Neurology Flashcards
What is syncope?
Temporary loss of consciousness due to a disruption of blood flow to the brain
What is syncope also known as?
Vasovagal episodes
Fainting
What causes a vasovagal episode?
A problem with the autonomic nervous system regulating blood flow to the bran.
What happens when the vagus nerve recieves a strong stimulus?
It stimulates the parasympathetic nervous system, causing the blood vessels in the brain to relax and the cerebral blood pressure to drop, leading to hypoperfusion of brain tissue
What is prodrome?
The events/ feelings prior to fainting
What symptoms may be included in prodrome?
Hot or clammy Sweaty Heavy Dizzy/ lightheaded Blurred/ dark vision Headache
What are the causes of primary syncope (simple fainting)?
Dehydration
Missed meals
Extending standing in warm environment
Strong stimuli
What events may strongly stimulate the vagus nerve?
Emotional event
Painful sensation
Change in temperature
What are the secondary causes of syncope?
Hypoglycaemia Dehydration Anaemia Infection Anaphylaxis Arrhythmias Valvular heart disease Hypertrophic obstructive cardiomyopathy
What are the key points to take in a syncope history?
Differentiate it from seizure Triggers? Concurrent ilnness? Injuries caused by colapse? Associated cardiac or neurological symptoms?
What investigations may be done after a vasovagal episode?
ECG
24 hour ECG
Echo
Bloods (FBC, electolytes, blood glucose)
What is epilepsy?
Umbrella term for the tendency to have seizures
What are seizures?
Transient episodes of abnormal electrical activity in the brain
What are the different types of seizure?
Generalised tonic-clonic Focal Absence Atonic Myoclonic Infantile spasms Febrile convulsions
What is the tonic phase?
Muscle tensing
What is the clonic phase?
Muscle jerking
What phase comes first in a generalised tonic-clonic seizure?
Tonic phase
What may also occur in a tonic-clonic seizure?
Tongue biting, incontinence, groaning and irregular breathing
What happens after a tonic-clonic seizure?
Prolonged post-ictal period
What happens in the post-ictal period?
Person is confused, drowsy and feels irritable/ low
What is the first line management of tonic-clonic seizures?
Sodium valproate
What is the second line management of tonic-clonic seizures?
Lamotrigine or carbamazepine
What are focal seizures?
Seizures that affect hearing, speech, memory and emotions
Where to focal seizures start?
In the temporal lobes
What are the different ways focal seizures can present?
Hallucinations
Memory flashbacks
Deja Vu
Strange actions on autopilot
What is the first line management of focal seizures?
Carbamazepine or lamotrigine
What is the second line management of focal seizures?
Sodium valproate or Levetiracetam
What are absence seizures?
When patient becomes blank, stares into space and then abruptly returns to normal
Who most commonly gets absence seizures?
Children
How long do absence seizures typically last?
10-20 seconds
What is the first line treatment for absence seizures?
Sodium valproate
What are atonic seizures?
Drop attacks- brief lapses in muscle tone
How long to atonic seizures usually last?
Less than 3 minutes
What syndrome may be the cause of atonic seizures?
Lennox-Gastaut syndrome
What is the first and second line management of atonic seizures?
First line= Sodium valproate
Second line= Lamotrigine
What are myoclonic seizures?
Sudden brief muscle contractions
When do myoclonic seizures usually manifest?
In children as part of juvenile myoclonic epilepsy
What is the management of myoclonic seizures?
1st line= sodium valproate
2nd= lamotrigine/ levetiracetam/ topiramate
What are infantile spasms?
Clusters of full body spasms
What are infantile spasms also known as?
West syndrome
When do infantile spasms usually start?
Around 6 months
What is the prognosis of infantile spasms?
1/3 die by 25
1/3 are seizure free
What are febrile convulsions?
Seizures that occur in children whilst they have a fever
In what age range do febrile convulsions begin?
6 months- 5 years
What key investigations may be done into epilepsy?
