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Pediatrics > Neuro > Flashcards

Neuro Flashcards

1
Q

Brain Abscess: fever, h/a, focal neuro deficits, seizure

Risk factors include:

  1. what conditions related to the head?
  2. what conditions related to the heart?
  3. Nocturnal symptom?
  4. Morning symptom?
A

Brain Abscess

Presentation: fever, h/a, focal neuro deficits, seizure

  • -Nocturnal h/a
  • -Morning vomiting

Risk Factors

  • -direct spread: sinusitis, otitis, dental infection
  • -hematogenous spread: infection, cyanotic heart dz
  • Cyanotic Heart Disease
  • -R2L shunt
  • -bacteria in blood bypass pulmonary circulation, where they’re removed by phagocytosis

NB: similar presentation to tumor; imaging alone cannot tell difference; requires biopsy

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2
Q

DDx: flaccid paralysis

  1. descending flaccid paralysis
  2. descending flaccid paralysis preceded by n/v, abdominal pain, diarrhea
  3. ascending flaccid paralysis
A

DDx: flaccid paralysis

  1. Infant Botulism
    –ingestion of C. botulinum spores
    Tx: human-derived botulism Ig
  2. Foodborne Botulism
    –ingestion of preformed botulinum toxin
    Tx: equine-derived botulism anti-toxin
  3. Guillain-Barre Syndrome
    –autoimmune peripheral nerve demyelination
    Tx: IVIGs
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3
Q

Neuroblastoma

  • -N-myc
  • -usually adrenal or retroperitoneal
  • -palpable, painful mass in flank or midline
  • -calcification and hemorrhage on CT/Xray
  • -elevated urine HVA, VMA
  1. Derived from what embryonic structure?
  2. Metastases most common in what bones?
A

Neuroblastoma

  • -derived from neural crest cells
  • -N-myc
  • -usually adrenal or retroperitoneal
  • -palpable, painful mass in flank or midline
  • -calcification and hemorrhage on CT/Xray
  • -metastases to skull, orbital bone
  • -elevated urine HVA and VMA

NB: Wilms tumor arises from metanephros, which is precursor to renal parenchyma

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4
Q

–ataxia at less than 10 y.o.; loss of DTRs; extensor plantar reflex; weakness in hands and feet; explosive, dysarthric speech; hypertrophic cardiomyopathy

Dx?
Mode of Inheritance?

A

Friedrich Ataxia
–AR

Neuro

  • -ataxia
  • -loss of DTRs
  • -extensor plantar reflex
  • -dysarthria

Cardiovascular

  • -hypertrophic cardiomyopathy
  • -diabetes

MSK

  • -scoliosis
  • -hammer toes
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5
Q

–liver disease in a child with neurologic, behavioral, or psychiatric changes; Kayser-Fleischer rings; inborn error of copper metabolism

Tx: chelation with penicillamine

Dx?
Mode of Inheritance?
Screening test?

A

Wilson Disease

  • -AR
  • -decreased serum ceruloplasmin
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6
Q

Tay-Sachs Disease

  • -normal development until 6m, then regress; seizures, hypotonia, blindness; cherry-red macula
  • -accumulation of GM2

Enzyme deficiency?
Mode of Inheritance?

A

Tay-Sachs Disease

  • -defiency of beta-hexosaminidase A
  • -AR
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7
Q

Lesch-Nyhan Disease
–self-mutilation and dystonia; gouty arthritis; tophi; renal calculi

Mode of Inheritance?
Elevated levels of what marker?
Mutation in gene for what enzyme?

A

Lesch-Nyhan Disease

  • -XLR
  • -elevated uric acid
  • -mut in gene for HPRT enzyme
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8
Q

Intracranial Pathology s/s

  1. Nocturnal symptom?
  2. Morning symptom?
A

Intracranial Pathology s/s

  • -nocturnal h/a
  • -morning vomiting

*Evaluate with CT, MRI

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9
Q

Dx and Tx: Seizures

  1. asynchronous tonic or clonic movements; 10-20s; no post-ictal period; EEG: spike and sharp waves or multifocal spikes
  2. LOC; automatisms; post-ictal state; EEG: sharp waves or focal spikes in temporal lobe; MRI: abnormality in temporal lobe
  3. aura with focal findings; LOC; tonic-clonic activity; post-ictal state
A

Partial Seizures

  1. Simple Partial Seizure
    –no LOC; deja-vu; patient may remember
    –asynchronous tonic or clonic movements; 10-20s
    –no post-ictal period
    –EEG: spike and sharp waves or multifocal spikes
    Tx: phenytoin
  2. Complex Partial Seizure
    –LOC
    –automatisms (lip smacking, chewing, etc.)
    –post-ictal state
    –EEG: sharp waves or focal spikes in temporal lobe; MRI: abnormality in temporal lobe
    Tx: carbamazepine
  3. Partial Seizure with Secondary Generalization
    - -LOC
    - -tonic-clonic activity
    - -post-ictal state
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10
Q

