Neurology/Neurosurgery Flashcards
what are the features of wernicke’s encephalopathy?
- opthalmoplegia
- reduced GCS/confusion
- nystagmus
- ataxia
- peripheral sensory neuropathy
how would you manage Charcot-Marie-Tooth disease?
there is no treatment to cure or slow the disease
management is for symptomatic relief, and should be coordinated by a specialist neurologist involving the MDT of orthopaedic surgeons, and PT/OT
what is the role of steroids in managing guillian-barre syndrome?
should not use steroids
only treatment is IVIG or plasma exchange
what does coning mean?
coning = herniation of the cerebellar tonsils through the foramen magnum
what is usually the first feature of parkinsons?
unilateral or bilateral?
unilateral pill-rolling tremor (60%)
asymmetrical signs support parkinson’s rather than parkinsonism
what sort of medication leads to acute dystonia?
what is the neural mechanism?
what is the management?
anti-dopaminergics (typical antipsychoitcs and metaclopramide)
blockade of negrostriatal D2 leads to compensatory excess production of basal ganglia catecholamines, resulting in prolonged, forceful contractions of the muscles around the face, head, neck and arms
treatment is with anticholinergic - procyclidine
written paper 2014
does pain behind the ear increase or decrease the likelihood of a diagnosis of bell’s palsy?
pain behind the ear is consistent with Bell’s palsy
what are the features of vestibular schwannoma (acoustic neuroma)?
- cranial nerve VIII: hearing loss, vertigo, tinnitus
- cranial nerve V: absent corneal reflex
- cranial nerve VII: facial palsy
what are the causes of parkinsonism?
- idiopathic
- lewy body dementia
- drugs/toxins
- MPTP
- typical > atypical antipsychotics
- chlorpromazine, prochlorperazine
- metaclopramide
- basal ganglia tumour
- wilson’s disease
name two Parkinson’s Plus syndromes
- progressive supranuclear palsy
- multisystem atrophy
- corticobasal degeneration
other than better soft tissue resolution, what is the benefit of MR imaging over CT in the brain?
MR has better sensitivity for posterior fossa masses/bleeds
what two conditions increase the rate of Bell’s palsy?
pregnancy and diabetes
what are the causes of bilateral facial nerve palsy?
what are the causes of unilateral facial nerve palsy?
lyme disease, giullian-barre syndrome, sarcoidosis or trauma
bell’s, ramsey-hunt, parotid surgery/tumour, diabetes, stroke, middle ear (otitis media or cholesteatoma)
typical side effects of lamotrigine
- skin: maculopapular rash / SJS / TEN
- head: diplopia, blurred vision, photosensitivity
- tremor
side effects of valproate
pregnancy: teratogenic
GI: liver failure, pancreatitis
skin: hair loss, regrows curly
nerves: tremor, ataxia, encephalopathy
haem: thrombocytopenia
what is the definition of ‘parkinsonism’
movement disorder characterised by bradykinesia and at least one of tremor, rigidity, postural instability
describe what you see (3)
- wasting of the distal leg muscles, specifically the anterolateral compartment with relative sparing of the quadraceps, giving an ‘inverted champagne bottle’ appearance
- bilateral pes cavus
- clawing of the toes
the diagnosis is Charcot-Marie-Tooth disease
cerebellar lesions - hyper or hypotonia?
hypotonia
loss of supplimental cerebellar bacgkround reinforcing signals leaves motor cortex to send tonic signals out unaided, resulting in temporary hypotonia in the early/medium term. eventually the motor cortex adjusts and the hypotonia becomes less marked
what physcial sign helps identify the location of the lesion in Horner’s syndrome?
anhidrosis
- central lesion - anhidrosis of arm, trunk and face
- pre-ganglionic lesion - anhidrosis of the face
- post-ganglionic lesion - no anhidrosis
what are the investigations for guillian-barre syndrome?
