Paeds - neuro (textbook + 0-finals) Flashcards

1
Q

Headaches are sometimes split into primary, secondary and then other (cranial neuralgias, central and primary facial pain, etc)

Name some differentials for these groups

A

Primary headaches

  • migraine, tension type, cluster and other trigeminal autonomic cephalalgias, and other primary headaches

Secondary headaches = symptomatic of underlying pathology

  • medication overuse headache
  • secondary to head/neck trauma
  • cranial or cervical vascular disorder (ICH, vascular malformation, etc)
  • Non-vascular intracranial disorder (raised ICP, idiopathic intracranial htn)
  • substance misuse or withdrawal (alcohol, solvent, drug abuse)
  • infection (meningitis, encephalitis, abscess)
  • disorder of homeostasis (hypercapnia or htn)
  • disorder of facial or cranial structures (acute sinusitis)
  • associated with emotional disroders

Others

  • trigeminal and other cranial neuralgias and central causes of facial pain
  • other headaches
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

How would a tension headache present?

patient.info

A
  • a tight symmetrical band of pain across the forehead and around both sides of the head
  • nonspecific symptoms in younger children (quiet, stop playing, become tired)
  • last from a few hours to several days (resolve more quickly in chidlren, typically within 30 mins)
  • mild/mod pain (can be severe) but does not typically disturb sleep
  • usually gradual onset in the day, worsen as the day goes on (mildest in the morning)
    • unless the headache is caused by physical tension or tightness in the muscles at the back of neck and over scalp (eg. due to poor sleeping posture)
  • can worsen from emotional tension, fear, discomfort, tiredness, anxiety, hunger
  • can worsen from too much caffeine or alcohol or dehydration
  • not usually worse from physical activity
  • can be triggered by infection in children
  • most people work through it but eye strain can worsen it
  • can be sensitive to bright light or noise
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How would you manage a tension headache?

A

Reassurance, analgesia

regular meals, avoid dehydration

reduce stress

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How do migraine symptoms differ to tension headache symptoms?

A
  • unilateral
  • more severe
  • throbbing/ pulsing in nature
  • take longer to resolve

typically associated with…

  • visual aura
  • photophobia and phonophobia
  • nausea and vomiting
  • abdo pain
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How would you manage and prevent migraines in children?

A

Management:

  • rest, fluids, low stimulus environment
  • paracetamol
  • ibuprofen
  • sumatriptan
  • antiemetics eg. domperidone

Migraine prophylaxis = If migraines are freq or having a significant impact eg. missing lots of school…

  • propanolol (avoid in asthma)
  • pizotifen (SE = drowsiness)
  • topiramate (teratogenic so girls of child bearing age need contraception)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is an abdominal migraine?

A

Abdo migraines can occur in young children before they develop traditional migraines as they get older.

  • present with episodes of central abdo pain lasting >1 hour
  • examination will be normal
  • associated nausea/vomiting, anorexia, headache, pallor

If patient presents with possible migraines, ask about recurrent central abdo pain as a kid.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Infections are a common cause of headaches in kids.

The headaches typically resolve along with the infection.

What are 4 common ones?

A

Viral Upper Respiratory tract infections

Otitis Media

Sinusitis (usually viral)

  • inflammation in ethmoidal, maxillary, frontal or sphenoidal sinuses
  • produces facial pain behind nose, forehead, eyes
  • tenderness over affected sinuses helps to diagnose
  • resolves in 2-3 weeks

Tonsillitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is a seizure?

A

transient episodes of abnormal electrical activity in the brain.

There are many different types:

  • Generalised tonic-clonic seizures
  • Focal seizures
  • Absence Seizures
  • Atonic seizures
  • Myoclonic seizures
  • infantile spasms
  • febrile convulsions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is a generalised tonic-clonic seizure?

