Y4 Degenerative Diseases Flashcards
dementia - what two structures differences is there found on biopsy?
neurofibrillary tangles and β amyloid plaques
what are three risk factors of dementia?
ApoE4 allele, presenillin 1/2 mutations, Down’s
what score does the MMSE have to be to prescribe cholinesterase inhibitors in alkheimers dementia ?
10-20
what is the usual presenting complaint of lewy body dementia ?
Fluctuating cognitive dysfunction, visual
hallucinations, parkinsonism
what is found on biopsy of frontotemporal dementia?
Pick Bodies
what affect does picks bodies have on the brain?
cause cells to die
what are reversible causes on dementia ?
infection Vascular Chronic subdural haematoma Inflammation SLE Sarcoid Neoplasia Nutritional Thiamine deficiency B12 and folate deficiency Pellagra (B3 / niacin deficiency) Hypothyroid Hypoadrenalism Hypercalcaemia Hydrocephalus (normal pressure)
state 4 parkinsonism conditions ?
Multiple Systems Atrophy
Progressive Supra nuclear Palsy
CorticobasilarDegeneration
Lewy body dementia
what destruction causes parkinsons ?
Destruction of dopaminergic neurones in pars compacta
of substantia nigra.
what would be found on biopsy of parkinsons causing the pathophysiology?
β-amyloid plaques
Neurofibrillary tangles: hyperphosphorlated tau
state some reversible causes of parkinsons?
Infection Syphilis, HIV, CJD Vascular: Multiple infarcts in SN Drugs: Antipsychotics, metoclopramide Trauma: dementia pugilistica Genetic: Wilson’s disease
what are the four cardinal signs of parkinsons ?
rigidity
gait
bradykinesia
tremor
state the characterstics of parkinsons tremor ?
Worse at rest
Exacerbated by distraction
4-6hz, pill-rolling
state the characteristics of the bradykinesia of parksinons?
Slow initiation of movement ̄c reduction of amplitude on repetition
Expressionless face
Monotonous voice
Micrografia
what are the signs of multiple systems atrophy?
Autonomic dysfunction: post hypotension, bladder dysfunction
Cerebellar + pyramidal signs
Rigidity > Tremor
what are the signs of progressive supra nuclear palsy?
Postural instability → falls
Speech disturbance (+ dementia)
Palsy: vertical gaze
what are two signs of lewy body dementia ?
Fluctuating cognition
Visual hallucinations
what are the signs of Corticobasilar Degeneration?
Aphasia, dysarthria, apraxia
Akinetic rigidity in one limb
Astereognosis (cortical sensory loss)
Alien limb phenomenon
what is Astereognosis?
inability to identify an object by active touch of the hands without other sensory input
what is dementia pugilistica?
A variant of chronic traumatic encephalopathy (CTE), which is itself a serious type of brain damage resulting from repeated concussions and is found in many professional athletes and military personnel who have been subjected to multiple impacts to the head.
state some features of parkinsons ?
Tremor Rigidity Akinesia Postural instability: stooped gait ̄c festination Postural hypotension Sleep disorders: insomnia, EDS, OSA, RBD Psychosis: esp. visual hallucinations Depression / Dementia
sleep disorders what does EDS, OSA and RBD stand for?
EDS - excessive daytime sleepiness
OSA - obstructive sleep apnoea
RBD - rapid eye movement behaviour disorder
what could cause Early morning dystonia in parkinsons?
medication is wearing off
state some side effects of LDOPA? DOPAMINE
Dyskinesia On-Off phenomena = Motor fluctuations Psychosis ABP↓ Mouth dryness Insomnia N/V EDS
what is the on off effect in parkinsons ?
unpredictable fluctuations in motor
performance unrelated to timing of dose.
state some autonomic dysfunction with parkinsons ?
Postural hypotension Constipation Hypersalivation → dribbling (↓ ability to swallow saliva) Urgency, frequency, Nocturia ED Hyperhidrosis
what scale is used to measure disability in parkinsons ?
UPDRS: Unified Parkinson’s Disease Rating Scale
name a MOAB inhibitor?
rasagiline, selegiline
name a LDOPA?
co-careldopa or co-beneldopa
name a dopamine agonist ?
ropinirole, pramipexole
after how long do people loss response to LDOPA?
2-5years
what gene is associated with MS?
HLA-DRB1
what are the four common symptoms someone with MS presents with?
Tingling (paraesthesia)
eyes (optic neuritis)
ataxia (loss of control of voluntary movements)
motor (spastic paraparesis)
what is Lhermitte’s Sign?
when someone flexes their neck and gets shooting pain down arms/legs/trunk
what is Uhthoff’s sign in optic neuritis ?
when vision is worse with heat (hot meal, exercise)
what is INO?
Disruption of MLF connecting CN6 to CN3
Weak adduction of ipsilateral eye
Nystagmus of contralateral eye
when is INO seen?
lesion between CN6 and CN3 for example during MS when there is demyelination
what type of MRI is used for MS?
T2
what is found on LP for MS?
IgG oligoclonal bands (not present in serum)
what antibodies can be found with MS?
anti MBP abs
NMO IgG abs
what disease is NMO IgE abs specific for ?
Devics disease
what criteria is used for T2 MS MRIs?
macdonald criteria
what is another name for Devics?
neuromyelitis optica
what are two features of neuromyelitis optica?
transverse myelitis
optic atrophy
what is the treatment for an acute attack of MS?
steroids
what medication is available for treatment of MS?
steroids
DMARDS
Biologics
what medication is used to prevent relapses of MS?
DMARDS
- IFN-β:
- Glatiramer:
Biologics
Natalizumab: anti-VLA-4 Ab
Alemtuzumab: anti-CD52
what symptoms can be treated during MS?
fatigue bladder incontinence depression pain tremor erectile dysfunction
on examination what can be found for optic neuritis ?
↓ acuity, ↓ colour vision, white disc, central scotoma,
RAPD (relative afferent pupil defect)
