neuro (264) Flashcards
Ax Duchenne muscular dystrophy?
S/s
X-linked recessive - mutation in dystrophin gene results in dystrophin deficiency
s/s
* delay in motor development
* onset of weakness in 3-4 years
(Gower’s sign, toe walking, exaggerated lumbar lardosis, calf hypertrophy)
Dx DMD?
raised CK
genetic testing (dystrophin gene)
EMG
muscle biopsy
is DMD inherited?
2/3rds inherited from asymptomatic mother 1/3rd sporadic
huntington’s disease onset?
S/s?
Ax?
30-50 years
S/s
* dementia
* chorea
Ax = autosomal dominant CAG42
appearance of Huntington brain
flattening of normal convex curve of lateral ventricles - due to loss of ganglia from caudate nucleus
Spinal muscular atrophy Ax?
S/s?
Tx?
autosomal recessive - loss of anterior horn cells
* floppy/weak arms and legs
* breathing difficulties
Tx = synthetic oligonucleotide
Alzheimers brain appearance genes?
neurofibrillary tangles + amyloid plaques
AD (presenilin) in 5% of cases - usually multifactorial
ApoE4?
carriers more likely to get Alzhiemer’s in old age
classification meningitis
acute pyogenic (bacterial)
acute aseptic e.g. viral
chronic bacterial - TB
acute encephalitis
pyogenic meningitis dx?
suppurative exudate
neutrophils in subarachnoid space
Ax bacterial meningitis?
strep pneumo (gram +ve cocci)
neisseria meningitidis (gram -ve diplococci)
listeria (gram +ve bacilli) - seen in alcoholics, immunosuppressed, elderly or neonates
Tx bacterial meningitis
ceftriaxone! + dexamethasone
*if penicillin allergic = chloramphenicol
if at risk for listeria (alcohol, old, immunosuppressed) add amoxicillin
(ceftrizone does not cover listeria)
viral meningitis Ax?
Dx?
Tx?
enteroviruses e.g. ECHO virus
Dx = viral stool culture, throat swab, CSF PCR
Tx = supportive, self-limiting
encephalitis vs meningitis
s/s?
encephalitis = brain parenchyma
meningitis = meninges
encephalitis = confusion, behaviour disturbances, psychosis, altered consciousness, seizures
meningitis = fever, photophobia, headache, neck stiffness, ?rash
meningo-encephalitis = combo
Ax encephalitis
Ix
Tx?
Ax = herpes simplex
Ix = LP, EEG (abnormalities), MRI (temporal lobe)
Tx = IV aciclovir
Ax brain asbcess?
Tx?
strep + bacteroides
Tx = ceftriaxone
* if penicillin allergic = vancomycin
most likely cause of meningitis in
1 - neonates
2 - children
3 - ages 10-64
4 - over 65
5 - decreased immunity
6 - fracture of cribiform plate
7 - head trauma
neonates = listeria
children = haemophilus
10-64 = strep penumo
>65 = strep pneumo + listeria
decreased immunity = listeria
fracture of cribiform plate = strep pneumo
head trauma = staph
complications meningitis?
exudate around CN III + VI
brain abscess
cerebral oedema
communicting hydrocephalus
sources of infection meningitis
nasopharyngeal colonisation (sinusitis, otitis media)
remote foci of infection - endocarditis, pneumonia, UTI
when should lumbar puncture be avoided?
increased ICP
GCS <12
bacterial vs viral CSF
PCR meningitis?
used for viruses
are you more likely to see neisseria or haemophilus as cause of meningitis in children?
neisseria meningitidis because Hib vaccine?
most common cause of meningitis?
strep pneumo
tuberculous meningitis Tx?
isoniazid + rifampacin key
(pyrazinamide + ethamutol too)
Ax aseptic meningitis
Viral
malignancy
sarcoidosis
vasculitis
dural venous sinus thrombosis
drugs - co-trimox, NSAIDs
Tx listeria?
if penicillin allergic?
amoxicillin
if penicillin allergic = co-trimoxazole
contact prophylaxis meningitis?