(Grand mal seizures)

A
  • loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) movements
    • tonic phase typically comes before the clonic phase
  • Can be associated tongue biting, incontinence, groaning and irregular breathing
  • After the seizure there is a prolonged post-ictal period (confusion, drowsiness, irritable or low)

Management = sodium valproate 1st line

2nd line = lamotrigine or carbamazepine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is a focal seizure?

A
  • seizures that start in the temporal lobes
  • affect hearing, speech, memory, emotions
  • can present with various symptoms…
    • hallucinations
    • memory flashbacks
    • deja vu
    • doing strange things on autopilot

Management (reverse of tonic-clonic) = carbamazepine or lamotrigine 1st line

2nd line = sodium valproate or levetiracetam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are absence seizures?

A
  • patient becomes blank, stares into space and then abruptly returns to normal
  • during episdoes, they are unaware of their surroundings and won’t respond
  • episodes typically last 10-20 seconds
  • episodes typically stop happening as kids get older

Management = sodium valproate or ethosuximide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is an atonic seizure?

A
  • characterised by brief lapses in muscle tone.
  • they don’t usually last more than 3 minutes
  • typically begin in childhood and can indicate Lennox-Gastaut syndrome

Management = sodium valproate 1st line

2nd line = lamotrigine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is a myoclonic seizure?

A
  • they present as sudden brief muscle contractions like a suden “jump”
  • patient usually remains awake during the episode
  • typically happen in children as part of juvenile myoclonic epilepsy

Management = sodium valproate 1st line

2nd line = lamotrigine or levetiracetam or topiramate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are infantile spasms?

A
  • also known as West Syndrome (a rare disorder starting in infancy at around 6 months old)
  • characterised by clusters of full body spasms
  • poor prognosis (1/3 die by 25, however 1/3 are seizure free)

Management = prednisolone and vigabatrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How would you investigate epilepsy?

A
  • Electroencephalogram (EEG)
    • perform an EEG after the second simple tonic-clonic seizure
    • children are allowed one simple seizure before being investigated for epilepsy
  • MRI brain to visualise structural problems or other pathology like tumours. Consider this when…
    • first seizure is under 2 years old
    • focal seizures
    • no response to 1st line anti-epileptics

Others to consider…

  • ECG to exclude heart pathology
  • U&Es (Na+, K+, Ca2+, Mg2+)
  • Blood glucose for hypoglycaemia and diabetes
  • Blood cultures, urine cultures, LP if sepsis/encephalitis/meningitis is suspected
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How would you educate patients of epilepsy?

(Epilepsy is an umbrella term for a condition where there is a tendency to have seizures)

A

educate patients and families about safety precautions, recognising/managing further seizures, avoiding triggers…

  • take showers rather than baths
  • be cautious about swimming unless seizures are well controlled and they are closely supervised
  • be cautious about heights
  • be cautious about traffic
  • be cautious with hot/heavy/electrical equipment
  • avoid driving unless they meet specific criteria (DVLA for teens)

Generally valproate for generalised and Carbamazepine for focal.

Rescue medication = Buccal Midazolam/ PR Diazepam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are some common anti-epileptics you would prescribe?

A

Anti-epileptic medications…

  • sodium valproate is 1st line for most except focal seizures
    • increases activity of GABA
    • SE = teratogenic, liver damage, hepatitis, hair loss, termour
  • Carbamazepine is 1st line for focal seizures
    • SE = agranulocytosis, aplastic anaemia, induces P450 system so many drug interactions
  • Phenytoin
    • SE = folate and vit D deficiency, megaloblastic anaemia (folate), osteomalacia (vit d)
  • Ethosuximide
    • SE = night terrors, rashes
  • Lamotrigine
    • SE = stevens-johnson syndrome or DRESS syndrome, leukopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What would you do if you saw someone having a seizure?

A
  • put them down in a safe position eg. on a carpeted floor
  • place in recovery position
  • put something soft under their head to protect against head injury
  • remove obstacles that could lead to injury
  • make note of the time at start and end
  • call an ambulance if seizure lasts more than 5 mins or this is their first seizure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is status epilepticus and what would you do?

