Neurology Flashcards
Vasovagal syncope pathophysiology
Stimulus—> vagus nerve—> PNS Vasodilation of cerebral vessels Decreased pressure in cerebral circulation Hypoperfusion of brain tissue Loss of consciousness
Prodrome symptoms vasovagal syncope
Hot or clammy Sweaty Heavy Dizzy or light-headed Vision going blurry or dark Headache
Signs and symptoms during vasovagal syncope
Collateral history
Suddenly losing consciousness and falling to the ground
Unconscious on ground for a few seconds to a minute as blood returns to their brain
Twitching, shaking or convulsion activity
Incontinence
Postictal period
Prolonged period of confusion, drowsiness, irritability, disorientation
Causes of primary syncope
Dehydration
Missed meals
Extended standing in warm environment
Vasovagal response to stimulus
Secondary causes of syncope
Hypoglycaemia Dehydration Anaemia Infection Anaphylaxis Arrhythmias (electrolyte abnormalities) Valvular heart disease Hypertrophic obstructive cardiomyopathy
Syncope features
Prolonged upright position before the event
Lightheaded before event
Sweating before event
Blurring or clouding of vision before the event
Reduced tone during the episode
Return of consciousness shortly after falling
No prolonged post-ictal period
Seizures features
Epilepsy aura (smells, tastes, deja vu) before event Head turning or abnormal limb positions Tonic clonic activity Tongue biting Cyanosis Lasts >5 minutes Prolonged post-ictal period
Types of seizures
Generalised tonic-clonic seizures Focal seizures Absence seizures Atonic seizures Myoclonic seizures Infantile spasms Febrile convulsions
Generalised tonic-clonic seizures
Loss of consciousness
Tonic: muscle tensing followed by
Clonic: muscle jerking
Tongue biting, incontinence, groaning, irregular breathing
Post-ictal period: confused, drowsiness, irritable or low
General tonic-clonic seizures management
First line: sodium valproate
Second line: lamotrigine or carbamazepine
Focal seizures features
Temporal lobes: hearing, speech, memory, emotions Hallucinations Memory flashbacks Deja vu Doing strange things on autopilot
Focal seizures management
First line: carbamazepine or lamotrigine
Second line: sodium valproate, levetiracetam
Absence seizures features
Typical in children 4-8 Blank Stares into space Returns to normal abruptly Unaware of surroundings during episode Last 10-20seconds Most patients stop as they get older
Absence seizures management
First line: sodium valproate or ethosuximide
Atonic seizures features
Drop attacks Brief lapses in muscle tone <3 minutes Typically begin in childhood Indicative of Lennox-Gastaut syndrome
Management of atonic seizures
First line: sodium valproate
Second line: lamotrigine
Myoclonic seizures features
Brief muscle contractions
Sudden jump
Patients awake during episode
Part of juvenile myoclonic epilepsy
Myoclonic seizures management
First line: sodium valproate
Second line: lamotrigine, levetiracetam, topiramate
Lennox-Gastaut syndrome
Extension of infantile spasms Onset 1-5 years Atypical abscess, falls, jerks 90% moderate-severe mental handicap EEG: slow spike Ketogenic diet may help
Benign rolandic epilepsy
Most common in childhood M>F
Paraesthesia (e.g. unilateral face), usually on waking up
Juvenile myoclonic epilepsy (Janz syndrome)
Onset: teens, F:M = 2:1
Infrequent generalised seizures, often in morning/following sleep deprivation
Daytime absences
Sudden, shock like myoclonic seizures (these may develop before seizures)
Usually good response to sodium valproate
Infantile spasms features
West’s syndrome Infancy->6months Clusters of full body spasms M>F Flexion of head, trunk, limbs-> extension of arms (Salaam attack); last 1-2secs, repeat up to 50times Progressive mental handicap EEG: hypsarrhythmia Usually 2nd to serious neurological abnormality (e.g. TS, encephalitis, birth asphyxia) or may be cytogenetic Poor prognosis
Infantile spasms, West syndrome management
Prednisolone
Vigabatrin
Investigations epilepsy
EEG, after 2nd tonic-clonic
MRI brain
