Neurology

Question 1

What is it important to ask about in history of seizures?

Answer 1

Do the seizures occur when the child is awake or asleep

Could be benign sleep myoclonus - not an epileptic seizure and will disappear with age; no change in colour, saturations, otherwise well

Was the child in distress before the episode?
= breath holding spells, reflex anoxic seizures

Question 2

What is epilepsy?

Answer 2

Umbrella term - tendency to have seizures - which are transient episodes of abnormal electrical activity in the brain

Question 3

What are the types of seizures?

Answer 3

Tonic-clonic
Focal seizures 
Absence seizures
Atonic seizures
Myoclonic seizures
Infantile spasms

Febrile convulsions

Question 4

What is seen in a tonic clonic seizure?

Answer 4

loss of consciousness, tonic muscle tensing, clonic muscle jerking
Associated tongue biting, incontinence, irregular breathing
Drowsy post-octal period

Question 5

What is seen in a focal seizure?

Answer 5

start in temporal lobe
Affect hearing, speech, memory, emotions
Hallucinations
Memory flashbacks
Deja vu
Doing strange things on autopilot

Question 6

What is the management of tonic clonic seizures?

Answer 6

First line - sodium valproate

Second line - lamotrigine or carbamazepine

Question 7

What is the management of focal seizures?

Answer 7

First line: carbamazepine or lamotrigine

Second line: sodium valproate, levetiracetam

Question 8

What are absence seizures?

Answer 8

Patient becomes blank
Stares into space then abruptly returns to normal
During episode unaware of surroundings
Lasts 10-20 seconds

Question 9

What is the management of absence seizures?

Answer 9

First line - sodium valproate or ethosuximide

Question 10

What are atonic seizures?

Answer 10

Drop attacks
Brief lapses in muscle tone
Usually don’t last more than 3 minutes
May be indicate of Lennox-Gastaut syndrome?

Question 11

What is lennox-gastaut syndrome?

Answer 11

rare type of severe childhood-onset epilepsy
associated with gene mutations, perinatal injuries, brain tumours, malformations
treatment does not tend to end seizure recurrence

Question 12

What is the treatment of atonic seizures?

Answer 12

First line sodium valproate

Second line lamotrigine

Question 13

What are myoclonic seizures?

Answer 13

Sudden brief muscle contractions like a sudden jump
Patient usually awake during
Typically happen as part of juvenile myoclonic epilepsy

Question 14

What is the management of myoclonic seizures?

Answer 14

First line - sodium valproate

Other options - lamotrigine, levetiracetam, topiramate

Question 15

What is West syndrome?

Answer 15

Infantile spasms
Clusters of full body spasms
Difficult to treat

First line prednisolone or vigabatrin

Question 16

What are febrile convulsions?

Answer 16

Seizures in children whilst they have a fever
Not caused by epilepsy or underlying neurological pathology

Occur only in children between the ages of 6 months - 5 years

Slightly increases risk of developing epilepsy in future

Question 17

What are the investigations for epilepsy?

Answer 17

Good history, type of seizure
EEG
MRI brain - first seizure in under 2, focal seizures, no response to first line drugs

ECG to exclude heart problems
Blood electrolytes
Blood glucose - hypoglycaemia and diabetes
Blood cultures, urine cultures, LP - infection

Question 18

What general advice is key in those with epilepsy?

Answer 18

Take showers rather than baths
Be cautious with swimming unless seizures well controlled and closely supervised
Cautious with heights
Cautious with traffic
Cautious with heavy, hot or electrical equipment
Likely will avoid driving

Question 19

What is the effect of sodium valproate?

Answer 19

Increasing GABA

Teratogenic
Liver damage, hepatitis
Hair loss
Tremor

Question 20

What are some of the side effects of carbamazepine?

Answer 20

Agranulocytosis
Aplastic anaemia
Induces P450 system

Question 21

What are some of the side effects of phenytoin?

Answer 21

Folate and Vit D deficiency
Megaloblastic anaemia
Osteomalacia - Vit D def

Question 22

What are some of the side effects of lamotrigine?

Answer 22

Stevens Johnson syndrome

Leukopenia

Question 23

What is the management of a seizure?

