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1 - Paeds AE > Neurological disorders > Flashcards

Neurological disorders Flashcards

1
Q

What are the different classifications of headaches?

A

Primary: migraine, tension, cluster and other primary headaches (primary malfunction of neurones)
Secondary: symptomatic of an underlying pathology (raised ICP, space-occupying lesion)
Trigeminal and other cranial neuralgia, and other headaches including root pain from herpes zoster

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2
Q

What is a tension headache?

A

Symmetrical headache of gradual onset, often described as tightness, a band or pressure. There are usually no other symptoms.

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3
Q

What is the presentation of the headache of a migraine? (with or without aura)

A

In children, episodes may last 1-72hrs.
Commonly bilateral but may be unilateral.
Characteristically pulsatile over temporal or frontal area.
Often accompanied by unpleasant GI disturbance such as nausea, vomiting and abdo pain and photophobia or photophobia. Aggravated by physical activity

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4
Q

What are the most common visual disturbances in an aura?

A

Negative phenomena - hemianopia or scotoma

Positive phenomena - fortification spectra

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5
Q

What can trigger a migraine?

A

Late nights
Early rises
Stress
Winding down after stress at home or school
Certain foods - cheese, chocolate and caffeine
Menstruation
COC

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6
Q

What can cause a secondary headache?

A 
Head and/or neck trauma
Crania or cervical vascular disorder
Non-vascular intracranial disorder
A substance or its withdrawal
Infection - meningitis, encephalitis
Disorder of homeostasis - HTN, hypercapnia
Disorder of facial or cranial structure
Psychiatric disorder
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7
Q

What is specific about headaches due to a space occupying lesion?

A

Worse when lying down and morning vomiting.
May also cause night-time wakening.
Often a change in mood, personality or educational performance.

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8
Q

What are some additional symptoms to a headache that may suggest a space occupying lesion?

A 
Visual field defect
Cranial nerve abnormalities causing diplopia, new-onset squint or facial nerve palsy
Abnormal gait
Torticollis
Growth failure
Pappiloedema
Cranial bruits
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9
Q

How can you acutely treat migraines?

A

Analgesia - paracetamol and NSAIDs
Anti-emetics - metoclopramide
Serotonin agonists - sumatriptan

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10
Q

How can you prevent migraines medically?

A

Pizotifen
Beta blockers - propranolol
Sodium channel blockers - valproate

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11
Q

What is a febrile seizure?

A

A seizure accompanied by a fever in the absence of intracranial infection due to bacterial meningitis or viral encephalitis

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12
Q

What age does febrile seizures cover?

A

6 months and 6 years

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13
Q

When is the course of the viral infection does the seizure occur?

A

Usually early in a viral infection when the temperature is rising rapidly.

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14
Q

What type of seizures happen in febrile seizures?

A

Usually brief, generalised tonic-clonic seizure

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15
Q

What makes a seizure complex?

A

Focal, prolonged or repeated in the same illness

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16
Q

Does having febrile seizures mean you’re at an increased risk of epilepsy?

A

Simple: no
Complex: yes

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17
Q

How would you manage febrile seizures?

A

Treat cause.
First aid taught to the family
If a history of prolonged seizures, rescue therapy with rectal diazepam or buccal midazolam can be supplied

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18
Q

What are some causes of paroxysmal disorders? (funny turns)

A

Breath holding attacks (temper)- treat with distraction therapy
Reflex anoxic seizures (head trauma, cold food, fright, fever)
Syncope
Migraine
Benign paroxysmal vertigo

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19
Q

What are the two classifications of seizures?

A

Generalised - absence, myoclonic, tonic, tonic-clonic, clonic
Focal - frontal, temporal, occipital, parietal

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20
Q

What are the symptoms of a frontal seizures?

A

involve the motor or premotor cortex. May lead to clonic movements, which may travel proximally (Jacksonian march). Asymmetrical tonic seizures can be seen, which may be bizarre and hyperkinetic and can be mistakenly dismissed as non-epileptic seizures. Atonic seizures may arise from mesial frontal discharge

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21
Q

What are the features of a temporal seizure?

A

Strange warning feelings or aura with smell and taste abnormalities and distortions of sound and shape. Lip-smacking, plucking at one’s clothing and walking in a non-purposeful manner may be seen. Deja-vu and jamais-vu are described

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22
Q

What are the features of occipital seizures?