EEG
MRI brain
What is an EEG and when would it be performed?
Electroencephalogram, performed after the second instance of simple tonic-clonic seizure
When would an MRI brain be considered?
If the first seizure is in a child <2
Focal seizures
No response to medications
What can an MRI brain help rule out?
Structural problems and pathology such as tumours
What additional investigations may be considered to exclude causes of seizures?
ECG Blood electrolytes Blood glucose Blood cultures Urine cultures LP
What general advice is given to patients/ families with new epilepsy diagnosis?
Don’t take baths
Be cautious with swimming, heights, traffic and heavy/ electrical equipment
Avoid driving
What is the first line medication for most forms of epilepsy?
Sodium valproate
Which form of epilepsy is sodium valproate not the first line in?
Focal seizures
What is the action of sodium valproate?
Increases the activity of GABA, which has a relaxing effect on the brain
What are the key side effects of sodium valproate ?
Teratogenic
Liver damage
Hair loss
Tremor
In which patients should sodium valproate be avoided and why?
Girls because it is severely teratogenic
What is the first line medication for focal seizures?
Carbamazepine
What are the notable side effects of carbamazepine?
Agranulocytosis
Aplastic anaemia
Many drug interactions
What are the notable side effects of Phenytoin?
Folate & vitamin D deficiency
Megaloblastic anaemia
Osteomalacia
What are the notable side effects of Ethosuximide?
Night terrors
Rashes
What are the notable side effects of Lamotrigine?
Stevens-Johnson syndrome
DRESS syndrome
Leukopenia
How should you manage someone having a seizure?
Safe position
Put something soft under head
Remove obstacles/ danger
Make note of start and end
When do you call an ambulance with a seizure?
If it lasts more than 5 minutes or is their first seizure
What is status epilepticus?
Medical emergency- seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness
How is status epilepticus managed in hospital?
ABCDE
IV lorazepam
IV phenytoin if seizure persists
What are simple febrile convulsions?
Generalised tonic, clonic seizures caused by a high fever
How long do simple febrile convulsions last?
Less than 15 minutes
What are complex febrile convulsions?
Partial or focal seizures, lasting more than 15 minutes or occurring multiple times during the same febrile illness
What are the differential diagnoses of a febrile convulsion?
Epilepsy Meningitis/ encephalitis Intracranial space occupying lesions (brain tumour/ haemorrhage) Syncopal episode Electrolyte abnormalities Trauma
What is the typical presentation of a febrile convulsion?
18 month year old child presenting with 2-5 minute tonic clonic seizure during high fever
How are febrile convulsions managed?
Identify underlying source of infection
Paracetamol/ ibuprofen
What are breath holding spells?
Involuntary episodes during which a child holds their breath
What usually triggers breath holding spells?
Something upsetting or scary
In what age range do breath holding spells usually occur?
6-18 months
What are the two types of breath holding spells?
Cyanotic breath holding spells
Pallid breath holding spells
What are cyanotic breath holding spells?
When the child gets really worked up, lets out a long cry, then stops breathing, becomes cyanotic and loses consciousness
What are reflex anoxic seizures?
When the child is startled, the vagus nerve sends strong signals to the heart that causes it to stop beating/
What happens after a cyanoti breath holding spell?
The child regains consciousness and starts breathing within a minute
What happens during a reflex anoxic seizure?
The child goes pale, loses consiousness and may have some muscle twitching
How long do reflex anoxic seizures typically last?
30 seconds
How are breath holding spells managed?
Exclude other pathology
Treat any iron deficiency anaemia
Educate and reassure
What are the causes of headaches in children?
Tension headaches Migraines ENT infection Analgesic headache Vision problems Raised ICP Brain tumours Meningitis Encephalitis Carbon monoxide poisoning
What is the main cause of headaches in children?
Tension headaches
What are tension headaches?
Common type of headache producing mild ache across forehead (band- like pressure)
What may trigger tension headaches?
