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
2
Q
Infection is the second most common cause of seizure in neoates T/F
A
False
1st HIE
2nd Cerebral infarction
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%)
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)
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
6
Q
What is Devic’s disease AKA?
A
Neuromyelitis optica
7
Q
What is the treatment of neuromyelitis optica?
A
Plasmapheresis (as opposed to high dose steroids for MS)
8
Q
What two features are suspicious for NMO (neuromyelitis optica).
Lab test?
A
Optic neuritis and transverse myelitis
Lab: aquaporin antibodies
9
Q
What lab tests can help with the diagnosis of sydenham’s chorea?
A
Anti-streptolysin O titres and anti-DNAse B antibodies
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
11
Q
SMA type one is typically caused by a del on chr 10q T/F
A
F - del on chr 5q (SMN1 gene)
12
Q
Centrotemporal spikes on an EEG are diagnostic of what seizure disorder?
A
Benign rolandic epilepsy (BECTS - benign epilepsy with centrotemporal spikes)
13
Q
3/s spikes on EEG what sz disorders?
A
Absence
AKA spike and wave pattern
Note: can be induced by hyperventilation
14
Q
Vertical nystagmus where is the lesion?
A
Brainstem at pontomedullary junction (eg Arnold Chiari malformation)
15
Q
In vestibular nystagmus the slow phase is directed to the side of the lesion T/F
A
True
16
Q
In cerebellar nystagmus the fast component is directed toward the side of the lesion T/F
A
True
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
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
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
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
21
Q
Common side effects of carbamazepine
A
Diplopia
Ataxia
Also link with SJS
Note: lamotrigine also commonly causes diplopia and ataxia
22
Q
What AED causes: Nystagamus Gum hypertrophy Hirsutism Anaemia Liver problems Rash (SJS assoc) Decreased folic acid Decreased bone density
A
Phenytoin
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
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
25
Panayiotopoulous syndrome is characterised by very low frequency of episodes, sleep onset, prolonged duration and preserved neurodevelopment. T/F
T
Note: AKA early onset benign partial epilepsy with occipital paroxysms
26
How does Landau-Kleffner present?
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
27
1st and 2nd line tx of absence seizures?
1st: ethosuximide or valproate
2nd: lamotrigine
28
Which is the most likely AED to cause problems in the foetus?
Sodium valproate
Note: increased risk of 6 specific malformations- spina bifida, ADS, cleft lip, hypospadias, polydactyly and craniosynostosis
29
Which AED - facial dysmorphism, DD, cleft lip and palate, microcephaly, hypo plastic nails and skeletal malformations?
Phenytoin
Note: fetal hydantoin syndrome
30
Auditory brain stem evoked response is the most appropriate way to check the hearing of a 5 yr old T/F
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.
31
Typical presentation and age for infantile spams?
Flexion-extension spasm
3 months - 1 yr
EEG hypsarrhythmia
M > F
32
What is Werdnig Hoffman disease
Spinomuscular atrophy type 1
33
What is the gold std to differentiate epileptic sz from non-epileptic attack disorder
Video telemetry
34
Features of central cord syndrome?
Weakness which is more pronounced in the upper limbs than the lower
Sensory loss may also be present
35
Features of Brown Sequard syndrome
Ipsilateral corticospinal and dorsal column loss
| Contralateral spinothalamic loss
36
Bitemporal hemianopia where is the lesion?
Optic chiasm
37
Homonymous hemianopia where is the lesion?
Optic tract
38
Homonymous hemianopia with macular sparing/sparing of central vision, where is the lesion?
Occipital cortex
39
Tongue biting can be present on pseudoseizures T/F
T - tongue biting is not a good way to differenciate pseudo from true sz
40
Features of cataplexy?
Loss of muscle tone without loss of consciousness
| Usually occur in assoc with narcolepsy
41
Night terrors are common in adolescents T/F
F - the typically present around 5 yrs old and rarely persist into adolescence
42
What is the recurrence rate for ADEM?
About 2-4%
43
What percentage of patients have long term sequelae post ADEM?
Less than 1/3
44
Rett syndrome is assoc with what pulmonary abnormality?
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
45
DDx of complete vision loss with preservation of light reflexes and an otherwise normal neuro exam?
Cortical blindness vs conversion disorder
46
Tramline calcifications on skull xray, what pathology?
