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Paediatrics > Paediatrics Neurology > Flashcards

Paediatrics Neurology Flashcards

1
Q

What is syncope?

A

Temporarly loss of consciousness due to a disruption of blood flow to the brain

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2
Q

What are syncopal episodes also known as?

A

Vasovagal episodes

Fainting

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3
Q

What is a vasovagal episode cause by?

A
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4
Q

Name some prodromes of fainting?

A
  • Hot and clammy
  • Sweaty
  • Heavy
  • Dizzy or lightheaded
  • Vision going blurry / dark
  • Headache
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5
Q

What is the difference between a postictal period following a seizure and period following a faint?

A

Postictal = longer periods of confusion, drowsiness, irritability, disorientation

(Incontinence may occur with both seizures and syncopal episodes)

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6
Q

What are some causes of primary syncope (simple fainting)?

A

Dehydration

Missed meals

Extended standing in a warm environment e.g. school assembly

Vasovagal response to stimuli e.g. sudden surprise or pain in the sight of blood

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7
Q

What are some secondary causes of syncope?

A

Hypoglycaemia

Dehydration

Anaemia

Infection

Anaphylaxis

Arrhythmias

Valvular heart disease

Hypertrophic obstructive cardiomyopathy

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8
Q

What questions to ask for a syncope history?

A

Syncope vs a seizure?

After exercise? (More like secondary cause)

Triggers?

Concurrent illness? Do they have a fever or signs of infection?

Injury? Do they have a head injury?

Associated cardiac symptoms, such as palpitations or chest pain?

Associated neurological symptoms?

Family history, particularly cardiac problems or sudden death?

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9
Q

Whats the difference between syncope or seizure?

A
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10
Q

What to look for on examination for syncopal episode?

A

Physical injuries?

Concurrent illness, for example an infection or gastroenteritis?

Neurological examination

Cardiac examination, specifically assessing pulses, heart rate, rhythm and murmurs

Lying and standing blood pressure

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11
Q

What investigations for syncopal episode?

A

ECG (arrhythmias and long QT)

24 hour ECG if paroxysmal arrhythmias are suspected

Echocardiogram if structural heart disease is suspected

Bloods, including a full blood count (anaemia), electrolytes (arrhythmias and seizures) and blood glucose (diabetes)

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12
Q

What is the management of a simple vasovagal episode?

A
  • Avoid dehydration
  • Avoid missing meals
  • Avoid standing still for long periods
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13
Q

What is epilepsy?

A

Umbrella term where there is a tendency to have seizures

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14
Q

What are seizures?

A

Transient episodes of abnormal electrical activity in the brain

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15
Q

What is a generalised tonic-clonic seizure?

A

Loss of consciousness

Tonic (muscle tensing)

Clonic (muscle jerking) movements

Along with tongue biting, incontinence, groaning and irregular breathing

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16
Q

What is the post ictal period?

A

Time after seizure when the person is confused, drowsy and feels irritable or low

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17
Q

What is the management of tonic-clonic seizures?

A

First line: sodium valporate

Second line: lamotrigineorcarbamazepine

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18
Q

What are focal seizures?

A

Seizures which start in the temporal lobes affecting hearing, speech, memory and emotions

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19
Q

How do focal seizures present?

A

Hallucinations

Memory flashbacks

Déjà vu

Doing strange things on autopilot

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20
Q

What is the treatment for focal seizures?

A

First line: carbamazepine or lamotrigine

Second line: sodium valproate or levetiracetam

(opposite of generalised tonic-clonic seizures)

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21
Q

What are absence seizures?

A

Typically in children - patient is blank, stares into space and then abruptly returns to normal

Lasts 10 to 20 seconds (most stop having seizures as they get older)

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22
Q

What is the treatment for absence seizures?

A

First line: sodium valporate or ethosuximide

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23
Q

What are atonic seizures?

A

Also known as drop attacks characterised by brief lapses in muscle tone (don’t usually last more than 3 months) typically begin in childhood

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24
Q

What may atonic seizures be indicative of?

A

Lennox-Gastaut syndrome

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25
Q

What is the management of atonic seizures?

A

First line: sodium valproate

Second line: lamotrigine

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26
Q

What are myoclonic seizures?

A

Sudden brief muscle contractions like a jump - patient usually remains awake during the episode

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27
Q

What type of epilepsy do myoclonic seizures occur in?

