Neurology Flashcards
MRI sequences for stroke/ demyelination/myositis
DWI/FLAIR/STIR
Abs for generalised myasthenia gravis
AChR (85%); MUSK (10%)
Abs for occular Myasthenia
50% AChR
Mgmt of thymoma/hyperplasia in MG
Resect
Characteristics of ACh-ve and MUSK +ve MG
Female, any age, oculobulbar form (not purely ocular); no thymic pathology and uncertain role of thymectomy
Thymectomy is recommended in whom in MG
in absence of thymoma when generalised MG and AChR Ab +ve aged <60. And those with thymoma
Initial therapy for MG
Pyridostigmine
MOA of pyridostigmine
Oral acetylcholinesterase inhibitor
Rx for severe /rapidly worsening/ crisis MG
steroids, PLEX, IVIG, steroid sparing- MMF, Aza.
Rx for refractory MG
Eculizumab, Rituximab
MuSK MG Mgmt is less responsive to…
AChesterase inhibitors and thymectomy
ADEM triggers
vaccination, infection
ADEM Ab
Anti-MOG
ADEM rx
Methylprednisolone
Severe form of ADEM
Acute Hemorrhagic Leukoencephalitis
Glatiramer MOA
4AA that mimics myelin basic protein, shifts TH1 -> Th2
Natalizumab MOA
alpha 4 integrin inhibitor; molecule that traffics lymphocytes across BBB
Ocrelizumab MOA
Anti-CD20
Alemtuzumab MOA
Anti-CD52 (found on lymphocyte cell surface)
Fingolimod MOA
SIP-1 receptor inhibitor; retains Th17 lymphocytes within LNs
Fingolimod side effects
Bradycardia, PML, lymphopaenia
Dimethyl Fumarate MOA
unknown
Teriflunomide MOA
Pyrimidine synthesis inhibitor
MOG Ab disease presentation in children vs. adults
kids- ADEM; adults- NMO
HMSN inheritance pattern
Any, depends on type
Characteristic findings of HMSN
pes cavus, stork leg
HMSN type 1 = CMT1 type/NCS finding/Biopsy
demyelinating/slowed conduction velocity/ onion bulbs
When 2 alleles are affected, get HMSN type 1; when 1 affected get…
Hereditary neuropathy with pressure palsy
HMSN type 2: type/ NCS
axonal/ reduced CMAP/SNAP
Main cancer that causes paraneoplastic syndrome
SCLC
Disease of Anti-Hu
Peripheral neuropathy w. limbic encephalitis- SCLC
Disease of Anti-Purkinje cell Ab
Cerebellar
Disease of anti-amphiphysin
Stiff man like syndrome
Disease of Anti-Ma2
Limbic Encephalitis (testicular)
Abs in LEMS are against…
Are against pre-synaptic voltage Calcium Channel channels
HLA of LEMs
HLAB8 DR3
Hallmarks of LEMs
Proximal weakness that improves with exercise, LL worse than UL, sensory neuropathy, arreflexia that improves with exercise, autonomic
NCS of LEMs
low CMAP that increases with exercise
LEMs mgmt
3-4 diaminopyridine: K channel blocker -> blocks efflux -> prolongs depol; IVIg, Prednisolone, rx of underlying Ca
Anti-NMDA features
oro-facial dyskinesias, seizures, drowsy, young female, psych
Anti-NMDA mgmt
high dose steroid, cyclophosphamide -> PLEX -> IVIg and rituximab. remove tumour
Huntington’s new drug
Tominersin- anti-sense oligonucleotide, intrathecal
SMA 2 specific new rxs
Nusinersin- anti-sense oligonucleotide, intrathecal. Zolgensma- gene replacement by adenoviral vector
CJD hallmarks
rapidly progressive dementia, cerebellar, myoclonus, visual disturbance
CJD MRI changes
Are in putamen and head of caudate
CSF finding in CJD
14-3-3 protein
EEG in CJD
periodic sharp wave complexes
MND is associated with what dementia?
FTD
MND most common familial gene
C9-ORF, esp. assoc. w. FTD-ALS
MND specific drugs
Riluzole- inhibits glutamate excitatory damage; Edaravone- inhibits oxidative stress
AD genes increasing risk
PSEN 1 and 2, APP have 100% penetrance. APO E4/E4 increases risk 15x, Late onset AD.