FVC every 4 hours
LP demonstrates elevated protein (>5.5 g/L), normal WCC
if the diagnosis is parkinson’s, what other tests would you like to complete the examination? (4)
- assess handwriting - micrographia
- lying and sitting blood pressure - autonomic dysfunction
- seborrhoeic dermatitis - autonomic dysfunction
- mini-mental test score - cognition/dementia with Lewy bodies
what is dysmetria?
incorrect velocity and amplitude of planned movements
what is the eye sign in parkinson’s disease?
blepharoclonus
tremor of the eyelid when the eyes are gently closed
what are the driving rules for epilepsy?
what is other general safety advice for a newly diagnosed epileptic?
must contact the DVLA and stop driving until they are seizure free for >1 year
avoid swimming, becoming pregnant, driving (esp HGVs), extreme sport, occupational hazards (pylon engineers, window cleaners etc)
how can you demonstrate bradykinesia in parkinsonism?
patient to open and close hands repeatedly. movements become slower and smaller in amplitude
patient to tap foot on the floor repeatedly. movements become slower and smaller in amplitude
what is Todd’s palsy?
temporary weakness following a focal seizure in the primary motor cortex
what nerve roots are affected in Erb’s palsy?
what are the classic findings on inspection of the upper limb?
C5-6
upper limb held in pronation and medial rotation
what are the types of seizures?
-
focal - start in a specific part of the brain and remain on one side
- focal aware, focal impaired awareness , focal awareness unknown
- focal motor (Jacksonian march), focal non-motor (deja-vu, jamais-vu)
- aura - implies the seizure starts in the temporal lobe
- focal to bilateral
-
generalised - involves both hemispheres of the brain, consciousness is lost immediately
- motor or absence
- motor subtypes: tonic-clonic, tonic, clonic, atonic, myoclonic
what is the gold standard diagnostic test for degenerative cervical myelopathy?
MRI cervical spine
disc degeneration, ligament hypertrophy, cord signal change
side effects of levetiracetam/keppra
- psych: depression and agitation (common)
- GI: dyspepsia, D&V
- head: diplopia, drowsiness
- haem: blood dyscrasia
what are the complications of ventriculoperitoneal shunt placement?
failure/malfunction requiring revision
shunt infection with staph epidermidis
other: over/under drainage, subdural haematoma, seizures
what does an aura preceeding a seizure tell you about the neuropathology?
often, but not always, indicates focal seizure starting in the temporal lobe
what is the principle of investigating epilepsy?
trying to identify a provoking factor - unprovoked is basically diagnosing epilepsy and that needs prophylactic medication and a driving ban
bedside - emergency EEG to include non-convulsive status epilepticus
bloods: U&E, glucose, anti-epileptic drug level
urine: dip, MC&S and 5-panel drug screen
images: MRI brain for ?SOL
special tests: LP and blood cultures if infection is suspected
what tests should be done for facial nerve palsy/Bell’s ?
rule out more sinister causes
look in the ear for vesicular rash on/aorund tympanic membrane (site of viral replication is the geniculate nucleus)
ESR and glucose.
varicella and boriella antibodies
what is classically found in Weber’s syndrome (stroke)?
- ipsilateral: CN III palsy
- contralateral: limb weakness
what is the summary of clinical findings that strongly suggests a diagnosis of motor neuron disease?
what are some types of motor neuron disease?
this patient has a combination of upper and lower motor neuron signs in the face, arms and legs with no sensory involvement. the diagnosis is motor neuron disease.
- amyotrophic lateral sclerosis (UMN and LMN)
- primary lateral sclerosis (mostly UMN)
- progressive muscular atrophy (mostly LMN)
- progressive bulbar palsy (mostly bulbar/pseudobulbar)
what sort of drugs are used to treat movement disorders (e.g. huntington’s)?
dopamine antagonists
unlicenced use of antipsychotics that have an anti-DA mechanism of action can also help control movement disorders
(e.g. haloperidol, olanzapine, risperidone)
note on Huntington’s - no treatment can slow the progression of disease
(e.g. tetrabenazine)
where is the tumour?
