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Neuro Flashcards

1
Q

Seizures are twice as common in preterm neonates as term T/F

A

False - they are 4 times more common in preterm

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2
Q

Infection is the second most common cause of seizure in neoates T/F

A

False
1st HIE
2nd Cerebral infarction

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3
Q

AEDs used in the neonatal period achieve good seizure control in 80% of patients T/F

A

False

Relatively low rates of control (about 40-60%)

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4
Q

Prognosis of neonatal seizure is very poor T/F

A

False

Favourable outcomes in about 70% (although decreased to 50% for seizures secondary to HIE)

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5
Q

Studies suggest that AEDs used to control seizures in the neonatal period may themselves lead to neuronal death with lasting effects on development T/F

A

True

However a balance needs to be struck between treatment side effects and the risks of ongoing seizures

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6
Q

What is Devic’s disease AKA?

A

Neuromyelitis optica

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7
Q

What is the treatment of neuromyelitis optica?

A

Plasmapheresis (as opposed to high dose steroids for MS)

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8
Q

What two features are suspicious for NMO (neuromyelitis optica).
Lab test?

A

Optic neuritis and transverse myelitis

Lab: aquaporin antibodies

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9
Q

What lab tests can help with the diagnosis of sydenham’s chorea?

A

Anti-streptolysin O titres and anti-DNAse B antibodies

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10
Q

Nerve conduction studies are the best way to diagnosis SMA (spinal muscular atrophy) type 1 T/F

A

F - neonatal nerve conduction studies are difficult to interpret and may be delayed until 6 months old

Diagnosis: genetic testing for del on chr 5q (SMN1) - 95% of cases; muscle biopsy may be needed for genetic testing is not definitive

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11
Q

SMA type one is typically caused by a del on chr 10q T/F

A

F - del on chr 5q (SMN1 gene)

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12
Q

Centrotemporal spikes on an EEG are diagnostic of what seizure disorder?

A

Benign rolandic epilepsy (BECTS - benign epilepsy with centrotemporal spikes)

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13
Q

3/s spikes on EEG what sz disorders?

A

Absence

AKA spike and wave pattern

Note: can be induced by hyperventilation

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14
Q

Vertical nystagmus where is the lesion?

A

Brainstem at pontomedullary junction (eg Arnold Chiari malformation)

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15
Q

In vestibular nystagmus the slow phase is directed to the side of the lesion T/F

A

True

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16
Q

In cerebellar nystagmus the fast component is directed toward the side of the lesion T/F

A

True

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17
Q

Triad of spasmus nutans

A

Head nodding
Torticollis
Pendular nystagmus

Note: presents in the first 2 yrs of life and can resolve spon

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18
Q

The first sz in a child with significant HA needs urgent eval preferably with a CT brain T/F

A

F - needs an MRI, CT may miss some small lesions

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19
Q

What type of epilepsy presents with seizure with prominent autonomic features (pallor, emesis or retching)

A 
Panayiotopoulous syndrome (AKA early-onset benign partial epilepsy with occipital paroxysms)
Note: Typically in those 1-14yrs, 10% or more off all epilepsy in childhood
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20
Q

Common side effects of sodium valproate

A

Metabolic: weight gain, increased appetite, insulin resistance, metabolic syndrome, hair loss. Link with PCOS
Haem: easy bruising due to thrombocytopenia and other coag abnormalities
Also tremor

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21
Q

Common side effects of carbamazepine

A

Diplopia
Ataxia
Also link with SJS

Note: lamotrigine also commonly causes diplopia and ataxia

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22
Q 
What AED causes: 
Nystagamus 
Gum hypertrophy
Hirsutism
Anaemia
Liver problems
Rash (SJS assoc)
Decreased folic acid
Decreased bone density
A

Phenytoin

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23
Q

Weight loss is a side effect of what AED

A

Topiramate

Note: other side effects - slow thinking, paraesthesia, metabolic acidosis, calcium phos nephrolithiasis (need to keep hydrated)
Also inhibits renal carbonic anhydrase and so can cause proximal and distal acidification defects

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24
Q

Panayiotopoulous syndrome has a poor prognosis T/F

A

F - prognosis is excellent and most children usually go into spon remission after a few sz and treatment if often unneccessary

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25
Q

Panayiotopoulous syndrome is characterised by very low frequency of episodes, sleep onset, prolonged duration and preserved neurodevelopment. T/F

A

T

Note: AKA early onset benign partial epilepsy with occipital paroxysms

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26
Q

How does Landau-Kleffner present?