A

A medical emergency!!!

A seizure lasting more than 5 minutes or more than 3 seizures in 1 hour

Management in hospital…

  • A-E
  • IV lorazepam, repeat after 10 mins if seizure continues
  • If seizure persists, then try an infusion of IV phenobarbital or phenytoin
    • at this point, consider intubation and ventilation to secure the airway
    • consider transfer to ICU if appropriate
20
Q

What are the medical options to treat seizures in community?

A
  • Buccal midazolam
  • Rectal (PR) diazepam
21
Q

What is a febrile convulsion?

A

Seizures that occur in children with a high fever.

Only occur in kids between 6 months old and 5 years old.

Simple febrile convulsions = generalised tonic clonic seizures (last <15 mins and only occur once during a single febrile illness)

Complex febrile convulsions = consist of partial or focal seizures (last >15 mins or occur multiple times during the same febrile illness)

22
Q

How would you diagnose febrile convulsions?

A

You need to rule out other neurological pathology.

Other differentials are…

  • epilepsy
  • meningitis, encephalitis, or other neuro infection like cerebral malaria
  • intracranial space occupying lesions eg. brain tumours or ICH
  • syncopal episode
  • electrolyte abnormalities
  • trauma (also think about non-accidental injuries in kids)

The typical presentation is of a kid presenting with 2-5mins tonic clonic seizures during a high fever.

Fever is usually caused by an underlying viral/bacterial illness like tonsillitis.

23
Q

How would you manage febrile convulsions?

A
  • identify and manage the underlying source of infection
  • control the fever via simple analgesia like paracetamol and ibuprofen
  • simple febrile convulsions = reassure and educate parents
  • complex febrile convulsions may need further investigation

Advise the parents for further seizures…

  • stay with child
  • put child in a safe place eg. on a carpeted floor with pillow under head
  • place them in recovery position away from obstacles that can injur
  • don’t put anything in their mouth
  • call an ambulance if seizure lasts >5 mins
24
Q

What is muscular dystrophy?

A

An umbrella term for genetic conditions that cause gradual weakening and wasting of muscles.

Do a muscle biopsy to confirm.

Types:

  • Duchennes muscular dystrophy
  • Beckers muscular dystrophy
  • Myotonic dystrophy
  • facioscapulohumeral muscular dystrophy
  • oculopharyngeal muscular dystrophy
  • limb-girdle muscular dystrophy
  • emery-dreifuss muscular dystrophy
25
Q

What is Duchennes muscular dystrophy?

(most important for exams)

A
  • caused by a defective gene for dystrophin on x-chromosome
  • dystrophin is the protein that holds muscles together at the cellular level
  • females have a spare x chromosome that may not have a defective dystrophin gene, so female carriers are typically asymptomatic
    • therefore Duchennes muscular dystrophy is X-Linked Recessive
  • Presents with Gowers sign (walking hands up legs)
  • Boys with Duchennes present around 3-5 years old with…
    • progressive weakness in muscles around their pelvis
    • become wheelchair bound before puberty
    • die of resp failure in early 20s

Management = oral steroids slow progression of muscle weakness. Creatine supplements improve muscle strength

26
Q

What is Beckers Muscular Dystrophy?

A
  • similar to duchennes but the dystrophin gene is less severely affected and maintains some function
  • symptoms appear around 8-12 years old
  • some are wheelchair bound by late teens, some may walk with assistance into later adulthood
  • survive til 30s
  • management = oral steroids, creatine supplements
27
Q

What is myotonic dystrophy?

A
  • a genetic condition that presents in adulthood…
    • progressive muscle weakness
    • prolonged muscle contractions
      • eg. patient is unable to let go after shaking someones hand, unable to release their grip on a doorknob after opening a door, etc
    • cataracts
    • cardiac arrhythmias
28
Q

What is facioscapulohumeral muscular dystrophy

A
  • presents in childhood with weakness around the face, progressing to shoulders and arms
  • classical initial symptom = sleeping with eyes slightly open and weakness in pursing their lips
    • unable to blow their cheeks out without air leaking from their mouth
29
Q

What is oculopharyngeal muscular dystrophy?