AD protective gene
APOE2
AD specific drugs x2 and MOA
Donepezil- Cholinesterase inhibitors, NOT for MCI; memantine- NMDA antagonist
Stiff person syndrome sx
trunk mainly- pain, increased tone and spasms
Stiff person aetiology
idiopathic, autoimmune
Abs in stiff person
Anti-GAD (glutamic acid decarboxylase, Anti-GAD/other Abs inhibit GABA receptors -> stiffness)
EMG of stiff person
continuous activity in trunk and limbs
Stiff person mgmt
Benzos, anti-spasmodics, immunosuppress if life threatening
Myotonic Dystrophy Type 1 pattern of weakness
facial, distal limb, and myotonia
Myotonic dystrophy Type 1 extra-muscle features
frontal balding, cataracts, DM, low IQ
McArdles Disease MOA
Glycogen storage disease V; myophosphorylase deficiency
McArdle’s classical presentation
exercise induced myalgia, stiffness, cramping, rhabdo and myoglobinuria
Duchenne’s muscular dystrophy inheritance
X linked
Duchenne’s muscular dystrophy weakness pattern
proximal and limb girdle
Duchenne’s gene affected
Dystrophin on X chromosome
Becker’s mutation
Dystrophin gene Xp21
Keppra MOA
inhibits SV2A
AED Na channel blockers
Phenytoin, CBZ, valproate, lamotrigine
AED for generalised
Valproate, Keppra
Best AED for focal seizures
CBZ, lamotrigine
Best AED for pregnancy
lamotrigine in pregnancy
AED causing weight gain
valproate
AED longest half life
Phenytoin
AED that induces own metabolism
CBZ
AED that causes hyperammonaemic encephalopathy
Valproate
Most significant two AED interaction
Lamotrigine and Valproate (are a good combo for refractory epilepsy, VA doubles serum Lam)
Sx of Lamotrigine toxicity
dizziness/ ataxia
Lamotrigine metabolism
via glucoronidation -> produces inactive metabolite that is renally excreted
OCP decreases concentration of which AED
Lamotrigine
Which AED should be avoided with Carbapenems?
Na Val (get 0-100% decrease in Na Val level)
AED for absence seizures only
Ethosuxamide; can use Lamotrigine for absence seizures
JME AED
Na Val/ Lamotrigine if child bearing demographic
What is a Jacksonian March?
focal seizure that progresses up limb
EEG findings of HSV encephalitis
temporal spike and wave discharged with diffuse b/g slowing
Treatment of seizures in neurocystericercosis
Rx seizures before rx the parasite
AED that worsens JME
CBZ
Alpha synucleinopathies
PD, MSA, LBD
Tau-opathies
PSP and CBD
PSP MRI sign
Hummingbird
In Encephalitis what is the biggest predictor of mortality?
conscious state
Top viral causes of encephalitis
HSV, VZV, enteroviruses
What is the most common form of HSV to cause encephalitis?
HSV1
Nerve injured in THR
sciatic
Gerstman’s symptoms
acalculia, agraphia, finger agnosia, L-R disorientation
Gerstmann’s affects what area
dominant parietal lobe
1st order neuron in cervical sympathetic chain is from
hypothalamus to C-spine (C8-T2)
Second order neuron in cervical sympathetic chain is from
T1 -> synapse at superior cervical ganglion at bifurcation of common carotid
Third order neuron in cervical sympathetic chain is from
superior cervical ganglion to eye
PSP defining sx
backwards falls, applause sign, supranuclear palsy/ophthalmoplegia w. slowed vertical saccades first
PSP differences from PD
Resting tremor RARE, symmetric
MSA defining features
pre-motor issues ++ behaviour/urinary dysfunction/REM sleep, Autonomic instability profound, then stridor/ parkinsonism/cerebellar features
MSA subtypes
Parkinsonism; cerebellar
CBD defining features
MARKEDLY asymmetric parkinsonism; can barely use affected limb- myoclonus, dystonia; prominent cognitive/behavioural problems