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Flashcards in Neurology q-bank Deck (71):
1

What gene is associated with Rett syndrome?

MECP2

2

What are clinical features of Rett syndrome?

Normal development until 1
Ataxic gait, tremor of hands early
Repetitive hand wringing, autistic behaviour and eventually GTC seizures.
Acquired microcephaly.

3

What antibiotics do you have to avoid when taking carbamazepine?

Macrolides, they decrease the AED metabolism.

4

What features and timeline must be present to diagnose Tourettes?

Multiple motor tics, at least 1 vocal tic, present almost everyday for >1 year.
(onset before 18 and not secondary to an alternate diagnosis)

5

What medication classes are considered for treating Tourettes?

alpha-2 agonists: clonidine, guanfacine
atypical neuroleptics: risperidone, haloperidol
if comorbid OCD, anxiety or depression: SSRI

6

What comorbidities go with Tourettes?

OCD
ADHD
Anxiety
Depression
Learning difficulties

7

What EEG finding is typical for absence seizures?

3 Hz spike and wave slow discharge
can be precipitated by hyperventilating

8

Do absence seizures have a post-ictal period?

No they go right back to what they were doing.
Sometimes the seizure can be associated with an automatism like lip smacking, picking at clothing, eye fluttering.

9

What meds are most effective in absence seizures?

ETHOSUXIMIDE
Valproic acid
Lamotrigine
Clonazepam

10

What is the prognosis of childhood absence epilepsy?

Generally excellent, with minimal cognitive or developmental sequelae. Most patients outgrow it before adulthood, in those with:
- if myoclonic jerks too, usually more difficult to treat
- 1/4 will develop GTC seizures

11

What meds are best for treating infantile spasms?

ACTH
Vigabatrin

12

What meds are best for treating Dravet syndrome?

Benzodiazepines
Valproate

13

What meds are best for treating focal seizures and epilepsies?

Carbamazepine
Oxcarbazepine

14

What meds are best for treating juvenile myoclonic epilepsy?

Valproate
Lamotrigine

15

What meds are best for treating Lennox-Gastaut?

Topiramate
Valproate

16

What are risk factors for febrile seizures recurrence?
major and minor

MAJOR: age <1 year, duration for fever <24h, fever 38-39

minor: fam hx of febrile seizures or epilepsy, complex febrile seizure, day care, male, lower serum sodium at presentation

17

How do the febrile seizure risk factors play in to recurrence rate?

Recurrence risk with
none: 12%
1 risk factor: 25-50%
2 RFs: 50-59%
>/= 3 RFs: 73-100%

18

Who should get an LP for a febrile seizure?

Any kid < 12 months, consider in 12-18 months.
Depending on history and physical, obvs.

19

What meds for acute migraine?

NSAIDs
+/- triptans
+/- anti-emetics ie: Maxeran

20

What meds for preventing migraines?

Amitriptyline- give for 4-6 months and wean
in adults: VPA, Topamax, beta-blockers, cyproheptadine

21

Triad of developmental delay, multiple seizure types and EEG findings of 1-2 Hz spike–and-slow waves, polyspike bursts in sleep, and a slow background in wakefulness

Lennox-Gastaut
often develop mental retardation and long-term intractable seizures.

22

EEG findings in juvenile myoclonic epilepsy?

4-5 Hz polyspike and slow wave discharges

23

5 year old boy with loss of language and new onset seizures, what syndrome to consider?

Landau-Kleffner
(rare) unknown cause
previousy normal child develops aphasia, receptive or expressive

24

When to start prophylaxis for migraines?

Headaches are frequent (>1/week)
Headaches interfere with daily activities

25

What anti-epileptic has the following side-effect profile?
pancreatitis, hepatitis, thrombocytopenia, weight gain, menstrual irregularities

Valproic acid

26

What anti-epileptic has the following side-effect profile?
SJS, agranulocytosis, aplastic anemia, liver toxicity, tics, hypoNa, nausea/dizziness, spina bifida in offspring

Carbamazepine

27

What anti-epileptic has the following side-effect profile?
glaucoma, metabolic acidosis

Topiramate

28

What anti-epileptic has the following side-effect profile?
gingival hyperplasia, SJS, liver toxicity, hirsutism, cerebellovestibular symptoms

Phenytoin

* gingival hyperplasia reduced with folic acid, only real treatment is to stop

29

What is Lemierre syndrome?

septic thrombophlebitis of the jugular vein, often a complication on parapharyngeal space or odontogenic infection.
Presents wtih fever, toxicity, neck pain, stiffness, resp distress (septic emboli)
Rx: abx and heparin

30

When does the parachute reflex appear? And disappear?