- grasp reflex
- dysphasia, perseveration
- decreased verbal fluency
…what is the grasp reflex?
frontal lobe
dysphasia - expressive dysphasia (Broca’s), receptive/fluent (Wernicke’s)
grasp reflex - running fingers across palm triggers the hand to grasp the examiner’s fingers
typical side effects of carbemazepine
- haem: leucopenia
- head: diplopia & blurred vision, balance, drowsiness
- skin: generalised erythematous rash
what is the treatment for Bell’s palsy?
conservative
- education - 95% make a full recovery but can take up to 6 months in some cases
- protect the eye - taping shut at night, sunglasses, arteficial tears
medical
- prednisolone given within 72 hours of onset of symptoms
- 60 mg OD PO for 5 days, then taper down by 10 mg/day
- if ramsey-hunt, give aciclovir
what is the consideration in migraine management for a woman of childbaring age?
for prophylaxis - propranolol is preferred to topiramate
usually topiramate is first class though
where is the tumour?
- hemisensory loss, decreased two-point descrimination
- sensory inattention
- astereognosis
- dysphasia
…what is astereognosis?
parietal lobe
astereogonosis - inability to descriminate objects based on touch alone
what are the causes of cerebellar syndrome?
ADULT
- posterior circulation CVA
- demyelination (MS)
- alcoholic cerebellar degeneration
PAEDS
- hereditary
- fredrich ataxia
- ataxia telengiectasia
- developmental malformations
- arnold-chiari
- dandy-walker
- posterior fossa tumour
- medulloblastoma
- astrocytoma
*
what are some complications of epilepsy?
sudden unexpected death in epilepsy (SUDEP) - apnoea or asystole during nocturnal seizure
5% risk of foetal abnormalities in women suffering from epilepsy during pregnancy
what are the eye signs of cerebellar disease?
how would you elicit these clinically?
nystagmus, fast beat toward the side of the lesion
test as usual
loss of smooth persuit: hyper/hypometric saccades
ask patient to alternate gaze between two fingers held up shoulder width apart
where is the tumour?
- dysphasia
- homonomous hemianopia
- amnesia
temporal lobe
what medication is used for acute termination of migraine?
what medication is used first line for prophylaxis?
termination - triptans + NSAID or triptan + paracetamol
prophylaxis: topiramate or propranolol
what are the localising signs of cerebellar disease?
-
vermis - truncal ataxia
- test with propulsion and retropulsion when standing
- neocortex - ipsilateral limb ataxia
what is the gait in cerebellar disease?
broad-based, ataxic, ‘drunken’ gait
tendency to fall toward the side of the lesion
what is the management plan for a CNS tumour?
conservative
- ?palliation maybe the best option
medical
- prevent seizures - anti-epileptics
- treat headaches
- opiates appropriate (codeine 60 mg prn max QDS)
- cerebral oedema?
- chronic = dexamethasone
- acute = mannitol
surgical
- resection if possible
- debulking and carmustine wafer insertion (deilvery of local chemotherapy)
- biopsy for tissue diagnosis and genotyping to optimise medical treatment
- ventriculo-peritoneal shunt placement for hydrocephalus
- neoadjuvant chemo/radiotherapy, or curative/palliative when tumour is inaccessable surgically
what is the prognosis of a CNS tumour?
poor: <50% survival at 5 years
…getting better
what is the result of Romberg’s test in cerebellar disease?
mechanism?
Romberg negative
Romberg is a test of proprioception: closing the patient’s eyes removes the second of three position senses, so with only the vestibular system functioning they will lose balance quickly
cerebellar lesions do not affect proprioception.
N.B. alcoholic cerebellar degeneration with co-morbid vit B12 deficiency and subacute combined degeneration of the spinal cord
what is the prognosis of guillian-barre syndrome?