A
  • presents in late adulthood with weakness of the ocular muscles and pharynx
    • bilateral ptosis
    • restricted eye movement
    • swallowing problems
    • muscles around limb girdles are also affected
30
Q

What is limb girdle muscular dystrophy?

A

usually presents in teenage years with progressive weakness around the limb girdles (hips and shoulders)

31
Q

How does Emery-Dreifuss Muscular Dystrophy present?

A
  • presents in childhood with contractures most commonly in elbows and ankles
  • patient also suffers progressive weakness and wasting of muscles, starting with upper arms and lower legs
32
Q

What is Gower’s sign?

A

a sign in children with proximal muscle weakness

  • from a lying position, these kids will get onto their hands and knees, push their hips up and backwards like the “downward dog” yoga post.
  • then they will shift their weight backwards and transfer their hands to their knees
  • whilst keeping legs mostly straight, they walk their hands up their legs to get their upper body erect
  • this is because the muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms
33
Q

How would you manage muscular dystrophies?

A

just try and give them the highest quality of life for the longest time

  • occupational therapy
  • physiotherapy
  • medical appliances like wheelchairs and braces
  • treat complications like spinal scoliosis and heart failure
34
Q

What is spinal muscular atrophy?

A

An autosomal recessive condition that causes a progressive loss of motor neurones, resulting in progressive muscular weakness.

  • affects the lower motor neurones in the spinal cord

LMN signs…

  • fasiculations
  • reduced muscle bulk
  • reduced tone
  • reduced power
  • reduced or absent reflexes
35
Q

What are the 4 categories of spinal muscular atrophy?

A

SMA type 1 = onset in the first few months of life, progresses to death within 2 years

SMA 2 = onset within the first 18 months, most never walk but survive into adulthood

SMA 3 = onset after first year of life, most walk without support but will lose that ability. Resp muscles are less affected, life expectancy is close to normal

SMA 4 = onset in the 20s, most retain the ability to walk short distances but require a wheelchair for mobility. Everyday tasks can cause significant fatigue. Resp muscles and life expectancy not affected.

36
Q

How would you manage spinal muscular atrophy?

A
  • physiotherapy to maximise strength in muscles and retain resp function
    • learn to use splints, braces, wheelchairs to maximise function
  • Resp support with non-invasive ventilation to prevent hypoventilation and resp failure (especially during sleep)
    • SMA 1 may require a tracheostomy with mechanical ventilation to extend life by supporting failing resp muscles
  • Percutaneous endoscopic gastrostomy (PEG feeding)
    • required when a weak swallow makes swallowing unsafe
37
Q

What is the pathophysiology of hydrocephalus?

A

This is when cerebrospinal fluid build up within the brain and spinal cord.

It is a problem of overproduction of CSF or a problem with draining or absorbing CSF.

  • usually CSF is produced by the walls of the ventricles and the 4 choroid plexuses (1 in each of the 4 ventricles = 2 lateral, 3rd and 4th ventricle)
  • CSF is usually absorbed into the venous system by the arachnoid granulations
38
Q

What are some common causes of hydrocephalus?

A
  • most common = aqueductal stenosis
    • insufficient draining of CSF
    • the cerebral aqueduct (connects 3rd and 4th ventricle) is stenosed so causes CSF to build up in the lateral and 3rd ventricles
  • large arachnoid cysts can block outflow of CSF
  • Arnold-Chiari malformation
    • cerebellum herniates downwards through foramen magnum, blocking outflow of CSF
  • Chromosomal abnormalities and congenital malformations can also cause obstruction to CSF drainage
39
Q

How would hydrocephalus present?