Answer 23

Safe position
Recovery position if possible
Something soft under head
Remove obstacles that could lead to injury
Record start and end time
Call ambulance if lasts more than 5 mins, or first seizure

Question 24

What is the definition of status epilepticus?

Answer 24

Seizures lasting more than 5 minutes

More than 3 seizures in one hour

Question 25

What is the management of status epilepticus?

Answer 25

``` Secure airway High conc oxygen Assess cardiac and respiratory function Check blood glucose levels Gain IV access IV lorazepam repeated after 10 mins if continues ``` If still persists - infusion of IV phenobarbital or phenytoin Then intubation, ventilation

Question 26

What are the medical options in the community for status epilepticus?

Answer 26

Buccal midazolam | Rectal diazepam

Question 27

What questions need to be asked about episodes - fits, faints or funny turns?

Answer 27

Freq - how many in day, week, month Timing - certain time of day or behavioural states Relationship to sleep - on falling asleep, during sleep, waking Triggers - tired, travel, excitement, exercise Warnings beforehand Duration - if clustered how long between episodes Colour change - pallor, early cyanosis Alterations in conscious level Motor phenomena - act it out Recovery and symptoms after Family history

Question 28

What defines epilepsy?

Answer 28

At least two unprovoked seizures occurring more than 24 hours apart One unprovoked seizure and a probability of further seizures similar to the general recurrence risk after two unprovoked seizures occurring over next 10 years Diagnosis of an epilepsy syndrome

Question 29

What are acute asymptomatic seizures?

Answer 29

Result of acute insults | e.g. hypoglycaemia, hypoxia, electrolyte derangements, infection, trauma

Question 30

What are the common paediatric epilepsy syndromes?

Answer 30

``` West Syndrome - infantile spasms Age 4-7 Sudden jerks of neck, trunk Shortly after wakening, or when falling asleep, clustered Cries at end Loss of visual alertness ``` Benign epilepsy with Centro-temporal spikes 3-12 years, spontaneous remission by adolesence From sleep, focal onset Sensory or motor Tingling, drooling, aphasia ``` Childhood absence epilepsy 4-8 years, more common in girls Brief arrest of speech, activity Perioral, periocular flickering Unrousable, rapid recovery Can be induced by hyperventilation ``` Juvenile myoclonic epilepsy 12-18 years Present with first generalised tonic-clonic seizure Awareness retained during jerks

Question 31

What are examples of non-epileptic paroxysmal episodes which can occur during sleep?

Answer 31

Benign neonatal sleep myoclonus Myoclonic movements not involving the face Occur in infants few wks old ``` Parasomnias Sleep paralysis Confusional arousal Sleep walking Night terrors ```

Question 32

What are examples of non-epileptic paroxysmal episodes which can occur during feeding?

Answer 32

GORD/Sandifer syndrome GORD can cause apnoea and dystonic posturing of the head, neck and back Associated with feeding is Sandifer syndrome

Question 33

What are examples of non-epileptic paroxysmal episodes which can occur during fevers?

Answer 33

Febrile seizures Brief, generalised convulsions 6 months to 6 years Vaso-vagal syncope In older children Triggers e.g. intercurrent illness, heat, low food and water intake, prolonged standing Prodromal pallor, nausea, visual greying, dizziness

Question 34

What are examples of non-epileptic paroxysmal episodes which can occur due to pain, shock or startles?

Answer 34

Reflex asystolic syncope/reflex anoxic seizure: Sudden unexpected pain or discomfort e.g. headbanging Child becomes pale, hypotensive, loses consciousness May have limb stiffening, clonic jerking, vagally mediated Cyanotic breath holding End expiratory apnoea following crying Hyperekplexia: Whole body stiffening in response to sudden noises, being touched or handled Severe neonatal form can result in life threatening apnoea - forcible flexion of the neck can terminate it

Question 35

What are examples of non-epileptic paroxysmal episodes which can occur due to boredom, tiredness, stress?