A

Causes distortion of vision

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23
Q

What are the features of parietal seizures?

A

Contralateral dysaesthesias or distorted body image

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24
Q

What happens to consciousness during focal seizures?

A

It may be retained, consciousness may be lost or the seizure may be followed by generalised tonic-clonic seizures

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25
Q

When are MRI or CT brain scans indicated in seizures?

A

If there are neurological signs between seizures or if seizures are focal, in order to identify a tumour, vascular lesion or area of sclerosis which could be treated.

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26
Q

What functional scans can be used in epilepsy?

A

PET or SPECT

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27
Q

What happens during absence seizures?

A

Transient loss of consciousness, with an abrupt onset and termination, unaccompanied by motor phenomena except for some flickering of the eyelids and minor alterations in muscle tone.

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28
Q

What happens during myoclonic seizures?

A

Brief, often repetitive, jerking movements of the limbs, neck or trunk.

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29
Q

What happens during tonic seizures?

A

Generalised increase in tone

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30
Q

What happens during tonic-clonic seizures?

A

Rhythmical contraction of muscle groups following the tonic phase.
Tonic - child may fall to ground, sometimes injuring themselves, they do not breath and become cyanosed.
Clonic - Jerking of the limbs, breathing is irregular, cyanosis persists and saliva may accumulate in the mouth.

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31
Q

What happens during atonic seizures?

A

Often combined with a myoclonic jerk, followed by a transient loss of muscle tone causing a sudden fall to the floor or drop of the head.

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32
Q

What seizures should you not use carbamazepine in?

A

Absence and myoclonic seizures, it can make them worse

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33
Q

What is the first-line treatment for generalised seizures?

A

Valproate

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34
Q

What advice should you give when managing epilepsy?

A

Avoid deep baths, swimming alone, discuss driving, contraception and pregnancy

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35
Q

What are the three main brain areas controlling movement?

A

Motor corte
Basal ganglia
Cerebellum

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36
Q

What are some examples of corticospinal (pyramidal) tract disorders?

A 
Global hypoxia-ischaemia
Arterial ischaemic stroke
Cerebral tumour
Acute disseminated encephalomyelitis
Post-ictal paresis
Hemiplegic migraine
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37
Q

What are some examples of basal ganglia disorders?

A

Acquired brain injury - acute and profound hypoxia-ischaemia, carbon monoxide poisoning
Post-strep chorrea
Wilsons disease
Huntington disease

38
Q

What are some examples of cerebellar disorders?

A

Acute - medication and drugs -alcohol and solvent abuse
Post-viral - particularly varicella
Ataxis cerebral palsy
Friedreich ataxia

39
Q

What are the presentations of a neuromuscular disorder?

A 
WEAKNESS - progressive or static
Floppiness
Delayed motor milestones
Muscle weakness
Unsteady/abnormal gait
Fatiguability
Muscle cramps
40
Q

How can you tell if a child has a myopathy?

A

Waddling gait and Gowers sign (proximal muscle weakness)

41
Q

What is Gowers sign?

A

The need to turn prone to rise to a standing position from a supine position. ‘Climb up the legs to get to the hands’

42
Q

What type of neuromuscular disorder does increasing fatiguability throughout the day, often with opthalmoplegia and ptosis point towards?

A

Depletion at the motor end-plate and a diagnosis of myasthenia gravis

43
Q

What are some categories of neuromuscular disorders?

A

Disorders of the anterior horn cell
Disorders of the peripheral nerve
Disorders of neuromuscular transmission
Muscle disorders

44
Q

What are some disorders of the anterior horn cell?

A

Spinal muscular atrophy

Poliomyelitis

45
Q

What are some disorders of the peripheral nerve?

A

Hereditary motor sensory neuropathies
Acute post-infectious polyneuropathy (Guillain-Barre)
Bell Palsy

46
Q

What are some disorders of neuromuscular transmission?

A

Myasthenia gravis

47
Q

What are some muscle disorders?

A

Muscle dystrophies - Duchenne/Becker/congenital
Inflammatory myopathies - benign acute myositis, polymyositis, dermatomyositis
Myotonic disorders - dystrophia myotonic
Metabolic myopathies
Congenital myopathies

48
Q

What are some myopathy investigations and what do they show?