A

Juvenile myoclonic epilepsy

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28
Q

What is the management of myoclonic seizures?

A

First line: sodium valproate

Other options: lamotrigine, levetiracetam or topiramate

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29
Q

What are infantile spasms also known as?

A

West syndrome (rare 1 in 4000)

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30
Q

What are the features of infantile spasms?

A

Begin at 6 months of age

Clusters of full body spasms

Poor prognosis 1/3 die by 25, 1/3 are seizure free

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31
Q

What are the treatments for infantile spasms?

A

Prednisolone

Vigabatrin

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32
Q

What are febrile convulsions?

A

Seizures which occur in children when they have a fever (not caused by epilepsy or meningitis or tumours - other underlying neurological pathology)

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33
Q

In what age children do febrile convulsions occur?

A

Between 6 months and 5 years (no lasting damage - slightly increases risk of developing epilepsy in the future)

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34
Q

What are the investigations for epilepsy?

A

Clear history (as opposed to febrile convulsion or vasovagal episode)

Electroencephalogram (EEG) show patterns in different forms of epilepsy - perform after second simple tonic-clonic seizure

MRI brain (tumours) - when first seizure is in children under 2, focal seizure, no response to first-line anri-epileptics

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35
Q

Which additional investigations considered to exclude pathology?

A

ECG to exclude problems in the heart

Blood electrolytes including sodium, potassium, calcium and magnesium

Blood glucose for hypoglycaemia and diabetes

Blood cultures, urine cultures and lumbar puncture where sepsis, encephalitis or meningitis is suspected

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36
Q

What is the general advice to patients with epilepsy?

A
  • Showers rather than baths
  • Cautious with swimming unless seizures are well controlled
  • Cautious with heights/traffic/heavy, hot or electrical equipment
  • Avoid driving unless they meet specific criteria
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37
Q

What is sodium valporate?

A

First line option for most forms of epilepsy

Increases activity of GABA which relaxes the brain

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38
Q

What are the side effects of sofium valporate?

A

Teratogenic so patients need careful advice about contraception

Liver damage and hepatitis

Hair loss

Tremor

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39
Q

What is first line for focal seizures?

A

Carbamazepine

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40
Q

What are some side effects of carbamazepine?

A

Agranulocytosis

Aplastic anaemia

Induces the P450 system so there are many drug interactions

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41
Q

What are the side effects of phenytoin?

A

Folate and vitamin D deficiency

Megaloblastic anaemia (folate deficiency)

Osteomalacia (vitamin D deficiency)

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42
Q

What are the side effects of ethosuximide?

A

Night terrors

Rashes

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43
Q

What are the notable side effects of lamotrigine?

A

Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.

Leukopenia

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44
Q

What is the management of seizures?

A

Put the patient in safe position (on a carpeted floor)

Put in recovery position

Put something under their head

Remove obstacles

Make a note of start and end time

Call an ambulance if more than 5 mins or if first seizure

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45
Q

What is status epilepticus?

A

Seizure lasting more than 5 mins or more than 3 seizures in one hour

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46
Q

What is the management of status epileptics?

A

ABCDE approach

Secure the airway

Give high-concentration oxygen

Assess cardiac and respiratory function

Check blood glucose levels

Gain intravenous access (insert a cannula)

IV lorazepam, repeated after 10 minutes if the seizure continues

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47
Q

What is the medication if seizures persist in status epileptics?

A

IV phenobarbital or phenytoin (consider intubation at this point and transfer to ICU)

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48
Q

What are the medical treatment options for status epileptics in the community?

A

Buccal midazolam

Rectal diazepam

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49
Q

What are simple febrile convulsions?

A

Generalised, tonic clonic seizures - last less than 15 minutes and only occur once during a single febrile illness

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50
Q

What are complex febrile convulsions?

A

Complex = partial / focal seizures

Last more than 15 minutes or multiple times during same febrile illness

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51
Q

What are the differentials diagnosis for febrile convulsion?

A

Epilepsy

Meningitis, encephalitis or another neurological infection e.g. cerebral malaria

Intracranial space occupying lesions e.g. brain tumours or intracranial haemorrhage

Syncopal episode

Electrolyte abnormalities

Trauma (think about non accidental injury)

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52
Q

What is the management of febrile convulsion?

A

Identify and manage the underlying source of infection

Control fever with simple analgesia e.g. paracetamol and ibuprofen

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53
Q

What is the general advice for managing a febrile convulsion?

