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Paediatrics > Neurology - TLOC/Ataxia/Brian Tumours/Devlopmental Regression/Other > Flashcards

Neurology - TLOC/Ataxia/Brian Tumours/Devlopmental Regression/Other Flashcards

1
Q

What are the potential causes of funny turns?

A
  • breath holding attacks - toddler holds breath when crying but rapidly recovers
  • reflex anoxic seizures - occurs when infant are in pain and they become hypoxic which may stimulate tonic clinic seizure
  • syncope
  • migraine
  • benign paroxysmal vertigo
  • cardiac causes - cardiomyopathy or prolonged QT
  • pseudoseizure
  • NAI
  • paroxysmal movement disorder
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2
Q

What is ataxia?

A

incoordination of movement, speech and posture due to either cerebellar or posterior pathway problems

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3
Q

What are the common causes of ataxia?

A

Causes of cerebellar ataxia

  • acute causes - meds, drugs, alcohol, solvents, trauma
  • post viral - seen with varicella infection
  • posterior fossa lesions - CPA syndrome
  • genetic and degenerative disorders

Genetic and degenerative disorders:

  • ataxic CP
  • friedreich’s ataxia
  • ataxia telangiectasia
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4
Q

What are the investigations for ataxia?

A

genetic testing
LP
brain scans to check for tumours and damage

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5
Q

What are the presenting features of brain tumours?

HEADSMART

A
  • persistent/recurrent vomiting
  • persistent/recurrent headache
  • balance/co-ordination/walking problems
  • abnormal eye movements or suspected loss of vision
  • double vision
  • behaviour change, particularly lethargy
  • fits/seizures
  • abnormal head position
  • increasing head circumference
  • delayed or arrested puberty
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6
Q

What is the usual history of breath holding?

A

brief periods where the young child stops breathing
cause child to pass out
usually occurs when child is angry, in pain or afraid
occurs in children most commonly between 1-3 years
should not cause serious damage
eventually resolve

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7
Q

What are the common causes of developmental regression?

A 
battens disease 
retts syndrome 
leukodystrophies 
wilsons disease 
SSPE (subacute sclerosis panencephalitis)
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8
Q

What is obstructive hydrocephalus?

A

obstruction may be within ventricular system or aqueduct

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9
Q

What is communicating hydrocephalus?

A

the obstruction may be at the arachnoid villi, the site of absorption for CSF (can also be due to CSF overproduction or venous drainage insufficiency)

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10
Q

What is external hydrocephalus?

A

this is a benign condition with a self limiting absorption deficiency of infancy and early childhood that leads to increased ICP.

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11
Q

What is macrocephaly?

A

head circumference above the 98th centile

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12
Q

What are the common causes of macrocephaly?

A 
tall stature 
familial macrocephaly 
raised ICP 
hydrocephalus 
chronic subdural haematoma 
cerebral tumour 
neurofibromatosis 
cerebral gigantism (sotos syndrome) 
CNS storage disorders (hurler syndrome)
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13
Q

What are the common causes of microcephaly (<2nd centile)?

A
  • familial - when present at birth and development normal
  • autosomal recessive condition - when associated with developmental delay
  • caused by congenital infection
  • acquired after an insult to the developing brain e.g. perinatal hypoxia, hypoglycaemia
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14
Q

How are the different types of headache divided?

A

primary - migraines, tension-type headache, cluster headache

secondary - symptomatic of underlying pathology

trigeminal and other cranial neuralgias - including nerve root pain

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15
Q

What are the headache red flag symptoms for a SOL?

A 
headache worse on lying down/coughing 
wakes up child 
confusion 
morning/persistent N&amp;V 
personality, behavioural or educational change
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16
Q

What are the headache red flag signs for a SOL?

A 
growth failure 
visual field defects - crainopharyngioma 
squint 
cranial nerve abnormality 
torticollis 
abnormal coordination - cerebellar
gait change 
papilledema in fundus 
bradycardia 
cranial bruits - AVM
17
Q

What are the treatment options for raised ICP?