A

Serum creatine phosphokinase - markedly elevated in Duchenne and Becker muscular dystrophy and inflammatory myopathies
DNA testing - to identify abnormal genes
Muscle biopsy, needle or open

49
Q

What are sone neuropathy investigations?

A

Nerve conduction studies - to identify delayed motor sensory nerve conduction velocities seen in neuropathy
DNA testing
EMG (electromyography) - helps in differentiating myopathic and neuropathic disorders

50
Q

What are the symptoms of anterior horn cell disorders?

A

There are signs of denervation: weakness, loss of reflexes, fasciculation and wasting as the nerve supply to the muscle fails

51
Q

What are the symptoms of neuropathy?

A

Often distal nerves affected.
Motor neuropathy will give weakness
Sensory neuropathy will give impaired perception of pain and temperature, or touch
Loss of reflexes in either

52
Q

What are the symptoms of myopathy?

A

There is weakness (often proximal), wasting, gait disturbance

53
Q

What are the symptoms of neuromuscular junction disorders?

A

As end-plate ACh stores become depleted, there is diurnal worsening through the day, leading to fatiguability

54
Q

What is spinal muscular atrophy?

A

An autosomal recessive degeneration of the anterior horn cells, leading to progressive weakness and wasting of skeletal muscles due to mutations in the survival motor neurone gene.

55
Q

When does Guillain Barre present?

A

2-3 weeks after an URTI or campylobacter gastroenteritis

56
Q

How does Guillain Barre present?

A

There may be fleeting abnormal sensory symptoms in the legs, but the prominent feature is an ascending symmetrical weakness with loss of reflexes and autonomic involvement.

57
Q

What are the serious complications of Guillain Barre?

A

Involvement of bulbar muscles leads to difficulty in chewing and swallowing and the risk of aspiration.
Respiratory depression may require artificial ventilation

58
Q

What is the prognosis of Guillain Barre?

A

Although full recovery may be expected in 95% of cases, this may take up to 2 years

59
Q

What investigations would you do in Guillain Barre and what would they show?

A

CSF protein is characteristically markedly raised, but this may not be seen until the second week of illness.
CSF WCC is NOT raised.
Nerve conduction velocities are reduced

60
Q

How would you manage Guillain Barre?

A

Supportively, regarding respiration.

Corticosteroids have NO beneficial effect and may delay recovery

61
Q

What is Bell palsy?

A

An isolated motor neurone paresis of the VIIth cranial nerve leading to facial weakness.

62
Q

How would you treat Bell palsy?

A

Corticosteroids may be of value in reducing oedema in the facial canal during the first week.
Recovery is complete in the majority of cases but it may take several months

63
Q

What is the main complication of Bell palsy?

A

Conjunctival infection due to incomplete eye closure on blinking.

64
Q

What is an important differential for Bell palsy?

A

Compressive lesion in the cerebellopontine angle (if VIIIth nerve paresis is also present)

65
Q

What cardiac condition in Bell palsy associated with?

A

Coarctation of the aorta

Check for HTN because of this

66
Q

What is myasthenia gravis?

A

Abnormal muscle fatiguability with improves with rest of ACh drugs

67
Q

What is juvenile myasthenia ?

A

Similar to adult autoimmune myasthenia.

Due to binding of antibody to ACh receptors on the post-junctional synaptic membrane.

68
Q

When and how does juvenile myasthenia present?

A

Usually after 10 years of age.
Opthalmoplegia and ptosis, loss of facial expression and difficulty chewing. Generalised, especially proximal weakness may be seen

69
Q

How can you diagnose juvenile myasthenia?

A

Improvement following IV edrophonium.

Testing for ACh receptor antibodies or anti-MuSK.

70
Q

How do you treat juvenile myasthenia?

A

With cholinesterase inhibitors - neostigmine, pyridostigmine.
In the longer term - immunosuppressive therapy with prednisolone and azathioprine

71
Q

What are some muscular dystrophies?

A

Duchenne muscular dystrophy
Becker muscular dystrophy
Congenital muscular dystrophies

72
Q

How is Duchenne muscular dystrophy inherited and what is the chromosomal abnormality?

