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Pediatrics > Neurology > Flashcards

Neurology Flashcards

1
Q

Neurocutaneous syndromes

A

Neurofibromatosis
Sturge Weber
Tuberous sclerosis

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2
Q

Slowly developing back pain in child +
Neuro dysfunction +
Step off in lumbosacral area

What is it?

A

Spondylolisthesis

= developmental DO caused by forward slip over vertebrae (usually over L5 or S1)

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3
Q

MIgraine ppx

A

Amitryptyline
Propranolol
Valproate
Topiramate

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4
Q

Myelomenigocele

A

Increased AFP
- screen at 16-18 wks

Usually in lumbosacral region

Meninges + spinal cord protrude

Physio issues:

  • +/- bowel, bladder incontinence
  • +/- flaccid paralysis
  • absent DTRs
  • hydocephalus assoc w/ type 2 Chiari defect
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5
Q

Meningocele

A

Meninges herniate through defect in posterior vertebral arches

+/- assoc w/ hydrocephalus

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6
Q

Spina bifida

A

Defect in closure of posterior vertebral arches adn laminae usually at L5 and S1

Most are asymptomatic

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7
Q

How test for neural tube defects?

A

AFP

Acetylcholinesterase

@ 16-18 wks

AFP elevated in meningocele and myelomeningocele. OK in spina bifida

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8
Q

Febrile seizure

A

1 seizure disorder of childhood (9 mo - 5 yo)

Usually generalized, tonic-clonic lasting few seconds to minutes

Atypical febrile seizures > 15 min

Dx

  • EEG NOT needed
  • Always consider meningitis!!!
  • no anticonvulsants
  • use antipyretics
  • rectal diazepam or lorazepam if febrile seizure prolonged
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9
Q

Infantile spasms

A

Generalized seizure

Start 4-8 mo age

Possible etiology: corticotropin releasing hormone overproduced –> neuronal hyperexcitability and seizures

3 types:

  • flexor spasms (flexion head, arms, neck ontro trunk)
  • extensor spasms (extension of tunk and extremities)
  • mixed spasms (flexion and extension)

EEG - hypsarrhythmia

Tx

  • ACTH
  • prednisone
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10
Q

Absence seizure

A

Generalized seizure

Not common < 5 yo
girls > boys
No aura, no postictal

EEG pattern = generalized, symmetrical 3 Hz spike and wave activity on nl background (provoke by hyperventilate during EEG)

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11
Q

Tx absence

A

Valproate

Ethosuximide

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12
Q

Myoclonic episodes

A

Brief, symmetric contractions w/ loss of body tone

Pts fall or slump forward

Benign myoclonus of infancy can be confused with infantile spasms - but EEG is normal and seizures resolve by 2 year

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13
Q

Partial seizures

A

3 types

  • simple
  • complex
  • benign focal

Simple

  • 10-20s
  • confused with tics
  • motor activity most common sx, no automatisms

Complex

  • auras
  • automatism common

Benign (rolandic)

  • focal motor w/ generalized spread
  • start @ 5 yo –> stop at adolescence
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14
Q

Cerebral palsy

- general info

A

D/o impaired motor functioning and posture

Onset before birth or year 1

NOT progressive

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15
Q

Spastic diplegia

A

Seen in LBW infants

Lower extremities more involved

Increased muscle tone
Increased DTR
Contractures
Seizures

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16
Q

Spastic quadriplegia

Type of CP

A

ALl 4 extremities equally involved

Scoliosis common

Assoc w/

  • severe asphyxia
  • LBW
  • MR
  • seizures
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17
Q

Spastic hemiplegia

Type of CP

A

Involves 1 side of body only

Cognitive function spared? seizures commom

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18
Q

Extrapyramidal form of CP

A

Hypotonia
Choreoathetosis (involuntary mvmts)
Dystonia

Can be assoc w/ kernicterus

19
Q

Ataxic form of CP

A

Hypotonia
Brisk reflexes
Severe cognitive delays

20
Q

Any lab workup for CP

A

NO

21
Q

Rett syndrome

A

Neurodegenerative DO of unknown cause

ONLY in girls

Onset < 1 yo

  • loss of developmental milestones
  • acquired microcephaly

Complications:
- generalized tonic clonic seizures

22
Q

Hydrocephalus

  • types
  • dx
  • tx
A

Usually 2/2 impaired absorption of CSF

Obstructive vs Nonobstructive

Obstructive - obstruction in ventricular system

Nonobstructive - obliteration of subarachonid cisterns or malfunction of villi

Dx

  • US
  • CT
  • MRI

Tx:

  • acetazolamide or furosemide for temporary relief
  • shunt

Complications = infection 2/2 steph epi at shunt

23
Q

Macewen sign

A

the is a sign used to help to diagnose hydrocephalus and brain abscesses.

Tapping (percussion) the skull near the junction of the frontal, temporal and parietal bones will produce a stronger resonant sound when either hydrocephalus or a brain abscess are present.