Answer 35

``` Self gratification behaviour: Self comforting Can occur in older children with a neurological disability Adducted, crossed legs Unresponsive to interruption ``` Tics: Compulsive movements Gestures Occur in isolation, or may have vocal tics or Tourette's Daydreaming

Question 36

What are examples of non-epileptic paroxysmal episodes which can occur due to excitement?

Answer 36

Shuddering spells Excitement from toy or food - provokes shivering as if chilld Benign ``` Cataplexy Strong emotion e.g. laughter provokes sudden loss of tone Temporary Brief sagging of the legs Associated with sleep-wake fragment ```

Question 37

What are the types of primary headaches?

Answer 37

Migraine Tension type headache Cluster headache and other trigeminal autonomic cephalagias other primary headaches

Question 38

What are causes of secondary headaches?

Answer 38

``` Headaches due to an underlying pathology Head or neck trauma Intracranial haemorrhage Vascular malformation Substance or withdrawal Infection Disorder of homeostasis Psychiatric disorder ```

Question 39

What are tension headaches?

Answer 39

Mild ache across forehead Pain or pressure in band like pattern Come on and resolve gradually Children become quiet, stop playing, turn pale, tired Resolve quicker ``` Triggers include stress, fear, discomfort Skipping meals dehydration infection ```

Question 40

What is the management of tension headaches in children?

Answer 40

Reassurance Analgesia Regular meals Avoid dehydration, stress

Question 41

What are the types of migraine?

Answer 41

``` Migraine with aura Migraine without aura Silent migraine - migraine with aura but no headache Hemiplegic migraine Abdominal migraine ```

Question 42

What is an abdominal migraine?

Answer 42

May occur in younger children before they develop traditional migraines Episodes of central abdominal pain lasting more than one hour Examination normal May have associated N&V, anorexia, headache, pallor In child with possible migraines, ask about history of recurrent central abdominal pain as a child

Question 43

What may be the presentation of migraine?

Answer 43

Unilateral More severe Throbbing Take longer to resolve Associated with visual aura Photophobia, phonophobia Nausea and vomiting Abdominal pain

Question 44

What is the management of migraines in children?

Answer 44

``` Rest, fluids, low stimulus environment Paracetamol Ibuprofen Sumatriptan Antiemetics e.g. domperidone unless contraindicated ```

Question 45

What are options for migraine prophylaxis?

Answer 45

Propranolol - but avoid in asthma Pizotifen - causes drowsiness Topiramate - girls with child bearing potential need good contraceptive as teratogenic

Question 46

What are options for migraine prophylaxis?

Answer 46

Propranolol - but avoid in asthma Pizotifen - causes drowsiness Topiramate - girls with child bearing potential need good contraceptive as teratogenic

Question 47

How can infections cause a headache in a child?

Answer 47

Sinusitis causes headaches with inflammation of ethmoidal, maxillary, frontal or sphenoidal sinuses Viral URTI, otitis media, sinusitis, tonsillitis

Question 48

How might a headache suggest a space occupying lesion?

Answer 48

Worse when lying down Morning vomiting Night time wakening Change in mood, personality ``` Visual field defects Cranial nerve abnormalities Abducens often affected - squint with diplopia Abnormal gait Torticollis - tilting of head Growth failure Papilloedema - late feature Cranial bruits ```

Question 49

What are other causes of funny turns to consider in a child?

Answer 49

``` Cardiac arrhythmia - prolonged QT interval Non epileptic attack disorder Pseudoseizures - child feigns seizures Fabricated Paroxysmal movement disorders ```

Question 50

What are the types of muscular dystrophies?

Answer 50

Umbrella term for genetic conditions causing gradual weakening and wasting of muscles ``` Duchennes Beckers Myotonic dystrophy Fascioscapulohumeral Limb-girdle Emery-Dreifuss ```

Question 51

What is Gower's sign?

Answer 51

Standing up from a lying position - using hands on their legs to help them stand up Shift weight backwards Muscles around the pelvis not strong enough to keep upper body erect without arms

Question 52

What is the management of muscular dystrophy?

Answer 52

No curative treatment Aid quality of life Occupational therapy, physio, medical appliances e.g. wheelchairs, braces Management of complications e.g. spinal scoliosis, heart failure

Question 53

What is the inheritance of Duchennes?

Answer 53

Defective gene for dystrophin on the X chromosome X-linked recessive If mother is carrier with one faulty gene - 50% chance being carrier as female, 50% chance of having condition if male

Question 54

What is the presentation of Duchennes?

Answer 54

Waddling gait and/or language delay Mount stairs slowly Psuedohypertrophy of the calves, due to replacement of muscle fibres with fat and fibrous tissue ``` Progressive muscle atrophy and weakness No longer ambulant by 10-14 Respiratory failure Associated cardiomyopathy Scoliosis ```

Question 55

What is the management of Duchennes?

Answer 55

Exercise to maintain muscle power and mobility Passive stretching and night splints prevent contractures Orthoses for walking Truncal brace for scoliosis CPAP or NIPPV for nocturnal hypoxia, due to weakness of intercostal muscles Oral steroids shown to slow progression of weakness Creatine supplementation

Question 56

What is Beckers muscular dystrophy?

Answer 56

Similar to Duchennes Dystrophin gene less severely affected Clinical course less predictable, starts later

Question 57

What is myotonic dystrophy?

Answer 57