A

Regression in language skills, subacute onset of acquired aphasia in a child with previously normal development and cognition
Seizures usually accompany progression of syndrome

Note: characterised by EEG abnormalities which are seen in sleep
Differentiate from Lennox Gastaut - regression usually starts after the seizures

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27
Q

1st and 2nd line tx of absence seizures?

A

1st: ethosuximide or valproate
2nd: lamotrigine

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28
Q

Which is the most likely AED to cause problems in the foetus?

A

Sodium valproate

Note: increased risk of 6 specific malformations- spina bifida, ADS, cleft lip, hypospadias, polydactyly and craniosynostosis

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29
Q

Which AED - facial dysmorphism, DD, cleft lip and palate, microcephaly, hypo plastic nails and skeletal malformations?

A

Phenytoin

Note: fetal hydantoin syndrome

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30
Q

Auditory brain stem evoked response is the most appropriate way to check the hearing of a 5 yr old T/F

A

F - it can be done at any age but it is a long test.

Note: Pure tone audiometry or play audiometry would be more appropriate for this age (uses headphone and plays different tones so some cooperation is required).
Otoacoustic emissions is a useful screening test but cannot distinguish between conductive and sensory deficits.

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31
Q

Typical presentation and age for infantile spams?

A

Flexion-extension spasm
3 months - 1 yr
EEG hypsarrhythmia
M > F

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32
Q

What is Werdnig Hoffman disease

A

Spinomuscular atrophy type 1

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33
Q

What is the gold std to differentiate epileptic sz from non-epileptic attack disorder

A

Video telemetry

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34
Q

Features of central cord syndrome?

A

Weakness which is more pronounced in the upper limbs than the lower
Sensory loss may also be present

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35
Q

Features of Brown Sequard syndrome

A 
Ipsilateral corticospinal (power) and dorsal column (proprioception) loss 
Contralateral spinothalamic (pain and temperature) loss
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36
Q

Bitemporal hemianopia where is the lesion?

A

Optic chiasm

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37
Q

Homonymous hemianopia where is the lesion?

A

Optic tract

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38
Q

Homonymous hemianopia with macular sparing/sparing of central vision, where is the lesion?

A

Occipital cortex

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39
Q

Tongue biting can be present on pseudoseizures T/F

A

T - tongue biting is not a good way to differenciate pseudo from true sz

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40
Q

Features of cataplexy?

A

Loss of muscle tone without loss of consciousness

Usually occur in assoc with narcolepsy

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41
Q

Night terrors are common in adolescents T/F

A

F - the typically present around 5 yrs old and rarely persist into adolescence

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42
Q

What is the recurrence rate for ADEM?

A

About 2-4%

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43
Q

What percentage of patients have long term sequelae post ADEM?

A

Less than 1/3

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44
Q

Rett syndrome is assoc with what pulmonary abnormality?

A

Abnormal breathing pattern - during wakefullness patients have periods of hyperventilation followed by hypoventilation/apnea

Note: it is also associated with microcephaly and increased risk of sudden cardiac death

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45
Q

DDx of complete vision loss with preservation of light reflexes and an otherwise normal neuro exam?

A

Cortical blindness vs conversion disorder

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46
Q

Tramline calcifications on skull xray, what pathology?

A

Sturge Weber

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47
Q

What is the first line treatment of spasticity assoc with CP?

A

Referral for physical therapy

If child has discomfort, pain, muscle spasms and of worsening of function can start baclofen but usually start with PT first

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48
Q

Children with albinism can have congenital nystagamus

A

T

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49
Q

Signs of increased ICP and cerebellar signs, likely aetiology?

A

Posterior fossa mass
If insidious/long history low grade astrocytoma
If more acute medulloblastoma

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50
Q

Typical exam findings in neurogenic shock

A

Hypotension with bradycardia or an inappropriately normal HR and warm peripheries
They have lost sympathetic response

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51
Q

What age would you use play audiometry vs pure tone audiometry for hearing tests in children?

