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RC Studying > Neurology > Flashcards

Neurology Flashcards

1
Q

What are the features of congenital type 1 myotonic dystrophy?

A
  • Presents in neonatal period

- Hypotonia, arthrogryposis, poor feeding, and respiratory failure

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2
Q

What is the inheritance pattern/genetics of myotonic dystrophy?

A
  • Myotonic dystrophy type 1 is caused by expansion of a CTG-trinucleotide repeat
  • Autosomal dominant inheritance
  • Anticipation: trinucleotide repeat count increases over successive generations = next generation presents earlier
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3
Q

What is the inheritance pattern of muscular dystrophy (DMD and BMD)?

A
  • DMD and BMD are caused by mutations in the gene for dystrophin
  • X linked disorder
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4
Q

What is the clinical presentation of DMD?

A
  • Weakness b/w 2-3 years of age

- Proximal>distal, lower extremities

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5
Q

Which antiepileptic can cause bilateral renal calculi?

A

Topiramate

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6
Q

A child presents with progressive weakness of the legs over the last 2 days. He also complains of urinary retention. On exam, you note a sensory level and increased tone. What are you most likely to see on CSF?

A

Transverse Myelitis

  • Postinfectious (WNV, herpes, HIV, mycoplasma)
  • Rapidly progressing B/L weakness over hours to days
  • Typically involves legs and sometimes arms (if C-spine involved)
  • Most have sensory level
  • Bowel/bladder dysfunction (often retention)
  • UMN signs: initially low tone and DTRs that progress to increased tone and hyperreflexia
  • if C-spine may have resp. arrest (C345 keeps diaphragm alive)
  • CSF: lymphocytosis with elevated protein
  • Rx: glucocorticoids
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7
Q

A child presents with a 2 week history of progressive, symmetric weakness that started in the legs but has ascended upwards. O/E DTRs are absent. He complains of numbness and tingling to toes. There are no infectious symptoms but he recently had diarrhea. What is noted on your CSF?

A

Guillain-Barré

  • Progressive, symmetric weakness that typically starts in legs and ascends upwards with loss of DTRs
  • Clinical diagnosis
  • CSF: albuminocytologic dissociation: elevated CSF protein w/ N WBC
  • Increased F wave latency on NCS
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8
Q

What are the side effects of phenytoin and fosphenytoin?

A

Side effects of both phenytoin and fosphenytoin include cardiac arrhythmias, bradycardia, and hypotension, such that continuous BP and electrocardiogram (ECG) monitoring is recommended during infusion.

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9
Q

A child is less than 18 months of age with status epilepticus and continues to seize despite delivery of first line medications. In addition to consideration of second line medications, what might you also consider giving?

A

Pyridoxine

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10
Q

A child has status epilepticus and a bedside glucose shows a glucose of 2.1. What do you do?

A

If glucose <2.6 give:

  • 5mL/kg D10W via periph. IV
  • 2mL/kg D25W via central line
    0. 5g/kg glucose
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11
Q

What is the number one cause of status epilepticus?

A

Prolonged febrile seizure

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12
Q

What factors categorize a patient as low risk when they have a BRUE?

A
  • Age >60 days
  • Born >/=32 weeks gestation and cGA >/=45 weeks
  • No CPR by trained medical provider
  • Event lasted <1 minute
  • First event
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13
Q

What management would you consider offering for a patient with a low risk BRUE?

A
  • Educate caregivers and shared decision making for plan
  • Offer CPR training resources
  • May obtain pertussis testing and ECG
  • May briefly monitor patients with cont. pulse oximetry and serial observations
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14
Q

A child with tuberous sclerosis is diagnosed with infantile spasms. What is the treatment of choice?

A

Vigabatrin

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15
Q

What is the best test for identifying the etiology of infantile spasms?

A

MRI

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16
Q

The absence of which brain stem reflexes is required as part of the minimum clinical criteria for neurological determination of death?

A
  • Gag (Glossopharyngeal/CNIX)
  • Cough (Vagus/CN X)
  • B/L absence of corneal responses (CN VII) , pupillary responses to light , vetibulo-ocular responses (CN III, VI)
17
Q

How do you manage a child who just had a concussion?

A
  1. Physical and congitive rest until sx free x7-10d
  2. Return to learn before return to play
  3. Each step of protocol should take a minimum of 24h to progress to next step, if any symptom recurs, should rest until it resolves before trying again
    - No routine meds are recommended
    - Do not need to wake child from sleep but should be monitored for first 24-48 hours
18
Q

What serious side effects are associated with carbamazepine?

A

SJS, agranulocytosis, aplastic anemia, liver toxicity
Nuisance: tics, transient leukopenia, hypoNa, weight gain, nausea, dizziness
-Note SJS and liver toxicity is a serious SE of multiple AEDs

19
Q

Which AED typically causes pancreatits/pancreatitic toxicity?

A

Valproate

20
Q

An EEG shows generalized 3Hz spike-wake morphology. What is the most likely diagnosis?

A

Absence seizures

21
Q

An EEG shows bilateral centrotemporal sharp waves. What is the most likely seizure type?

A

Rolandic/Benign childhood epilepsy with centro-temporal spikes
-Typically outgrown by age 16

22
Q

An EEG shows 4-6Hz B/L polyspike and slow wave discharge. What is the most likely seizure type?

A

Juvenile myoclonic

  • EEG can also show photoparoxysmal response to photo stimulation
  • Morning myoclonus, absence seizures, eventually GTCs
  • Typically life long
23
Q

How are acute dystonic reactions treated?

A
  • Antihistamines (diphenhydramine), anticholinergics and benzos
  • Can be secondary to dopamine agonists such as metoclopramide, prochlorperazine, neuroleptics
24
Q

How is hypsarrhythmia described?

A

High amplitude, slow, chaotic background with mutlifocal spikes, intrahemispheric-interhemispheric asynchrony

25
Q

A 5 year old wakes in the night with unilateral facial droop, and are having difficulty speaking. They try to explain their face is numb. What is the most likely diagnosis?

A

Benign Rolandic Epilepsy

-Self limited childhood epilepsy with centrotemporal spikes

26
Q

Which medications are commonly associated with pseudotumor cerebri/idiopathic intracranial hypertension?

A

Minocycline, tetracycline, doxycycline, sulfa drugs, Vit A & retinoids, lithium

27
Q

A child has gingival enlargement. What seizure medication are the likely taking?

A

Phenytoin

28
Q

What is the recommended treatment for absence seizures?

A

Ethosuximide (first line) or valproate (second line)
-AVOID: carbamazepine, oxcarbazepine, vigabatrin, possibly phenytoin (these WORSEN generalized szrs and absence is a generalized szr)

29
Q

What provocative test can you do in clinic to help with the diagnosis of an absence seizure?

A

Hyperventilation can cause an absence seizure to occur (can get them to blow a piece of paper)

30
Q

Which anti epileptic is associated with behavioural issues?

A

Keppra/Levetiracetam (and clobazam - which has more sedation issues)

RC Studying (24 decks)

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