ECG to exclude heart problems
Blood electrolytes: sodium, potassium, calcium, magnesium
Blood glucose: hypoglycaemia, diabetes
Blood cultures, urine cultures, lumbar puncture
When should MRI brain be considered in epilepsy
First seizures in <2 years
Focal seizures
No response to first-line anti-epileptic medications
General advice for epilepsy
Showers > baths Cautious with swimming Cautious with heights Cautious with traffic Cautious with heavy, hot or electrical equipment
Sodium valproate side effects
Teratogenic, contraception advice
Liver damage and hepatitis
Hair loss
Tremor
First line for most except focal seizures
Increases activity of GABA
Carbamazepine side effects
Agranulocytosis
Aplastic anaemia
Induces P450 system- many drug interactions
First line for focal seizures
Phenytoin Side effects
Folate and vitD deficiency Megaloblastic anaemia (folate deficiency) Osteomalacia (vitD deficiency)
Ethosuximide side effects
Night terrors
Rashes
Lamotrigine side effects
Stevens-Johnson syndrome or DRESS syndrome
Leukopenia
DRESS syndrome
Drug reaction with eosinophilia and systemic symptoms
Management of seizures
Put patient in safe position
Place in recovery position
Put something soft under head
Remove obstacles that could lead to injury
Make a note of time at start and end
Call ambulance if >5 minutes or first seizures
Status epilepticus definition
Seizures >5minutes or
>3 seizures in one hour
>=2 seizures within a 5 minute period without the person returning to normal between them
Management of status epileptic in hospital
Initial management
Secure airway High-concentration oxygen Assess cardiac and respiratory function Check blood glucose levels Gain IV access IV lorazepam, repeat after 10minutes if seizure continues
Management of status epilepticus in hospital if seizures persist after initial management
Infusion of IV phenobarbital or phenytoin
Intubation and ventilation
Transfer to ICU
Options in community to treat status epilepticus
Buccal midazolam
Rectal diazepam
Febrile seizure definition
Seizure associated with a febrile illness
Not caused by CNS infection
No previous neonatal seizures or previous unprovoked seizure
Not meeting criteria for acute symptomatic seizure
6months-6years
Febrile seizures epidemiology
12-18months peak incidence
Febrile seizure pathophysiology
Viral infections: URTI, LRTI, otitis media, UTI most common
Also gastroenteritis and fever post-vaccination associated
Febrile seizures risk factors
FH
Socio-economic causes
Seasonal: viruses more common in winter
Zinc and iron deficiency
Clinical features of febrile seizures
Fever >38
Age 6months-6 years
Tonic-clonic seizure
Symptoms of infection
Simple febrile seizure
<15 minutes
Generalised tonic-clonic
Isolated event- doesn’t recur within the same febrile illness
Post-ictal state: uneventful recovery from seizure
Complex febrile seizure
> 15 minutes
Focal, or focal with secondary generalisation
Recurrence within 24 hours of the same febrile illness
May suffer from Todd’s paresis
Febrile status epilepticus
Subgroup of complex febrile seizures
Duration >30minutes
Multiple seizures lasting 30 minutes with no recovery between each one
History of febrile convulsions
Has child been vaccinated Are they at school? Previous treatment with antimicrobials Any history of trauma or toxin ingestion Any family history Developmental history
Examination in febrile convulsions
Find source of infection External ear examination with auroscope- look for signs of otitis externa or otitis media Throat examination- for signs of URTI (inflamed tonsils) Full respiratory examination- for signs of LRTI Check fontanelles Brudzinskis or Kernigs sign Nuchal rigidity (neck stiffness) Mental status of child Full neurological examination Cardiovascular examination Abdominal examination Urine dipstick and microscopy Superficial infective skin lesions
DD febrile seizures
CNS infection Delirium Syncope Epilepsy Intracranial space occupying lesions, brain tumours or intracranial haemorrhage Electrolyte abnormalities Trauma