500mg ciprofloxacin
vaccines meningitis?
neisseria - ACYW
Haemophilus - Hib
strep pneumo
glial cells CNS?
astrocytes - BBB
oligodendrocytes - myelin
epdenymal cells - ventricles
microglia - macrophages
red neuron?
hallmark of acute neuronal injury due to hypoxia e.g. stroke
example of inclusions?
neurofibrillary tangles in Alzheimer’s
oligodendrocite damage is a feature of?
demyelinating disorders
Gliosis?
astrocytes - scarring
most important indicator of CNS injury
types of microglia
M1 = acute
M2 = chronic
why does hypxoxia afect brain so much?
needs 20% total body oxygen
can only increse bloodflow 2-fold in response to hypoxia
excitotoxcity?
makes ischaemia worse
when can autoregulary brain mechanisms not compensate for BP?
when MAP falls below 50 mmHg
where in the brain are thromboembolic events most common?
middle cerebral artery
which areas of the brain are particularly sensitive to ischaemia?
watershed areas - zone between 2 arterial territories i.e. parieto-occipital
stroke?
sudden disturbance of cerebral function of vascular origin that causes death or lasts over 24 hours
cerebral infarction Ax
thrombotic
* atherosclerosis - most common MCA!!
embolic
* from atherom ain internal carotid artery/aortic arch
rarer
* osteophytes compressing vertebral arteries
* vasculitis
* septal defects - right to left shunts
risk factors stroke
atheroma
hypertension
cholesterol
obesity
diabetes
drugs
smoking
septal defects
stroke after 12 hours
48 hours
a week
few weeks
12 hours = red neuron
48 hours = lots of micoglia
a week = gliosis begins
few weeks = gliotic scar
symptoms carotid artery infarct?
contra-lateral weakness or sensory loss
sometimes aphasia/apraxia
sudden severe headache
sudden vision loss
symptoms middle cerebral artery infarct?
weakness of contralateral face and arm
anterior cerebral artery infarct?
weakness and sensory loss in contralateral leg
vertebro-basilar artery infarct?
vertigo
ataxia
dysarthria
dysphasia
complex brain stem syndromes
lacunar infarcts Ax?
Affect?
multiple lacunar infarcts?
atherosclerosis/thrombus or distal embolism
affect basal ganglia
multiple = multi-infarct dementia
hypertensive encephalopathy symptoms?
signs?
symptoms
* severely hypertensive
* symptoms of raised ICP - headache, vomiting, altered balance, fits
signs
* global cerebral oedema
* tentorial and tonsillar herniation
* petechiae
types of intracrnaial haemorrhage
extradural haematoma = does not cross suture line
subdural haematoma = crosses suture line
subarachnoid hemorrhage
intracerebral haemorrhage
Ax intracerebral haemorrhage
causes of vascular injury - hypertension, amyloid angiopathy, diabetes, drugs (cocaine, alcohol), vasculitis
aneurysms
vascular malformations
coagulation disorders/iatrogenic anticoagulation
neoplasms
amyloid angiopathy Ax?
complication?
most commobly due to Alzheimers
complication = intracereberal haemorrhage (amyloid deposited in vessels causes rupture)
examples of vascular malformations in the brain
complications?
which are most likely to cause these complications?
arteriovenous malformations
cavernous angiomas
venous angiomas
capillary telangectases
can cause intracerebral haemorrhage, headaches, seizures + focal neurological deficits
arteriovenous and cavernous angiomas are most likely to cause intracrnaial haemorrhage
most common vascular abnormality?
where are they found?
arteriovenous malformation
MCA
subarachnoid haemorrhage Ax?
areas effected?
associated with?
most common cause is rupture of berry aneurysm
90% in territory of carotid artery
associated with ADPKD
subarachnoid haemorrhage risk factors
S/s?
common complication?
smoking, hypertension, drugs
s/s
* severe thunderclap headache
* vomiting
* loss of consciousness
complication = hydrocephalus
what seperates the 2 hemispheres?
what covers cerebellum
falx cerebri seperates 2 hemispheres
tentorium cerebelli covers cerebellum
normal CSF cytology
clear fluid
tiny amounts of lymphocytes
no neutrophils
no RBCs
no protein
glucose >2.2
will see neutrophils in bacterial meningitis
lymphocytes in viral meningitis
RBCs in SAH
hydrocephalus Ax
obstruction to CSF - inflammation, pus, tumours
decreased reabsorption of CSF - SAH, meningitis
overproduction of CSF - tumours of choroid plexus (very rare)
hydrocephalus classification
non-communicating = obstruction to flow of CSF within ventricular system
communicating = obstruction to flow of CSF outside ventricular system e.g. subarachnoid space or arachnoid granulations
timing of hydrocephalus
hydrocephalus ex vacuo
dilation of ventricular system + increase in CSF volume secondary to loss of brain parenchyma - Alzheimer’s disease
Ax raised ICP
increased CSF (hydrocephalus)
SOL
oedema
increased venous volume
physiological (hypoxia, hypercapnia, pain)
consequences raised ICP
intracranial shifts + herniations (e.g coning)
midline shift
pressure on cranial nerves
impaired cerebral blood flow
examples of herniations
tonsillar through foramen magnum
signs of raised ICP
papilloedema
headache
nausea + vomiting
neck stiffness
reduced consciousness
examples of SOLs
tumours
abscesses
haematomas
localised brain swelling i.e. oedema around infarct
symptoms + signs brain tumour
symptoms = heaahce (worse in morning), vomiting, seizures, visual disturbances
signs = focal deficit, papilloedema
brain tumours children?
