conditions Flashcards
pathophysiology behind Parkinson’s
loss of dopaminergic neurons in substantia nigra
if someone presents with Parkinson’s and autonomic dysfunction eg pissing themselves or postural hypotension what is diagnosis
multi system atrophy
is the tremor in Parkinsons symmetrical or asymmetrical
asymmetrical
how is rigidity distinguished from spascity
rigidity is velocity dependent
what test is used to identify bradykinesia in Parkinson
tap index finger and thumb together should have decreased amplitude
what scan helps to distinguish Parkinson’s from dystonia
DAT scan
what test should be done in a younger patient presenting with Parkinson’s symptoms
DAT, MRI , bloods as Wilsons disease can cause Parkinsonism
what is motor neurone disease
cluster of degenerative diseases assoc with selective loss of motor neurons in either motor cortex, CN nuclei and anterior horn of spinal cord
what kind of MND is most common
Amyotrophic lateral sclerosis ALS or Lou Gehrig
median survival after onset of MND
3 years
average age of diagnosis of MND
64 years
how is MND most likely to present
muscle weakness most commonly upper limb. may be bulbar onset
describe ALS and classic symptoms
mixed upper and lower motor neurone deficit weak grip and arm abduction stumbling gait foot drop drooling and dysarthria
what drug can be given in MND to prolong life
riluzole
what criteria system is used in MND
EL ESCORIAL
what additional medication is commonly given in MND
anticholinergic for drosling and baclofen for muscle spasms and cramps
what kind of dementia is MND associated with
frontotemporal dementia
what would distinguish MND from myasthenia graves
and doesn’t affect the eyes
what would distinguish MS from MND
no sensory or sphincter involvement
what is syndenhams chorea
chorea seen In children after infection (strep throat )
what is a stroke defined as
sudden disturbance of cerebral function of vascular origin that causes death or lasts 24 hours
whats more likely a haemorrhage or thrombosis embolic stroke
thrombosis embolic
what artery is most commonly affected in thrombotic cerebral infarction
MCA
where is atheroma most likely to originate from in emboli cerebral infarction
ICA or aortic arch
what is charcot Marie foot also known as
hereditary sensory motor neuropathy
high arched foot, champagne bottle deformity and lower limb distal muscle weakness
charcot marie tooth
early viral exposure to what may predispose to MS
EBV
when does MS present
30-40s
F:M ratio of MS
3:1
describe progressive relapsing form of MS
slowly gets worse and has flair ups. After flair up is worse than prior to it
clinical features of MS
optic neuritis , pyramidal dysfunction (weakness) , sensory symptoms, lower urinary tract dysfunction, cerebella r and brain stem features, cognitive impairment
symptoms of optic neuritis
painful eye movements and visual loss that resolves
how many episodes have to have occurred for diagnosis of MS
minimum 2 episodes
what is seen in CSF in MS and not in serum
IgG oligoclonal bands
what test may be used to diagnose MS
MRI, CSF, neurophysiology and bloods - PV, FBC CRp should all be normal
what is first line treatment for remitting relapsing MS
tefidera and interferons and Glitiramer Acetate
what is second line treatment for MS
fingolimed/cadrabine and monoclonal antibodies
what is seen macroscopically in the brain in MS
plaques of inflammation - well demaricated- ill defined if active
a GCS of less than what on initial assessment would indicate CT within an hour
<13
a CT within 1 hour of head injury would be indicated if how many episode of vomiting
> 1
what traumatic haematoma would be suspected if they present with a lucid interval
extradural haematoma
what neurotransmitter is released in excitotoxicity and subsequently what ion
glutamate and calcium
who can certify brainstem death
2 doctors one must be a consultant
what immune cells are activated in MS
t-cells
are gliosis seen in MND
yes
what protein does CAG code for
glutamine
below what age is Alzheimers classed as early onset
<65
what genes are implicated in Alzheimers
Amyloid precursor protein (APP)
presnilin 1 and 2
what dementia is seen in downs syndrome
alzheimers
what are neuritic plauques formed of in Alzheimers
A-beta amyloid core and astrocyte peripherally
what protein is dysregulated in Alzheimers
tau
what is seen microscopically in Alzheimers
neurofibrillary tangles and neuritic plaques
what does amyloid precursor protein cleave ?
a-beta (aka b-amyloid protein)
what chromosome is the APP gene found on
21
what apoliipoportine is associated with Alzheimers
e4
what area of the brain atrophies 1st in Alzheimers
hippocampus found in temporal lobe
what is a Lewy body
intracytoplsmic eosinophilic inclusion with dense core surrounded by alpha-synuclei fibrils
describe features of Lewy body dementia
progressive dementia along with hallucinations and fluctuating levels of attention and cognition
what neurotransmitter is release at NMJ
ACh
name 2 pre-synaptic disorders of the NMJ
botulism and Lambert eaton myasthenia syndrome
describe pathophysiology of myasthenia gravis
autoimmune antibodies against post synaptic Each receptor
conditions of what other organ are seen in myasthenia graves
thymus abnormalities
describe Lambert eaton
antibodies to pe-synpatic calcium channel
what carcinoma is associated with Lambert eaton syndrome
small cell carcinoma
treatment of Lambert eaton
3,4, diaminopyridine
treatment of myasthenia graves
pyridostigmine
IV Ig
thymectomy
diagnosis of myasthenia graves
nerve conduction with repetitive stimulation
what drug should be avoided in myasthenia graves
gentamicin
marker of polymyositis and treatment
increased CK and steroids
what drugs can cause myopathies
statins, amiodarone, diuretics and steroids
what spills from muscle into plasma in rhabdomyolysis
potassium , PO4, myoglobin
describe transiten global amnesia
abrupt onset anterograde >retrograde amnesia
repetition
transit 4-6 hours
generally a one off
describe sporadic CJD
rapid onset dementia , neurological signs and myoclonus
prognosis for sporadic CJD
4 months
what atypical kind of Alzheimers did Terry prachett have
posterior cortical atrophy
symptoms of posterior cortical atrophy
visuospatial disturbances, visual agnosia
what type of Alzheimers results in loss of language first
primary progressive aphasia