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Neurology Flashcards

(44 cards)

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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

Study These Flashcards
A

Hypotonia
Choreoathetosis (involuntary mvmts)
Dystonia

Can be assoc w/ kernicterus

19
Q

Ataxic form of CP

Study These Flashcards
A

Hypotonia
Brisk reflexes
Severe cognitive delays

20
Q

Any lab workup for CP

21
Q

Rett syndrome

Study These Flashcards
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
Study These Flashcards
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

Study These Flashcards
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…

Study These Flashcards
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
Hydrocephalus + myelomeningocele suggest...
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
Cystic expansion of 4th ventricle of posterior fossa + hydrocephalus + prominent occiput
Dandy walker malformation - absence or partial cerebellar vermis Papilledema Gait abnormalities
27
Werdnig-Hoffman Disease
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
Guillain Barre
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
Charcot Marie Tooth
#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
Myasthenia gravis
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
2 forms of neonatal myasthenia
Transient - born to moms with myasthenia - only a few weeks Congenital - rare - not caused by receptor antibodies - poor response to therapy - no remission
32
Tx myasthenia gravis
``` Neostigmine Physostigmine ( need greater dosage but has longer activity) ``` Long term steroids Thymectomy Plasmapheresis IvIG
33
Complications of MG | Prognosis?
Don't use: - neuromuscular blocking agents - avoid aminoglycosides - potentiates Prognosis - remission vs permanent disease; varies
34
Neurofibromatosis - definition - etiology - PE
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
Lisch nodules
pigmented hamartomatous nodular aggregate of dendritic melanocytes affecting the iris
36
Neurofibromatosis - dx - tx
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
Sturge Weber
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
Hx of trauma to soft palate with foreign body Hemiparesis now...what is going on?
Internal carotid A dissection --> stroke Confirm with MRI/MRA of brain
39
Medullobastomas are most commonly found
From cerebellar vermis
40
Infants, event if increased ICP, have decreased risk of herniation s/p LP because
of open fontanels
41
Malignant hyperthermia
Myopathy inherited as AD trait on ch 19 Codes for ryanodine receptor = Ca release channel Dx: - caffeine contracture test --> + muscle spasm
42
Acute disseminated encephalomyelitis
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
Tumor locations for kids
< 1 yo - supratentorial 1-10 yo - infratentorial > 10 yo - supratentorial
44
Botulism in infant commonly presents as
constipation

Pediatrics (17 decks)

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