Febrile convulsions investigations
If child >1
Or symptoms of intracranial infection
General observations Clear source of infection Bedside testing- urinalysis Bloods: FBC, CRP, U&E, calcium, glucose, magnesium, blood cultures Stool cultures Lumbar puncture Imaging: CXR CT/ MRI/ EEG in complex seizures
Red flags of CNS infection
Complex febrile seizures
History of lethargy, irritability or decreased feeding
Prolonged post-ictal altered consciousness or neurological deficit >1 hour
Any physical signs of meningitis/ encephalitis
Previous/ current treatment with antibiotics which may have masked full clinical presentation of meningitis
Incomplete immunisation in children 6-18months against Haemophilus influenza B and streptococcus pneumoniae
Acute management febrile seizure
A-E Monitor child and prevent injury Keep child well-hydrated Paracetamol or ibuprofen In-depth explanation
Treatment febrile seizure >5 minutes
Benzodiazepine rescue
Febrile seizures risk factors for recurrence
Age <18 months at onset
Shorter duration of fever before seizure (<1 hour)
Relatively lower grade of fever associated with seizure (<40degrees)
Multiple seizures during the same febrile illness
Day nursery attendance
FH of febrile seizure in a first degree relative
Risk of epilepsy after febrile seizure
FH epilepsy
Complex focal seizure
Neurodevelopmental impairment
Breath holding spells
Breath holding attacks Involuntary episodes Trigger: something scaring or upsetting them Occur 6-18months of age Not harmful in long term
Cyanosis breath holding spells
Pallid breath holding spells
Cyanotic breath holding spells
Occur when child is really upset, worked up, crying
Stop breathing after letting out a long cry
Become cyanosis and lose consciousness
Regain consciousness within a minute and start breathing again
Tired and lethargic after an episode
Reflex anoxic seizures
Occur when child is startled
Vagus nerve sends strong signals to heart that causes it to stop breathing
Child will suddenly go pale, lose consciousness, seizure-like muscle twitching
<30seconds heart restarts and child becomes conscious again
Management of breath holding spells
Treat iron deficiency anaemia
Causes of headache in children
Tension headaches Migraines ENT infection Analgesic headache Problems with vision RICP Brain tumours Meningitis Encephalitis Carbon monoxide poisoning
Tension headaches features
Mild ache across forehead
Pain/pressure in band-like pattern around head
No visual changes or pulsating sensations
Typically symmetrical
Symptoms in young children:
Quiet, stop playing, turn pale or become tired
Resolve quickly in children compared with adults
Within 30 minutes
Triggers for tension headaches
Stress, fear or discomfort
Skipping meals
Dehydration
Infection
Management tension headaches
Reassurance Analgesia Regular meals Avoid dehydration Reduce stress
Migraines types
Migraine without aura Migraine with aura Silent migraine Hemiplegic migraine Abdominal migraine
Symptoms of migraines
Unilateral
More severe
Throbbing in nature
Take longer to resolve
Migraine associations
Visual aura
Photophobia and phonophobia
Nausea and vomiting
Abdominal pain
Management of migraines in children
Rest, fluids and low stimulus environment Paracetamol Ibuprofen Sumatriptan Antiemetic, domperidone
Migraine prophylaxis
Propanolol (avoid in asthma)
Pizotifen (drowsiness SE)
Topiramate: teratogenic
Abdominal migraine
Young children Occur before the develop traditional migraines Central abdominal pain >1 hour N/v Anorexia Headache Pallor
Sinusitis
Headache Inflammation in sinuses Facial pain Tenderness over affected sinuses Sinusitis resolves within 2-3weeks Mostly viral
Causes of neonatal hypotonia
Neonatal sepsis
Werdnig-Hoffman disease (spinal muscular atrophy TY1)
Hypothyroidism
Prader-Willi
Maternal drugs, e.g. benzodiazepines
Maternal myasthenia gravis
Neurological conditions causing hypotonia
Central hypotonia:
Cerebral palsy
Brain and spinal cord injury
Serious infections; meningitis, encephalitis