A

Stay with child

Put child in safe place (carpeted floor)

Place in recovery position

Don’t put anything in their mouth

Call ambulance if lasts more than 5 minutes

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54
Q

What is the risk of developing epilepsy?

A

1.8% for gen population

2-7.5% for simple febrile convulsion

10-20% after complex febrile convulsion

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55
Q

What are breath holding spells?

A

Involuntary episodes where child holds breath (trigged by something upsetting/scary) between 6-18 months of age - not harmful in long term, don’t lead to epilepsy

Most outgrow by 4-5 years

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56
Q

What are the two types of breath holding spells?

A

Cyanotic breath holding spells

Pallid breath holding spells (also known as reflex anoxic seizures)

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57
Q

What are cyanotic breath holding spells?

A

When child is really upset, worked up and crying, after a long cry they some breathing, become cyanotic and lose consciousness - within a minute regain consciousness - may be tired and lethargic after episode

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58
Q

What are reflex anoxic seizures?

A

Occur when child is startled - vagus nerve sends strong signals to the heart to cause it to stop beating

Child suddenly goes pale, loses consciousness and may start to have some seizure-like muscle twitching

Within 30 seconds the heart restarts

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59
Q

What is the management of breath holding spells

A

Reassure after excluding other pathologies

Treat if iron deficiency anaemic (as this has been linked)

60
Q

What are some causes of headaches in children?

A

Tension headaches

Migraines

Ear, nose and throat infection

Analgesic headache

Problems with vision

Raised intracranial pressure

Brain tumours

Meningitis

Encephalitis

Carbon monoxide poisoning

61
Q

How do tension headaches present?

A

Mild ache across forehead

Band-like symmetrical pattern

Resolve gradually

No visual changes / pulsating sensation

62
Q

What are the triggers for tension headaches in children?

A

Stress, fear or discomfort

Skipping meals

Dehydration

Infection

63
Q

What is the management of stress headaches?

A

Reassurance

Analgesia

Regular meals

Avoiding dehydration

Reducing stress

64
Q

What are the types of migraine?

A

Migraine without aura

Migraine with aura

Silent migraine (migraine with aura but without a headache)

Hemiplegic migraine

Abdominal migraine

No simple explanation for why these occur

65
Q

What are the features of a headache caused by a migraine?

A

Unilateral

More severe than tension headaches

Throbbing in nature

Take longer to resolve

66
Q

What are migraines associated with?

A

Visual aura

Photophobia and phonophobia

N&V

Abdo pain

67
Q

What is the management of migraines in children?

A

Rest, fluids and low stimulus environment

Paracetamol

Ibuprofen

Sumatriptan

Antiemetics e.g. domperidone

68
Q

What are some options for migraine prophylaxis?

A

Propranolol (avoid in asthma)

Pizotifen (often causes drowsiness)

Topiramate (girls with child bearing potential need highly effective contraception as it is very teratogenic).

69
Q

What are the features of an abdominal migraine?

A

Central abdominal pain lasting more than 1 hour

Examination will be normal

May also have: N&V, anorexia, headache, pallor

70
Q

What infections can cause headaches?

A

Viral upper respiratory tract infection

Otitis media

Sinusitis

Tonsillitis

71
Q

What can be given for headaches caused by infection?

A

Paracetamol and ibuprofen

72
Q

Where is the inflammation in sinusitis?

A

Ethmoidal

Maxillary

Frontal

Sphenoidal

73
Q

What are the features of sinusitis?

A

Facial pain behind the nose, forehead and eyes

Tenderness over the affected sinuses - helps to diagnose

Resolves in 2-3 weeks

Mostly viral

74
Q

What is cerebral palsy?

A

Name of the permanent neurological problems resulting from damage to the brain around time of birth - not a progressive condition

75
Q

How does cerebral palsy vary?

A

Huge variation in symptoms from wheelchair bound to para-olympic athletes with only subtle problems with coordination / mobility

76
Q

What are the causes of cerebral palsy?

A

Antenatal:

Maternal infections

Trauma during pregnancy

Perinatal:

Birth asphyxia

Pre-term birth

Postnatal:

Meningitis

Severe neonatal jaundice

Head injury

77
Q

What are the types of cerebral palsy?

