basal ganglia (substantia nigra)
- shortage of dopamine results in difficulty of messages passing from thinking to doing the the brain
- slow passage from cortex to cerebellum/brainstem
- underlying cause is unknown
- repeated head trauma considered
What are the clinical signs of Parkinson’s disease?
- bradykinesia
- slow movement
- slow initiation of movement
- rigidity
- increased muscle tone
- joints are stiffer and move in a jerky manner
- tremor
- slow amplitude
- felt in hands, arms and mandible
- rest tremor and intentional movement inhibits
- can progress from some poor movement to no movement very quickly
What are the effects of Parkinson’s disease?
- tremor
- pain
- visuospatial dysfunction
- dysphagia
- gastrointestinal dysfunction
- frequent falls
- high cholesterol levels
- low urate levels
- mild cognitive impairment
- postural problems
- mobility problems
How does Parkinson’s disease manifest?
- impaired gait and falls
- unsteady walking
- impaired use of upper limbs
- mask like face
- reduced function of facial expression muscles
- swallowing problems
- voluntary muscles of pharynx affected
- loss of cognitive function
- non-motor changes are particularly distressing
What is the treatment for Parkinson’s disease?
- physiotherapy and occupational therapy
- maintain function at as high a level for as long as possible
- maximise living ability with remaining function
- dopamine
- levodopa
- initially effective but dose must be continually increased
- side effects become intolerable
- dopamine analogues
- mimic effect of dopamine
- tablets - promipexole, selegiline
- injection - apomorphine (subcutaneous)
- infusion - duodena (direct to gut)
- some medications cause abnormal compulsions
- gambling addiction
How is Parkinson’s disease treated?
- surgery
- stereotactic surgery
- deep brain stimulation with electrodes
- allows some return of function
- stem cell transplant
- aims to deposit dopamine into substantia nigra
What are the dental aspects of Parkinson’s disease?
- difficulty accepting treatment
- tremor at rest of body
- mouth opening often stops tremor
- increased time needed to comply with requests
- dry mouth
- anticholinergic effects of drugs
- water and saliva substitutes
- drug interactions
- check for Parkinson’s and dental drug interactions