Neurology Flashcards
Parkinson’s disease RFs
- REM sleep disorder
- sleep apnoea
- older males
- CVD risk factors
Vertebral artery dissection symptoms
- neck pain / headache
- ataxia, dysarthria, vertigo, diplopia
- lateral medullary syndrome: ataxia, nystagmus, ipsilateral horner’s & loss of facial sensation; contralateral limb sensory loss
- cerebellar infarction
Botulism features
- patient usually fully conscious with no sensory disturbance
- flaccid paralysis (descending)
- diplopia
- ataxia
- bulbar palsy
- may have autonomic features e.g. dry mouth, dilated, slow reacting pupils
- may have false positive tensilon test
- NMJ problem therefore no conduction block (vs GBS)
Sporadic CJD clinical features
neuropsychiatric: dementia, anxiety, fluctuating behavioural changes, frontal lobe syndromes, apraxia, aphasia (rapid onset)
myoclonus
cerebellar: ataxia, incoordination
corticospinal: spasticity, hyperreflexia, babinski +ve
extrapyramidal features
NB: cranial nerve abnormalities and peripheral nerve involvement is rare
Sporadic CJD investigations
CSF is usually normal. CSF 14‐3‐3, S100 and tau may be positive. RT-QuIC
EEG: biphasic, high amplitude sharp waves (only in sporadic CJD). periodic sharp wave complexes
MRI: hyperintense T2 signals in the cortex, basal ganglia and thalamus. Cortical ribboning is a classic sign. DWI changes are most the most sensitive
Paroxysmal hemicrania
- unilateral
- retro-orbital / temporal
- autonomic: eye watering, red eye, runny nose, miosis
- specific: multiple short lasting atacks
- responds well to indomethacin
Anterior spinal artery syndrome
Features
- anterior 2/3 spinal cord stops working
- dorsal columns preserved - vibration / proprioception
- flaccid paresis, arreflexia
- autonomic features e.g. hypotension, bladder/bowel dysfunction
- cervical cord may -> ventilatory failure
Causes: aortic aneurysm / dissection (into anterior spinal), trauma, atherosclerosis, embolic stroke, disc herniation / cancer occluding anterior spinal artery
Anterior inferior cerebellar artery stroke
- lateral pontine infarct
- vertigo, nystagmus
- ipsilateral tinnitus
- ipsilateral facial LMN
- ipsilateral conjugate lateral gaze palsy
- cerebellar ataxia
- ipsilateral horner’s
- contralateral loss of pain / temp sensation
Posterior cerebral artery stroke
- distal occlusions can be symptomatic and variable
- agnosia
- prosopagnosia
- cortical blindness
Anterior cerebral artery stroke weakness pattern
- lower limb > upper limb
Posterior inferior cerebellar artery stroke
- ipsilateral loss temp / pain
- +/- ipsilateral horners
- +/- ipsilateral CN palsy
- ipsilateral ataxia
- nystagmus
- contralateral loss temp / pain
Weber syndrome
- ventrolateral midbrain infarction
- paramedian branches basilar / posterior cerebral artery
- ipsilateral CNIII palsy
- contralateral weakness
Carotid artery dissection
- pressure symptoms: CNIII and CNXII palsies, partial horners (miosis)
- thromboembolic symptoms: anterior circulation strokes
Friedreich’s ataxia aetiology
AR trinucleotide repeat disorder (GAA)
peak incidence 8-14 yrs
chromosome 9 defect
Friedreich’s ataxia features
- progressive condition
- ataxia
- diminished reflexes
- loss of vibration sense / proprioception
- spastic paraparesis with extensor plantars
- dysarthric speech
- optic atrophy
- kyphoscoliosis
- DM 10-20%
- CV abnormalities e.g. HOCM / LVH
- high arched palate, pes cavus
- NCS: low amplitude sensory, normal motor
MS relapses mx
- PO methylpred 500mg OD 5/7
- 2 relapses in less than 2 years? referral for DMARDs
1) beta-interferon
2) fingolimod
3) natalizumab (beware PML, ITP)
Muscular dystrophies general info
- X linked recessive
- Progressive muscular weakness
- Cardiomyopathies may occur
- Duchenne: cognitive impairment as well, cardiomyopathy more common
