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Medicine, Year 4, Paediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

What is the first-line and second-line management for generalised tonic-clonic seizures?

A

First line: Sodium Valproate

Second line: Lamotrigine / Carbamazepine

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2
Q

What is the first-line and second-line management for focal seizures?

A

First line: Carbamazepine / Lamotrigine

Second line: Sodium Valproate / Levetiracitam

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3
Q

How can focal seizures aka partial seizures be classified?

A
  • Awareness level i.e. focal aware, or focal impaired
  • Motor or non motor
  • Site in brain i.e. Temporal, frontal, parietal or occipital
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4
Q

What are the features characteristic of a focal seizure affecting the temporal lobe?

A

HEAD mnemonic:

  • H: Hallucinations
  • E: Epigastric rising, emotional
  • A: Automatisms i.e. lip smacking / grabbing, plucking
  • D: Dysphasia, De-ja vu
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5
Q

What are the features characteristic of a focal seizure affecting the frontal lobe?

A

Motor signs:

  • Leg / head movements
  • Jacksonian march
  • Postictal weakness
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6
Q

What are the features characteristic of a focal seizure affecting the parietal lobe?

A

Parietal = Paresthesia

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7
Q

What are the features characteristic of a focal seizure affecting the occipital lobe?

A

Visual disturbances

- Flashes & floaters

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8
Q

In simple terms, outline what a seizure is?

A

Transient episodes of abnormal electrical activity in the brain

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9
Q

How can Generalised Seizures be classified?

A
  • Not by awareness level, as all patients are unconscious
  • Tonic
  • Clonic
  • Tonic-Clonic
  • Atonic
  • Myoclonic
  • Absence
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10
Q

For Absence Seizures:

  • What is the typical onset in age?
  • What is the duration of them?
  • What are the EEG findings?
  • What is the prognosis?
  • What is the management?
A
  • Onset: 4-8 years old
  • Duration: up to 30s, fast recovery
  • EEG: 3Hz spike and wave
  • Prognosis: 90%+ become seizure free by adolescence
  • Treatment: Sodium Valproate / Ethosuxamide
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11
Q

For Febrile Seizures:

  • What is the typical onset in age?
  • What is the duration of them?
  • What is the usual cause?
  • What is the prognosis?
  • What is the classification?
A
  • Onset: 6mo - 5 years old
  • Duration: upto 5 mins usually
  • Cause: Caused by a fever, typically influenza, otitis media
  • Prognosis: Good prognosis, majority only have 1 episode
  • Classification: Simple & complex
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12
Q

Outline the classification of Febrile seizures into simple

and complex

A

Simple - Last less than 15mins, usually tonic-clonic, and only occur once during febrile illness

Complex - Last longer than 15mins, can have focal seizures, and may occur multiple times during febrile illness

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13
Q

What is type of generalised seizure is associated with Lennox-Gastaut Syndrome?

A

Atonic seizures

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14
Q

For Infantile Spasms:

  • What is it also known as?
  • What is the typical onset in age?
  • Describe the spasms?
  • What is the duration?
  • What are the EEG findings?
  • What is the prognosis?
  • What is the management?
A
  • Known as: West Syndrome
  • Onset: Few months of life
  • Spasm description: Flexion of the head, trunk, arms, followed by extension of the arms (Salaam attack)
  • Duration: 1-2secs, upto 50x a day
  • EEG: Hypsarrhythmia
  • Prognosis: Poor
  • Management: Vigabatran, ACTH
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15
Q

For Benign Rolandic Epilepsy:

  • Describe the epilepsy?
  • Prognosis?
A
  • Description: Parasthesia, usually on face

- Prognosis: Outgrown by puberty

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16
Q

State the various Seizures associated with children

A
  • Absence seizures
  • Febrile seizures
  • Infantile spasms (West Syndrome)
  • Lennox-Gastaut Syndrome
  • Benign Rolandic Epilepsy
  • Juvenile Myoclonic Epilepsy
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17
Q

For Juvenile Myoclonic Epilepsy:

  • What is it also known as?
  • What is the typical onset in age?
  • Describe the epilepsy?
  • Management?
A
  • Known as: Janz syndrome
  • Onset: Teen years, more common in GIRLS
  • Description:
    1. Generalised seizures in morning
    2. Daytime absences
    3. Sudden myoclonic seizures
  • Management: Sodium Valproate
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18
Q

For Lennox-Gastaut syndrome:

  • What is the typical onset in age?
  • What is it associated with?
  • Describe the seizure?
  • What are the EEG findings?
  • What is the management?
A
  • Onset: 1-5 years of age
  • Association: Infantile spasms
  • Description: Atypical absences
  • EEG: Slow spike
  • Management: Ketogenic diet
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19
Q

In simple terms, outline what a breath holding spell is?