Peripheral hypotonia: Muscular dystrophy Myasthenia gravis Spinal muscular atrophy Charcot-Marie-Tooth disease: affects myelin
Non-neurological causes of hypotonia
Down’s syndrome Prader-Willi Tay-Sachs disease Congenital hypothyroidism Marfan syndrome and Ehlers-Danlos Connective tissue disorders Premature
Causes of hypotonia in larger life
Multiple sclerosis
Motor neurone disease
Extradural haematoma pathophysiology
Collection of blood between skulls and dura
Caused by low-impact trauma
Collection in temporal region, pterion, middle meningeal artery
Haematoma expands
Uncus of temporal lobe herniates around tentorium cerebelli
Compression of parasympathetic fibres of CN3
Biconvex (or lentiform), hyperdense collection around the brain surface
Limited by suture lines of the skull
Extradural haematoma presentation
Loses, briefly regains, loses again consciousness
After low impact head injury
Lucid interval
Fixed and dilated pupil from compression of parasympathetic fibres CN3
Management of extradural haematoma
Craniotomy
Evacuation of haematoma
Subdural haematoma pathophysiology
Collection of blood deep to dural layer of meninges
High-impact injury
Bridging veins
Crescent collection on CT
Midline shift or herniation
Shaken baby syndrome in infants as they have fragile bridging veins
Subdural haematoma management
Surgical decompression with burr holes
Subdural haematoma signs and symptoms
Confusion
Reduced consciousness
Neurological deficit
Subarachnoid haemorrhage causes
Head injury (traumatic SAH) Absence of trauma (spontaneous SAH) Intracranial aneurysm: Berry aneurysm, adult polycystic kidney disease, Ehlers-Danlos, coarctation of aorta AV malformation Pituitary apoplexy Arterial dissection Mycotic (infective) aneurysm Perimesencephalic (idiopathic venous bleed)
Classic presenting feature of SAH
Headache: thunderclap N/V Meningism Coma Seizures Sudden death ECG changes: ST elevation
SAH diagnosis
CT head: acute blood
Lumbar puncture: if CT negative, 12 hours following onset of symptoms to allow for Xa tho chronic to develop
Referral to neurosurgery
CT intracranial angiogram
SAH treatment
Craniotomy and clipping
Bed rest
Control BP
Avoid straining to prevent re-bleed
21 days of nimodipine to treat vasospasm, treat with hyper volar is, induced-hypertension, haemodilution
Hydrocephalus is treated with external ventricular drain (CSF diverted into a bag at bedside) or long-term ventriculo-peritoneal shunt
Complications of aneurysms SaH
Re-bleeding: most common in first 12 hours, need repeat CT
Vasospasm: delayed cerebral ischaemia, 7-14days after onset
Hyponatraemia: SIADH
Seizures
Hydrocephalus
Death
Muscular dystrophy
Genetic conditions that cause gradual weakening and wasting of muscles
Types of muscular dystrophy
Duchennes muscular dystrophy Becker’s muscular dystrophy Myotonic dystrophy Facioscapulohumeral muscular dystrophy Oculopharyngeal muscular dystrophy Limb-girdle muscular dystrophy Emery-dreifuss muscular dystrophy
Gowers sign
Technique to stand up from a lying position
Proximal muscle weakness
Pelvic weakness
Use hands on their legs to help them stand up
Management of muscular dystrophy
No curative treatment Occupational therapy Physiotherapy Medical appliances Manage complications: spinal scoliosis and heart failure
Duchennes muscular dystrophy pathophysiology
Caused by defective gene for dystrophin on the X-chromosome
Dystrophin helps hold muscles together at cellular level
X-linked recessive
Duchennes muscular dystrophy presentation
Boys present at 3-5years Weakness in muscles around pelvis Progressive weakness Eventually all muscles will be affected Wheelchair bound by teenage years Life expectancy 25-35 Good management of cardiac and respiratory complications Associated with dilated cardiomyopathy Calf pseudohypertrophy
Duchennes muscular dystrophy management
Oral steroids: slow progression of muscle weakness as much as two years
Creatine supplementation: slight improvement in muscle strength
Genetic trials are ongoing