A

Play audiometry : 2-5 yrs

Pure tone: school age children and older

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52
Q

Age for distraction testing for hearing in children?

A

8 months to 1 yr old

Note: child must be able to sit unsupported

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53
Q

What is the initial presentation of ataxia telangiectasia?

A

Child is late at starting to walk

Note: 30% of these patients will have epilepsy

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54
Q

What is the cause of macrocephaly in achondroplasia?

A

Hydrocephalus is commonly due to stenosis of the foramen magnum

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55
Q

What is the 1st line treatment for benign partial epilepsy of childhood/rolandic seizures?

A

Do not need to treat unless episodes are frequent

If frequent use carbamazepine

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56
Q

Treatment of infantile spasms?

A

1st Steroids or ACTH - it is not clear which is better

If tuberous sclerosis vigabatrin is 1st line

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57
Q

1st line treatment for generalised seizures?

A

Valproate

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58
Q

ADEM risk factors?

A 
1-20 days post viral infection
Post vaccination (MMR)
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59
Q

How often should medications to abort a headache be used?

A

No more than 3 times a week

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60
Q

What is the management of medication overuse headache?

A

Complete and abrupt withdrawal of the medications

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61
Q

Which is better to use for treatment of acute migraine headache ibuprofen or paracetamol?

A

Ibuprofen is better than paracetamol

Note: triptans are also good

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62
Q

What is Kugelbery Walender disease AKA

A

Spinal muscular atrophy type 3

63
Q

When do patients with Duchenne Muscular Dystrophy become symptomatic?

A

In toddler age, normal at birth and infancy

Note: Becker Muscular dystrophy will not present until adolescence

64
Q

Most patients will juvenile myoclonic epilepsy will grow out of it and no longer need treatment in adult years T/F

A

F - most patients will need life long treatment

65
Q

What is communicating hydrocephalus AKA?

A

Non obstructive

Note: bacterial meningitis is a cause due to thickening of leptomeninges leading to reduced CSF absorption

66
Q

When does Friedreich’s ataxia typically present?

A

Onset of ataxia is typically around 10 yrs old (between 5-15 yrs)

Note: intelligence is preserved

67
Q

When does ataxia telangiectasia typically present?

A

Around 2 yrs old

Also have nystagmus and decreased IgA

68
Q

How does Abetalipoproteinaemia present?

A

Failure to thrive and steatorrhoea

Note: ataxia occurs later in childhood, Vit E is undetectable

69
Q

What is the most common cause of shunt failure in children?

A

Obstruction - occurs in around 40% of children within 2 yrs of shunt insertion.
Infection and abdo complications are less common

70
Q

Night terrors vs nightmares, timing and recollection?

A

Night terrors - occur within first 3 hours of sleep, no recollection
Nightmares - during 2nd half of night and are well remembered

71
Q

Sleepwalking is rare in prepubertal children T/F

A

F - commonly occurs in age 3-10 yrs, affects 30% of children

72
Q

Waiters tip deformity is seen in what injury?

A

Upper brachial plexus Erb-Duchenne (C5-6)

Note: lower brachial plexus is Klumpke (C8-T1)

73
Q

Port wine stain is associated with what neuro abnormality?

A

Sturge Weber

Note: in particular on face. Hence risk of glaucoma and/or leptomeningeal angiomatosis (that may cause seizures)

74
Q

Patients with Tuberous sclerosis are at increased risk for infantile spasms T/F

A

T

75
Q

Patients with myasthenia gravis are most likely to have what antibodies?

A

Antibodies to Acetylcholine receptors

76
Q

What is the first line treatment for juvenile myoclonic epilepsy?

A

Sodium valproate in males

Topiramate in females

77
Q

Cerebellar signs and a resting tremor is most likely Wilson’s disease in a teenage T/F

A

T

78
Q

3 yr old with myoclonic jerks, optic atrophy and progressive dementia. Bone marrow biopsy shows lipofuscin. What disease?

A

Batten

79
Q

What is symptomatic epilepsy?

A

Epilepsy occurring as a symptom of an underlying problem (eg HIE, infection, structural brain malformation or metabolic disorder)

80
Q

Patient’s with what type of epilepsy are high risk for SUDEP? (sudden unexplained death in epilepsy) ?

A

Symptomatic epilepsy (cause is known eg head injury), 20x increased mortality compared to those with other types of epilepsy.