adults?
children = below tentorium cerebelli
adults = above tenorium cerebelli
brain metastases
from?
far more common than primary brain tumours
* breast
* bronchus
* kidney
* thyroid
* colon
* melanoma
commonest primary brain tumour in adults?
children?
adults = astrocytoma
children = medulloblastoma
astrocytoma grading
grade 1 = pilocytic
* childhood, benign
grade 2 = well differntiated, some nuclear atypia but overall low grade appearance
grade 3 = more atypia
grade 4 = glioblastoma
* extreme atypia, necrosis
* 10-12 month survival
medulloblastoma?
found?
complication
Tx
most common tumour in children followed by pilocytic astrocytoma
occur in midline of cerebellum i.e. below tentorium cerebelli
complication = hydrocephalus
Tx = surgery + radiotherapy
brain abscess Ax
single abscesses = mastoiditis, chronic otitis media, sinusitis, dental infections, skull fracture (tend to occur adjacent to source)
multiple abscesses = haematogenous spread from bronchopneumonia, bacterial endocarditis, bronchiectasis, lung abscesses, congenital heart disease, IV drug use (tend to coccur at grey and white matter boundary)
appearance brain abscesses
S/s?
Dx?
Tx?
central necrosis, oedema, fibrous capsule
S/s = fever, raised ICP
Dx = CT, MRI (ring enhancing)
Tx = aspiration + antibiotics
brain abscess organisms?
strep, bacteriodes, staph
immunocompromised patients = fungi, toxoplasma in AIDS
why do contra-coup injuries tend to be worse despite being non-impact side?
denser CSF moves to coup side first
most common intracranial haematoma?
epidural
“burst lobe”
subdural + intracerebral haematoma
occurs mostly in frontal + temporal lobe
Ax epidural haematoma?
fracture of tempero-parietal region = midle meningeal artery
subdural haemorrhage Ax
disruption of bridging veins
(blood accumulates between dura and arachnoid mater)
acute vs chronic subdural haemorrhage
acute = elderly, cleat history of trauma
chronic = commin in injury but often no clear history of trauma
B - juvenile myoclonic epilepsy is generalised epilepsy so not associated with hippocampal sclerosis (focal)
important features in epilepsy history
onset - what were they doing? what did they look like? aura?
type of movements - rigor? clonus? tonic phase?
speed of recovery
ask about birth + development, family history, previous seizures (e.g. febrile fits), drugs, alcohol
social history - driving, occupation
what drug can make epilepsy worse
tramadol
Ix for epilepsy?
MUST DO ECG - prolonged Qt syndrome (most common presentation is episode of collapse)
CT to screen for injury
EEG
differential diagnoses epilepsy?
syncope
pseudoseizures, psychogenic
panic attacks
TIA
migraine
hypoglycaemia
parasomnia
paroxysmal movement disorders
cataplexy
tonic spasms of MS
driving rules epilepsy
1st seizure = 6 months (car), 5 years (HGV)
epilepsy = 1 year or 3 years during sleep (car), 10 years off medication (HGV)
types of epilepsy?
further classification?
focal + generalised (cortical network)
focal
* simple = without impaired consciousness
* complex = with impaired consciousness
generalised
* absence - petit mal
* myoclonic
* atonic
* tonic
* tonic clonic - grand mal
focal seizure locations + symptoms
Motor (frontal lobe) = rhythmic jerking, posturing, head and eye deviation, other movements (e.g. cycling), automatisms (e.g. plucking), vocalisation
(most common = movements of hands and face)
Sensory (parietal lobe) = omatosensory, olfactory, gustatory, visual, auditory
Psychic = memories, deja vu, jamais vu, depersonalisation, aphasia, complex visual hallucinations
generalised epilepsy onset?
tx?
e.g.?
onset = childhood + teens
Tx = sodium valproate
e.g. juvenile myoclonic epilepsy
* early monring jerks
* generalised seizures
* risk factors - sleep deprivation, flashing lights
focal epilepsy onset?