- Becker: presents later, arrhythmias occur
Myotonic dystrophy genetics
- AD trinucleotide repeat
- generational anticipation
Myotonic dystrophy features
- myotonic facies
- frontal balding
- b/l ptosis
- cataracts
- dysarthria
- slow relaxing reflexes (myotonia)
- limb weakness (usually distal initially)
- mild LD
- testicular atrophy
- heart block (primary)
- dysphagia
- IX: EMG (dive bomber), genetic testing
Post seizure time no driving
6 months
PML ix
CD4 <100 usually in HIV
Other immunosuppression
Widespread patchy demyelination
CNS lymphoma sx and ix
Fevers
Progressive GCS deterioration
LP: elevated lymphocyte count
PFO stroke mx
- PFO closure
- antiplatelet
- increased risk AF - anticoagulate if develops
Trigeminal neuralgia red flags which require MRI / neurologist review
- sensory changes
- deafness
- pain only in V1
- bilateral pain
- FHx MS
- age <40
ABX for meningitis post basal skull fracture
S Aureus and Epidermidis cover
e.g. IV linezolid
OR IV vancomycin (but worse CNS penetrance)
Carotid endarterectomy in strokes
Symptomatic stenosis for all >70%
Consider for symptomatic stenosis >50%
No indication for 100% symptomatic stenosis
Subacute combined degeneration of the cord sx
- dorsal and lateral columns
- loss of proprioception and vibration sensation first
- then paraesthesiae / numbness distal legs
- UMN signs lower limbs - weakness, brisk knee jerks, extensor plantars
- BUT absent ankle jerks
- neuropsych manifestations of b12 deficiency may also occur
Migraine treatment
1) triptan + NSAID / paracetamol (nasal triptan 12-17)
2) metoclopramide
NB: triptans CI in ischaemic heart disease or CVA
Migraine prophylaxis
1) propranalol
2) topiramate
3) amitriptyline
NB: topiramate is teratogenic and interferes with COCP therefore not preferable in women of childbearing age
Charcot Marie Tooth aetiologies
Hereditary sensorimotor neuropathies
- type 1: demyelinating (increased latency)
- type 2: axonal (decreased amplitude)
Charcot Marie Tooth presentation
- ascending weakness starting with intrinsic mucles of the feet
- champagne bottle legs
- foot drop
- pes cavus
Cerebellopontine angle lesion sx (e.g. acoustic neuroma)
- CN VIII: ipsilateral hearing loss, tinnitus, vertigo
- CN V (if involved): loss of corneal reflex
- CN VII (if involved): LMN facial palsy
Petrous temporal lesions sx
- CN V all divisions: loss of sensation / corneal reflex
- CN VI: lateral gaze palsy
Superior orbital fissure lesion sx
- CN V - opthalmic division only - loss of sensation / corneal reflex
Homocysteineuria
- marfanoid features e.g. arachnodactyly
- learning disability (not present in Marfan’s)
- livedo reticularis
- malar flush
- young stroke
Progressive supranuclear palsy sx
- parkinson plus
- cognitive impairment
- vertical gaze palsy
- slurred speech
- poor response to L-dopa
Multisystem atrophy sx
- parkinson plus
- parkinsonism +/- cerebellar signs (either may predominate)
- autonomic symptoms: postural hypotension, erectile dysfunction, bladder / bowel disturbance
Syringomyelia sx
- spinothalamic tracts (neuropathic pain / loss of temp sensation) -> anterior horn (power) -> usually spares dorsal columns (vibration / proprioception)
- flaccid paresis at the level of the lesion
- spastic paraparesis below level of the lesion as syrinx expands
- symptoms worse on coughing
- association with arnold-chiari malformation
Tropical spastic paraparesis
HTLV-1 transmission through blood / sexual contact
Caribbean / equatorial Africa / South America - regions endemic
5% of infected get tropical spastic paraparesis
No cure
Cerebral toxoplasmosis mx
Sulfadiazine, pyrimethamine, folinic acid
CMV encephalitis mx
Foscarnet & ganciclovir
Cryptococcus meningitis
CSF: high protein, lymphocyte predominance, glucose not too low