Note: poor AED compliance does not seem to be a risk factor
Other RFs: prone sleep and early onset epilepsy

81
Q

According to the NICE guidelines for treatment of epilepsy if initial AED is not successful?

A

Should still attempt monotherapy - start the 2nd AED and then gradually wean off the first one

82
Q

Ovarian teratoma is assoc with what neurological pathology?

A

Anti- NMDAR encephalitis

Note: about 1/3 of girls aged 14-18 years old with anti-NMDAR encephalitis are found to have an ovarian teratoma (paraneoplastic teratoma)

83
Q

Bells palsy upper or lower motor neurone lesion?

A

Lower

84
Q

Bells palsy responds well to steroids T/F

A

F - evidence is limited in children

85
Q

What is the typical triad of symptoms with craniopharyngioma?

A

Headache
Visual disturbance
Hormone imbalances

86
Q

Compare the timing of neonatal vs perinatal deaths?

A

Neonatal - death within first 28 days of life
Perinatal - still births plus death within 1st 7 days of life

Note: still birth = in utero death after 24 weeks gestation

87
Q

In a chronic subdural haematoma the trauma to the head is usually minor and often forgotten by the patient T/F

A

T

88
Q

What is the earliest feature of a chronic subdural haematoma?

A

Headache

89
Q

Decorticate vs decerebrate posturing

A

Decorticate: “mummy posture” - bent arms and outstretched legs

Decerebrate: extensor posturing

90
Q

Main way to differentiate breath holding spells from reflex anoxic seizures (pallid spells or reflex asystolic syncope )

A

Breath holding spellings: apnea and then loss of consciousness
Reflex anoxic seizures: loss of consciousness and then apnea

Note: both can be triggered by similar things

91
Q

Reflex anoxic seizures have a poor prognosis T/F

A

F - they are self resolving and development is normal

92
Q

Myotonic dystrophy is associated with what in addition to myotonia and weakness?

A

Diabetes, cardiac abnormalities, club feet and cataracts

Note: if the mother is affected and so is her newborn it is referred to as congenital myotonic dystrophy.
Weakness tends to spare proximal muscle groups

93
Q

Carbamazepine is the first line treatment of juvenile myoclonic epilepsy. T/F

A

F - it is not recommended for myoclonic jerks

Female: topiramate
Male: valproate

94
Q

What is the ideal head positioning in a patient with raised ICP?

A

Head elevated 20 degree and in midline (Aids venous drainage)

95
Q

Defining characteristics of a pt in a permanent veg state?

A

Brainstem function is still intact, normal resp effort and circulation
Cortical functioning has been significantly impaired or lost

NB: these patients cannot be legally declared dead

96
Q

Are night terrors are associated with significant autonomic arousal?

A

Yes - sweating, tachycardia and pupillary dilation

97
Q

During a night terror a child is easily roused and comforted T/F

A

F - difficult to fully rouse, do not respond to communication; often inconsolable

98
Q

What AEDs are most likely to cause neural tube defects?

A

Valproate, carbamazepine and phenytoin

99
Q

What type of mutation leads to congenital myotonic dystrophy?

A

A trinucleotide repeat

Note: AD and shows anticipation, gene is DMPK

100
Q

What AED is known to cause visual field defects

A

Vigabatrin - rarely both eyes

Note: lamotrigine and carbamazepine are known to cause visual disturbances such as diplopia

101
Q

70% of patients with Dravet syndrome have a mutation in what gene?

A

SCN1a

102
Q

Typical presentation of Dravet syndrome?

A

Seizures usually start within 1st year of life, often associated with a fever and often a prolonged seizure that effects one side of the body.
Usually are initially developing normally and start to regress around 2 yrs old

103
Q

Commonest cause of hypertension in NF1?

A

Renal artery stenosis

Can also be essential or due to pheochromocytoma

104
Q

What is the mechanism of action of vigabatrin?

A

Inhibits breakdown of GABA (NB does not function at the GABA receptor)

105
Q

What is the mechanism of flumazenil?

A

Antagonist at the GABA receptor

Note: benzo and barbiturates are agonists at the receptor

106
Q

Tetrad of features in classic narcolepsy?