A

Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones

Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia causing athetoid movements and oro-motor problems - caused by problems with basal ganglia

Ataxic: problems with coordinated movement resulting from damage to the cerebellum

Mixed: spastic / dyskinetic / ataxic features

78
Q

What is spastic CP also known as?

A

Pyramidal CP

79
Q

Dyskinetic CP is also known as?

A

Athetoid CP and extrapyramidal CP

80
Q

What limbs can be affected by cerebral palsy?

A

Monoplegia: one limb

Hemiplegia: one side of body

Diplegia: four limb are affected but mostly legs

Quadriplegia: four limb affected but more severely (seizures, speech distubance and other impairments)

81
Q

What children are at risk of developing cerebral palsy?

A

Hypoxic-ischaemic encephalopathy

82
Q

What are some signs or symptoms of cerebral palsy?

A
  • Failure to meet milestones
  • Increased / decreased tone, generally or specific limbs
  • Hand preference below 18 months
  • Proglems with coordination or speech
  • Feeding or swallowing problems
  • Learning difficulties
83
Q

What information about the cerebral palsy do the following gaits give you?

A

Hemiplegic / diplegic: UMN lesion

Broad based gait / ataxic: cerebellar lesion

High stepping gait: foot drop / lower motor neurone lesion

Waddling gait: pelvic muslce weakness due to myopathy

Antalgic gait: limp = localised pain

84
Q

What are the features of UMN lesions vs LMN lesions?

A
85
Q

What is a hemiplegic / diplegic gait?

A

Increase in muscle tone and spasticity in legs

Leg is extended with plantar flexion of feet and toes - swing leg around in large semicircle due to lack of space

86
Q

What is the differential diagnosis for an UMN lesion?

A

Acquired brain injury / tumour

87
Q

What are some complications and associated conditionstocerebral palsy?

A

Learning disability

Epilepsy

Kyphoscoliosis

Muscle contractures

Hearning and visual impairment

GORD

88
Q

What is the multi-disciplinary management of CP?

A

Physio to stretch and stregthen muscles, prevent contractures

OT help pts manage everyday activities - adaptations e.g. rails or hoist

Speech and language therapy for speech and swallowing (may require NG tube or PEG tube)

Dieticians to help ensure they meet nutritional requirements

Orthopaedic surgeons - profectures to lengthen tendons (tenotomy)

Paediatricians - regularly see child to optimise medications

Social workers for benefits and support

Charities and support groups to share info on condition

89
Q

What medications are used in CP?

A

Muscle relaxants e.g. baclofen for muscle spasticity and contractures

Anti-epileptic drugs for seizures

Glycopyrronium bromide for excessive drooling

90
Q

What is a squint?

A

Misalignment of the eyes also known as stabismus - images don’t match and person will experience double vision

91
Q

How is a squint managed in childhood?

A

Brain copes by reducing signal from less dominant eye - so “lazy eye” becomes progressively more disconnected from the brain - can become worse called amblyopia

92
Q

What are concomitant squints due to?

A

Differences in control of the extra ocular muscles - severity here can vary

93
Q

What are paralytic squints due to?

A

Paralysis in one or more of the extra ocular muscles

94
Q

Define the following terms:

Strabismus

Amblyopia

Esotropia

Exotropia

Hypertropia

Hypotropia

A

Strabismus: the eyes are misaligned

Amblyopia: the affected eye becomes passive and has reduced function compared to the other dominant eye

Esotropia: inward moving squint

Exotropia: outward moving squint

Hypertropia: upward moving affected eye

Hypotropia: downward moving affected eye

95
Q

What are the causes of squints?

A

Idiopathic

Hydrocephalus

Cerebral palsy

Space occupying lesions, for example retinoblastoma

Trauma

96
Q

What to examine for in a squint?

A

General inspection

Eye movements

Fundoscopy (or red reflex) to rule out retinoblastoma, cataracts and other retinal pathology

Visual acuity

97
Q

What is Hirschberg’s test?

A

Shine a pen torch at the patient from 1 meter away when they look at the - look at reflection of light on cornea (should be central - deviation indicates squint) make a note of results

98
Q

What is the cover test?

A

Cover one eye and ask patient to focus on object in front of them then switch and watch movement of previously covered eye - if this eye moves inwards then it had drifted outwards (exotropia) and if it moves outwards then it means it had drifted inwards when covered (esotropia)

99
Q

When does treatment for a squint need to begin?