Ax?
Tx?
any age
Ax = underlying structural cause e.g. hippocampal sclerosis
Tx = carbamazepine
how to AEDs work?
activate GABA system
side effects sodium valproate?
weight gain, hair loss, fatigue
teratogenic!!
what drug is avoided in primary generalised epilepsy?
carbamazepine - can make way worse!
which AEDs induce hepatic enzymes?
carbamazepine, phenobarbital + phenytoin
(so avoid in POP and COCP)
status epilepticus?
Ax?
medical emergency - seizure activity >30 mins
Ax = hyponatraemia, pyridoxine (B6) deficiency, infection, head trauma, SAH, abrupt withdrawal of anticonvulsants, treating absence seizures with carbamazepine
convulsive status?
Complications?
Tx?
most dangerous form of status epilepticus - generalised tonic clonic fits without cessation
comps = hypoxia, hypotension, hyperthermia, rhabdomyolysis
Tx = ABCDE
* identify cause - infection, SAH, etc - so FBC, CT
* phenytoin + benzodiazepines!!
Tx epileptic seziure
IV lorazepam (1st line)
once stabilised = restablish normal AED Tx
if seizures don’t stop = IV phenytoin/phenobarbital
if still don’t stop = transfer to ICU for general anaesthesia
patient presenting with acute confusional state?
always suspect partial status epilepticus in addition to septic encephalitis (as they are difficult to distinguish)
functional attacks?
Ax?
Dx?
not epilepsy but patient has no control of attacks
related to trauamatic events, physical/sexual abuse, other stress, anxiety/depression
Dx = EEG + history
types of aura
Somatosensory auras = numbness, tingling, electric shocks, thermal sensations, pain
Visual auras = simple shapes, static, flashing, moving lights, colours
Auditory auras
Vertiginous auras = temporal-parietal lobe
Autonomic auras = temporal lobe (insula, amygdala, etc)
Tx functional attacks?
removal of epiletpic drugs
CBT
Tx of anxiety or depression
motor neurone disease?
s/s?
Untreatable and rapidly progressive neurodegenerative condition
s/s = muscle weakness, problems with speech, swallow problems, breathing problems
upper/lower motor neuron signs WITHOUT sensory problems
most common subtype MND?
ALS
prognosis MND?
average survival time = 3 years
Ax MND?
Types?
features of these types?
90% sporadic, 10% familial
types = ALS, PLS, progressive musclar atrophy, progressive bulbar palsy
ALS with fronto-temporal dementia associated with?
c9orf72 gene
upper vs lower motor signs in MND?
ALS symptoms?
PLS symptoms?
PBP symptoms?
ALS = mixed UMN and LMN signs
PLS = pure UMN
PBP (LMN) = wasting of the tongue, dysphagia, difficulty speaking (slurring)
progressive bulbar palsy will eventually become?
ALS
split hand syndrome?
ALS
C9orf72 mutation?
ALS with FTD
which subtype of MND has best prognosis?
primary lateral sclerosis
Ix MND?
EMG
neurological exam
Tx MND?
riluzole - gives an extra 3 months
communication needs
nutritional needs (dietician, gastrostomy) - metabolic rate in MND is doubled so weight loss expected
respiratory needs
sialorrhoea tx MND?
muscle cramps?
muscle spasms?
sialorrhoea = hyoscine/buscopan, glycopyronium (esp in cognitive impairment), botox
muscle cramps = quinine, baclofen
muscle spasms = baclofen, gabapentin
SOB MND?
lorezepam
S/s MND?
UMN/LMN signs without sensory issues
emotional lability i.e. inappropriate crying/laughing (early UMN sign)
cognitive impairment (50%) - ALS associated with FTD
* apathy, disinhibition, poor planning/decision making
hypokinetic vs hyperkinetic movement disorder
hypokinetic (too little movement) = parkinsons
hyperkinetic (too much movement) = tremor, tics, chorea, myoclonus, dystonia, athetosis
important points in history for movement disorders?
birth history - anoxia, perinatal problems, milestones
family history
drug and toxin history - neuroleptics, antiepileptics, illicit substances, toxins
s/s parkinsonism/akinetic rigid syndrome?