Appears at 7-8 months
Never goes away.

31

When does the tonic neck reflex appear?
Disappear?

1 month
6-7 months

32

When does the Moro appear? Disappear?

37 weeks.
5-6 months

33

Rooting appearance? Disappearance?

36 weeks.
Less prominent after 1 month.

34

Palmar grasp appearance? Disappearance?

32 weeks.
2-3 months.

35

What are the categories of criteria in the DSM-V for the diagnosis of PTSD?

1) intrusion
2) avoidance
3) negative alterations in cognitions or mood
4) alterations in arousal and activity

36

What clinical features go with an EEG pattern of centrotemporal spikes?

Benign Epilepsy with Centro-Temporal Spikes
peak in 7-9 year olds
mostly focal seizures, no LOC: facial numbness, twitching, vocalizations, hypersalivation, dysphagia, speech arrest
75% at night or upon awakening
rare SE
Rx: carbamazepine if absolutely needed

37

What type of brain injury most commonly seen in shaken baby syndrome?

ICH (subdural)
retinal hemorrhage
HIE

38

What is the most common craniosynostosis affecting a single suture?

Sagittal = dolicocephaly or scaphocephaly (boat-shaped)

39

Severe rigidity combined with high fever, autonomic symptoms (tachycardia, diaphoresis), delirium and dystonia

Neuroleptic Malignant Syndrome
occurs within days of starting or increasing neuroleptic drugs or withdrawal of dopaminergic agent
Rx: dantrolene

40

What medication side effect present with torticollis, retrocollis, oculogyric crisis, or tongue protrusion +/- laryngospasm

Acute dystonic reaction
occurs in the FIRST days of exposure
Rx: diphenhydramine, 2nd line benzodiazepines

41

What is the most common cause of spastic diplegia?

Major causes: prematurity, ischemia, infection, endo/metabolic

represents 35% of CP

Periventricular leukomalacia on neuropath

42

What is the most common cause of spastic hemiplegia?

Major causes: thrombophilic disorders, infection, genetic/dev'tal, perventricular hemorrhagic infarction

25% of CP

stroke in utero or cortical malformations

43

What are the most common causes of athetoid and dyskinetic CP?

Major causes: BIRTH ASPHYXIA, kernicterus, mitochondrial, genetic/metabolic

15% of CP ** intellect preserved in most patients, muscular weakness and rigidity

neuropath: asphyxia with symmetric scarring of putamen and thalamus
kernicterus with scars in globus pallidus and hippocampus

44

What are the most common causes of spastic quadriplegia?

Major causes: ischemia, infection, endo/metabolic/genetic/dev'tal

20% of CP

neuropath: PVL, multicystic encephalomalacia and cortical malformations

45

What is a type 1 Chiari malformation and how does it present?

displacement of the cerebellar tonsils into the cervical canal
- patients present with headache, neck pain, urinary frequency and progressive LE spasticity in adolescence or adult life
NO hydrocephalus

46

What are type 2 Chiari malformations and how do they present?

elongation of the 4th ventricle and kinking of brainstem lead to displacement of the vermis, pons and medulla into the cervical canal
- progressive hydrocephalus with a myelomeningocele
- infants present with stridor, weak cry, apnea
- kids present with gait, spasticity, increasing incoordination

47

What are type 3 Chiari malformations and how do they present?

herniation of the cerebellum occurs through a cervical spina bifida defect
- occipital encephalocele (difficult to miss)

48

What is the main reason that diazepam isn't used as an AED in neonates?

decreased liver metabolism, rapid distribution into fat (brain)

49

Name some drugs known to cause pseudotumour cerebri?

tetracyclines
sulfonamides
corticosteroid withdrawal
nitrofurantoin
cyclosporine
phenytoin
levonorgestrel implants

50

What are the criteria of a developmental coordination disorder?

