Neuro-Paeds

Question 1

Whats a migraine without aura?

Answer 1

90% of migraines.
In children episodes last -1-71hrs, headache bilateral but can be uni.
Pulsatile over temporal or frontal area
Assc w/ unpleasent GI disturbance like N+V, abdo pain, photophobia.
Aggrevated by PE

Question 2

Whats a migraine with aura?

Answer 2

10%
Headache preceded by an aura(visual, sensory, motor) , although aura may occur without headache.
CF:
Abscence of problems between episodes and frequent presecence of premonitory sx (tiredness, difficulty concentrating, autonomic features,
Most common aura conprises visual disturbance:
Negative phenomena- henianopia (loss of half visual field) or scotoma (small areas of visual loss)
Positive phenomena- fortification spectra- seeing zig zag lines
Rarely:
Unilateral sensory or motor sx. Sx for some hours, kids prefer to lie down in dark place. Sleep helps.
Sx of tension type or migraine often overlap. Both from 1o neural dysfunction, usually channelopathies, with vascular phenomena as 2o events.
Genetic component-1/2 degree relatives
Bouts triggered by inherent biorhythms disturbance - late nights, early rises , stress, winding down after stress at home or school. Certain foods: cheese, chocolaye, caffeine- rarely.
G- can bebrelated to pill or menstruation.

Question 3

What are some uncommon forms of migraine?

Answer 3

Familial- calcium channel defect, dominant.
Sporadic hemiplegic migraine
Basilar type m- vomitting with nistagmus and cerebral signs
Periodic supyndromes: precursors of migraine:
Cyclical vomiting, looks well
Abdominal migraine- episodic midline pain , moderate to severe ,1-72hrs, assc w/ vasomotor sx- N+V . Well inbetween these episodes.
Benign paroxysmal vertigo
Heterogenous disorder- vertigo without warning. Normal. Resolves spontaneously.

Question 3

What would you ask in a history of headache?

Answer 3

Premonitory sx, aura, character, position, radiation, frequency, duration, trigger, relieveing and exacerbating fx.
Special consideration:
Triggers- stress, relaxation, food, menstruation?
Emotional or behavioural probs at home/dpsxhool?
Vision checked: refractive error?
Head trauma?
Alcohol, solvent, drug abuse?
Analgesia over use?

Question 4

Headache types?

Answer 4

Tension type- constriction band
Migraine w/o aura: bilateral, unilateral, pulsatile, GI distrurbance eh N+V, abdo pain, photophobia. Lies in quiet dark place. Relieved by sleep.
Migraine with aura-preceded by aura
Mixed type headaces- common

Question 5

What are some red flags sx of headaches?

Answer 5

Headache- worse with lying down, exertion or cough.
Headache wakes up child, different from awakening one. Not common with migraine
Assc w/confusion, and or morning or persistent nausea and vomitting
Recent changes in behaviour or personality or educational performance.

Question 6

Red flags physical signs??

Answer 6

Growth Failure
Visual defects- craniopharyngioma 
Squint
Cranial nerve abnormality
Torticollis (tilting of head) 
Abnormal coordination- for cerebellar lesions 
Gait- UMN, or cerebellar signs
Fundi- pappiloedema
Bradycardia
Cranial bruits- arteriovenous malformation

Question 7

What are some other physical signs of headaches?

Answer 7

Visual acuity- refractive erros
Sinus tenderness- for sinusitis
Paim on chewing- temporomandibular joint malocclusion
Blood pressure: for HTN

Question 8

When would you comsider investigating a headache?

Answer 8

Only if there are any ref flags

Question 9

What are some rescue tx for headaches?

Answer 9

Analgesia, NSAIDS,
Antiemetics- prochloperazine, metoclopramide
Serotonin- 5HT agonsist- sumatriptan>12Y nasal preparation.