A

Before 8 years (as visual fields are developing)

100
Q

What is the management of a squint?

A

Occlusive patch - cover the good eye and force the weaker to develop

Alternatively, atropine drops can be used in good eye, causing vision in that eye to be blurred

Management is coordinated by an ophthalmologist - important to treat any underlying pathology e.g. cataracts

Refractive errors can be corrected by corrective lenses

101
Q

What is hydrocephalus?

A

Cerebrospinal fluid building up abnormally within the brain and spinal cord (due to over-production of a problem with draining or absorbing)

102
Q

How many ventricles are there in the brain?

A

Four: 2 lateral, the third and fourth (these contain CSF)

103
Q

What is the purpose of the CSF?

A

Cushion for brain tissue

104
Q

Where is CSF created?

A

In the four choroid plexuses (one in each ventricle) by the walls of the ventricles

105
Q

Where is CSF absorbed into the venous system?

A

By the arachnoid granulations

106
Q

What is the most common cause of hydrocephalus?

A

Aqueductal stenosis leading to insufficient drainage of CSF - cerebral aqueduct that connects the third and fourth ventricle is narrowed (causing CSF to build up in the lateral and third ventricles)

107
Q

What are some other causes of hydrocephalus?

A

Arachnoid cysts - these block the outflow of CSF if large enough

108
Q

What is an Arnold-Chiari malformation?

A

Cerebellum herniates downwards through the foramen magnum blocking the outflow of CSF

109
Q

What is a final cause of hydrocephalus?

A

Chromosomal abnormalities and congenital malformations can cause obstruction to CSF drainage

110
Q

How does hydrocephalus present?

A

Large occipital-frontal circumference (as the cranial bones are not yet fused at the sutures)

Bulging anterior fontanelle

Poor feeding and vomiting

Poor tone

Sleepiness

111
Q

What is the treatment for hydrocephalus?

A

Ventriculoperitoneal shunt which drains CSF from the ventricles into another body cavity (usaully teh peritoneal cavity)

Catheter is placed through a hole in the skull at the back of the neck and into one of the ventricles - this then runs under the skin into the peritoneal cavity

112
Q

What are the complications of VP shunts?

A

Infection

Blockage

Excessive drainage

Intraventricular haemorrhage during shunt related surgery

Outgrowing them (replacement needed every 2 years)

113
Q

What is craniosynostosis?

A

Skull sutures close prematurely (causing abnormal head shapes and restriction in growth of the brain)

114
Q

What happens in untreated craniosynostosis?

A

Raised intracranial pressure - developmental delay, cognitive impairment, vomiting, irritability, visual impairment, neurological symptoms, seizures

115
Q

What are the different types of synostosis and resulting head shapes?

A
116
Q

What are some other presenting features of craniosynostosis?

A

Anterior fontanelle closure before 1 year of age

Small head in proportion to the body

117
Q

What is the first line investigation for craniosynostosis?

What is used to confirm the diagnosis?

A

Skull x-ray

CT head with bone views to confirm the diagnosis or exclude if doubt on x-ray

118
Q

What is the management of craniosynostosis?

A

Mild cases = monitored and followed up

Severe = surgical reconstruction of the skull

Lifelong scar on scalp where surgery was performed

119
Q

What are plagiocephaly and brachycephaly?

A

Common conditions which cause abnormal head shapes in otherwise normal healthy babies

120
Q

What is plagiocephaly?

A

Flattening of one area of baby’s head (plagio = oblique or slanted)

121
Q

What is brachycephaly?

A

Flattening at the back of the head (brachy translates as short - short head back to front)

122
Q

What is the cause of plagiocephaly or brachycephaly?

A

Baby has a tendency to rest head on a particular point - resulting in skull bones and sutures moulding with gravity (positional plagiocephaly)

123
Q

Why is plagiocephaly more common?

A

Parents are advised to rest babies on their backs to reduce the risk of sudden infant death syndrome?

124
Q

What is the typical presentation of plagiocephaly and brachycephaly?

A

Baby aged 3-6 months with abnormal shaped head (often with preferance to sleep on one side)

125
Q

What is the management of plagio/brachycephaly?

A

Exclude craniosynotosis with thorough history and properly palpating sutures

Look for congenital muscular torticollis - a shortening of SCM muscle on one side (physio can help)

Reassurance - as vast majority of cases the head shapre returns to normal

126
Q

What measures can be taken to encourage the baby to avoid resting on the flattened area?

