Neurology Flashcards
What is meningitis?
Inflammation of the arachnoid, pia and CSF.
Is meningitis normally viral or bacterial?
Usually viral
What are the viral/ bacterial causes of meningitis?
Most common is herpes simplex virus
Enteroviruses- coxsackie, echovirus, HIV
Bacterial- HAEMOPHILUS INFLUENZA TYPE B
Streptococcus agalactiae
Group B strep
Childhood- neisseria meningitidis, strep pneumoniae
What is the pathophysiology behind meningitis?
Bacteria and viruses tend to spread to the meninges via. The haemtogenous route, where they cause an inflammatory reaction.
The immune system is activated which causes an increase in cytokines
The increase in cytokines leads to an increased permeability of the blood brain barrier and also causes cerebral vasculature vasodilation (leading to reduced perfusion pressure and ischaemia)
The increased permeability of the blood brain barrier leads to cerebral oedema
The cerebral vasculature vasodilation can lead to a raised ICP.
How does meningitis lead to meningococcal septicaemia?
The bacteria releases an endotoxin that can result in
Increased vascular permeability which may lead to hypovolaemia
Coagulopathy which leads to petechiae and purpura
Myocardial dysfunction
Metabolic disturbance- acidosis, decrease in K+/Ca2+/MG2+
What are the risk factors for meningitis?
Maternal infection Premature rupture of membranes Low birthweight Prematurity Splenectomy Not immunised CSF shunt Immunocompromised
What are the signs and symptoms of meningitis?
Symptoms-
neck stiffness, photophobia (meningeal irritation)
Altered consciousness, N+V (cerebral oedema)
Cytokine release (Rigors, headache)
Signs-
Brudzinski sign, Kernig, seizures (meningeal irritation)
Decrease in GCS, bulging fontanelles, papilloedema
What are the signs and symptoms of meningococcal septicaemia?
Symptoms- hypovolaemia (dehydration) and non blanching rash
Signs- decrease in BP, increase in HR, petechiae and purpura
What investigations would you do in a child your suspecting of having meningitis?
Blood cultures, FBC, CRP, renal profile
LUMBAR PUNCTURE with CSF analysis is GOLD STANDARD (unless there are features of raised ICP!)
When you are doing lumbar puncture with CSF analysis, the glucose in the CSF is compared to glucose in plasma, so take plasma glucose levels at the same time.
What would the CSF analysis for bacterial meningitis likely show?
. The white cells present would be neutrophils
. Appearance would be turbid
. There would be an increased protein content
. There would be a decreased glucose content
What would the CSF sample for viral meningitis likely show?
Clear appearance
White cells would be lymphocytes
Protein content may or may not be increased
Glucose content may or may not be increased
What are the differential diagnoses for meningitis?
Any cause of sepsis Intracranial abscess Encephalitis Intracranial abscess Intracranial tumours Hydrocephalus
What are the differentials for meningitis?
Any cause of sepsis Intracranial abscess Encephalitis Intracranial tumours Hydrocephalus
What is the management of meningitis?
Do NOT delay starting empirical ABx whilst awaiting diagnostic tests
. A to E resuscitation
. IV ABx
. IV corticosteroids (if >3 months of age)
What are the complications of meningitis?
Sepsis, shock, SIADH. Ataxia/abscess DIC (disseminated intravascular coagulopathy) Retardation Epilepsy Paralysis Deafness
What is a febrile convulsion?
A seizure which is associated with fever and unrelated to other pathology.
What history would you expect with febrile convulsions?
Preceding febrile illness
A generalised tonic clinic seizure which lasts 5 minutes and less than 15 minutes
Full recovery within an hour
What are the investigations done for febrile convulsions?
So usually it will be just diagnosed clinically
Can do a lumbar puncture if suspecting something else
MSU and bloods
What are the differentials for convulsions?
If they are febrile- meningitis, encephalitis, sepsis, UTI
Not febrile- epilepsy, breath holding spells, reflex anoxic seizure, electrolyte disturbance, decreased glucose, epilsepsy
How do you manage febrile convulsions?