10% die
85% make a complete/near-complete recovery
10% are unable to walk alone at 1 year
what are the risk factors for degenerative cervical myelopathy?
smoking, genetics and occupation that exposes patients to high axial weight loads
what is classically found in lateral medullary syndrome? (stroke)
- ipsilateral: ataxia, dysphagia, facial numbness, cranial nerve palsy (horner’s)
- contalateral: loss of limb pain and temperature sensation
structures affected - nucleus ambiguous (CN IX and X), cerebellar peduncle, spinothalamic tract, CN VIII nucleus
what is the pathology of normal pressure hydrocephalus?
what is the definitive treatment?
decreased absorbtion of CSF by the arachnoid villi
ventriculo-peritoneal shunting
what are the drug treatments for parkinson’s?
-
dopaminergic drugs
- Levodopa
- dopamine agonists (bromocriptine, pramipexole)
- MAO-B inhibitors (rasagline)
- anticholinergics (procyclidine)
-
COMT inhibitors (entacapone, tolcapone)
- decrease the peripheral degradation of levodopa, increasing the on:off ratio
what are the complications of myotonic dystophy?
-
cardiac
- conduction (arrythmia, heart block)
- valves (mitral valve prolapse)
- cardiomyopathy (LVH and myocardial myotonia)
-
endocrine
- diabetes mellitus
- hypogonadism
-
gastrointestinal
- dysphagia and reflux
- hypomotility/delayed gastric emptying
-
respiratory
- respiratory failure following anaesthesia
- hypoventilation (weakness)
personal or family history of respiratory complications following anaesthesia should make you suspect…
myotonic dystrophy
define epilepsy
neurological condition characterised by recurrent episodes of abnormal, uncontrolled, intermittent electrical activity in the brain leading to multiple seizures.
convulsions are the motor sign of electrical discharges in the brain
what are the archetypal features of myotonic dystrophy
- myotonic facies
- wasting of small facial muscles, temporalis, masseter and SCMs
- frontotemporal balding
- cateracts
- delayed hand grip release
- delayed eye opening followng forceful closure
- distal muscle wasting, foot drop & high steppage gait
side effects of phenytoin
psych: decreased intellect, depression
skin: acne, gum hyperplasia, coarse facial features
haem: blood dyscrasia
nerves: polyneuropathy
what are the aetiologies of a space occupying lesion in the brain?
-
tumour
- primary
- metastasis
-
vascular
- aneurysm
- chronic subdural haematoma
-
infection
- abscess
- granuloma
- cyst (cysticersosis)
what are some disease that present with diffuse LMN signs ONLY?
- multifocal motor neuropathy
- chronic inflammatory demyelinating polyneuropathy
- old poliomyelitis
- syphilis
- spinal muscular atrophy
what are the principles of managing restless leg syndrome?
- stretch and massage the legs
- treat any iron deficiency
- dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
- benzodiazepines
- gabapentin
what are the investigations of Wernicke’s encephalopathy?
- decreased RBC transketolase (rarely done)
- MRI brain (periaqueductal punctate haemorrhage)
how do you describe the arms in parkinson’s?
how do you describe the gait in parkinson’s?
asymmetrical pill rolling temor, lead pipe rigidity at the elbow, cogwheel rigidity in the wrist, bradykinesia accentuated by voluntary movement of the opposite arm
unsteady, shuffling, narrow-based, stooped posture, accentuation of the pill-rolling tremor
what’s the diagnosis?
- pain
- opthalmoplegia
- horner’s syndrome
- trigeminal nerve (V1) palsy
- proptosis
cavernous sinus syndrome
what are the diagnostic findings that would support MS?
MRI - hyperintensities in the periventricular white matter
CSF - oligoclonal IgG bands that are not present in the serum, suggest CNS inflammation
what is the medical management of MS?