Ix: cryptococcal antigen, india ink stain
Amphotericin B (± abx cover for bacterial meningitis e.g. ceftriaxone)
Bell’s palsy treatment
60mg prednisolone OD for 1 week
Inclusion body myositis presentation
- usually men over 50
- slower progression than polymyositis
- affects quads and forearm flexors most - weakness and atrophy
- CK raised but less than polymyositis
- less responsive to immunosuppressive therapy vs polymyositis
Carbemazepine and COCP
Carbemazepine reduces COCP effectiveness
Advise take preparation containing at least 50mcg ethinyloestradiol
Juvenile myoclonic epilepsy
Presentation
- myoclonic seziures
- frequent absences
- infrequent GTCs
- onset teens, mostly females
- symptoms often on waking
- lifelong disease
Mx
- sodium valproate
- lamotrigine / keppra - esp for females as not teratogenic
- NB: carbamazepine WORSENS myoclonus / absences
Common peroneal nerve palsy symptoms
- weakness of foot dorsiflexion
- weakness of foot eversion / preservation of inversion (vs L5/S1 lesions)
- weakness of extensor hallucis longus
- sensory loss over the dorsum of the foot and the lower lateral part of the leg
Basilar artery stroke
ventral pons infarct -> locked in syndrome
Target bp in ICH
140/80
PCA aneurysm
Ipsilateral facial pain / headache
Oculomotor nerve palsy with progressive increase in size
Multifocal motor neuropathy
LMN signs Patchy motor neuron block on EMG / NCS Bland CSF Raised anti-GM1 ganglioside antibody Responds well to IVIG
Spinal muscular atrophy
AR diseases Flaccid paraparesis SMA1 - rapid perinatal death SMA3 - juvenile onset SMA4 - adult onset
Jugular foramen syndrome
- lesion e.g. glomus jugulare tumour
- compression and deficit of CN IX, X, XI
- NB: similar deficits caused by extracranial carotid artery aneurysm / haematoma expansion
Bickerstaff’s encephalitis
- progressive symmetrical opthalmoplegia
- ataxia
- change in gcs
+/- hyper-reflexia - anti-GQ1b ab positive
- preceding illness
- Mx: steroids, IVIG, PEX
Managing hallucinations in Lewy body dementia
AChE inhibitors e.g. rivastigmine
Lance-Adams syndrome
Intention myoclonus following cerebral hypoxia (days to weeks)
Mx: clonazepam / valproate
Idiopathic intracranial hypertension RFs
- raised BMI
- drugs: OCP, vitamin A, DAs, abx, phenytoin
Idiopathic intracranial hypertension mx
- therapeutic LP
- acetazolamide
- topiramate if co-existing migraines
Meningeal carcinomatosis
- lung, breast, GI, melanoma, lymphoma primary
- variable neurology
- headache
- sciatic pain
- cauda equina
- CN palsies
- focal neurology
- poly-radiculoneuropathy
- seizures
- confusion
Ix: CSF lymphocyte pleocytosis, malignant cells
Factors worsening myasthenia gravis
Beta blockers Phenytoin ABX: gent, macrolides, quinolones Penicillamine Procainamide Pregnancy
Neuroleptic malignant syndrome mx
- IV fluids
- Stop antipsychotic OR restart dopamine agonist
- dantrolene (in severe cases, reduces muscle excitation)
- bromocriptine
Lambert Eaton syndrome vs Myaesthenia
- improvement in weakness with repeated stimulation
- more likely to have autonomic involvement - e.g. dry mouth, erectile dysfunction
- more commonly paraneoplastic
- ophthalmoplegia and ptosis less commonly present
- Mx: 3,4-diaminopyradine (not pyridostigmine!) AND immunosupression with steroids and aza/mtx
Meralgia paraesthetica
Compression of the lateral cutaneous nerve of the thigh (passing through tunnel of inguinal ligament and ASIS) -> sensory loss antero-lateral thigh
Frontal lobe seizures
- usually nocturnal
- bizarre automatisms
- short duration
- less post ictal symptoms
Cluster headache mx
Acute 1) high flow oxygen 2) SC or nasal sumitriptan Prophylaxis 1) verapamil
Stiff person syndrome
- anti-GAD antibody
- T1DM association
- can be paraneoplastic
- progressive stiffness and spasms -> improve during sleep