A

Position on rounded side to sleep

Supervised tummy time

Using rolled towels or other props

Minimising time in pushchairs and car seats

127
Q

What are plagiocephaly helmets?

A

Treatment option - some limitations in that they need to be used for the vast majority of the day and can lead to skin problems (contact dermatitis) and psychosocial problems - not routinely available on NHS

128
Q

What is muscular dystrophy?

A

Genetic condition causing gradual weakening and wasting of muscles

129
Q

What is the main muscular dystrophy to know for the purpose of exams?

A

Duchennes muscular dystrophy

130
Q

What are the other types of muscular dystrophy?

A

Beckers muscular dystrophy

Myotonic dystrophy

Facioscapulohumeral muscular dystrophy

Oculopharyngeal muscular dystrophy

Limb-girdle muscular dystrophy

Emery-Dreifuss muscular dystrophy

131
Q

What is Gower’s sign?

A

The

132
Q

Who is involved in the management of muscular dystrophy?

A

OT

Physio

Medical appliances (wheelchairs and braces)

Surgial input for spinal scoliosis or heart failure

133
Q

What causes Duchennes muscular dystrophy?

A
134
Q

When do patients with Duchennes present? What is the prognosis?

A

3-5 years present (weakness around pelvis)

Eventually all muscles affected

Wheelchair bound by teenage

Life expectancy of 25-35 years with good management of cardiac and resp complications

135
Q

What treatment can be given for Duchennes?

A

Oral steroids - slows progression of muscle weakness by 2 years

Creatine supplementation gives a slight improvement in muscle strength

136
Q

What is Beckers muscular dystrophy?

A

Similar to Duchennes however dystrophin gene is less severely affected.

Clinical course is less predictable (symptoms appear at 8-12 years) some require wheelchairs in late 20s or 30s - others are able to walk with assistance into later adulthood

Management is similar to Duchennes

137
Q

What are the typical features of myotonic dystrophy?

A
  • Progressive muscle weakness
  • Prolonged muscle contractions
  • Cataracts
  • Cardiac arrhythimas
138
Q

What is facioscapulohumeral muscular dystrophy?

A

Muscular dystrophy which presents in childhood with weakness around the face progressing to the shoulders and arms

139
Q

How does facioscapulohumeral muscular dystrophy present?

A

Sleeping with eyes slightly open and pursing their lips

Unable to blow their cheeks out without air leaking from their mouth

140
Q

What is oculopharyngeal muscular dystrophy?

A

Presents in late adulthood with weakness of the ocular muscles (around the eyes) and pharynx (around the throat)

141
Q

How does ocularpharyngeal muscular dystrophy present?

A

Bilateral ptosis

Restricted eye movement

Swallowing problems

Muscles around the limb girdles are also affected to varying degrees

142
Q

What is limb-girdle muscular dystrophy?

A

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

143
Q

What is Emery-Dreifuss muscular dystrophy?

A

Presents in childhood with contractures mostly in the elbows and ankles (shortening of muscles and tendons that restrict the range of movement in limbs)

Progressive weakening and wasting of muscles, starting with upper arms and lower legs

144
Q

What is spinal muscular atrophy (SMA)?

A

Rare autosomal recessive condition which causes a progressive loss of motor neurones (causing progressive muscular weakness)

145
Q

What are the features of SMA?

A

Affects lower motor neurones in the spinal cord

Lower motor neurone signs = fasciculations, reduced muscle bulk, reduced tone, reduced power, reduced / absent reflexes

146
Q

What are the different categories of SMA?

A

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

SMA type 2 = onset in first 18 months, never walk, survive into adulthood

SMA type 3 = onset after first year of life - walk without support - loose that ability. Resp muscles less affected and life expectancy is close to normal

SMA type 4 = onset in 20s - most retain ability to walk short distances but require wheelchair for mobility - everyday tasks can lead to significant fatigue. Resp muscles and life expectancy are not affected

147
Q

What is the management of SMA?

A

No cure - management is supportive

Physio - to max the strength in the muscles and retain resp function (split, braces and wheelchairs used)

Resp support with non-invasive ventilation to prevent hypoventilation and resp failure (especially during sleep)

SMA type 1 may require tracheostomy with mechanical ventilation

PEG feeding when swallowing unsafe

Paediatrics (18 decks)

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