A

A breath holding spell refers to involuntary episodes during which a child holds its breath, and is usually triggered by something upsetting or stressful

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20
Q

What is the epidemiology of breath holding spells?

A

Common in children between 6-18 months. Usually outgrown by 4-5 years old

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21
Q

What are the two types of breath holding spells? Describe them?

A
  • Cyanotic breath holding spells
    Child is upset, and will cry followed by breath holding, will become cyanotic and lose consciousness
  • Pallid breath holding spells (aka reflex anoxic spells)
    Child is startled, stimulating the vagus nerve to send impulses to reduce heart contractility. Child will go pale and lose consciousness
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22
Q

In simple terms, outline what cerebral palsy is?

A

Cerebral palsy is a NON-PROGRESSIVE neurodevelopmental disorder causing loss of muscle control

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23
Q

How is Cerebral Palsy classified?

A

Classified on area of brain affected:

PYRAMIDAL
- Spastic (affects cortex)

EXTRA-PYRAMIDAL

  • Athetoid / Dyskinetic (affects basal ganglia)
  • Ataxic (affects cerebellum)

Mixed

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24
Q

Describe the features of Spastic Cerebral Palsy

A

Muscles are stiff and hypertonic, patient may have a scissor gait (adductor muscle flexion) or toe walk (calf muscle flexion)

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25
Q

Describe the features of Athetoid / Dyskinetic Cerebral Palsy

A

Slow, involuntary, writhing movements, dystonia, chorea

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26
Q

What are some treatments for muscle spasms exhibited in Cerebral Palsy?

A
  • Oral diazepam
  • Oral / intrathecal baclofen
  • Botox Type A
  • Orthopaedic surgery
  • Selective dorsal rhizotomy
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27
Q

What are the two types of Dystrinopathies?

A
  • Duchenne Muscular Dystrophy

- Beckers Muscular Dystrophy

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28
Q

Name all of the Muscular Dystrophies (7 types)

A
  • Duchenne Muscular Dystrophy
  • Beckers Muscular Dystrophy
  • Myotonic Muscular Dystrophy
  • Fascioscapulohumeral Muscular Dystrophy
  • Oculopharyngeal Muscular Dystrophy
  • Limb-girdle Muscular Dystrophy
  • Emery-Dreifus Muscular Dystrophy
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29
Q

What is the function of the Dystrophin protein?

A

Dystrophin helps anchor transmembrane proteins to the intracellular actin cytoskeleton

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30
Q

What is the inheritance pattern of Duchenne and Becker’s Muscular Dystrophy?

A

X-Linked recessive

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31
Q

What mutations are Duchenne and Becker’s Muscular Dystrophy caused by?

What is the difference is age of presentation and severity of symptoms?

A

Duchenne:

  • Nonsense mutation / Frameshift mutation
  • Age of presentation before age 5
  • More severe symptoms

Beckers: Missense mutation

  • Age of presentation between 10-20 years old
  • Less severe symptoms
32
Q

What two signs are typically seen in Duchenne / Becker’s Muscular Dystrophies?

A

Gower’s sign: Child using arms to stand up from a squatted position

Calf pseudohypertrophy: Enlarged calves from fat and fibrotic tissues

33
Q

What are tests which can help diagnose Duchenne / Becker’s Muscular Dystrophy?

A
  • Elevated creatinine kinase
  • Positive mutations in Dystrophin gene (DNA test, Western blot)
  • Muscle biopsy (positive stain for Dystrophin)
34
Q

What is the life expectancy for Duchenne and Becker’s Muscular Dystrophy?

A

25-35 years of age

35
Q

What is the management for Duchenne and Becker’s Muscular Dystrophy?