Stress, fear or discomfort
Skipping meals
Dehydration
Infection
How are tension headaches managed?
Reassurance Analgesia Regular meals Avoid dehydration Reduce stress
How long do tension headaches typically last in children?
30 minutes
What are the different types of migraine?
Migraine with or without aura
Silent migraine
Hemiplegic migraine
Abdominal migraine
What is a silent migraine?
Migraine with aura but without headache
What is a hemiplegic migraine?
Migraine that causes temporary weakness down one side of the body
What are the common presenting features of a migraine?
Unilateral, severe, throbbing pain Often photo/ phonophobia Visual auras N&V Abdominal pain
How are migraines in children managed supportively?
Rest/
Fluids
Low stimulus environment
What medications can be used to manage migraines in children?
Paracetamol
Ibuprofen
Sumatriptan
Antiemetics
What medications can be used as migraine prophylaxis?
Propanolol
Pizotifen
Topiramate
What are abdominal migraines?
Central abdominal pain lasting more than 1 hour. Tend to occur in young children before developing traditional migraines when they get older
What is cerebral palsy?
Permanent neurological problems resulting from damage to the brain around the time of birth
What are the antenatal causes of cerebral palsy?
Maternal infections
Trauma during pregnancy
What are the perinatal causes of cerebral palsy?
Birth asphyxia
Pre-term birth
What are the different types of cerebral palsy?
Spastic
Dyskinetic
Ataxic
Mixed
What is spastic CP?
Hypertonia and reduced function
What is dyskinetic CP?
Problems controlling muscle tone (both hyper and hypotonia), causing athetoid movements and oro-motor problems
What causes spastic CP?
Damage to upper motor neurones
What causes dyskinetic CP?
Damage to basal ganglia
What is ataxic CP?
Problems with coordinated movement resulting from damage to the cerebellum
What is spastic CP also known as?
Pyramidal CP
What is dyskinetic CP also known as?
Athetoid or extrapyramidal CP
What are the different patterns of spastic cerebral palsy?
Monoplegia
Hemiplegia
Diplegia
Quadraplegia
What is hemiplegia?
One side of the body affected
What is diplegia?
When all four limbs are affected, but mostly the legs
What are the signs and symptoms of CP during development?
Failure to meet milestones Increased/ decreased tone *Hand preference before 18 months Problems with coordination, speech or walking Feeding/ swallowing problems Learning difficulties
What gait may you find on examination of a child with CP?
Hemiplegic/ diplegic Ataxic High stepping Waddling Antalgic gait (limp)
What may be found on neurological examination of a patient with an upper motor neurone cerebral palsy?
Hemipledic/ diplegic gate
Hypertonia
Slightly reduced power
Brisk reflexes
What may be found on neurological examination of a patient with a lower motor neurone cerebral palsy?
High stepping gate Reduced muscle bulk with fasciculations Hypotonia Dramatically reduced power Reduced reflexes
What is a hemiplegic/ diplegic gait?
When there is increased muscle tone and spasticity in the legs, caused them to be extended with plantar flexion. This means they must swing leg around in a large semicircle.
What are the complications/ conditions associated with cerebral palsy?
Learning disability Epilepsy Kyphoscoliosis Muscle contractures Hearing/ visual impairment GORD
How is cerebral palsy managed?
MDT: Physiotherapy OT SALT Dieticians Paediatricians Social workers Orthopaedic surgeons
What is strabismus?
Misalignment of the eyes
What will a person with strabismus experience?
Double vision
What is amblyopia?
Lazy eye (affected eye has reduced function compared to dominant eye)
What is esotropia?
Inward positioned squint
What is exotropia?
Outward positioned squint
What is hypertropia?
Upward moving affected eye
What is hypotropia?
Downward moving affected eye
What are the causes of strabismus?
Idiopathic Hydrocephalus Cerebral palsy Space occupying lesion Trauma
What investigations do you do into strabismus?