Prophylactic agents: when headaches are frequent and inteusive
5-HT antagonists- Pizotifen: wt gain and sleepiness
Beta blockers- propranolol- CI in asthma
Na channel blockers

Psychosocial support:
Bullying? Anxiety over exams? Family?
Relaxation and other self regulatory techniques.

Question 10

What are some epileptic causes of seizures?

Answer 10

Idiopathic 70-80%, presumed genetic
Secondary:
Cerebral dysgenesis/malformation
Cerebral vascular occlusion
Cerebral damage- congenital infx, HIE, intraventricular haem/ischaemia
Cerebral tumour
Neurodegenerative disorders
Neurocutaneous syndromes (skin and Neural tube both arise from ectoderm)

Question 11

What are some non epileptic causes of seizures?

Answer 11

Febrile seizures
Metabolic: hypoglycaemia, hypocalcaemia/hypomagnesia, hypo/hypernatraemia
Head trauma
Meningitis/encephalitis
Poisons/toxins

Question 12

What happens in febrile seizures?

Answer 12

3% of kids affected, have genetic predisposition
6M-6Y of age
usually brief, generalised tonic clonic seizures occuring with a rapid rise in fever.
If a bacterial infx present like meningitis must be recognised and tx.
Advise family about management of seizures, comsider rescue therapy
If simple- does not affect intellectual performance or risk of developing epilepsy.there is 1-2% , like all other kids who never had one before.
If complex- (focal, prolonged or repeated in the same illness)
4-12% risk of subsequent epilepsy.

Examination: focus on fever, usually its viral.

Question 13

What are the classical sx of meningitis?

Answer 13

Neck stiffness, photophobia so use infection screen- blood cultures, urine culture and LP to asses CSF.
If child unconsious or has cardiovascular instability- NO LP- sudden release of high ICP will be harmfull. Antibiotics started immediately.

Question 14

What are some causes of funny turns?

Answer 14

These can mimic epilepsy.
1. Breath holding attacks–> temper-> upset toddlers- goes blue- no drugs, behaviour modification- distractions.
2. Reflex anoxic seizures-> (pain/discomfort from minor head trauma, cold food(ice cream, cold drinks) fright, fever) -> infants or toddlers-> many have a first degree relative with faints. After triggering effect- child becomes pale and falls to floor. The hypoxia may induce a generalised tonic-clonic seizure- the episodes are due to cardiac asystole due to vagal inhibition. Seizure is brief and child recovers quickly.
Ocular compression under controlled conditions- may cause it.

3. Syncope–> kids may faint if on a hot and stuffy day environment, standing for long periods , or from fear. Clonic movements may occur.
4. Migraine-
   May lead to paroxysmal headache involving unsteadniness or light-headeness as well as the more common visual and GI disturbances .
5. Benign paroxysmal vertigo
   Recurrent vertigo episodes 1-several mins, assc w/ nystagmus, umsteadiness or even falling (esp w/ closed eyes)
   It is a 1o headache disorder of childhood occasionally due to viral labyrinthitis.
6. Other causes: prolonged QT interval->
   Cardiac arythmia- may cause collapse or cardiac syncope related ro exercise.
   Tics, daydreaming, night terrors.
   Self- gratification-> young kids stimulate ther genitalia for a comfort feeling rather than sexual gratification
   Non- epileptic attack disorder (NEAD)
   Pseudoseizures- feign seizures
   Fabricated- by parent
   Induced illness-> Non accidental injury- seizures from hypoglycaemia from adult injecting insulin.
   Paroxysmal movement disorders- genetically determined episodes, no loss of consciousness.

Question 15

What are some sx of generalised seizures?

Answer 15

Always loss of consciousness
No warning
Symmetrical seizure
Bilateral synchronous seizure discharge on EEG

Onset from both hemispheres

Question 16

What are some different types of generalised seizures?