A
  1. Excessive daytime sleepiness
  2. Cataplexy (generalised muscle weakness leading to partial or complete collapse)
  3. Hypnagogic and/or hypnopompic hallucinations
  4. Sleep paralysis
107
Q

What is the mechanism of action of phenytoin?

A

Inhibits voltage gated Na channels

108
Q

What is the mechanism of action of ethosuximide and zonisamide?

A

T type Ca channel blocker

109
Q

Which is associated with decreased facial expression spinal muscular atrophy or congenital myotonic dystrophy?

A

Congenital myotonic dystrophy

110
Q

What is the commonest form of adrenoleukodystrophy? What age does it usually present?

A

Cerebral

Age 5-8 yrs

111
Q

Friedriechs ataxia vs cerebral adrenoleukodystrophy

A

FA: 5-15 yrs old at presentation, progressive ataxia and spasticity, can have extensor plantar responses but lower limb reflexes are typically absent. Preserved intelligence

Cerebral ADL: 5-8 yrs old at presentation, behaviour change and alternation in gait, upper motor neuro signs, results in neuro degeneration

112
Q

Hepatic dysfunction is a side effect of topiramate T/F

A

F - hepatic dysfunction is one of the main SEs of valproate

113
Q

Patients on topiramate are advised to maintain adequate hydration. Why?

A

Due to increased risk of nephrolithiasis

114
Q

In order to diagnosis menstruation associated migraine how many consecutive menstrual cycles does the headache need to be associate with?

A

2 out of 3 consecutive cycles

115
Q

A slow spike-and-wave pattern is seen on EEG in what pathology?

A

Lennox Gastaut

Can also have widespread background slowing and “generalised paroxysmal fast activity”

116
Q

What is the typical natural history of neonatal myoclonus?

A

Starts within a few days of life and resolves by 3 months

Happens when they are sleepy/falling asleep and stop when woken.

117
Q

Ciprofloxacin can lower seizure threshold T/F

A

T

Note: it can also interact with phenytoin

118
Q

Rapidly increasing head circumference is often the first sign of Rett syndrome T/F

A

F - the opposite, deceleration of head growth

119
Q

Criteria for diagnosis of Tourette’s syndrome?

A

Multiple motor and vocal tics for at least one year (although do not need to be present concurrently)
No tic free period > 3 months
Onset prior to 18 yrs
No underlying medical condition

120
Q

When Tourette’s typically present?

A

< 7 yrs old

Note: severity peaks around 10 -12 yrs

121
Q

Risperidone is indicated in the treatment of irritability associated with Autism Spectrum Disorder T/F

A

T

Note: Indicated in ages 5 - 16 yrs
Aripiprazole can also be used in age 6 - 17 yrs

122
Q

NSAIDs can increase serum lithium levels T/F

A

T - NSAIDs decrease the renal clearance of lithium, hence increasing the serum levels

123
Q

Common presentation of bilateral acoustic neuroma?

A

Tinnitus
Unsteady gait
Hearing loss
Facial weakness

124
Q

Features of NF 2

A

Bilateral or unilateral acoustic neuroma
Plus 2 or more of: meningioma, glioma, schwannoma, juvenile or cortical wedge cataract

Note: freckling, cafe au lait spots and lisch nodules are not typically present. Theses are features of NF 1

125
Q

In the setting of head trauma a lucid period followed by rapid deterioration to a near comatose state is typical of what pathology?

A

Epidural haematoma

Note: epidural haematoma enlarges much faster than subdural. Epidural is middle meningeal artery bleed and subdural is a venous bleed

126
Q

Compare benign neontal seizures to infantile spams

A

Benign neonatal: presents around 4-6 days old, lasts 1-3 minutes, often tonic and may be unifocal or multifocal, excellent prognosis remit on their own with no associated developmental delay

Infantile spams: present around 3-7 months, flexor or extensor seizures, only last seconds but clusters of > 100/day, poor prognosis and developmental delay

127
Q

Marcus Gunn phenomenon is due to abnormal innervation of which nerves?

A

Trigeminal (controls muscles of mastication)
Oculomotor (controls levator palpebrae superioris)

AKA jaw winking phenomenon

128
Q

Transverse myelititis is sometimes proceeded by non specific symptoms T/F

A

T

It is often associated with infection eg EBV, mumps, rubella, varicella, influenza, mycoplasma and Borrelia burgdorferi. Autoimmune aetiology is thought to be involved in other cases

129
Q

What are the CSF findings in transverse myelitis?