Sturge Weber
47
What is the first line treatment of spaasticity assoc with CP?
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
48
Children with albinism can have congenital nystagamus
T
49
Signs of increased ICP and cerebellar signs, likely aetiology?
Posterior fossa mass
If insidious/long history low grade astrocytoma
If more acute medulloblastoma
50
Typical exam findings in neurogenic shock
Hypotension with bradycardia or an inappropriately normal HR and warm peripheries
They have lost sympathetic response
51
What age would you use play audiometry vs pure tone audiometry for hearing tests in children?
Play audiometry : 2-5 yrs
| Pure tone: school age children and older
52
Age for distraction testing for hearing in children?
8 months to 1 yr old
Note: child must be able to sit unsupported
53
What is the initial presentation of ataxia telangiectasia?
Child is late at starting to walk
Note: 30% of these patients will have epilepsy
54
What is the cause of macrocephaly in achondroplasia?
Hydrocephalus is commonly due to stenosis of the foramen magnum
55
What is the 1st line treatment for benign partial epilepsy of childhood/rolandic seizures?
Do not need to treat unless episodes are frequent
If frequent use carbamazepine
56
Treatment of infantile spasms?
1st Steroids or ACTH - it is not clear which is better
| If tuberous sclerosis vigabatrin is 1st line
57
1st line treatment for generalised seizures?
Valproate
58
ADEM risk factors?
```
1-20 days post viral infection
Post vaccination (MMR)
```
59
How often should medications to abort a headache be used?
No more than 3 times a week
60
What is the management of medication overuse headache?
Complete and abrupt withdrawal of the medications
61
Which is better to use for treatment of acute migraine headache ibuprofen or paracetamol?
Ibuprofen is better than paracetamol
Note: triptans are also good
62
What is Kugelbery Walender disease AKA
Spinal muscular atrophy type 3
63
When do patients with Duchenne Muscular Dystrophy become symptomatic?
In toddler age, normal at birth and infancy
Note: Becker Muscular dystrophy will not present until adolescence
64
Most patients will juvenile myoclonic epilepsy will grow out of it and no longer need treatment in adult years T/F
F - most patients will need life long treatment
65
What is communicating hydrocephalus AKA?
Non obstructive
Note: bacterial meningitis is a cause due to thickening of leptomeninges leading to reduced CSF absorption
66
When does Friedreich's ataxia typically present?
Onset of ataxia is typically around 10 yrs old (between 5-15 yrs)
Note: intelligence is preserved
67
When does ataxia telangiectasia typically present?
Around 2 yrs old
Also have nystagmus and decreased IgA
68
How does Abetalipoproteinaemia present?
Failure to thrive and steatorrhoea
Note: ataxia occurs later in childhood, Vit E is undetectable
69
What is the most common cause of shunt failure in children?
Obstruction - occurs in around 40% of children within 2 yrs of shunt insertion.
Infection and abdo complications are less common
70
Night terrors vs nightmares, timing and recollection?
Night terrors - occur within first 3 hours of sleep, no recollection
Nightmares - during 2nd half of night and are well remembered
71
Sleepwalking is rare in prepubertal children T/F
F - commonly occurs in age 3-10 yrs, affects 30% of children
72
Waiters tip deformity is need in what injury?
Upper brachial plexus Erb-Duchenne (C5-6)
Note: lower brachial plexus is Klumpke (C8-T1)
73
Port wine stain is associated with what neuro abnormality?
Sturge Weber
74
Patients with Tuberous sclerosis are at increased risk for infantile spasms T/F
T
75
Patients with myasthenia gravis are most likely to have what antibodies?
Antibodies to Acetylcholine receptors
76
What is the first line treatment for juvenile myoclonic epilepsy?
Sodium valproate in males
Topiramate in females
77
Cerebellar signs and a resting tremor is most likely Wilson's disease in a teenage T/F
T
78
3 yr old with myoclonic jerks, optic atrophy and progressive dementia. Bone marrow biopsy shows lipofuscin. What disease?
Batten
79
What is symptomatic epilepsy?
Epilepsy occurring as a symptom of an underlying problem (eg HIE, infection, structural brain malformation or metabolic disorder)
80
Patient's with what type of epilepsy are high risk for SUDEP? (sudden unexplained death in epilepsy) ?