Answer 16

1. Abscence seizures: transient loss of consiousness, abrupt onsent and termination, unaccompined by motor phenomena except some eyelid flickering and minor muscle tone alterations. Can be typical (petit mal) or atypical, can be precipitated often by hyperventilation.
2. Myoclonic seizures: brief, often repetitive jerky movements of the neck, trunk and limbs. Non-epileptic myclonic movements are also seen physiologically in hiccoughs (myoclonus of the D) or on passinh through stage 2 sleep.
3. Tonic seizures- Generalised increase in tone.
4. Tonic clonic seizures
5. Atonic seizures - often combimed with myoclonic jerk, followed by a transient lodd of muscle tone causing a sudden fall to the floor or drop of the head.

Question 17

What are tonic-clonic seizures?

Answer 17

Rhytmical contraction of muscle groups followinh the tonic phase. In the rigit phase- kids may fall on the ground and injrure themselves. They do NOT ❌ breathe and become cyanosed.
This is followed by clonic phase- with jerkinh of the limbs. Breathing is irregular, cyanosis may perists, salive may accumulate to mouth.
There may be biting of tongue and incontinence of urine.
Few secs- mins followed by uncosciousness or deep sleep for up to several hours.

Question 18

What are sime sx of focal seizures?

Answer 18

Onsent in neural netwerok limited to one cerebral hemisphere (neurochannels)
Begin in one small dysfunctional neural group
May be heralded by aura- relfects origin site
May or may not be assc w/ consciousness levels change
Or more generalised tonic-clonic seizures.

Question 19

What are some common clues to the site of origin of a focal seizure?

Answer 19

Frontal seizure- motor phenomena (premotor cortex)
Temporal lobe- smell or taste phenomena
Occipital- positive or negative phenoma
Parietal- contralateral altered sensation (dysaesthesia)

Question 20

What are some epilepsy syndromes and when do they peak?

Answer 20

West syndrome: 4-6M, violent flexor spasms of head, trunk and limbs.
Lennox -Gastaut syndrome 1-3Y
Childhood abscence epilepsy: 4-12Y, stare and stop moving
Juvenile myoclonic epilepsy - adolescence- adulthood.

Question 21

What are the choices of antiepileptic drugs according to NICE?

Answer 21

Generalised seizures:
1. Tonic clonic- 1st line: Valproate, carbamazepine, 2nd line: Lamotrigine, Topiramate

2. Abscence- 1st: Valproate, ethosuximide, 2nd: Lamotrigine
3. Myoclonic:1st: Valproate, 2nd: Lamotrigine

Focal seizures: 1st: Carbamazepine, valproate, Lamotrigine( most effective must slow titration) ,2nd line: Topiramate, levetiracetam, oxcarbazepine, gabapectin, tiagabine
Vigabatrin

Question 22

What happens in childhood epilepsy?

Answer 22

Affects 1 in 200 children
Classified by type, syndrome identification, underlying aetiology.
If suspected; EEG
AED therapy only if seizures intrusive, selected according to seizure type , monotherapy when possible and with least potential SEs.

Requires lialosation to school about seizure management and avoiding situatioms which could lead to injury.

Question 23

What are some causes of movement disorders?

Answer 23

Corticospinal (pyramidal) tract disorders
Basal ganglia disorders
Cerebellar disorders

Question 24

What are some corticospinal tract disorders, causing movement dx?

Answer 24

Cerebral dysgenesis- neural migration problem
Global hypoxia ischaemia
Arterial ischaemic stroke
Cerebral tumor
Encephalomyelitis

Post-ictal paresis
Hemiplegic migraine

Question 25

What are some basal ganlia causes of motor dysfunction?

Answer 25

Acuired brain injury:
Acute and profound hypoxia- ischaemia
CO posioning
Post cardiopulmonary chorea

Post streptococcal chorea (rheumatic fever)
Mitochondrial cytopathies
Wilson Disease
Huntington disease

Question 26

What are some cerebellar causes for motor dysfunction?