General inspection Eye movement Fundoscopy Visual acuity Hirschberg's test Cover test
What is Hirschberg’s test?
Shine pen-torch at patient from 1 meter away. When they look at it, observe reflection of light source on their cornea (should be central and symmetrical)
What is a cover test?
Cover one eye and ask patient to focus on an object in front of them. Move the cover across the opposite eye and watch movements of previously covered eye. (Will show exotropia and esotropia)
How is strabismus managed?
Earlier the better Occlusive patch Atropine drops (blurs vision in good eye) Glasses Surgery Botox
What is hydrocephalus?
Abnormal build up of CSF in the brain and spinal cord
What causes hydrocephalus?
An overproduction of CSF or a problem with draining or absorbing it
How many ventricles are there in the brain and what are they?
4
Two lateral ventricles, third and fourth
What do the brain ventricles contain?
CSF
Where is CSF created?
In the four choroid plexuses (in each ventricle) and by the walls of the ventricles
Where is CSF absorbed?
Into the venous system by the arachnoid granulations
What is the most common cause of hydrocephalus?
Aqueductal stenosis, leading to insufficient drainage
What is the cerebral aqueduct?
Passage connecting the third and fourth ventricle
What happens in aqueductal stenosis?
There is stenosis of the aqueduct connect the third and fourth ventricle, blocking the normal flow of CSF out of the third ventricle, causing it to build up in the lateral and third ventricles
What are other congenital causes of acqueductal stenosis?
Arachnoid cysts
Arnold-Chiari malformation
Chromosomal abnormalities
Congenital malformations
What is an Arnold-Chiari malformation?
Where the cerebellum herniates downwards through the foramen magnum, blocking the outflow of CSF
At what age do the cranial sutures fuse?
2 years
How does hydrocephalus present in babies?
Enlarged and rapidly increasing head circumference Bulging anterior fontanelle Poor feeding/ vomiting Poor tone Sleepiness
What is the mainstay of treatment for hydrocephalus?
VP shunt
What is a VP shunt?
Catheter placed into ventricle and drains CSF into another body cavity (usually peritoneal)
Why is the peritoneal cavity the most commonly used to drain CSF?
There is plenty of space and it is easily reabsorbed
What are the complications of a VP shunt?
Infection Blockage Excessive drainage Intraventricular haemorrhage Outgrowing shunt
How often do VP shunts need replacing?
Every 2 years
What is craniosynostosis?
When the skull sutures close prematurely
What does craniosynostosis cause if left untreated?
Raised intracranial pressure
Abnormal head shapes
Restriction in brain growth
What is the main presenting feature of craniosynostosis?
Abnormal head shape
small head in proportion to body, anterior fontanella closure before 1
What is the head shape in saggital synostosis?
Long and narrow from front to back
What is the head shape in coronal synostosis?
Bulging on one side of forehead
What is the head shape in Metopic synostosis?
Pointy triangular forehead
What is the head shape in lambdoid synostosis?
Flattening on one side of the occiput
What investigations are done for suspected craniosynostosis?
Skull Xray
CT head
How are mild cases of craniosynostosis managed?
Monitored and followed up over time
How are more severe cases of craniosynostosis managed?
Surgical reconstruction of the skull
What is plagiocephaly?
Flattening in an area of the baby’s head
What is brachycephaly?
Flattening at the back of the head resulting in a short head from back to front
What causes plagio/brachycephaly?
Positional plagiocephaly: When a baby has a tendency to rest their head on a particular point, so the bones and sutures mould with gravity
When and how do babies usually present with How are mild cases of plagiocephaly?
3-6 months with abnormal head shape
What must be excluded when diagnosing plagiocephaly?
Craniosynotosis
Congenital muscular torticollis
What is congenital muscular torticollis?
Shortening of the sternocleidomastoid muscle on one side
How is plagiocephaly managed?