They can be managed at home
Advice on parental management
Manage fever (doesnt prevent recurrence)
What would the initial management of a seizure be in terms of parent/doctor?
Parents: protect the child, don’t restrain, recovery position after seizure, call 999 if it’s been more than 5 minutes.
Doctors: same as parents, of seizure is more than 5 mins then use IV lorazepam, this can be given 2x every ten mins
If IV lorazepam is unavailable then you can use buccal medazolam, if you cant get IV access then you can try get intraosseous
If lorazepam has been tried twice then you can try phenytoin IO/IV, this is an infusion
What is epilepsy?
Epilepsy is a common condition which affects the brain and causes frequent seizures (which are a burst of electrical activity in the brain, temporarily affecting how it works)
What is the pathophysiology behind epilepsy?
An increased activation of neurones or decreased inhibition can lead to an imbalance of being created, leading to an overall net excitation- paroxysmal discharge. The Brain region affected dictates the symptoms associated with the seizures.
What would the features of epilepsy be if it affected the frontal lobe?
If it affected the motor cortex- movement impairement
If it affected the frontal cortex- emotional/cognitive change
What features would you see if epilepsy affected the temporal lobe?
Auditory cortex- ringing or hissing/ hearing music
Wernickes area- dysphasia
Olfactory area- unusual taste or smell
Superior temporal gyrus- automatisms, lip smacking, chewing
What would the features of epilepsy be if the parietal lobe was affected?
If the sensory cortex was affected causing sensory disturbance
What are the features of epilepsy affecting the occipital lobe?
Flashes, Scotoma, blurring/ formed visual hallucinations
What are the features of epilepsy when the limbic system is affected?
Amygdala, thalamus, hypothalamus will be affected therefore you will get autonomic dysfunction.
What are the causes of epilepsy?
Risk factors…
. Learning disability
. First degree relative affected
Many aetiologies which may cause epilepsy…
. Idiopathic
Genetic syndromes- tuberous sclerosis, Rett syndrome and leader willi metabolic disease
Electrolyte disturbance
What is meant by tonic?
Stiffness in the limbs
What is meant by clinic?
Sustained rhythmical jerking of the limbs
What is meant by tonic clinic?
Initial stiffening which is followed by jerking.
What is atonic?
Sudden loss of muscle tone so the child will fall to the floor
What is meant by myoclonic?
A brief muscle jerk
What is meant by automatises?
Repetitive, purposeless actions (making sounds, picking at clothes, lip smacking, chewing) .
What are the clinical features of focal motor epilepsy?
Symptoms and signs will be up to a few minutes duration . Automatisms . Tonic . Clonic . Tonic clonic . Atonic . Myoclonic Above affects a specific region of the body
What are the clinical features of focal non motor epilepsy?
Altered sensation
An unsustainable smell/taste
Visual disturbance
Autonomic disturbance
What are the clinical features of a motor generalised seizure?
Loss of awareness
Associated with motor symptoms
Most common motor symptom is tonic clonic characterised by an initial rigid phase in which the child may bite their tongue
Followed by rhythmic jerking during which the child may lose control of the bladder and bowels.
Afterwards, there is a post ictal stage which lasts from 5-30 mins in which the child is drowsy and confused 🤷
What are the clinical features of a generalised (absence) non motor epilepsy?
Very brief period (<10s) during which the child
I’ll stops what they are doing, becomes unaware and stares into the distance. It self terminates and the child resumes what they were doing, there is no post ictal phase and it can be confused with day dreaming.
What is the treatment of focal seizure epilepsy?
Carbamazepine or lamotrigine
What is the treatment of generalised tonic clonic seizures?
Sodium valproate or lamotrigine
What is the treatment of generalised non motor (absence) epilepsy?
Ethosuximide or sodium valproate
What is the treatment of myoclonic/tonic/atonic?
Sodium valproate
What are the side effects of sodium valproate?
This can be remembered by VALPROATE V= valproate A= ataxia L= liver failure P= pancreatitis R= reversible hair loss O= oedema A= appetite increased T= teratogenic/thrombocytopenia E= enzyme inhibitor (CYP450)
What are two classifications of headaches?