A

Tumour – removal surgically or radio/chemotherapy
Subdural haematoma – surgical drainage Hydrocephalus – ventriculoperitoneal shunt
Idiopathic - specific diuretics can be prescribed by the neurologist.
Hypoventilation of the patient can temporarily
decreased ICP by decreased CO2 and causing vasoconstriction
Craniotomy may be performed if other procedures have not worked

18
Q

What are the causes of subdural haematoma?

A

characteristic of NAI caused by shaking or direct trauma to infants or toddlers

occasionally seen after a fall from height

19
Q

What are the symptoms of subdural haematoma?

A 
altered mental state 
seizures 
apnoea 
breathing difficulties 
headaches 
lethargy 
sudden cardiac arrest
20
Q

What is craniosynostosis?

A

premature fusion of one or more sutures

21
Q

What are the features of myotonic dystrophy?

A

poor feeding
failure to meet milestones and hypotonia
progressive distal muscular weakness, ptosis, weakness and thinning of the face and SCM along with a carp mouth

22
Q

What are the features of Duchenne Muscular Dystrophy (DMD)?

A 
waddling gait 
language delay 
slower than peers 
Gowers sign 
serum creatinine kinase is elevated
life expectancy is late 20s - respiratory failure, cardiomyopathy
23
Q

How is DMD treated?

A 
Physio to prevent contractures 
Overnight CPAP
Corticosteroids to preserve mobility and prevent scoliosis 
Night splints 
Passive stretching
24
Q

What is a congenital muscular dystrophy?

A 
heterogeneous group of disorders 
recessive inheritance 
present with weakness, hypotonia or contractures
present at birth 
longer life expectancy than DMD
25
Q

What are the features spinal muscular atrophy?

A 
progressive weakness 
denervaion 
areflexia 
fasciculations 
wasting 
severe form - death from resp failure in 12 months
26
Q

What is spinal muscular atrophy?

A

recessive degeneration of anterior horn cells

SMN gene

27
Q

What is the most severe type of spinal muscular atophy?

A

spinal muscular atrophy type 1 (Werdnig-Hoffman disease)

28
Q

How does Gullain-Barre syndrome present?

acute post-infectious polyneuropathy

A

presentation is typically 2-3 weeks after an URTI or gastroenteritis
ascending symmetrical weakness
areflexia
difficulty swallowing

29
Q

How is Gullain-Barre managed?

A

full recovery in 95% of cases

management is supportive esp with respiration

30
Q

What is Bell’s Palsy?

A

LMN - facial weakness

must rule out compressive lesion in cerebellopontine angle and sarcoidosis if bilateral weakness

31
Q

How is Bell’s Palsy managed?

A

corticosteroids to reduce oedema
associated with HSV infection
usually full recovery

32
Q

What is Charcot Marie Tooth disease?

A

distal muscle wasting and sensor loss with proximal progression over time
usually autosomal dominant but can occur without family history

33
Q

What are the features of Charcot Marie tooth disease?

A 
muscle weakness and wasting starting distally 
sensory loss is similar
generalised tendon areflexia 
may be foot drop and difficulty walking 
spinal deformities in 50%
34
Q

How is Charcot Marie tooth disease managed?

A

supportive management

no effective treatment

35
Q

What is CIDP (chronic inflammatory demyelinating polyneuropathy)?

A

Acquired, immune mediated inflammatory disorder of the PNS

Thought to be a chronic version of Charcot Marie Tooth disease

36
Q

What are the features of CIDP?

A 
Relapsing symptoms 
Proximal and distal limbs are affected 
Weakness 
Tingling and numbness 
Deep tendon reflexes reduced and gait is abnormal
37
Q

How to tension headaches present?

A

symmetrical
bilateral
gradual evening onset

38
Q

How do migraines present?

A 
unilateral 
relieved by sleep 
worse on exertion 
photophobia 
90% in children without an aura 
10% aura - most commonly visual disturbance
39
Q

What is Becker Muscular Dystrophy?

A

onset 11 years
similar to duchenne but slower progression
life expectancy late 40s - normal

Paediatrics (14 decks)

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