Encourage baby to avoid resting on flattened area
Plagiocephaly helmets
What is muscular dystrophy?
Umbrella term for genetic conditions that cause gradual weakening and wasting of muscles
What is the main type of muscular dystrophy?
Duchennes muscular dystrophy
What are other types of muscular dystrophy?
Beckers Myotonic Facioscapulohumeral Oculopharyngeal Limb-girdle Emery-Dreifuss
What sign will be present in children with proximal muscle weakness?
Gower’s sign
What is Gower’s sign?
When standing up from sitting, they get on their hands and knees, then push their hips up and backwards, before transferring weight backwards and transferring hands to knees
How is muscular dystrophy managed?
MDT:
OT, PT, equipment (wheelchairs)
Surgical/ medical management of complications (e.g. scoliosis, heart failure)
What kind of inheritance pattern is duchennes muscular dystrophy?
X-linked recessive
What causes Duchennes muscular dystrophy?
Defective gene for dystrophin on X-chromosome
What is Dystrophin?
Protein that helps hold muscles together at the cellular level
In which patients does Duchennes muscular dystrophy pretty much solely present and why?
Boys- girls have a spare X chromosome
At what age does Duchennes usually present?
3-5
How does Duchennes usually first present?
With weakness in muscles around pelvis
What is the prognosis of Duchennes?
Progressive disease and eventually all muscles will be affected
Wheelchair bound by teenager
Life expectancy of 25-35 years
What can be used to slow the progression of muscle weakness in Duchennes?
Oral steroids
Createnine can improve muscle strength
How does Beckers muscular dystrophy differ from Duchenens?
Dystrophin gene is less severely affected and maintains some function
Presents later and less predictable
What are the typical features of myotonic dystrophy?
Progressive muscle weakness
Prolonged muscle contractions
Cataracts
Cardiac arrhythmias
What is the key feature of myotonic dystrophy to remember?
Prolonged muscle contraction- e.g. may be unable to let go after shaking hands or opening doorknob
What kind of condition is myotonic dystrophy and when does it typically present?
Genetic disorder
Presents in adulthood
What is facioscapulohumeral muscular dystrophy?
Weakness around face, progressing to shoulderns and arms
When and how does facioscapulohumeral muscular dystrophy usually present?
In childhood with child sleeping with eyes slighlty open and weakness in pursing lips
What is oculopharyngeal muscular dystrophy?
Weakness of ocular muscles and pharynx
When and how does oculopharyngeal muscular dystrophy usually present?
Bilateral ptosis, restricted eye movements and swallowing problems in late adulthood
What is limb-girdle muscular dystrophy?
Progressive weakness around the limb girdles (hips and shoulders) presenting in teenage years
What is Emery-Dreifuss muscular dystrophy?
Dystrophy presenting in childhood with contractures in elbows and ankles, as well as progressive muscle weakness and wasting
What is spinal muscular atrophy?
Rare genetic condition causing progressive loss of motor neurones, leading to progressive muscular weakness
What kind of genetic condition is spinal muscular atrophy?
Autosomal recessive
What neurones does SMA affect?
Lower motor neurones in spinal cord
What lower motor neurone signs will you get in SMA?
Fasciculations Reduced muscle bulk Reduced tone Reduced power Reduced/ absent reflexes
What are the 4 categories of spinal muscular atrophy?
SMA type 1, 2 4 & 4
What is SMA type 1?
Onset in first few months of life and progresses to death within 2 years
What is SMA type 2?
Onset within first 18 months of life. Most never walk but survive into adulthood
What is SMA type 3?
Onset after first year of life. Most walk without support but lose ability. Life expectancy close to normal
What is SMA type 4?
Onset in 20s. Most retain ability to walk short distances but everyday tasks can lead to significant fatigue
How is spinal muscular atrophy managed?
Supportive with MDT
What may be required to prevent respiratory failure in those with SMA?
Non-invasive ventilation
Tracheostomy with mechanical ventilation (SMA T1)
