Degenerative Disease of the CNS Flashcards
Definition of dementia
Progressive impairment of multiple domains of cognitive function in an alert patient leading to loss of acquired skills and interference in occupational and social role
Parkinsonism is a clinical syndrome with >2 of;
Bradykinesia
Rigidity
Tremor
Postural instability
Definition of bradykinesia
Slowness of movement
Where is the pathology in parkinsons?
Basal ganglia
What is predominately lost is the basal ganglia in parkinsons?
Dopamine
What is the 2nd most common neurodegenerative disease?
Parkinsons
Types of late onset (65 +) dementia
Alzheimer’s (55%)
Vascular (20%)
Lewy body (20%)
Others (5%)
Types of young onset (<65 years old) dementia
Alzheimers (33%) Vascular (15%) Frontotemporal (15%) Other (33%) - toxic (alcohol) - genetic (huntingtons) - infection (HIV, CJD) - inflammatory (MS)
Treatment causes of dementia
Vitamin deficiency - B12
Thyroid disease
HIV
Syphillis
What conditions mimic dementia?
Hydrocephalus
Tumour
Depression (pseudodementia)
Types of parkinsonism
Idiopathic parkinsons disease (IPD) Lewy body dementia (LBD) Drug induced (e.g. dopamine antagonists) Vascular parkinsons (lower half) Parkinsons plus syndromes
What are some parkinsons plus syndromes?
Multiple system atrophy
Progressive supranuclear palsy
Corticobasal degeneration
What is examined when looking at cognitive function?
Memory Attention Language Visuospatial Behaviour Emotion Executive function Apraxia Agnosias
What is ataxia?
A motor disorder in which the individual has difficulty with the motor planning to perform tasks or movements when asked, provided that the request or command is understand and he/she is willing to perform the task
Definition of agnosia
Inability to process sensory information
What screening tests are done for cognitive function?
Mini mental (MMSE) Montreal (MOCA)
What type of dementia has a stepwise progression?
Vascular
What would dementia with abnormal movements indicate?
Huntington’s
What type of dementia also has parkinsonism?
Lewy body dementia
What type of dementia comes with myoclonus?
CJD
How do you get a definitive diagnosis of parkinsonism?
Post morteom
Presentation of IPD
BRADYKINESIA \+ At least one of the following - tremor - rigidity - postural instability Slowly progressive (>5-10 years) Asymmetric rest tremor
Does parkinsons have a good response to dopamine replacement treatment?
Yes
Treatment of PD
Dopamine replacement treatment
- Levodopa (L-dopa) = CO-CARLEDOPA OR CO-BENELDOPA
- dopamine agonists = ROPIRINOLE OR PRAMIPEXOLE OR ROTIGOTINE PATCH
Treatment of Alzheimer’s (+/- Lewy body dementia)
Cholinesterase inhibitors (cholinergic deficit) - donepezil - rivastigimine - galantamine NMDA antagonist - memantine
Treatment for frontotemporal dementia
None
Complications for parkinsonism
Drug induced
- motor fluctuations - levodopa wears off
- dyskinesias - involuntary movements (levodopa)
- psychiatric - hallucinations, impulse control
Non drug induced especially non motor
- depression
- dementia
- autonomic; BP, bladder, bowel
- speech, swallow
- balance
Late treatment of parkinsonism
Prolong levodopa half life - MAO-B inhibitors - COMT inhibitor - Slow release levodopas Add oral dopamine agonist Continous infusion - apomorphine - duodopa Functional neurosurgery (Deep brain stimulation)
What is the commonest neurodegenerative condition?
Alzheimer’s disease
Mean onset age of Alzheimer’s?
70 y/o
Presentation of temporo-pareital dementia
Early memory disturbance
Language and visuospatial problems
Personality preserved until later
Presentation of frontotemporal dementia
Early change in personality/behaviour
Often change in eating habits
Early dysphagia
Memory/visuospatial relatively preserved
Presentation of vascular dementia
Mixed picture
Stepwise decline
What are the degenerative causes of parkinsonism?
IPD
LBD
Parkinsons plus syndromes
What are the 2ndry causes of parkinsonism?
Vascular
Drug induced
What is IPD responsive to?
Levodopa
Exclusion criteria for PD
History of
- repeated strokes with stepwise progression of parkinsonism features
- repeated head injury
- definite encephalitis
Oculogyric crises
Neuroleptic treatment at onset of symptoms
More than one affected relative
Sustained remission
Strictly unilateral features after 3 years
Supranuclear gaze palsy
Cerebellar signs
Early severe autonomic involvement
Early severe dementia with disturbance of memory, language and praxis
Babinskis sign
Prescence of cerebral tumour or communicating hydrocephalus on imaging study
Negative response to large doses of levodopa in absence of malabsorption
MPTP exposure
Supportive prospective criteria for PD
Unilateral onset Rest tremor present Progressive Persistent asymmetry affecting side of onset most Excellent response (70-100%) to levodopa Severe levodopa induced chorea Levodopa response for 5 years or more Clinical course of 10 years or more
Pathological changes of IPD
Deposits of Lewy bodies
Loss of pigmented dopaminergic neurones in brainstem nuclei
Alpha synuclein changes thought key
Over time changes spread - involving more of brainstem, then cortex etc
What is the most common cause of parkinsonism?
IPD
What cause of parkinsonism is more likely to have cognitive problems than parkinsonism?