``` Type of muscular dystrophy Progressive muscle weakness Prolonged muscle contractions e.g. unable to let go after shaking hand, cannot release grip Cataracts Cardiac arrhythmias ``` Usually presents in adulthood

Question 58

What is facioscapulohumeral muscular dystrophy?

Answer 58

``` Presents in childhood Weakness around face Progresses to shoulders and arms Sleeping with eyes open Weakness pursing lips Cannot blow cheeks out without air leaking ```

Question 59

What is oculopharyngeal muscular dystrophy?

Answer 59

``` Presents in late adulthood Weakness of ocular muscles Weakness around pharynx Bilateral ptosis Swallowing problems ```

Question 60

What are the central movement control centres?

Answer 60

Motor cortex in the pre-central gyrus which passes down corticospinal tracts to basal ganglia Basal ganglia - stores patterns of movement Cerebellum

Question 61

What are examples of corticospinal tract disorders?

Answer 61

``` Cerebral dysgenesis Global hypoxia ischaemia Cerebral tumour Hemiplegic migraine Arterial ischaemic stroke ```

Question 62

What are examples of basal ganglia disorders?

Answer 62

Acquired brain injury e.g. CO poisoning, ischaemia, post cardiopulmonary bypass chorea Wilson disease Huntington's

Question 63

What are examples of cerebellar disorders?

Answer 63

Medication, drugs Post viral Medulloblastoma Genetic and degenerative disorders e.g. ataxia-telangiectasia

Question 64

How might a child present with a neuromuscular disorder?

Answer 64

``` Weakness - progressive or static Floppiness Delayed motor milestones Muscle weakness Unsteady-abnormal gait Fatiguability Muscle cramps - metabolic myopathy ```

Question 65

How may a myopathy present?

Answer 65

Waddling gait or positive Gowers | Gowers positive is normal up until the age of 3

Question 66

What are examples of neuromuscular disorders?

Answer 66

Disorders of anterior horn: Poliomyelitis Spinal muscular atrophy Disorders of peripheral nerve: Hereditary motor sensory neuropathies Acute post-infectious polyneuropathy - Guillan-Barre Bell's Disorder of neuromuscular transmission: myasthenia gravis ``` Muscle disorders: Muscle dystrophies Inflammatory myopathies e.g. benign acute myositis, polymyositis Myotonic disorders Metabolic myopathies Congenital myopathies ```

Question 67

What are the investigations for a myopathy?

Answer 67

Serum creatine phosphokinase elevated in Duchenne and Becker's, and in inflammatory myopathies Muscle biopsy DNA testing USS or MRI of muscles

Question 68

What are the investigations for a neuropathy?

Answer 68

Nerve conduction studies DNA testing Nerve biopsy (rare) EMG - electromyography e.g. shows fatiguability in repetitive nerve stimulation in myasthenia

Question 69

What is spinal muscular atrophy?

Answer 69

Rare autosomal recessive condition Progressive loss of motor neurones and progressive muscular weakness Affects lower motor neurones in spinal cord - LMN signs: fasciculations, reduced muscle bulk, reduced tone and power Reduced or absent reflexes

Question 70

What are the types of SMA?

Answer 70

1 - onset in first few months of life, death within 2 years 2 - onset within first 18 months, most never walk, survive to adulthood 3 - onset after first year of life, most walk without support 4 - onset in 20s, retain ability to walk short distances, tasks are tiring, rest muscles and life expectancy not affected

Question 71

What is the management of spinal muscular atrophy?