Answer 26

Acute: medication and drugs: includinh alcohol and solvent abuse
Post viral- esp varicella
Posterior fossa lesions or tumors- medulloblastoma

Genetic and degenerative: ataxic cerebral palsy, Friedreich ataxia and ataxia- telangiectasia

Question 27

What are some neuromuscular disorders?

Answer 27

Dx of anterior horn:

1. Spinal miscular atrophy,
2. Polymyelitis

Dx of peripheral nerve:

1. Hereditory motor sensory neuropathies
2. Acute post- infx polyneuropathy (Guillaine-Barre)
3. Bell palsy

Dx of neuromuscular transmission
1. Myasthania gravis

Muscle disorders:

1. Muscle dystrophies: Duchenne (M) / Becker/ congenital
2. Inflamatory myopathies: benign acute myositis, polymyositis, dermatomyositis.
3. Myotonic disorders: dystrophia myotonica
4. Metabolic myopathies
5. Congenital myopathies

Question 28

What happens in neuromuscular disorders?

Answer 28

They present with miscle weakness, may manifest in flopimess, delayed motor milestones, unsteady gait, or muscle fatiguability.
Anterion horn cell: spinal miscular atrophy: progressive weakness and wasting of skeletal muscles; tongue fascuculation may aid diagnosis

Peripheral nerve: HMSN: symmetrical wasting of distal muscles
Guillan -Barre- ascending symmetrical weakness, may be bulbar palsy and resp depression.

Neuromuscular transmission: juvenile myasthenia: >10Y, opthalmoplegiamand ptosis, loss of facial expressiom and diffivultu chewing.

Muscle: Duchenne muscular atrophy: X linled recessive, presents with waddling gait and difficulty climbing stairs.

Question 29

Whatbare some reasons for the floppy (hypotonic) infant?

Answer 29

Central:

1. Cortical: HIE, cortical malformations.
2. Genetic: Downs, Prader -Willi syndrome
3. Metabolic : hypothyroidism, hypovalcaemia

Peripheral:
1. Neuromuscular:
Spinal miscular atrophy
Myopathy
Myotonia
Congenital myasthenia

Question 30

What happens in cerebral ataxia?

Answer 30

Causes: meds, drugs, varicella infx, postiriot fossa lesions or tumors, genetic and
Degenerative disorders: eg. Ataxic CP, Friedreich ataxia, (autosomal recessive- pyramidal involvment, impairment in joint positions, vibration sense, often optic atrophy, ataxia telangiectasia; DNA repair disorder-> oculomotor dx, difficulty in balance and coordination evident at school age.
Wheelchair in early adolescnce :(

Question 31

What happens in cerebral haemorrhage?

Answer 31

Hx of significant head injury- remember that extradural haem may be present even if lucid afterwards.

Subdural heamatoma and retinal haemorrhages in an infant: comsider non accidental head injury caused by shaking or trauma?

Question 32

Strokes??

Answer 32

Occur in infants and children
Occur in antenatal or perinatal period and may be present in late infancy with hemiplagia or at seizure time

Are often seen in cardiac or sickle cell disease, but varicella infx is another cause.

Question 33

What happens in neural tube defects (NTD) ?

Answer 33

Include anencephaly, encephalocele, spina bifida occulta, meningocele and myelomeningocele.
Dropped due to screening
Birth prevalence reduced by periconceptual folic acid.

Myelomeningioceles can cause paralysis of legs, disclocation of hips and talipes, sensory loss, neuropathic bladder and bowel, scoliosis, hydrocephalus from the Chiari malformation.

Question 34

What are some causes of hydrocephalus?