Headaches can be classified as primary (attributable to a headache disorder) or secondary (due to another underlying disease)
What are examples of primary headaches?
Tension type
Cluster
Migraine
What are the examples of secondary headaches?
Trauma=post concussion headache
Intracranial bleeds
Fracture
MSK= temperomandibular joint, cervical spine
Vascular= subarachnoid (ruptured aneurysm), parenchymal (vascular malformation)
Raised ICP= space occupying lesion, hydrocephalus
Meningeal irritation- meningitis, encephalitis
Sinuses- sinusitis
What are the features of a tension headache?
Bilateral or unilateral
Tight
Unaffected by routine activity
What are the features of migraine?
May be unilateral or bilateral Pulsating Nausea and vomiting Photophobia, photophobia \+/- aura
What are the clinical features of a cluster headache?
Unilateral around the eye, stabbing throbbing and burning
You get a unilateral red eye
Epiphora
Nasal congestion and sweating
What are the features of sinusitis?
Usually frontal, usually there is facial tenderness, nasal congestion, sore ear, cough and fever
What clinical features would suggest encephalitis?
Fever
Altered mental status (Confused/drowsy)
+/- seizures
+/- decreased GCS
What are the clinical features of a space occupying lesion?
The headache may be localised or generalised
Headache will be worse in the mornings, worse with the valsalva manoeuvre, slowly worsening.
Pt may also experience vomiting, change in personality
How would you manage headaches?
Secondary- treat underlying cause
Tension type; paracetemol/NSAIDS
Migraine;
Acute= oral Triptan + NSAID or paracetemol +/- anti emetic
Prophylaxis= topiramate or propranolol
Cluster;
Acute= oxygen + SC/IM triptan
Prophylaxis= verapamil
What is muscular dystrophy?
An umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.
What is Gowers sign?
This is when children with proximal muscle weakness use a specific technique to stand up from a lying position.
They do this because because their muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms.
If there is a 5 year old boy presenting with vague symptoms of muscular weakness and the description is that you notice them using their hands on their legs to help them up, what would your diagnoses be and what is the underlying genetic inheritance of the most likely cause?
Duchennes muscular dystrophy
The inheritance is most likely X linked recessive
What is the cause of duchennes muscular dystrophy?
It is caused by a defective gene on the X chromosome, this gene normally codes for dystrophin which is a protein that holds muscles together. Due to girls having two X chromosomes girl carriers will not normally have symptoms whereas male carriers do.
When does duchennes muscular dystrophy normally show?
Usually shows around 3-5 years with weakness in the muscles around the pelvis? The weakness is progressive and eventually all muscles are affected. They are usually wheelchair bound by the time they are teenagers.
They have a life expectancy of about 25-35 :(
Can any treatment be given for duchennes muscular dystrophy?
Oral steroids can be shown to slow progression
Genetic trials are ongoing
Creatine supplements give a slight improvement in muscle strength.
What is Becker’s muscular dystrophy?
Very similar to duchennes, however the dystrophin gene is less severely affected and maintains some of its function.
Clinical course is less predictable than duchennes
What is myotonic dystrophy?
Genetic disorder which usually presents in adulthood
Progressive muscle weakness
Cataracts
Prolonged muscle contractions (unable to let go after shaking someone’s hand)
Cardiac arrhythmia.
What is facioscapulohymeral muscular dystrophy?
This usually presents in childhood with weakening around the face which spreads to the shoulders and arms.
A classic symptom is sleeping with their eyes slightly open and weakness in pursing their lips. They are unable to blow their cheeks out without air leaking from the mouth.
What is oculopharyngeal muscular dystrophy?
Usually presents with weakness in the muscles of the eyes and weakness of the pharynx- bilateral ptosis, restricted eye movement and swallowing problems.
What is limb girdle muscular dystrophy?
Usually presents in teenage years with progressive weakness around the limb girdles (the hips and the shoulders)
What is Emery Dreyfus muscular dystrophy?
Usually presents in childhood with contractures (shortening of the muscles and tendons which 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.
What is spinal muscular atrophy?