Beckers muscular dystrophy presentation
Similar to Duchennes
Dystrophin gene is less severely affected and maintains some function
Symptoms appear 8-12years
Some patients require wheelchairs in their last 20s or 30s
Others able to walk with assistance into later adulthood
Management similar to Duchennes
Features of myotonic dystrophy
Progressive muscle weakness
Prolonged muscle contractions
Cataracts
Cardiac arrhythmias
Unable to let go after shaking hand, unable to release doorknob grip
Facioscapulohumeral muscular dystrophy
Usually presents in childhood with weakness around face
Progressing to shoulders and arms
Classic initial symptom: sleeping with eyes initially open, weakness in pursing their lips
Unable to blow their cheeks our without air leaking from their mouth
Oculopharyngeal muscular dystrophy
Usually presents in late adulthood
Weakness of ocular muscles (around eyes)
Weakness of pharynx (around throat)
Typically presents with bilateral ptosis, restricted eye movements, and swallowing problems
Muscles around limb girdles are also affected to varying degrees
Limb-girdle muscular dystrophy
Usually presents in teenage years
With progressive weakness around limb girdles (hips and shoulders)
Emery-Dreifuss muscular dystrophy
Usually presents in childhood with contracture
Most commonly in the elbows and ankles
Contractures: shortening of muscles and tendons that restrict the range of movement in limbs
Patients also suffer with progressive weakness and wasting of muscles, starting with the upper arms and lower legs
Raised intracranial pressure pathophysiology
Normal ICP is 7-15mmHg in adults in the supine position
Cerebral perfusion pressure is the net pressure gradient causing cerebral blood flow to the brain
CPP= mean arterial pressure - ICP
RICP causes
Idiopathic intracranial hypertension Traumatic head injuries Infection: meningitis Tumours Hydrocephalus
RICP features
Headache Vomiting Reduced level of consciousness Papilloedema Cushings trial: widening pulse pressure, bradycardia, irregular breathing
RICP investigations and monitoring
Neuro imaging: CT/ MRI
Invasive ICP monitoring
Catheter placed into lateral ventricle of brain to monitor the pressure
May also be used to take collect CSF samples and also to drain small amounts of CSF to reduce the pressure
Cut-off of >20mmHg is often used to determine if further treatment is needed to reduce the ICP
RICP management
Investigate and treat underlying cause
Head elevation to 30degrees
IV mannitol for osmotic diuresis
Controlled hyperventilation, reduce pCO2-> vasoconstriciton of cerebral arteries-> reduced ICP. Rapid, temporary lowering of ICP
Removal of CSF:
Drain from intraventricular monitor
Repeated lumbar puncture
Ventriculoperitoneal shunt
Spinal muscular atrophy
Rare autosomal recessive condition
Progressive loss of motor neurones
Progressive muscular weakness
Affects LMN in spinal cord
Spinal muscular atrophy signs
LMN signs Fasciculations Reduced muscle bulk Reduced tone Reduced power Reduced or absent reflexes
SMA TY1
Onset in first few months of life
Usually progressing to death within 2 years
SMA TY2
Onset within the first 18 months
Most never walk, but survive into adulthood
SMA TY3
Onset after the first year of life
Most walk without support, but subsequently loose that ability
Respiratory muscles are less affected and life expectancy is close to normal
SMA TY4
Onset in 20s
Most will retain ability to walk short distances
May require wheelchair for motility
Everyday tasks can lead to significant fatigue
Respiratory muscles and life expectancy aren’t affected
Management of spinal muscle atrophy
MDT management
Physiotherapy: maximising strength in muscles and retaining respiratory function. Splints, braces and wheelchairs can be used to maximise function
Respiratory support with non-invasive ventilation, may be required to prevent hypoventilation and resp failure
SMA TY1: may require a tracheostomy with mechanical ventilation
Percutaneous endoscopic gastrostomy: unsafe swallow
