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Pediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Tourette Syndrome definition

A

motor AND vocal tics
> 1 year

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2
Q

What are the red flags for headaches and what is the next step if they are present?

A

Acute/subacute
Progressive
Posterior
Pressure
Early morning/awakening
Constant
Emesis > nausea
Diplopia
Provoked by pressure, papilledema, pregnancy
Younger <6yo

MRI, LP if you’re thinking pseudomotor cerebrii

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3
Q

Benign rolandic epilepsy

A

Biphasic independently bilateral centrotemporal spikes, near central culcus (in light sleep)
Focal motor epilepsy
5yo - 10 yo
Infrequent brief focal motor seizures – in sleep
Arm and facial twitching, speech arrest, oropharyngeal guttural sounds, hypersalivation, preservation of consciousness

+/- GTC

No need to treat but if you want, oxcarbazepine and keppra
Prognosis: remit by 16 yo

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4
Q

3 Hz spine and slow wave

A

Absence
Ethosuximide –> valproic acid –> lamotrigine

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5
Q

Triad of juvenile myoclonic epilepsy and age of onset

A
  1. myoclonic seizures in early morning
  2. GTCs
  3. Abscence

late school-age & adolescent
Precipietated by alcohol, stress, sleep deprivation

Prognosis: persists into adulthood

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6
Q

Panayiotopoulos syndrome

A

Infrequent autonomic seizures
1-4 yo
Ictal emesis, mydriasis, pallor, cyanosis
Out of sleep
Preservation of consciousness
–> status epilepticus or hemiclonic/clonic movements
Frequently Mistaken for encephalitis

Prognosis: grow out of it after few years

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7
Q

Sx of Chiari II malformation

A

swallowing difficulties, stridor, hoarse voice, disordered breathing, choking, breath0holding spells

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8
Q

What is myelomeningocele

A

incomplete closure of posterior neural tube
Exposed meningeal sac
Results in hydrocephalus and Chiari II

Associated with agenesis of corpus callosum, polymicrogyria, heterotopia, structural micro-anomalies

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9
Q

Sx of hydrocephalus

A

increased head circ
bulging fontanelle
Restricted upward gaze (sun-setting eyes)
VI nerve palsies (inability to abduct eyes)
Lethargy

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10
Q

Rapidly progressing scending paralysis after hiking, ddx and treatment

A

Tic paralysis

Rocky Mountain Spotted Fever Dermacentor andersoni or dermacentor variabilis

Tx: removal of tic (improvement in 1h to few days)

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11
Q

Transverse myelitis sx

A

both MOTOR and SENSORY deficit, ofter bowel and bladder dysfunction

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12
Q

Dandy Walker malformation

A

congenital malformation of 4th ventricle and cerebellum

delayed walking/crawling
hydrocephalus

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13
Q

Someone comes in lethargic, found to have brain abscesses on MRI, what is the next step (per PREP)

A

blood cultures
prior to administering IV abx and calling neurosurgery

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14
Q

If a child has painful torticollis, not improving, cant move head past midline, what are you suspicious for? What imaging should you get

A

Atlantoaxial rotary subluxation (C1-C2)

CT cervical spine
Tx: conservative, resolve in 1-2 weeks

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15
Q

Febrile seizure 5 criteria

A

OOGLO

Occur in child 6mo-5yo
Occur in developmentally normal child
Generalized
Last <5 min
Once in a 24-hour period

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16
Q

Risk of devepoling epilepsy after febrile seizure

A

slightly higher

Recurrence:
1/3 of children with simple febrile seizure
More likely if first seizure <12mo

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17
Q

Diagnosis of migraine

A

> 4 lifetime attacks lasting 2-72 hours
2 of 4: photophobia, phonophobia, nausea, vomiting
+/- aura

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18
Q

Tx of migraine (stepwise)

A
  1. lifestyle migraine management
  2. abortive medications and plan
  3. Preventive medications
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19
Q

Acute flaccid myelitis

A

acute paralysis and areflexia of 1 or more limbs
preserved sensation
normal mental status
Onset: within 1 week of viral symptoms
MRI: central inflammation
LP: lymphocytic pleocytosis
Tx: supportive

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20
Q

Acute disseminated encephalomyelitis (ADEM)

A

Post-infectious
Needs encephalopathy
Seizure, headache, ataxia, paralysis, paresthesias
Brain MRI: demyelination, focal/multifocal lesions in white matter without prior lesions
Tx: steroids

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21
Q

Guillain Barre tx

A

IVIG
plasmapharesis

22
Q

Idiopathic transverse myelitis MRI findings and tx

A

longitudinal lesion, multiple segments involving white and gray matter

Tx: steroids

23
Q

Juvenile myoclonic epilepsy EEG findins and Tx

A

polyspike and wave at 4-6Hz frequency
Tx: valproic acid (not in adolecent girls –> teratogenic), zonisamide, topiramate, keppra

24
Q

Which AED exacerbates myoclonus in some children

A

lamotrigine

25
Q

Progressive myoclonic epilepsy

A

myoclonic
GTC
progressive neurodegeneration

Cognitive decline, ataxia, neuropathy, myopathy

26
Q

When does postconcussive syndrome start?