Rare autosomal recessive condition which causes a progressive loss of motor neurones, causing progressive muscular weakness.
What signs would you get with spinal muscular atrophy?
As it affects the lower motor neurones, it will lead to lower motor neurone signs... . Fasciculations . Weakness . Reduced muscle bulk . Reduced tone . Reduced power . Reduced reflexes
How do you categorise spinal muscular atrophy?
There are four categories from most to least severe. Type 2 is the most common
SMA type 1 has an onset in the first few months of life and usually progresses to death within 2 years
Type 2 never walk, survive into adulthood, onset is 18 months (most common)
Type 3 onset after the first year of life, most walk without support but then lose it
Resp muscles are not very affected and the life expectancy is actually close to normal
Type 4 has onset in the 20s most will retain the ability to walk short distances but require a wheelchair for mo ilium
Everyday task are tiring
Resp muscles and life expectancy are not affected
What is the management of spinal muscular atrophy?
Physio can be helpful in maximising strength in the muscles and retaining resp function
Splints, braces and wheelchairs can also be used
Resp support with non invasive ventilation may be required to help prevent hypoventilation and resp failure, Particularly during sleep.
Children with SMA type 1 may require a tracheostomy with mechanical ventilation
PEG feeding if they have a weak swallow
Headaches often raise the fear of brain tumours, what would be characteristic of headaches caused by brain tumours?
Worse when lying down Morning vomiting Nigh tame waking Change in mood, personality, educational performance Visual field defects Cranial nerve abnormalities Abnormal gait Torticollis Growth failure Papilloedema Cranial bruits Early or late puberty
What type of space occupying lesion would cause vision problems?
Pituitary rumour
Craniopharyngioma
What are the re flag symptoms for headaches?
The symptoms which suggest a space occupying lesion..l
Headache worse on lying down, with coughing or straining
A headache that wakes up the child
Any associated confusion and/or morning or persistent nausea or vomiting
Recent change in personality, behaviour, educational performance
What are the red flag physical signs for a space occupying lesion?
Growth failure Visual field defects- craniopharyngioma Squint Cranial nerve abnormality Torticollis Abnormal co ordination Gait Fundi- papilloedema Bradycardia Cranial bruits
What is a convulsion?
A seizure (epileptic or non epileptic with motor components.
What is status epilepticus?
An epileptic seizure which lasts 30 minutes or repeated epileptic seizures for 30 minutes Without recovery of consciousness.
How is an extradural haemorrhage usually caused?
Usually follows direct head trauma and is often associated with a skull fracture, it results from arterial or venous bleeding into the extra DURAL space.
What is the usual presentation of extradural haemorrhage?
Often a lucid interval until the conscious level deteriorates
Seizures secondary to the increasing size of the haematoma
May be: focal neurological signs with dilatation of the ipsilateral pupil, paresis of the contralateral limbs, false localising unilateral or bilateral 6th nerve paresis. (6th nerve is the abducens nerve which controls the movement of the lateral rectus muscle which is responsible for an outward gaze).
In young children, the first presentation may be with anaemia and shock.
How do you manage an extradural haemorrhage?
Correct the hypovolaemia, urgent evacuation of the HAEMATOMA, arrest of the bleeding.
What artery is usually affected in an extradural haemorrhage?
The middle meningeal artery as it passes through the Foramen spinosum of the sphenoid bone. It results from arterial or venous bleeding into the extradural space.
What type of haemorrhage is usually caused in NAI?
Subdural haemorrhage- tearing of the bridging veins as the cross the subdural space
Retinal haemorrhages are typical of shaking injury also
What other causes of subdural haematoma other than NAI are there?
Brain shrinkage through atrophy
Over draining of hydrocephalus
How does subarachnoid haemorrhage present?
This is actually much more commo;in adults than children
Presentation is usually with a severe headache with rapid onset (thunderclap), vomiting, confusion or a lowered level of consciousness, sometimes seizures and comma.
What are the usual causes of subarachnoid haemorrhage and how can you identify it?
The cause is often an aneurysm or arteriovenous malformation
Can be identified on MR angiography, CT of conventional angiography
How do you treat subarachnoid haemorrhage?