A
  • cranial bones in babies are not fused at the sutures until around 2 years old
    • therefore skull is able to expand to fit the cranial contents
    • when a baby has hydrocephalus it causes outward pressure on the cranial bones
    • therefore hydrocephalus babies have an enlarged and rapidly increasing head circumference (occipito-frontal circumference)
  • Bulging anterior fontanelle
  • poor feeding and vomiting
  • poor tone
  • sleepiness
40
Q

How would you manage hydrocephalus?

A

Ventriculoperitoneal shunt will drain CSF from the ventricles into another body cavity

  • usually the peritoneal cavity is used to drain CSF as there is plenty of space and it is easily reabsorbed

Complications…

  • infection
  • blockage
  • excessive drainage
  • intraventricular haemorrhage during shunt related surgery
  • outgrowing shunt (needs replacing every 2 years)
41
Q

What is craniosynostosis?

A
  • when skull sutures close prematurely, resulting in abnormal head shapes and restriction to the growth of the brain.
  • if untreated, results in raised ICP and symptoms
    • eg. developmental delay, cognitive impairment, vomiting, irritability, visual impairment, neuro symptoms, seizures
42
Q

How would a child present depending on which suture was synostosed?

  • Sagittal
  • Coronal
  • Metopic
  • Lamboid
A
  • Some typical presenting features = small head in proportion to body, anterior fontanelle closing before 1 years old

Sagittal synostosis = head is long and narrow from front to back

Coronal synostosis = head bulges on one side of the forehead

Metopic synostosis = pointy triangular forehead

Lamboid = flattening on one side of the occiput

43
Q

How would you manage a suspected craniosynostosis?

A
  • skull Xray
    CT head with bone views to confirm diagnosis or exclude it if there is doubt on the Skull Xray

Management…

  • monitor and follow up mild cases over time
  • severe cases = surgical reconstruction of the skull
44
Q

What are Plagiocephaly and Brachycephaly?

A

conditions that cause abnormal head shapes in normal healthy babies.

Plagiocephaly = flattening of one area of the babys head

Brachycephaly = flattening at the back of the head, resulting in a short head from back to front

Positional plagiocephaly = where a baby has a tendency to rest their head on a particular point, resulting in skull bones and sutures moulding with gravity to create an abnormal head shape

(this is more common now as parents are being advised to rest babies on their back to reduce sudden infant death syndrome)

45
Q

How would you manage plagiocephaly and brachycephaly?

A
  • Exclude craniosyntosis with a thorough hx and palpation of sutures
  • look for congenital muscular torticollis (shortening of SCM on one side), which can be the reason the baby always rests on one side of their head
    • physiotherapy and movement exercises can treat this
  • Reassurance!!!

Measures to encourage baby to avoid resting on flattened area:

  • positioning them on rounded side for sleep
  • supervised tummy time
  • use rolled towels or other props
  • minimise time in pushchairs and car seats

Plagiocephaly helmets can be considered but need to be used for the majority of the day, can cause skin problems like contact dermatitis, and cause psychosocial problems.

46
Q

What are some causes and types of non-epileptic seizures?

A

Causes

  • febrile conunvulsions, meningitis/ encephalitis, toxins/ poisons, head trauma, metabolic

Types

  • Reflex anoxic seizures (sudden drop in cerebral perfusion due to shock eg. falling over, head trauma)
  • Breath holding attacks (unthreatening episodic apnoea common in 12-18 months)
  • Pseudoseizures (diagnosis of exclusion, exclude safeguarding issues)
47
Q

What are infantile spasms?

A

Also known as West Syndrome

  • present as “salaam” attacks of flexion of head, trunk and arms, followed by ext of arms
  • lasts 1-2 seconds for up to 50 times
  • progressive mental handicap
  • EEG = hypsarrhythmia in 2/3
  • CT = typically shows brain disease like tuberous sclerosis in 70%
  • 1st line = vigabatrin
    • ACTH also used