A

4 weeks after concussion, lasts 3 mo

27
Q

Risk factors for developing post-concussive syndrome

A

Pre-existing psych disorders
Multiple prior concussions
History of migraine HAs
Learning disorder
Severe initial symptom, LOC for >1 min, vestibular symptoms

28
Q

Defining features of Rett

A

only GIRLS
Normal development, then REGRESSION with loss of speech, stereotyped hand motion, and gait abnormalities
Acquired microcephaly (was well then falls off curve)
MECP2 on X chromosome

29
Q

X-linked adrenoleukodystrophy

A

genetic peroxisomal disorder affecting CNS myelin (no oxydation of very long chain fatty acids

BOYS between 4-8
Sx: ADHD, vision complaints, global developmental regression + adrenocortical dysfunction

30
Q

MRI of x-linked drenoleukodystrophy

A

bilateral posterior-predominant inflammatory demyelination affecting parieto-occipital lobes

31
Q

Tx of X-linked adrenoleukodystrophy

A

HSCT if early enough

32
Q

Landau-Kleffner Syndrome

A

Acquired epileptiform aphasia
Early childhood language regression 2/2 freq epileptiform discharges in parieto-occipital regions
Activated by sleep

33
Q

Acute transverse myelitis is associated with?

A

neuromyelitis optica
MS

34
Q

Diagnosis and tx of duchenne muscular dystrophy

A

CK
corticosteroids

35
Q

Diagnosis of narcolepsy

A

4 clinical features for at least 3 mo
Excessive daytime sleepiness
Cataplexy
Hypnagogic hallucinations
Sleep paralysis

36
Q

What is myasthenia gravis associated with and what imaging should be done?

A

Thymoma or thymic hyperplasia
Get a Chest CT

37
Q

Treatment of juvenile myasthenia gravis

A

thymectomy
pyridostigmine (acetylcholinesterase inhibitor prevents degradation of acetylcholine in NM junction)
Immunomodulation to decrease Ab (steroids, cellcept, azathioprine)

38
Q

Management of myasthenia gravis exacerbations

A

IVIG
Plasma exchange

39
Q

What do you use metaiodobenzylguanidine (MIBG) scans to evaluate?

A

opsoclonus-myoclonus-ataxia paraneoplastic syndrome to evaluate for neuroblastoma

40
Q

In Anti-NMDA encephalitis, what further imaging should you obtain?

A

CTAP to look for occult tumors

41
Q

What is the Miller Fisher variant of guillan-barre syndrome?

A

triad of ophthalmoplegia, areflexia, ataxia
Autoantibodies to ganglioside GQ1b, GT1a and GD3

42
Q

What is albuminocytologic dissociation

A

high protein, normal cell count in CSF
supportive of acute inflammatory demyelinating polyneuropathy (AIDP)

43
Q

Signs and sx of acute cerebellar ataxia

A

HEAD BOBBING (titubation), dysarthria, dysmetria
Prior infection
Affects 2-5 years
Classically 2/2 varicella infection, but can be others

44
Q

Presentation of SMA

A

Regression of milestones
Muscle weakness, hypotonia, diminished reflexes
Pathophys: anterior horn cell dysfunction and loss

Legs>arms

45
Q

Definition of status epilepticus

A

continuous seizure activity or clustering of seizures without return to baseline for longer than 30 min

early SE: sz lasting between 5-30 m
Refractory: continuous despite treatment with 2-3 AED loads

46
Q

Closed-lip schizencephaly

A

congenital disorder of cortical migration

47
Q

Spastic diplegia, where is the predominant site of spasticity and most common cause?

A

legs

Premature (periventricular leukomalacia), prenatal/postnatal infections, brain malformations, anoxic injury

48
Q

Extrapyramidal/dyskinetic CP, what does it effect and what are symptoms?

A

arms > legs

bradykinesia, choreoathetosis, hemiballismus, dystonia

Causes: hypoxic-ischemic encephalopathy and kernicterus

49
Q

What are fifth-day fits and how do you treat it

A

benign neonatal seizure
tx: AED
familial, should go away within few days to week after birth

50
Q

Most common cause of neonatal seizure

A

hypoxic ischemic encephalopathy

51
Q

Infantile epileptic encephalopathies EEG pattern

A

burst suppression pattern on EEG
treatment-resistant

52
Q

Pyridoxine dependent epilepsy tx

A

not AED
pyridoxine +/- folinic acid

Pediatrics (26 decks)

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