A

X-linked recessive disorder, although a third are from new mutations.
Deletion from the short arm of the X chromosome

73
Q

What is the pathophysiology of Duchenne muscular dystrophy?

A

Dystrophin deficiency.
Several aberrant intracellular signalling pathways associated with an influx of calcium ions, a breakdown of the calcium calmodulin complex and an excess of free radicals, ultimately leading to myofibril necrosis

74
Q

How does Duchenne muscular dystrophy present?

A

Waddling gait and/or language delay.
Mount stairs one by one and run slowly compared to friends.
Gowers sign.

75
Q

What age does DMD normally present?

A

5.5 years old

76
Q

What is the prognosis of DMD?

A

Can’t walk by age 10-14.

Life expectancy is reduced to late twenties from respiratory failure or the associated cardiomyopathy.

77
Q

How do you manage DMD?

A

Appropriate exercises - passive stretching and provision of night splints
Overnight CPAP or NIPPV
Ambulant children are increasingly treated with 10 days prednisolone each month to preserve mobility and prevent scoliosis

78
Q

What is the difference between Duchenne muscular dystrophy and Becker muscular dystrophy?

A

Becker is similar but the disease progresses more slowly.

The average age of presentation is 11, inability to walk in late 20s and life expectancy being from late 40s to normal

79
Q

What are the causes of the floppy infant?

A

Cortical - hypoxic-ischeemic encephalopathy, cortical malformations
Genetic - downs syndrome, prader-willi syndrome
Metabolic - hypothyroidism, hypocalcaemia
Neuromuscular - spinal muscular atrophy, myopathy, myotonia, congenital myasthenia

80
Q

What is Friedrich ataxia?

A

An autosomal recessive condition, caused by a trinucleotide repeat disorder.

81
Q

How does Friedrich ataxia present?

A

Worsening ataxia, distal wasting in the legs, absent lower limb reflexes but extensor plantar response because of pyramidal involvement, pes cavus and dysarthria.
Impairment of joint position and vibration sense, often optic atrophy.
Life expectancy - 40/50 years

82
Q

What are the causes of childhood stroke?

A

Cardiac - CHD
Haematological - sickle cell
Post-infective - following varicella or viral infection
Inflammatory - autoimmune disease e.g. SLE

83
Q

What are the symptoms of spina bifida?

A

Overlying skin lesion - hair tuft, lipoma, birth mark or small dermal sinus.
Neurological defects - bladder function and lower limb

84
Q

What are the symptoms of myelomeningocele?

A 
Paralysis of the legs
Dislocation of the hip and talipes
Sensory loss
Neuropathic bladder and bowel
Scoliosis
Hydrocephalus
85
Q

What is hydrocephalus?

A

Obstruction to the flow of CSF, leading to dilatation of the ventricular system proximal to the site of obstruction.

86
Q

What are the types of hydrocephalus?

A

The obstruction may be within the ventricular system or aqueduct - non-communicating/obstructive
The obstruction may be at the arachnoid villi, the sit of absorption of the CSF - communicating

87
Q

What are the causes of hydrocephalus?

A

Obstructive - congenital malformation, posterior fossa neoplasm or vascular malformation, intraventrical haemorrhage in preterm infant
Communicating - subarachnoid haemorrhage, meningitis

88
Q

What are the clinical features of hydrocephalus?

A

Infants - head circumference may be disproportionately large, anterior fontanelle bulges and scalp veins become distended, fixed downward gaze or sun setting of the eyes.
Older children - signs and symptoms of raised ICP

89
Q

How can you diagnose hydrocephalus?

A

Infants - routine cranial US scanning

Imaging with CT/MRI

90
Q

How do you treat hydrocephalus?

A

Symptomatic relief of raised ICP.

Insertion of ventriculo-peritoneal shunt.

91
Q

You need two or more of these criteria to diagnose neurofibromatosis type 1, what are they?

A

> 6 cafe-au-lait spots >5mm in before puberty, >15mm after puberty
1 neurofibroma
Axillary freckles
Optic glioma - may cause visual impairment
One Lisch nodule
Bony lesions from sphenoid dysplasia
A first-degree relative with NF1

92
Q

What are some neurocutaneous syndromes?

A

Neurofibromatosis
Tuberous sclerosis
Sturge-Weber syndrome

1 - Paeds AE (24 decks)

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