A
  • Creatinine supplementation: Improves muscule strength

- Oral steroids: Slow progression of muscle weakness by 2 years

36
Q

Becker’s Muscular Dystrophy is characterised by early involvement of what in the heart?

A

Right ventricle

37
Q

What are the two most common causes of death in DMD patients?

A
  • Dilated cardiomyopathy

- Respiratory failure due to weakened diaphragm

38
Q

Duchenne and Becker’s Muscular Dystrophy is more common in which gender? What about the opposite gender?

A

Males

Females are asymptomatic or can be manifesting carriers

39
Q

Outline the features of Fascioscapulohumeral Muscular Dystrophy?

A
  • Weakeness around face -> progresses to shoulder and arms

- Patients may sleep with eyes slightly open, and struggle to blow out cheeks without air leaking

40
Q

Outline the features of Oculopharyngeal Muscular Dystrophy?

A

Weakness of ocular muscles and phargynx -> Ptosis and dysphagia

41
Q

Outline the features of Limb-girdle Muscular Dystrophy?

A

Progressive weakness around hips and shoulders

42
Q

Outline the features of Emery-Dreifuss Muscular Dystrophy?

A

Contractures in elbows and ankles

43
Q

What is the inheritance pattern of Myotonic Muscular Dystrophy?

A

Autosomal Dominant

44
Q

What are the two types of Myotonic Dystrophy and what is the genetic basis of them? Which is more severe?

A

Type 1 (DM1) - MORE SEVERE

  • Trinucleotide expansion repeat of CTG on DMPK gene (>50 repeats)
  • Type 1 has a congenital form and an adult form
Type 2 (DM2)
- Tetranucleotide expansion repeat of CCTG on CNMP gene (>75 repeats)
45
Q

Outline how Myotonic Dystrophy demonstrates genetic anticipation?

A

Increased number of CTG/CCTG repeats due to slipped misparing, hence with each generation they have a higher number of repeats presenting earlier and with more severe symptoms

46
Q

What is the classic symptom associated with Myotonic Dystrophy?

A

Sustained muscle contractions, and difficulty relaxing -> unable to release grip from handshakes / doorknobs

47
Q

What facial features are associated with Myotonic Dystrophy?

A

Long, haggard myotonic face

Frontal balding

48
Q

What are some associations with Myotonic Dystrophy?

A
  • Insulin resistance / T2D
  • Cataracts
  • Cardiac arrhythmias: Prolonged PR interval
49
Q

Outline in simple terms, what Spinal Muscular Atrophy is?

A

A neuromuscular disorder characterised by premature death of the alpha-LMNs which supply the motor end plate, causing muscle atrophy

50
Q

What are the clinical features of Spinal Muscular Atrophy (SMA)?

A 
Reduced muscle bulk
Reduced muscle power
Reduced muscle tone
Reduced / absent deep tendon reflexes
Fasciculations
51
Q

Inheritance pattern of Spinal Muscular Atrophy?

A

Autosomal Recessive

52
Q

What are the different types of Spinal Muscular Atrophy? What is the genetic basis of this condition?

A

Type 1A, 1B, 2, 3, 4 (most -> least severe)
Caused by homozygous deletion of SMN1 gene. Some people do have a SMN2 gene which produces small amounts of functional protein

No. of SMN2 copies determines severity of Spinal Muscular Atrophy. If more SMN2 copies, less severe SMA

53
Q

Outline what Reye’s Syndrome is?

A

A hepato-encephalopathy caused by aspirin used during a viral illness (VZV, Influenza B)

54
Q

What condition would it be acceptable to prescribe Aspirin in children?

A

Kawasaki’s disease, where benefits outweigh the negatives

55
Q

What lab results may be suggestive of Reye’s Syndrome?

A
  • Increased ALT, AST
  • Increased Billirubin
  • Increased serum ammonia
56
Q

What other drugs aside from Aspirin can cause Reye’s Syndrome?

A
  • Tetracycline ABX
  • Sodium Valproate
  • HAART for HIV
57
Q

In simple terms, outline what Hydrocephalus is?

A

Refers to CSF abnormally building up in the brain and the spinal cord, due to overproduction or inadequate drainage

58
Q

Outline how CSF is produced and drained?