Interventional radiology
Neurosurgical
What is syncope?
Temporarily losing consciousness due to a disruption of blood flow to the brain, often leading to a fall. Syncopal episodes are also known as vasovagal episodes or simply as fainting.
What are the features that distinguish syncope from a seizure?
Syncope…
- prolonged upright position before the event
- lightheaded before event
- sweating before
- blurred vision
- reduced tone during episode
- return of consciousness shortly after falling
- no prolonged post ictal period
Seizure
- epilepsy aura (smells, tastes or de ja vu)
- head turning or abnormal limb positions
- tonic clonic activity
- tongue biting
- cyanosis
- lasting >5mins
- prolonged post ictal period
What investigations would you do for a child with syncope?
ECG
24 hour ECG if paroxysmal arrythmias are suspected
Echocardiogram if structural heart disease is susoexted
Bloods- including FBC (anaemia), electrolytes (arrythmias and seizures) and blood glucose (diabetes)
What advice can be given regarding vasovagal episodes?
Avoid dehydration
Avoid missing meals
Avoid standing for long periods
When experiencing prodromal symptoms then have some water or something to eat and rest until feeling better.
What are breath holding spells?
These are INVOLUNTARY episodes during which a child holds their breath, this is usually triggered by something upsetting and child will grow out of them by age 4/5.
What are the two types of breath holding spells to know?
Cyanotic breath holding spells
Reflex anoxic seizures
What is a reflex anoxic seizure?
These occur when the child is startled, child will let out a long cry, will stop breathing, become cyanotic and lose consciousness.
What is a reflex anoxic seizure?
When the child is startled, vagus nerve sends strong signals to the heart that cause it to stop beating.
The child will suddenly go pale, lose consciousness and may start to have some seizure like muscle twitching. Within 30 seconds the heart restarts and the child becomes conscious again.
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 cerebral palsy?
Permanent but not progressive neurological damage due to injury to the brain around the time of birth.
What are the causes of cerebral palsy?
Antenatal= maternal infections, traumatic birth
Perinatal= birth asphyxia, premature
Postnatal= trauma (NAI), meningitis, severe neonatal jaundice
What are the types of cerebral palsy?
Spastic (pyramidal)
Dyskinetic (extra pyramidal/athetoid)
Ataxic
Mixed
What are the patterns of cerebral palsy?
Monoplegia- one limb affected
Diplegia- four limbs affected, mostly the legs
Hemiplagia- one side of the body affected
Quadriplegia- four limbs are affected more severely, often with seizures, sleep disturbance and other impairments
What is the spastic type of cerebral palsy?
Hypertonia
(Increased tone) and reduced function resulting from damage to UMN, so you get tight and stiff muscles
What is the dyskinetic type of cerebral palsy?
This is due to damage of the basal ganglia
Athetoid movements
Hypertonia and hypotonia
What is ataxic type of cerebral palsy?
This is due to damage of the cerebellum and results in lack of co ordination.
What is the presentation of cerebral palsy?
Failure to meet milestones
Increased or decreased tone generally or in specific limbs
Hand preference below 18 months is a key thing to remember for exams
Problems with co ordination, speech, walking
Feeding or swallowing problems
Learning difficulties
What would a hemiplegic/diplegic gait indicate?
Indicates an upper motor neurone lesion
What would a broad based gait/ ataxic gait look like?
Indicates a cerebellar lesion
What does a high stepping gait indicate?
Indicates foot drop or a lower motor neurone lesion
What would a waddling gait?
Indicates pelvic muscle weakness due to myopathy
What does an antalgic gait indicate?
Indicates localised pain
What would an upper motor neurone look like?
On inspection muscle bulk would be preserved
Hypertonia
Brisk reflexes
Slightly reduced power
What would a lower motor neurone lesion look like?
On inspection there would be reduced muscle bulk with fasciculations
There would be hypotonia
Power would be dramatically reduced
Reduced reflexes
What gait would you get with cerebral palsy?
Diplegic/hemiplegic gait
What would be the differential for an UMN lesion other than cerebral palsy?
