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Flashcards in Neurology Deck (44)
1

Neurocutaneous syndromes

Neurofibromatosis
Sturge Weber
Tuberous sclerosis

2

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

What is it?

Spondylolisthesis

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

3

MIgraine ppx

Amitryptyline
Propranolol
Valproate
Topiramate

4

Myelomenigocele

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

5

Meningocele

Meninges herniate through defect in posterior vertebral arches

+/- assoc w/ hydrocephalus

6

Spina bifida

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

Most are asymptomatic

7

How test for neural tube defects?

AFP

Acetylcholinesterase

@ 16-18 wks

AFP elevated in meningocele and myelomeningocele. OK in spina bifida

8

Febrile seizure

#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

9

Infantile spasms

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

10

Absence seizure

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)

11

Tx absence

Valproate

Ethosuximide

12

Myoclonic episodes

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

13

Partial seizures

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

14

Cerebral palsy
- general info

D/o impaired motor functioning and posture

Onset before birth or year 1

NOT progressive

15

Spastic diplegia

Seen in LBW infants

Lower extremities more involved

Increased muscle tone
Increased DTR
Contractures
Seizures

16

Spastic quadriplegia

Type of CP

ALl 4 extremities equally involved

Scoliosis common

Assoc w/
- severe asphyxia
- LBW
- MR
- seizures

17

Spastic hemiplegia

Type of CP

Involves 1 side of body only

Cognitive function spared? seizures commom

18

Extrapyramidal form of CP

Hypotonia
Choreoathetosis (involuntary mvmts)
Dystonia

Can be assoc w/ kernicterus

19

Ataxic form of CP

Hypotonia
Brisk reflexes
Severe cognitive delays

20

Any lab workup for CP

NO

21

Rett syndrome

Neurodegenerative DO of unknown cause

ONLY in girls

Onset < 1 yo
- loss of developmental milestones
- acquired microcephaly

Complications:
- generalized tonic clonic seizures

22

Hydrocephalus
- types
- dx
- tx

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

Macewen sign

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

No Hydrocephalus + foreshortened occiput suggests...

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