DMARDs
- if mild… dimethyl fumarate
- alemtuzumab (anti-T cell)
- natalizumab (anti-leukocyte migration)
acute
- pulse methylprednisolone
symptom control
- tremor - botox A
- spasticity - baclofen/gabpentin
- urinary retention, PVRV >100 mL = self-catheterisation
- urinary retention, PVRV <100 mL = tolterodine
- fatigue = amantadine, CBT, exercise
what are Lhermitte’s sign and Uhthoff phenomenon?
both seen in MS
Lhermitte’s sign = flexion of the neck causes electric shock like feeling down the trunk and limbs
Uhthoff phenomenon = worsening of sypmtoms with heat
what are the layers of the scalp?
use SCALP
Pericranium
- Skin
- Connective tissue (dense)
- Aponeurotic layer (galea)
- Loose connective tissue
what is the origin of the middle meningeal artery?
external carotid
maxillary artery
middle meningeal
which branches of the trigeminal nerve are commonly affected in trigeminal neuralgia?
V2 and V3
opthalmic division is rarely affected.
pain felt in the upper nose, eyelid, forehead and scalp are unusual.
what is the normal CSF opening pressure?
8-14 mmHg
what are the layers through which the needle will pass for a lumber puncture?
skin
subcut fat
supraspinous ligament and intraspinous ligament
ligamentum flavum
extradural space
dura mater
arachnoid mater
subarachnoid space (with CSF)
compression of which nerve root affects knee jerk reflex?
compression of which nerve root affects ankle jerk reflex?
L4
S1
what are the causes of peripheral neuropathy?
metabolic
- diabetes
- vitamin B12 deficiency
drugs/toxins
- isoniazid
- phenytoin
- nitrofurantoin
- metronidazole
- alcohol
- heavy metals: lead/mercury
inflammatory
- chronic inflammatory demyelinating neuropathy
infectious
- guillian-barre syndrome
- neurosyphilis
- leprosy
hereditary
- charcot-marie-tooth disease
patient has UMN and LMN signs.
what 3 things should you look for/ask the examiner to confirm the diagnosis of MND?
never invovlement of the bladder
never involvement of the extra-ocular muscles
never any sensory signs
what are the causes of a ptosis?
unilateral
- CN III
- horner’s
- multiple sclerosis
(distiguishing question: is the pupil large, small or normal?)
bilateral
- dystrophia myotonica
- myaesthenia gravis, lambert eaton myaesthenic syndrome
- bialteral horner’s syndrome
what are the causes of a CN III palsy?
medical (eye movements first)
- diabetes
- hypertension
- microembolism from carotid arteries
surgical (mydriasis first)
- painful = aneurysm (posterior communicating artery)
- trauma
- bleed
what are the causes of a CN VI palsy?
medical
- diabetes
- hypertension
- multiple sclerosis
- intracranial hypertension
- has longest intracranial course. pressure will compress the nerve against the petrous part of the temporal bone -> palsy
what are the physical signs associated with intracranial hypertension?
- loss of retinal vein pulsation on fundoscopy
- reduced GCS
- CN VI palsy
- CN III palsy
- papilloedema (late)
- cushing’s triad
- hypertension
- bradycardia
- Cheyne-Stokes respiration)
what is the differential diagnosis for spastic paraparesis?
brain
- stroke
- multiple sclerosis
cord
- cervical spondylosis
- cerebral palsy
- motor neuron disease
- syringomyelia
emergency
- cord compression
what are the questions you have to ask to rule out a sinister cause of spinal back pain?
- erectile dysfunction (nocturnal erections)
- urinary retention
- faecal incontinence
- change in the sensation as you pass urine?
- perianal sensation
genetics of neurofibromatosis
type 1 - autosomal dominant, micodeletion syndrome of a gene on chromosome 17
type 2 - autosomal dominant, mincrodeletion syndrome of a gene on chromosome 22
both can arise de novo in a significant proportion of patients
what are the rules for the cafe-au-lait spots in NF?
must have more than 5
must be >15 mm diameter
what are the medical complications of type 1 NF?
- hypertension (phaeochromocytoma)
- SpLD
- malignant transformation in 10% of cases
- thoracic kyposis, T2RF
which is the muscle in the inner ear affected by CN VII lesions that leads to hyperacusis?
stapedius
