neuro 2 Flashcards
guillain barre
acute inflammatory polyneuropathy
motor difficulty, absence of deep tendon reflexes
parasthesias without objective sensory loss
inc CSF albumin + normal cell count.
2/3 have history of gastroenteritis or influenza like ting in the weeks leading up to it.
progressive symmetric weakness in lower before upper, pox before distal. parasthesias can preceed onset of weakness.
80% reach nadir by 2 weeks, 97% by 4 weeks.
Ix: nerve conduction studies
Lumber puncture
LFTs
Spirometry
Rx: 1st line- IvIG 400mg/kg/day 5/7
OR
plasma exchange - usually 2-5. 50ml/kg every other day.
trigeminal neuralgia
pain in one of trigeminal branches (of 3)
generally sudden intense bursts of pain– with no pain in between.
atypical is where there is some pain in between
may be infrequently, or 100s a day.
RF: woman, over 40 (60-50 peak), FH, HTN/ stroke, MS.
caused by vascular compression of trigeminal nerve (90%).
can occur regularly, then remiss for years, then come back.
Ix:
severe, unilateral, short, recurrent, triggered by; cold, light touch, eating, talking. — clinical diagnosis based on these.
may need to R/O tumour.
Rx: carbamazapine (usually 200mg t/Qds)
start at 100 and titrate up in 100mg steps.
If severe pain/ wt loss (unable to eat)/ depression ref to neurologist.
vestibular migrane
sudden onset vertigo +/- the classic migrane headache.
- common cause of spont vertigo.
as with migraine unsure of cause.
Rf: FH, Female, sleep dep, stress, caffiene.
S+S: dizzy, motion sensitivity, headache, N+V, sound sensitivity.
Ix: symptoms diary, role out other causes (meniers etc)- audiometry, imaging if needed.
Rx: lifestyle changes
Acute- sumatriptan 50-100mg
prophylaxis: propanolol- 80-160mg
topiramate 50-100mg
amitryptaline 25-75mg at night.
vestibular neuronitis
inflammation of the vestibular nerve- diffs to labrynthitis as thats the vestibular apparatus not the nerve.
causes: viral infection(herpes most com)
S+S: sudden onset severe vertigo, N+V, imbalance, - NO HEARING LOSS OR TINNITUS.
Ix/Dx: hx and physical, head inpulse test, vestibular function tests, imaging if persists
Rx: antiemetics/ histamines
steroids if severe
vestibular rehabilitation therapy.
antivirals are rare
Diff verses labrynthitis– no hearning loss in this, no tinnitus, no abx/ antivirals for this.
hypoxic-ischaemic encephalopathy
inflammation of the parenchyma due to hypoxia.
common in babies at birth- asphyxia, prolongued labour/ umbilical issues, placental abruption, congen heart defects.
adults- cardiac arrest, hypotension, near drowning.
S+S: babies- low apgar scores, weak cry, floppy, seziure.
adults: altered mental stat, seiz, paralysis etc etc.
Ix: physical, neuro ex, MRI, EEG, umbilical cord blood.
Rx: supportive care, reversal of anything reversable.
therapeutic hypothermia- 33.5 deg for 72 hrs.
rehab as needed.
idiopathic intercranial hypertension
increaced intercranial pressure without known cause.
median age of diag=30, obese,
S+S: headache- worst in morning, throbbin, blur vis, pulsatile tinnitus, shoulder pain.
pressure dependant- e.g cough sneeze aggs.
pappilodema
can cause traction on 6th (abducens)- double vis when looking to affectid side.
Ix/Dx: history and exam + MRI/CT (normal) MR venogram, LP with opening pressure.
Rx: aim is to prevent vis loss + blindness.
wt loss
LP
acetazolamide
venous sinus stenting- transvers sinus
shunt- either LP or VP usually.
discuss duchenne muscular dystrophy
mutation in DMD gene on X chromosone. – lack of dystrophin protein (muscle fibre stability)
X- linked inheritance- women can be carriers but as they have 2X chromosones, arent affected.
S+S: muscle weakness starting in early childhood (gowers sign, running issues), progressive loss of motility- wheelchair use by 12 usually. vent support by 20.
cardiomyopathy.
Ix: genetic testing –> DMD mutation
CK levels (usually elevated)
muscle biopsy, EMG to ax muscle activity.
Rx: similar for all muscle atrophies.
steds- pred- 0.75mg/kg.
physio
if heart concern- ACEi.
what is becker muscular dystrophy
mutation in same gene as DMD but less severe– later onset of symptoms (early adult hood)
rx similar but tailored to slower disease progression.
discuss spinal muscular dystrophy
autosomal recessive
SMN1 gene mutations– crucial for survival of motor neurons
multiple classifications- usually on age of onset (06,6-18,18-early adulthood, older adulthood)
Ix: examination, genetic testing, emg
Rx: some specific DMARDS
nusinersen
inasemnogene
risdiplam – all fairly funky and specialised.
discuss MND, phenotypes, what it is
degeneration of motor neurons in both the central and peripheral nervous system.
hereditary components to the disease.
S+S: mixed upper and lower motor signs.
4 main phenotypes, based on severity + location of origin
ALS
progressive bulbar palsy
progressive muscular atrophy
primary lateral sclerosis.
diagnosis of MND
hinges on clinical observation
aymmetric or symetric?
prox vs distal
upper vs lower
bulbar symptoms?
ALS- often limb onset disease. 1/3 bulbar involvement. usually upper motor signs initially then goes to lower (flaccidity) as disease progresses. – distal and asymmetric– finer hand mvts.
Progressive muscular atrophy– ‘the lower motor neurone’ subtype. 5% of cases. – better prognosis and slower progression. distal asymmetric again.
investigation of MND
physical examination
look for fasiculations, tounge wasting (B/L), hand muscle wasting, head drop
emotionallity
dysarthria, dysphagia
Labs: complete bloods, est, crp, tft, ck, mri brain + spine.
CSF can show biomarkers. - neurofilament light peptide, phosphorylated neurofilament heavy peptide.
Rx of mnd + prognosis
cramps - quinine 2nd- baclofen
exercise regemes
generally symptoms directed treatment i.e pain, cramps, dysphagia, spacicity,
prognosis: 3 years after diagnosis of als
myasthenia gravis
autoimmune condition affecting neuromuscular junction.
S+S: progressive worsening muscle strength (fatiguability) which improves with rest
blur vision, jaw fatigue, slur speech, ptosis.
link with thymomas
Ix: Achr antibodies (85% positive)
edrophonium test- (prevent breakdown of ach) +ve indicated MG
Rx: pyridostigmine- a cholinesterase inhibitor.
immunosuppression
thymectomy
rituximab.
acetylcholine receptor autoantibodies - blocking them.
signs of respiratory muscle weakness
diaphragmatic muscle weakness- breathless when in water
intercostal- breathless when upright.
therefore semi reclined is often most comfortable positon.
rapid shallow breathing, use of accessories, reduced chest expansion, abdo paradox, weak cough.
if acute- intubate
if chronic- NIV