A) Learning and execution of coordinated motor skills below level expected for age, given opportunity for skill learning
B) Motor skills significantly interfere with ADLs, academic/school productivity, prevocational and vocational activities, leisure and play
C) Onset early in development
D) Motor skills not better explained by intellectual disability, visual impairment or other neuro conditions that affect movement (medical practitioner involvement)

51

What is the risk of VPA in pregnancy?

Neural tube defects
CVS, GU malformations
minor craniofacial, skeletal anomalies

52

What is the risk of Phenytoin in pregnancy?

Orofacial clefts, CVS & GU malformations
reports of neuroblastoma

53

What is the risk of Phenobarbital in pregnancy?

CVS, GU, orofacial anomalies

54

What is the risk of Carbamazepine in pregnancy?

Neural tube defects
GU malformations

55

How many brachial plexus injuries recover in one month? When to refer?

75% in one month
refer if in the other 25%

56

What type of cardiac involvement is seen in myotonic dystrophy?

heart block and arrhythmias

57

Describe the systems involved in myotonic dystrophy

obviously muscular weakness but also:
intellectual impairment
swallowing difficulties, slow gastric emptying
cardiac involvement
endocrinopathies
immunologic deficiencies
cataracts

58

What are the signs and symptoms associated with Sturge-Weber?

Facial capillary malformation
Abnormal blood vessels in the brain
Abnormal blood vessels in the eye

59

What are complications of Sturge-Weber?

Seizures, glaucoma, hemiparesis, stroke-like episodes or visual defects, mental retardation or severe learning disabilities

60

How to manage Sturge Weber if suspecting?

MRI w/ contrast
CT for calcification evaluation
Ophthalmo
Pulsed dye laser therapy

61

In kids who have febrile seizures, what is their lifetime risk of epilepsy?
What is it in the general population?

Febrile seizures: 2-7%
Gen population: 1%

62

AUTISM:
a) Persistent deficits in social communication and social interaction across multiple contexts, as manifested by all of the following (4), currently or by history

1) deficits in social-emotional reciprocity
2) deficits in nonverbal communicative behaviours used for social interaction
3) deficits in developing, maintaining and understanding relationships

63

AUTISM:
b) Restricted, repetitive patterns of behavior, interests, or activities, as manifested by at least two of the following,

1) stereotyped or repetitive motor movements, use of objects or speech
2) insistence on sameness, inflexible adherence to routines or ritualized patterns of verbal or nonverbal behaviour
3)highly restricted, fixated interests that are abnormal in intensity or focus
4) hyper or hyporeactivity to sensory input or unusual interest in sensory aspects of the environment

64

What are the distinguishing features of Neuroleptic Malignant Syndrome vs Serotonin Syndrome?

The presence of neuromuscular excitation such as clonus and hyperreflexia are strongly predictive of serotonin syndrome.

In contrast, NMS is characterised by muscular 'lead-pipe' rigidity, haemodynamic dysregulation and hyporeflexia.

65

Best treatment for panic attacks?

SSRI & CBT

66

What are the DSM criteria for a PANICS attack?

At least four of the following:
P-alpitation, P-aresthesia
A-bdominal distress, A-ltered sensation
N-umbness , Nausea
I-ntense fear of death
C-hoking , C-hills , C-hest, C-razy
S-weating , S-haking , S-hortness of breath .

67

What are the criterion for symptoms to diagnose depression?

At least 5 of the following symptoms have been present during the same 2-week period and represent a change from previous functioning; at least one of the symptoms is either (1) depressed mood or (2) loss of interest or pleasure.
DAWSPEGIS

68

What does the CRAFFT mnemonic tool stand for?

Car
Relax
Alone
Forget
Family/Friends
Trouble

At least 2 YESs is a positive screen

69

What are key features in the diagnosis of CP?

Abnormal motor development and posture
Motor impairment is permanent and nonprogressive
Motor impairment is attributed to an insult that occurred in the developing or infant brain
Motor impairment results in limitation in functional abilities and activity
Motor impairment is often accompanied by secondary MSK problems, epilepsy, disturbed sensation/perceptions...

70

How to differentiate post-VZV cerebellar ataxia from meningoencephalitis?

Cerebellar ataxia have a gradual onset of gait disturbance, nystagmus, and slurred speech.
Nuchal rigidity, altered consciousness, and seizures characterize meningoencephalitis

71

What are contraindications to LP?

1) suspected mass lesion in the brain, ICP
2) critical illness
3) skin infection at site
4) thrombocytopenia <20