Answer 34

Enlargment of cerebral ventricles due to accumulation of CSF. Caused by obstruction and very rarely by overproduction of CSF.
Non-communicating- obstruction to ventricular system
(Intra- ventricular)
1. Congenital malformation:
Aqueduct stenosis, ventriculitis, brain tumor.
Atresia of the outflow foramina of the 4th ventricle (Dandy- Walker malformation)
Chiari malformation
2. Posterior fossa neoplasm or vascular malformation
3. Intraventricular haemmorhage in preterm infant.

Communicating causes: Failure to absprb CSF:

1. Subarachnoid haemorrhage
2. Meningitis: e.g. Pneumococcal, tuberculous
3. Arnold- Chiari malformation (obstruction blocks outflow of 4th ventricle)

Question 35

What happens in hydrocephalus?

Answer 35

In infants: presents with Xss increase in head circumference rapidly, separatiom of skull sutures, bulging of anterior frontanelle, distenstiom of scalp veins and sun setting of eyes. (Eyes deviating downwards)

Older kids present with raised ICP

Tx- Ventriculo-peritoneal shunt (VP shunt- into peritoneal cavity to allow for the childs growth. Right atrial Catheters require revision during growth)

Dx- on ANC , if suspected:cranial USS/CT/MRI
Monitor head circumference.

Question 36

What are some neurocutaneous syndromes?

Answer 36

Neurofibromatosis
Tuberous sclerosis
Sturge- Weber syndrome (port -wine stain in the distribution of the trigeminal nerve)

Question 37

What happens in neurodegenerative disorders?

Answer 37

Cause deterioration in motor and intellectual fx.
Abn neuro features including seizures, spactisity..
1.Lysosomal storage disorders- lipid sorage
2. Peroxisomal enzyme defects/ accumulation of very long chain fatty acids (VLCFAs)
3. Heterodegenerative disorders- Huntingtons- progressive dystonia, dementia, seizures, corticospinal tract signs.
4. Wilsons disease: accumulation of Cu- behaviour changes, neurological and hepatic signs
5. Subacute sclerosing panencephalitis (SSPE)
Delayed adolescence response to measels causing neuro regression, with characteristic EEG - rare due to measels immunisation.

Developmental regression with evolution of abnormal neurological signs.

Question 38

What happens in Cerebral Palsy (CP)

Answer 38

Disorser of motor function due to non- progressive lesion (but signs evolve as Nervous system develops) that occurred as a result of a single insult.
Main causes:
Congenital infections (CMV, Rubella, TP), birth asphyxia, IVH, HIA, hyperbilirubinaemia, hypoglycaemia, head injury, intracranial infection.
Most cases have antenatal cause.
RFs:
Maternal pre-eclampsia, prematurity, fetal distress in labour, neonatal cyanosis, fits, hypothermia.

CFs:
Delayed motor milestones, abnormal tone, feeding difficulties, speech delay.
Dx: clinically by abnormalities of tone, power reflexes, movement or posture.

Mx- physiotherapy (reduce hypotonia, prevent contractures) , braces to allow walking, surgery e.g. For contractures, support and education,
Antispasmotics

Assc problems- learning difficulty, seizures, reflex, feeding problems, recurrent pneumonias.

Question 39

What are some different types of CP?

Answer 39

Spastic CP: damage to UMN , increased tone, brisk tendon reflexes, extensor plantar response.
Divide into hemiparesis: (damage to internal capsule, one side, arm worse) , diplegia (periventricular infraction, legs), quadriplegia.
Most cases.

Dykinetic CP- damage to extrapyramidal or basal ganglia pathways e.g. Kernicterus . Get abnormal movements e.g. Chorea, atherosis, dystonia.

Ataxic CP- damage to cerebellum, early hypotonia w/ poor balance and lack of coordination.

Question 40

What happens in prolonged convolusions?

Answer 40

Cause brain damage due to hypoxia. Maintain airway, check glucose, give PR diazepam or IV lorazepam, then PR paraldehyde, then IV phenytoin.
If fails, intubate and ventilate.

Usually due to epilepsy, head trauma, febrile, intracranial infx or metabolic.