- EMG: continuous motor unit activity
- Mx: benzos or baclofen
Huntington’s disease
- AD trinucleotied CAG repeat
- huntingtin gene chromosome 4
- disease exhibits anticipation
- symptom onset after 35 yrs
- chorea
- personality changes
- saccadic eye movements
- dystonia
- death 20 years after symptom onset
- no cure
Kearns-Sayre syndrome
- mitochondrial inheritance
- onset <20 yrs old
- external ophthalmoplegia
- assoc retinitis pigmentosa
Refsum’s disease
- AR inheritance
- accumulation of phytanic acid
- cerebellar ataxia, peripheral neuropathy, deafness
- ichthyosis (dry skin, horns)
Venous sinus thrombosis symptoms
General
- headache, nausea, vomiting
Saggital: seizures, hemiplegia, haemorrhagic infarcts
Lateral: CN VI and VII palsies
Cavernous: CN III, IV, V1/V2, VI palsies
- NB: cavernous sinus syndrome causes include infection from sinusitis spreading, expanding ICA aneurysm, trauma
MELAS: mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes
- mitochondrial inheritance
- short stature, DM2
- recurrent migraines
- vomiting, muscle weakness, seizures
- strokes start in 40s
- progressive dementia
- lactic acidosis
- muscle biopsy: ragged red fibres
CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
- AD inheritance
- initially presents with migraines
- then multiple TIAs/strokes
- progressive dementia
- psychiatric problems
- widespread white matter infarcts
Causes of CN III palsy
- infarct e.g. midbrain e.g. Weber syndrome
- haemorrhage
- tumour
- aneurysm: posterior communicating artery, ICA, basilar
- as part of cavernous sinus syndrome
- demyelination
- vasculitis
- diabetes
McArdle’s disease
- glycogen storage disease
- AR inheritance
- painful cramps on exercising
- elevated CK
- myoglobinuria
- low lactic acid levels during exercise
- Ix: muscle biopsy
- Mx: carbohydrate rich diet
Drug of choice when considering pregnancy
1) lamotrigine
2) keppra
Variant CJD
- acquired through eating infected meat (e.g. bovine spongiform encephalopathy)
- earlier onset vs sCJD
- early symptoms often more neuropsychiatric -> neurological symptoms later
- CSF RT-QuIC often negative
- EEG often no periodic sharp waves
- PrP immunochemistry - cluster plaques
Subacute sclerosing panencephaliits
- progressive CNS degeneration following early measles infection
- intelectual deteriorating
- myoclonic jerks
- choroidoretinitis -> deteriorating vision
- seizures
- pyramidal signs, rigidity
- ultimately death, no cure
Median nerve muscles
- most forearm flexors
- pronators
LOAF muscles - lateral two lumbricles
- opponens pollicis brevis
- abductor pollicis brevis
- flexor pollicis brevis
Ulnar nerve muscles
- flexor carpi ulnaris
- flexor digitorum profundus
- small muscles of the hand (except LOAF)
Radial nerve muscles
- triceps
- brachioradialis
- supinator
- extensors of the wrist and fingers
CIDP (chronic inflammatory demyelinating polyneuropathy)
- chronic version of GBS type symptoms -> symmetrical flaccid weakness > sensory deficit
- can relapse and remit OR be progressive
- IX: NCS show conduction delay, MRI shows inflamed nerve roots
- Mx: corticosteroids, azathioprine, IVIG, PEX
Best anti-convulsant in alcoholics
Lamotrigine OR keppra
- as carbemazapine is cyp450 inducer
- valproate is cyp450 inhibitor
Acute dystonia and tardive dyskinesia mx
Procyclidine, stop offending agent
ABCD2 score
A 1) Age ≥60 B 1) SBP ≥140; DBP ≥90 C 1) speech impairment C 2) unilateral weakness Du 1) 10-59 mins Du 2) ≥60 mins Di 1) Diabetes
≥4 is high risk -> admit
Exacerbating factors in myaesthia
- B blockers
- antibiotics: gent, clary, cipro, doxy
- lithium
- phenytoin
- penicillamine
- quinidine
- procainamide
- magnesium
Restless legs mx
1) ropinerole / other dopamine agonists
2) Gabapentinoids
3) ? carbemazepine
3) Mild opioids / benzos