24
Q

No Hydrocephalus + foreshortened occiput suggests…

A

Arnold Chiari malformation I

  • downward displacement of the cerebellar tonsils through the foramen magnum
  • Is generally asymptomatic during childhood, but often manifests with headaches + urinary frequency + neck pain
  • less pronounced then type II
  • not associated w/ hydrocephalus
25
Q

Hydrocephalus + myelomeningocele suggest…

A

Arnold Chiari malformation II

  • there is a larger cerebellar vermian displacement.
  • low lying torcular herophili; The position of the torcular herophili is important for distinction from Dandy-Walker syndrome in which it is classically upturned
26
Q

Cystic expansion of 4th ventricle of posterior fossa + hydrocephalus + prominent occiput

A

Dandy walker malformation
- absence or partial cerebellar vermis

Papilledema
Gait abnormalities

27
Q

Werdnig-Hoffman Disease

A

Degnerative disease of anterior horn cells in spinal cord (motor neurons + motor nuclei in brains tem

Autosomal recessive

Happens < 2 yo, often in utero

Sx:

  • often lie in frog leg position
  • hypotonia, flaccid quadriplegia
  • normal intelligence
  • fasiculations of tongue
  • spares extraocular muscles - EOM will be intact

Tx: none

28
Q

Guillain Barre

A

Post infectious polyneuropathy
- demyelination in PERIPHERAL motor and sometimes sensory nerves

Assoc with:

  • C jejuni
  • Mycoplasma pneumoniae

Ascending weakness + paralysis
Usually 10 days after virus
can last days to weeks

PE:

  • DTR lost early
  • resp insufficiency can happen!

Dx:

  • CSF protein elevated
  • nl glucose, WBC

Tx:

  • admit for observation if resp distress
  • IVIG
  • steroids
29
Q

Charcot Marie Tooth

A

1 genetic neuropathy

Autosomal dominant

Peroneal muscular atrophy / tibial N atrophy too

  • gait distrubance
  • clumsy
  • trip over feet
  • pes cavus
  • tremor
  • variable sensory loss

Dx:

  • decreased motor and sensory conduction
  • sural N biopsy –> onion bulb formations surround axons
  • interstitial hypertrophic neuropathy**

Tx:
- orthotics

30
Q

Myasthenia gravis

A

Autoimmune

Destroy Ach receptors at postsynaptic junction
- Ach release is normal

Presentation:

  • ptosis
  • extraocular muscle weakness
  • dysphagia
  • weakness of limb-girdle and distal muscles of hands and feet

Rapid muscle fatigue

Dx:

  • EMG more diagnostic than muscle bx - decremental response to repetitive nerve stimulation
  • Anti-Ach antibody (but not in all)
  • hashimoto thyroiditis
  • edrophonium test
31
Q

2 forms of neonatal myasthenia

A

Transient

  • born to moms with myasthenia
  • only a few weeks

Congenital

  • rare
  • not caused by receptor antibodies
  • poor response to therapy
  • no remission
32
Q

Tx myasthenia gravis

A 
Neostigmine
Physostigmine ( need greater dosage but has longer activity)

Long term steroids

Thymectomy

Plasmapheresis

IvIG

33
Q

Complications of MG

Prognosis?

A

Don’t use:

  • neuromuscular blocking agents
  • avoid aminoglycosides - potentiates

Prognosis
- remission vs permanent disease; varies

34
Q

Neurofibromatosis

  • definition
  • etiology
  • PE
A

AD

Abnormality of neural crest differentiation during embryogenesis

2 types: 1 and 2

PE:

  • cafe au lait spots
  • Lisch nodules
  • optic N gliomas
  • scoliosis
35
Q

Lisch nodules

A

pigmented hamartomatous nodular aggregate of dendritic melanocytes affecting the iris

36
Q

Neurofibromatosis

  • dx
  • tx
A

Dx NF1 (need 2 of the following)

  • > = 5 cafe au lait spots > 5 mm prepubertal or > 6 cafe au lait spots > 15 mm postpubertol
  • axillary/infuinal freckling
  • > 2 iris Lisch nodules
  • > 2 neurofibromas
  • ossous lesion-kyphoscoliosis, sphenoid dysplasia
  • optic gliomas

Dx NF-2 (1 needed):

  • bilateral 8th N masses
  • parent, sibling, child with NF2

Tx

  • no specific
  • genetic counseling
  • annual ophtho
37
Q

Sturge Weber

A

Neurocutaneous syndrome

Assoc w/ vascularization that occurs sporadically in embryonic period

Characteristics:

  • port wine stain in trigeminal area**
  • intracranial calcifications
  • hemiparesis contralateral to port wine stain
  • +/- MR

Tx:

  • seizure control
  • cosmetic control of port wine stain

At risk for:

  • seizures –> hemiparesis
  • glaucoma
38
Q

Hx of trauma to soft palate with foreign body

Hemiparesis now…what is going on?

A

Internal carotid A dissection –> stroke

Confirm with MRI/MRA of brain

39
Q

Medullobastomas are most commonly found

A

From cerebellar vermis

40
Q

Infants, event if increased ICP, have decreased risk of herniation s/p LP because

A

of open fontanels

41
Q

Malignant hyperthermia

A

Myopathy inherited as AD trait on ch 19

Codes for ryanodine receptor = Ca release channel

Dx:
- caffeine contracture test –> + muscle spasm

42
Q

Acute disseminated encephalomyelitis

A

Autoimmune demyelinating disease

Children < 10 yo

Can follow different infections (URI, varicella, mycoplasma)

MRI shows multiple white plaques like MS

MOrtality is high

Tx - corticosteroids

43
Q

Tumor locations for kids

A

< 1 yo - supratentorial

1-10 yo - infratentorial

> 10 yo - supratentorial

44
Q

Botulism in infant commonly presents as

A

constipation

Pediatrics (17 decks)

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