TRAP
tremor
rigidity
akinesia/bradykineasia
postural disturbances (flexed posture, postural instability)
how to distinguish rigidity from spasticity?
rigidity = no increase with higher mobilising speed
spacticity = yes!
positive froment’s maneuver?
parkinson’s (rigidity increases in examine dbody segment by movement of other body parts)
not to be confused with froment’s test for ulnar nerve palsy (adductor pollicis)
camptocornia?
extreme anterior truncal flexion sometimes seen in Parkinson’s
parkinsonian gait?
slow shuffling steps
decreased arm swing
slow turning with multiple small steps
festination
postural instability (pull test)
types of tremor?
resting - occurs when body part at rest
postural - occurs when arms outstretched
kinetic - occurs during movement of body part
dystonia?
Ax?
sustained intermittent muscle contractions
chorea?
Ax?
irregular purposeless movement
can be difficult to distinguish chorea from myoclonus (chorea shifts from one body part to another)
Ax = drugs (COCP), basal ganglia lesions, Sydenham’s chorea (rheumatic fever), APS, Huntington’s, neuroacanthyocytosis
Myoclonus
electric shock like jerks
caused by brief activation of a group of muscles leading to jerk of affected body part
ballism?
suggestive of?
severe type of chorea
involves proximal joints resulting in large movements
strongly suggestive of contralateral lesion involving striatum of subthalamic nucleus
essential tremor?
seen when?
what makes better?
most common type of postural tremor (not associated with Parkinson’s = resting tremor)
seen in hands (5Hz) while holding outstretched
often made better with alcohol intake
pathological hallmarks of parkinson’s?
lewy bodies
loss of dopaminergic neurons from substantia nigra
non-motor symptoms Parkinson’s?
hypomimia
sleep disorders
hallucinations
GI dysfunction (constipation)
bladder problems
depression
congitive imapirment/dementia
anosmia
3 subtypes parkinsons
tremor-dominant (slower rate of progression)
non-tremor dominant (akinetic-rigid syndrome + postural instability gait disorder)
mixed
leadpipe vs cogwheel
Lead pipe = constant resistance throughout passive movement
Cogwheel = jerky resistance throughout movement as muscles contract and relax
Dx Parkinsons
DAT scan (SPECT)
does parkinsons reduce life expectancy?
barely - by 1 or 2 years
greatest risk factor parkinsons?
what decreases risk?
advancing age
smoking and alcohol decreases risk
Tx parkinsons?
levodopa
dopamine agonists
monamine oxidase B inhibitors
amantadine
anticholinergics for tremor
side effects parkinsons Tx?
dopamine agonists
* nausea
* daytime sleepiness
* oedema
* impulse control - gambling, hypersexuality, binge eating, compulsive spending
* hallucinations
levodopa
* greatest symptoamtic benefit but associated with dyskinesia (involuntary movments) and fluctuations in motor symptoms
red flag symptoms PD?
i.e. suggests not Parkinson’s
absence of asymmetry
severe axial or lower limb involvement
frequent falls
eye movement disorder
other unexpected movement disordrs (tics, myoclonus, chorea)
bulbar features
apraxia
severe cognitive deterioration or psychosis
marked autonomic dysfunction
negative levodop challenge
differential diagnoses Parkinson’s?
vascular Parkinsons
drug induced Parkinsons
tremor disorders
multi system atrophy
progressive supranuclear palsy
fragile X tremor ataxia syndrome
vascular parkinsonism features?
Dx?
affects predominantly lower limbs
rest tremor uncommon
poor levodopa response
Dx = brain imaging
drug induced parkinsonism?
symmetrical
coarse postural tremor
presence of other drug-induced disorders - orolingual dyskinesias, tardive dystonia, akathasia
usually narcoleptic/antipsychotic drugs
essential tremor vs parkinsons?
symmetrical, higher frequency (12Hz)
infrequently observed at rest
alcohol responsiveness
head tremor (mild)
multi system atrophy vs parkinsons?
Dx?
core triad = dysautonomia, cerebellar features, parkinsonism
60-70 y/o
jerky postural tremor, pyramidal signs (hyperreflexia and extensor plantar responses)
suboptimal levodopa response
other suggestive features = severe dysarthria/dysphonia, marked antecollis, inspiratory sighing, orofacial dystonia
Dx = MRI “hot cross bun” sign
progressive supraneuclear palsy vs parkinsons
symmetric akinetic-rigid syndrome
predominantly axial involvement
falls
tremor not usually seen
vertical gaze supranuclaer palsy
pseudobulbar symptoms
retrocollis
eyes wide open (staring)
no response to levodopa