Brain tumour
Brain injury
What are the complications of cerebral palsy?
Learning disability Epilepsy Kyphoscoliosis Muscle contractures Hearing and visual inpairment GORD
What is the management of cerebral palsy?
Management should involve a multi disciplinary team approach
Physiotherapy
Occupational therapy (help patients with their day to day lives)
Speech and language therapy (help with speech and swallowing)
Dietitian’s help with their nutritional requirements
Orthopaedic surgeons perform procedures to release contractures or lengthen tendons (tenotomy)
Paediatrics optimise the childs meds
Social worker
Charities and support groups
What is squint?
This is misalignment of the eyes, it is also known as strabismus.
When there is misalignment the images on the retina do not match and the person will experience double vision.
What is amblyopia?
The brain copes with the misalignment in squint by reducing the signal from the less dominant eye. This results in the dominant dye being the one they use to see and one eye they ignore (the lazy eye).
What are the types of squint?
Concomitant- due to the differences in the control of extra ocular muscles whereas paralytic squints are due to paralysis in one or more of the extra ocular muscles?
What is meant by A) esotropia squint B) exotropia C) hypertropia D) hypotropia
Esotropia= in Exo= out Hyper= up Hypo= down
What are the causes of squint?
Usually idiopathic Can be caused by cerebral palsy Space occuping lesions like retinoblastoma Trauma Hydrocephalus
What would be included in the examination of a child with squint?
General inspection
Eye movements
Fundoscopy (red reflex)
Visual acuity
Hirschspergs and cover test
What is the management of squint?
Visual field are still developing until the age of 8, therefore treatment should start before this and the earlier the better.
An occlusive patch can be used to cover the good eye and force the weaker ones to develop.
An alternative to the patch may be atropine drops in the good eye, causing vision in that eye to be blurred.
Ophthalmologist should co ordinate management
What is hydrocephalus?
Cerebrospinal fluid building up excessively within the brain and spinal cord
This is a result of either over production of CSF or a problem with draining/absorbing CSF
What is the normal drainage physiology of CSF?
There are four ventricles in the brain- two lateral, a third and a fourth. The ventricles contain CSF which provides a cushion for the brain tissue. CSF is created in the four choroid plexuses (one in each ventricle) and by the walls of the ventricles. CSF is absorbed into the venous system by the arachnoid granulations
What is the most common cause of hydrocephalus?
Aqueductal stenosis
This is where the cerebral aqueduct which connects the third and fourth ventricle is stenosed (narrowed), this blocks the normal flow of CSF out of the third ventricle causing CSF to build up in the lateral and third ventricles.
What are other causes of hydrocephalus other than the most common?
Arachnoid cysts
Arnold chiari malformation
Chromosomal abnormalities/congenital malformations
How does hydrocephalus present?
Cranial bones in babies are not fused at the sutures until around 2 years of age, therefore the skull expands and babies will have an enlarged and rapidly increasing head circumference.
Other signs: bulging fontanelle, poor tone, poor feeding snd vomiting, sleepiness
How do you manage hydrocephalus?
VP shunt, this drains CSF from the ventricles into another body cavity.
Usually the peritoneal cavity is used
What are the complications of VP shunt?
Infection
Blockage
Excessive drainage
Intraventricular haemorrhage during the shunt related surgery
Outgrowing them- need them replacing around every 2 years as the child grows
What is craniosynostosis?
This is when skull sutures close prematurely, resulting in an abnormal head shape and restriction to the growth of the brain.
How do you diagnose craniosynotosis?
The first line is a skull X ray
CT head can be used to confirm the diagnosis or exclude it if there is doubt on the x ray
What is the management of craniosynostosis?
Surgical reconstruction
What is plagiocephaly and bracycephaly?
Very common conditions that cause abnormal head shapes in otherwise healthy babies
Plagio= oblique
Brachy= short
It tends to occur due to the baby resting their head on a particular point and has become more common as parents are advised to rest babies on their back to reduce SIDS.
What is the management of plagiocephaly and brachycephaly?
Exclude craniosynotosis
Look for congenital muscular torticollis (shortening of the SCM on one side)
Reassure