A

Mild lymphocyte pleocytosis

Normal or slightly elevated protein

130
Q

X linked adrenoleukodystrophy is due to accumulation of what?

A

Very long chain fatty acids

Note: it is often initially mis diagnosed as ADHD

131
Q

Accumulation of sulphated glycosphingolipids is seen in what disease?

A

Metachromatic leukodystrophy.

Presents earlier to X linked leukodystrophy (1-2 yrs of age vs 7 yrs)

Note: due to a deficiency in arylsulfatase A.

132
Q

What diseases is Moyamoya associated with in additions to sickle cell?

A

T 21
NF 1
Collagen vascular disorders

133
Q

Presentation of moyamoya

A

Varied. TIA, acute stroke, chronic headaches or new onset seizure

134
Q

Pes cavus is associated with what neurological disease?

A

Charcot Marie tooth (AKA hereditary motor sensory neuropathy)

135
Q

Guillan Barre may be associated with autonomic dysfunction T/F

A

T - bradycardia, dysrhythmias and labile BP

136
Q

CSF finding in Guillan Barre?

A

Elevated protein

N glucose and N WBC

137
Q

Most common risk factors for sinus venous thrombosis in an infant?

A

Infection

Dehydration

138
Q

Presentation of sinus venous thrombosis?

A 
Diffuse or focal neuro defects
Seizures (common)
Headache 
Lethargy
N + V
Signs of increased ICP
139
Q

Differentiate Soto syndrome from Marfan’s in terms of growth?

A

Marfan’s: increased growth velocity continues through adolescence
Soto: growth velocity normalises by 5 yrs

140
Q

Soto syndrome is associated with hypotonia and poor feeding T/F

A

T

Other features: macrocephaly (at birth), increased growth velocity, large hands and feet, prominent forehead, down slanting palpebral fissures, hypertelorism, high arches palate, ASD or PDA, aggression

141
Q

Which AED is known to cause hyperammonemic encephalopathy?

A

Valproic acid

142
Q

What AED is known to cause acute-angle closure glaucoma?

A

Topiramate

143
Q

Which vit deficiency is associated with neonatal seizures that are resistant to traditional AEDs?

A

Vit B 6

Note: AKA pyridoxine; this vit is required for the synthesis of GABA

144
Q

Diagnostic test and mgmt of idiopathic intracranial HTN?

A

Diagnosis: CT and then LP
Mgmt: carbonic anhydrase inhibitors

145
Q

Individuals with Friedreich ataxia have a high prevalence of what AI disease?

A

Diabetes

About 20%

146
Q

6 week old with poor growth, hypoglycaemia, nystagmus, micropenis and direct hyper-bili. Diagnosis?

A

Septo-optic dysplasia

Note: hypopit is must often seen as growth hormone def. Imaging would show genesis of the septum pellucidum. Vision ranges from normal to complete blindness

147
Q

Headaches that are primarily occipital are an indication for imaging T/F

A

T

148
Q

Developmentally normal child
Seizures generally occur at night
Usually simple focal motor seizures, often involve face
Often sensory paraesthesia, drooling, grunting

A

Benign epilepsy with centrotemporal spikes

149
Q

Internal carotid artery dissection can present with Horner syndrome T/F

A

T

Note: may also present with ipsilateral headache, neck pain or eye pain

150
Q

Excessive Vit A is a risk factor for idiopathic intracranial HTN T/F

A

T

Other RF: increased BMI; OCP; tetracycline and isotretinoin; prolonged use or rapid withdrawal of corticosteroids

151
Q

Oxcarbazepine can cause what electrolyte disturbance? What is the management?

A

HypoNa

Mgmt: usually asymptomatic and requires no tx. If symptomatic or Na < 120 a reduction in dose and/or mild fluid restrictions usually returns Na to an acceptable level

152
Q

What nerve palsy if often seen in increased ICP?

A

6th nerve (abducens)

153
Q

Tongue fasciculations in a floppy neonate?

A

SMA type 1

Note: no fasciculations in congenital muscular dystrophy

154
Q

What is the most common cause of CP?

A

Prematurity

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