Symptomatic epilepsy, 20x increased mortality compared to those with other types of epilepsy.
Note: poor AED compliance does not need to be a risk factor
Other RFs: prone sleep and early onset epilepsy
81
According to the NICE guidelines for treatment of epilepsy if initial AED is not successful?
Should still attempt monotherapy - start the 2nd AED and then gradually wean off the first one
82
Ovarian teratoma is assoc with what neurological pathology?
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
Bells palsy upper or lower motor neurone lesion?
Lower
84
Bells palsy responds well to steroids T/F
F - evidence is limited in children
85
What is the typical triad of symptoms with craniopharyngioma?
Headache
Visual disturbance
Hormone imbalances
86
Compare the timing of neonatal vs perinatal deaths?
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
In a chronic subdural haematoma the trauma to the head is usually minor and often forgotten by the patient T/F
T
88
What is the earliest feature of a chronic subdural haematoma?
Headache
89
Decorticate vs decerebrate posturing
Decorticate: "mummy posture" - bent arms and outstretched legs
Decerebrate: extensor posturing
90
Main way to differentiate breath holding spells from reflex anoxic seizures (pallid spells or reflex asystolic syncope )
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
Reflex anoxic seizures have a poor prognosis T/F
F - they are self resolving and development is normal
92
Myotonic dystrophy is associated with what in addition to myotonia and weakness?
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
93
Carbamazepine is the first line treatment of juvenile myoclonic epilepsy. T/F
F - it is not recommended for myoclonic jerks
Female: topiramate
Male: valproate
94
What is the ideal head positioning in a patient with raised ICP?
Head elevated 20 degree and in midline (Aids venous drainage)
95
Defining characteristics of a pt in a permanent veg state?
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
Are night terrors are associated with significant autonomic arousal?
Yes - sweating, tachycardia and pupillary dilation
97
During a night terror a child is easily roused and comforted T/F
F - difficult to fully rouse, do not respond to communication; often inconsolable
98
What AEDs are most likely to cause neural tube defects?
Valproate, carbamazepine and phenytoin
99
What type of mutation leads to congenital myotonic dystrophy?
A trinucleotide repeat
Note: AD and shows anticipation, gene is DMPK
100
What AED is known to cause visual field defects
Vigabatrin - rarely both eyes
Note: lamotrigine is known to cause visual disturbances such as diplopia
101
70% of patients with Dravet syndrome have a mutation in what gene?
SCN1a
102
Typical presentation of Dravet syndrome?
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
Commonest of hypertension in NF1?
Renal artery stenosis
Can also be essential or due to pheochromocytoma
104
What is the mechanism of action of vigabatrin?
Inhibits breakdown of GABA (NB does not function at the GABA receptor)
105
What is the mechanism of flumazenil?
Antagonist at the GABA receptor
Note: benzo and barbiturates are agonists at the receptor
106
Tetrad of features in classic narcolepsy?
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
What is the mechanism of action of phenytoin?
Inhibits voltage gated Na channels
108
What is the mechanism of action of ethosuximide and zonisamide?
T type Ca channel blocker
109
Which is associated with decreased facial expression spinal muscular atrophy or congenital myotonic dystrophy?
Congenital myotonic dystrophy
110
What is the commonest form of adrenoleukodystrophy? What age does it usually present?
Cerebral
| Age 5-8 yrs
111
Friedriechs ataxia vs cerebral adrenoleukodystrophy
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
Hepatic dysfunction is a side effect of topiramate T/F
F - hepatic dysfunction is one of the main SEs of valproate
113
Patients on topiramate are advised to maintain adequate hydration T/F
T - due to increased risk of nephrolithiasis
114
In order to diagnosis menstruation associated migraine how many consecutive menstrual cycles does the headache need to be associate with?
2 out of 3 consecutive cycles
115
A slow spike-and-wave pattern is seen on EEG in what pathology?
Lennox Gastaut
Can also have widespread background slowing and "generalised paroxysmal fast activity"
116
What is the typical natural history of neonatal myoclonus?
Starts within a few days of life and resolves by 3 months
| Happens when they are sleepy/falling asleep and stop when woken.
117
Ciprofloxacin can lower seizure threshold T/F
T
Note: it can also interact with phenytoin