A

CSF is produced from the four ventricles of the brain, specifically the choroid plexus. The CSF drains into the venous system via the arachnoid granulations

59
Q

What connects the 3rd and 4th ventricle in the brain?

A

Cerebral aqueduct

60
Q

What are causes of Hydrocephalus in babies?

A
  • Cerebral aqueduct stenosis
  • Arachnoid cysts
  • Arnold-Chiari malformations
  • Chromosomal abnormalities and congenital malformations
61
Q

Outline what an Arnold-Chiari malformation is?

A

This refers to herniation of the cerebellum downwards into the foramen magnum, blocking CSF outflow

62
Q

What is the presentation of Hydrocephalus in babies?

A
  • Increased occipito-frontal circumference
  • Failure of UPWARD gaze “sunsetting eyes”
  • Bulging anterior fontanelle
  • Hypotonia
  • Sleepiness
63
Q

What is the management of Hydrocephalus in babies?

A

Ventriculo-peritoneal shunt, which drains CSF -> Peritoneal cavity

External ventricular drain, which drains CSF from R-lateral ventricle -> bedside

Surgery

64
Q

What are complications of a VP shunt?

A
  • Outgrowing it / replacement
  • Infection
  • Blockage
  • Excessive drainage
  • Intraventricular haemorrhage
65
Q

What is Craniosyntosis?

A

Skull sutures close prematurely, causing abnormal head shape and brain growth restriction

66
Q

What are the types of Craniosyntosis?

A
  • Saggital syntosis (most common)
  • Coronal syntosis
  • Metopic syntosis
  • Lambdoid syntosis
67
Q

What is Plagiocephaly and Brachycephaly?
Why does it occur?
What is the age of onset in babies?
Why has its prevalence increased?

A
  • Plagiocephaly - Oblique shaped head
  • Brachycephaly - Short shaped head

Occurs when a child has a tendency to rest their head on a particular point

Presents in babies aged 3-6 months

Prevalence has increased due to parents resting their babies in supine position to minimise risk of SIDS

68
Q

For Plagiocephaly and Brachycephaly, what is important to exclude?

A

Must exclude Craniosyntosis by palpating suture lines, and must exclude Congenital Muscular Torticollis (shortening of sternocleidomastoid muscles)

69
Q

How can Plagiocephaly and Bracycephaly be managed?

A
  • Reassurance that the majority will improve with time
  • Supervised time lying prone
  • Position on rounded side for sleep
  • Using rolled towels to support head
  • Minimise time in carseats / pushchairs
  • Plagiocephaly helmets (not routinely on NHS)
70
Q

In simple terms, outline what “Syncope” is?

A

Syncope refers to an event of temporarily losing consciousness due to disruption of blood flow to the brain, often causing a fall

71
Q

Outline how the pathophysiology of a Vasovagal attack?

A

A vasovagal attack is a problem with the autonomic nervous system regulating blood flow to the brain.

When the vagus nerve receives a strong stimulus -> stimulates PNS. PNS activation overrides SNS, causing blood vessels to relax, the BP in cerebal circulation to drop and hypoperfusion

72
Q

Outline symptoms of Prodome, which precipitates a Vasovagal attack?

A
  • Being hot and clammy
  • Sweaty
  • Vision going blurry
  • Feeling heavy
  • Feeling dizzy, light headed
  • Headache
73
Q

Is incontinence specific to syncope, or seizures?

A

Both syncope and seizures can bring on incontinence

74
Q

What are some primary causes of syncope?

A
  • Dehydration
  • Fasting
  • Extended standing in a warm environment
  • Vasovagal response to pain, sight of blood etc
75
Q

What are some secondary causes of syncope?

A
  • Hypoglycaemia
  • Anaemia
  • Infection
  • Anaphylaxis
  • Arrhythmias
  • Valvular heart disease
  • HOCM
76
Q

What things would you examine in a patient with syncope?

A
  • Cardiovascular exam for pulse, HR, rhythm, murmurs
  • Physical injuries due to the syncope
  • Lying and standing blood pressures
  • Neurological examination
  • Assess concurrent illness i.e. Infection / Gastroenteritis

Medicine, Year 4, Paediatrics (16 decks)

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