Answer 71

Supportive with MDT Physio to maximise muscles Retain resp function Use of splints, braces, wheelchairs Resp support with NIPPV to prevent hypoventilation and resp failure, part in sleep SMA 1 may need tracheostomy and mechanical ventilation Percutaneous endoscopic gastrostomy PEG tube if weak swallow

Question 72

What are signs of SMA Type 1?

Answer 72

Lack of antigravity power in hip flexors Absent deep tendon reflexes Intercostal recession Fasciculation of the tongue

Question 73

What is Guillain Barre syndrome?

Answer 73

Acute post infectious polyneuropathy 2-3 weeks after URTI or campylobacter gastroenteritis Fleeting abnormal sensory symptoms in legs Ascending symmetrical weakness, loss of reflexes Involvement of bulbar muscles can lead to difficulty chewing Difficulty swallowing Recovery can take up to 2 years Management supportive Resp support

Question 74

What is Bell's palsy?

Answer 74

Isolated lower motor neurone paresis of facial nerve Probably post infectious Corticosteroids can reduce oedema in first week Main complication conjunctival infection due to inability to close eye

Question 75

What is a key differential of Bell's palsy?

Answer 75

If symptoms of vestibulocochlear nerve paresis present; due to compressive lesion Herpes virus can invade geniculate ganglion, cause facial palsy treated with aciclovir HTN excluded as Bell's palsy can be associated with coarctation of the aorta Bilateral facial weakness can be due to sarcoidosis or Lyme disease

Question 76

What is juvenile myasthenia?

Answer 76

Similar to adult autoimmune myasthenia Due to binding of antibodies to acetylcholine receptors on post synaptic membrane Reduces no. of functional receptors ``` Presentation usually after 10 Ophthalmoplegia, ptosis Loss of facial expression Difficulty chewing Facial weakness ``` Diagnosis confirmed with acetylcholine receptor antibody tests or anti muscle specific kinase antibodies.

Question 77

What is the treatment for myasthenia gravis/juvenile myasthenia?

Answer 77

Anti-muscarinic drugs neostigmine or pyridostigmine Immunosuppressive therapy with prednisolone, azathioprine

Question 78

What are metabolic myopathies and causes?

Answer 78

Metabolic conditions can affect muscles Lead to floppy infant Or child with muscle weakness or cramps on exercise Glycogen storage disorders Disorders of lipid metabolism Mitochondrial cytopathies

Question 79

What is found on examination of a floppy infant?

Answer 79

Hang like a rag doll Marked head lag Central hypotonia associated with poor truncal tone, but preserved limb tone Dysmorphic features suggest genetic cause Lower motor neurone lesions - frog like posture, poor antigravity movements and absent reflexes

Question 80

What are causes of a central hypotonic infant (floppy)?

Answer 80

Cortical: Hypoxic ischaemic encephalopathy Cortical malformations Genetics: Down's Prader-Willi Metabolic: Hypothyroidism Hypocalcaemia

Question 81

What are the causes of a peripheral hypotonic infant?

Answer 81

Spinal muscular atrophy Myopathy Myotonia Congenital myasthenia

Question 82

What is Friedreich ataxia?

Answer 82

``` Autosomal recessive condition Presents with worsening ataxia Distal wasting in legs Absent lower limb reflexes Extensor plantar responses due to pyramidal involvement Pes cavus - high arch Dysarthria ``` Kyphoscoliosis and cardiomyopathy can worsen with age, cause death at 40-50

Question 83

What is ataxia telangiectasia?

Answer 83

Autosomal recessive Mild delay in motor development Oculomotor problems Incoordination Difficulty with balance and coordination Dystonia, cerebellar signs Telangiectasia in conjunctiva, neck and shoulders Increased susceptibility to infection Develop malignant disorders Raised AFP Increased white cell sensitivity to irradiation

Question 84

What are causes of cerebellar ataxia?

Answer 84

Medication, drugs Varicella infection Tumours Degenerative disorders - ataxic cerebral palsy, Freidreich ataxia, ataxia telangiectasia

Question 85

What is an extradural haemorrhage?

Answer 85

Follows direct head trauma | Skull fracture and tearing of middle meningeal artery

Question 86

What is the presentation of an extradural haemorrhage?

Answer 86

Lucid interval Then consciousness drops Seizures secondary to increasing size of haematoma Focal neurological signs may be present - dilatation of ipsilateral pupil, paresis of contralateral limbs. In young child, may be anaemic and in shock

Question 87

What is the diagnosis and management of an extradural haemorrhage?

Answer 87

CT scan Correct hypovolaemia Urgent evacuation of haematoma, arrest bleeding

Question 88

What is a subdural haematoma?

Answer 88

Tearing of veins as they cross subdural space Retinal haemorrhages present Characteristic non-accidental injury e.g. shaking or direct trauma

Question 89

What is a subarachnoid haemorrhage and its presentation?

Answer 89