LBD
What presymptomatic changes can people get in PD before they get their symptoms?
Anosmia
What can be used to assess bradykinesia?
Finger taps
What can indicate rigidity?
Loss of arm swing whilst walking
What type of signs to start off with indicate IPD?
Unilateral
Assessment of parkinsonism
Finger tap
Full neurological assessment
What happens to a finger tap in PD?
Do for 20 seconds, gets slower as time goes on and gets smaller
Worse on DOMINANT SIDE
Extra indications of PD
Loss of facial expression
Micrographia
What is micrographia?
Small and tremulous writing
Diagnosis of PD
Clinical
What must also be asked about in PD?
Non motor symptoms
What are the non motor symptoms that may occur in PD?
Cognition Cramps Restless legs Constipation Speech Mood changes
When is a Dat-SPECT scan used?
To differentiate essential tremor from parkinsonian tremor
When is Dat-SPECT scan +ve?
Degenerative parkinsonian conditions, but DOES NOT tell you which one
Drug interactions of Dat-SPECT that decreases the binding
Cocaine Amphetamines Methylphenidate Ephedrine Fentanyl
Drug interactions of Dat-SPECT that increase the binding
SSRIs
Why give levodopa in PD?
Replaces the chemical
Why give dopamine agonists in PD?
Boots any chemical left over in the brain
What are some other Parkinson therapies?
Apomorphine
Duodopa
What is apomorphine used to treat?
Disabling motor fluctuations - if off spells
How is apomorphine given?
SC infusion with pump or pen injection
What is depression thought to be in PD?
2ndry to the disease
Why may someone with PD need a dietician?
Due to loss of calories due to constant trembling
What is duodopa?
Gel for continuous administration into the duodenum or upper jejunum via a pump and tube
Triggers of freezing of gait
Different terrain
Doorways
Busy / loud environment
When does freezing of gait occur in PD?
Advanced disease
What is common in freezing of gait?
On and off spells
What make freezing of gait better?
Cueing techniques
- visual
- rhythmic
A slow shuffling turn then improves with what?
Walking
Features of essential tremor
FH Head can be involved Action tremor May have had for many years Mobility issues/falls/constipation/speech/swallow should NOT be affected
What may help an essential tremor?
Alcohol
What is commonly used to treat an essential tremor?
Propanolol
What is drug induced parkinsonism difficult to differentiate from?
IPD
Can you still get drug induced parkinsonism if off the meds?
Yes, still may have symptoms for months after
DAT SPECT Scan result in drug induced parkinsonism
Negative
Can you fully recover after drug induced parkinsonism if stop meds?
Will not progress any further but will not fully recover
Top 3 drugs causing drug induced PD
Metoclopradmide (anti emetic)
Prochlorperazine (anti emetic)
Haloperidol (antipsychotic)
Causes of drug induced PD
Metoclopramide (anti emetic) Procholrperazine (anti emetic) Haloperidol Antipsychotics Lithium Amoidarone Valproate Cinnarizine
Features of vascular parkinsonism
Legs > arms Stepwise progression Sudden onset Gait affected EARLY Cognitive impairment EARLY
Levodopa response in vascular parkinsonism
Poor
2 predominant types of multiple system atrophy
- MSA-P = predominant Parkinson features
2. MSA-C = predominant cerebellar features
Features of multiple system atrophy
Parkinsonism Autonomic disturbance - erectile dysfunction (usually early feature) - postural hypotension - atonic bladder Cerebellar signs
What is the antiemetic choice in PD?
Domperidone
Features of PD tremor
Unilateral
Improves with voluntary movements
Parkinsonism with autonomic disturbance points towards what diagnosis?
Multiple system atrophy
What can parkinsons lead to and why?
Postural hypotension
Due to autonomic failure
What neurodegenerative condition is associated with MND?
Frontotemporal dementia
As well as hands and fingers, what can an essential tremor also affect?
Vocal cords
Presentation of progressive supranuclear palsy
Impairment of vertical gaze - down gaze worse than up gaze - may complain of difficulty reading or climbing stairs Parkinsonism Falls Slurring of speech Cognitive impairment
Which treatment of parkinsons has been linked to impulse control disorders?
Dopamine receptor agonists
Treatment of vomiting caused by radio / chemotherapy
Ondansteron
Treatment of vomiting caused by intracranial causes e.g. raised ICP, direct effect of a tumour
Haloperidol
Treatment of vomiting caused by vestibular causes
Prochloperazine
Treatment of vomiting caused by GI problems
Metoclopramide
What drugs can cause neuroleptic malignant syndrome?
Antipsychotics
If dopaminergic drugs are stopped or have their dose reduced
When does neuroleptic malignant syndrome start?
Within hours to days of starting an antipsychotic
Presentation of neuroleptic malignant syndrome
Pyrexia Muscle rigidity Autonomic lability - HTN - tachycardia - tachypnoea Agitated delirium with confusion
What is often raised in neuroleptic malignant syndrome?
Creatinine kinase
What may develop in neuroleptic malignant syndrome secondary to raised creatinine kinase?
AKI due to rhabdomyolysis
Treatment of neuroleptic malignant syndrome
Stop drug
IV fluids to prevent renal failure
Dantrolene
Bromocriptine (dopamine agonist)
What psychiatric condition is the most common in PD patients and what % of them have it?
40%
Describe ataxic gait
A wide based gait with loss of heel to toe walking