``` Less common in kids Acute onset of head pain Neck stiffness Occasional fever Retinal haemorrhage Seizures and coma may occur ```

Question 90

What are the investigations and treatment for a subarachnoid haemorrhage?

Answer 90

CT scan identifies blood in CSF LP in acute situation avoided - haemorrhage may then extend following releasing of ICP Cause often aneurysm or arteriovenous malformation identifiable with MR angiography, CT or conventional angiography Treatment with neurosurgery or interventional radiography

Question 91

What are causes of rising intracranial pressure?

Answer 91

``` Meningoencephalitis Head injury Subdural/extradural bleeds - NAI? Hypoxia e.g. near drowning Ketoacidosis Tumours Thrombosis Reye's ```

Question 92

What are the sections of children's coma scale?

Answer 92

Best motor response Best verbal response Eye opening

Question 93

What are the signs of raised intracranial pressure?

Answer 93

``` Irritable Drowsy Headache Diplopia Vomiting Tense fontanelle Level of responsiveness ``` If unconscious look for: pupil changes (ipsilateral dilatation) abnormal posturing, Cushing's triad warns of imminent coning

Question 94

What are the types of abnormal posturing?

Answer 94

Decorticate - arms flexed over chest | Decerebrate - arms extended at sides, and head and back arched backward

Question 95

What is Cushing's triad?

Answer 95

Slow pulse, widened pulse pressure Raised BP Breathing pattern abnormalities

Question 96

What is the management of rising ICP?

Answer 96

``` Prevent ischaemia - keep head in midline elevated at 25 degrees Give O2 Treat any hypoglycaemia Control seizures Don't do LP - risk of coning Intubate, dexamethasone Fluid restriction and diuresis ```

Question 97

What is the main rule of visual field defects?

Answer 97

Pre-chiasmal lesions cause ipsilateral monocular defect | Post-chiasmal leads to homonymous visual field defects on contralateral side

Question 98

What do lesions at the optic chiasm cause?

Answer 98

Bitemporal hemianopia

Question 99

Why is there sparing of the macula in the occipital lobe?

Answer 99

As there is dual blood supply in the anterior portion of the visual centre - PCA and MCA Homonymous hemianopia

Question 100

What are typical lesions of the optic nerve?

Answer 100

Optic neuritis Amaurosis fugax Trauma Retrobulbar optic neuropathy Leads to ipsilateral monocular blindness

Question 101

What are typical lesions of the central portion of the optic chasm?

Answer 101

Pituitary adenoma Suprasellar aneurysm Leads to bitemporal hemianopia

Question 102

What are typical lesions of the optic tract?

Answer 102

MCA stroke Tumours Contralateral homonymous hemianopia

Question 103

What are typical lesions of the optic radiations?

Answer 103

MCA stroke Tumour Trauma Contralateral homonymous quadrantopis

Question 104

What are causes of acquired spinal cord injury?

Answer 104

Trauma Inflammatory - post infectious, abscesses, tuberculous Vascular - acute anterior spinal artery infarction - sparing of dorsal cord leads to preservation of dorsal column - vibration, joint position, sensation Compression - SOL, paraspinal neuroblastoma, syringomyelia

Question 105

What is the acute management of a spinal cord injury?

Answer 105

``` Neurosurgical evaluation High dose steroids Ventilatory support for high lesions Urinary catheterisation, management of constipation Aggressive contracture prevention ```

Question 106

What is autonomic dysreflexia?

Answer 106

Important problem of complete lesions above T6 Stimuli below this level, e.g. even full bladder and not aware can lead to increased reflex sympathetic activity, HTN, vasoconstriction This is sensed by CNS above lesion, causing increased vagal tone and bradycardia so vasodilation above lesion leads to pounding headache and sweating Needs emergency treatment, relief of cause, sit upright

Question 107

What are long term complications of a spinal cord injury?

Answer 107

``` Motor Weakness, spasticity, contractures Spinal deformity Chest infection Postural abnormalities Pathological fractures ``` Sensory Skin breakdown due to lack of pain sensation from pressure Scalds, hot weather etc Sphincters Incontinence, bladder dysfunction Unsafe bladder not well managed - renal failure Emotional Lack of independence, depression, sexual dysfunction

Question 108

What is syringomyelia?

Answer 108

Reversible cause Expansion of central cavity of cord due to abnormal CSF flow and pressure Loss of pain, pinprick and temperature

Question 109

What are some causes of vision loss pre-chiasmatic/chiasmatic?

Answer 109

Cornea - scarring, vit A deficiency, keratoconus (Trisomy 21) Lens - cataract Macula - damage, dystrophy Retina - trauma, NAI, detached retina, intraocular tumour Optic nerve - inflammation, optic neuritis, optic atrophy Compression; raised ICP, trauma, bone disease, infection, hypertension Ischaemia, optic glioma Chiasm - tumour

Question 110

What are examples of neural tube defects?

Answer 110

Anencephaly - failure of most of cranium and brain Encephalocele - extrusion of brain and meninges through midline skull defect Spina bifida: Spina bifida occulta - vertebral arch does not fuse properly, underlying tethering of cord causes neurological defects Meningocele - sac of fluid, meninges herniate but nervous system remains undamaged Myelomeningocele - spinal cord protrudes out as well as meninges containing it

Question 111

What problems is myelomeningoceles associated with?

Answer 111

``` Variable paralysis Muscle imbalance Dislocation of hips, talipes Sensory loss Bladder dernervation Bowel denervation Scoliosis Hydrocephalus ```

Question 112

What is seen in spina bifida occulta?

Answer 112

Associated overlying skin lesion Tuft of hair, lipoma, birth mark Small dermal sinus

Question 113

What is the criteria for immediate request of CT head in children?

Answer 113

LOC more than 5 mins Amnesia more than 5 mins Abnormal drowsiness Three or more discrete episodes of vomiting Clinical suspicion of NAI Post traumatic seizure but no hx of epilepsy GCS <14 or if less than 1 <16 Suspicion of open or depressed skull injury, tense fontanelle Any sign of basal skull fracture Focal neurological deficit Presence of bruise, swelling, laceration >5cm on head if less than 1 year Dangerous mechanism of injury

Question 114

What are some of the causes of microcephaly?

Answer 114

``` Normal variation, familial Congenital infection Perinatal brain injury e.g. hypoxic ischaemic encephalopathy Fetal alcohol syndrome Patau's Craniosynostosis ```

Question 115

What is hydrocephalus?

Answer 115

CSF building up abnormally within the brain and spinal cord | Either due to overproduction or problem draining and absorbing

Question 116

What is normal CSF physiology?

Answer 116

Four ventricles - two lateral, third and fourth Ventricles contain CSF Created in four choroid plexuses passes through to arachnoid granulations where it is absorbed

Question 117

What are congenital causes of hydrocephalus?

Answer 117

Aqueductal stenosis - stenosed connection in cerebral aqueduct between third and fourth ventricle so there is a build up in third and lateral ventricles Arachnoid cysts can block outflow Arnold-Chiari malformation, seen in spina bifida - cerebellum herniates downwards towards foramen magnum causing blockage Chromosomal abnormalities and congenital malformations

Question 118

What is the presentation of hydrocephalus?

Answer 118

Outward pressure on cranial bones if sutures not fused They fuse at 2 years Enlarged and rapidly increasing head circumference Bulging anterior fontanelle Poor feeding and vomiting Poor tone Sleepiness

Question 119

What is a VP shunt?

Answer 119

Drains CSF from ventricles into another body cavity | Usually peritoneal cavity as plenty of space, easily reabsorbed

Question 120

What are the complications of a VP shunt?

Answer 120

Infection Blockage Excessive drainage Intraventricular haemorrhage during shunt related surgery Outgrowing them - typically need replacing every 2 years