Dx and Tx: Seizures

  1. sudden cessation of motor activity or speech with blank stare and flickering eyes; no aura; less than 30s; no postictal period; more common in girls; EEG: 3 Hz spike and wave activity
  2. aura may demonstrate focal onset; loss of consciousness; eyes roll black; tonic contractions, then clonic contractions; tongue biting; loss of bladder control; semi-comatose for up to 2 hrs after with vomiting and frontal headache
    Dx? Tx? (4)
A

Generalized Seizures

  1. Absence (petit mal) Seizures
    Tx: ethosuximide, (2nd line - valproic acid)
  2. Tonic-Clonic Seizures
    Tx: valproic acid, phenobarbital, phenytoin, carbamazepine
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11
Q

Seizure Pharmacology

  1. First line tx for simple partial seizures?
  2. First line tx for complex partial seizures? Can cause SJS/TEN
  3. First line tx for absence seizures?

Tx Options for Tonic-Clonic Seizures
4. unique sfx include gingival hypertrophy, hirsutism, rash, lymphadenopathy; SJS/TEN

  1. three other options for T-C seizures?
A

Seizure Pharmacology

  1. Phenytoin
    - -simple partial seizures
  2. Carbamazepine
    - -complex partial seizures
    - -SJS/TEN
  3. Ethosuximide
    - -absence seizures

Tonic-Clonic Seizures

  1. Phenytoin
    - -gingival hypertrophy, hirsutism, rash, lympadenopathy; SJS/TEN
  2. Valproic acid, Phenobarbitol, Carbamazepine
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12
Q

DDx: Pediatric Brain Tumor

  • s/s: h/a, seizures, deteriorating school performance, new onset neuro abnormality, behavioral changes
    1. s/s of brain tumor; endocrine syndrome; visual field defect; Xray shows calcification at sella turcica; derived from remnants of Rathke pouch
    2. often in cerebellum; GFAP-positive; Rosenthal fibers (eosinophilic corkscrew fibers); cystic and solid components
    3. midline cerebellar; highly malignant; compress 4th ventricle –> hydrocephalus; “drop metastases” to spinal cord’ Homer-Wright rosettes; small blue cells
    4. found in 4th ventricle –> hydrocephalus; perivascular rosettes
    5. unilateral visual disturbance; strabismus; associated with neurofibromatosis
A

DDx: pediatric brain tumor

  1. Craniopharyngioma
    - supratentorial
  2. Benign pilocytic astrocytoma
  3. Medulloblastoma
  4. Ependymoma
  5. Optic nerve glioma
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13
Q

Myoclonic Seizures

  • -brief, symmetric muscle contraction and loss of body tone with falling forward
  • -Tx?

Infantile Spasms

  • -symmetric contractions of neck, trunk, extremities at 4-8m
  • -due to increased CRH
  • -EEG: hypsarrythmnia (asynchronous, chaotic bilateral spike and wave pattern)
  • -Tx?
A

Myoclonic Seizures
–tx: valproic acid

Infantile Spasms

  • -tx: ACTH
  • -add prednisone if no response
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14
Q

Cerebral Palsy

  • -uncoordinated motor dysfunction; rigidity and spasticity
  • -seizures
  • -abnormalities of speech, vision, intellect
  • -equinovarus deformity: feet point down and in

RFs: prematurity, LBW, intrapartum infxn, maternal EtOH and tobacco use, placental pathology, multiple gestation

Tx for spasticity includes dantrolene and what two other drugs?

A

Cerebral Palsy

Spasticity tx: dantrolene, baclofen, botulinum toxin

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15
Q

Hematoma: epidural v subdural

Epidural

  • -lens-shaped
  • -lucid interval precedes rapid neurologic deterioration
  • classically due to fracture of temporal bone with rupture of what vessel?

Subdural

  • -crescent-shaped
  • -progressive neurologic signs
  • tearing of what vessels?
A

Epidural Hematoma

  • -lens-shaped
  • -lucid interval precedes rapid neurologic deterioration
  • -fracture of temporal bone with rupture of middle meningeal artery

Subdural Hematoma

  • -crescent-shaped
  • -progressive neurologic signs
  • -tearing of bridging veins
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16
Q

Neurocutaneous Syndromes

  • -Neurofibromatosis Type 1
  • -Neurofibromatosis Type 2
  • -Tuberous Sclerosis
  • -Sturge-Weber Syndrome
  1. Which two are inherited AD, but require a second sporadic mutation?
  2. Which one is inherited AD or sporadic?
  3. Which one is caused by a sporadic mutation?
A

Neurocutaneous Syndromes

AD + 2nd sporadic mutation

  • -NF Type 1
  • -NF Type 2

AD or sporadic
–Tuberous Sclerosis

Sporadic
–Sturge-Weber Syndrome

17
Q

Sturge-Weber Syndrome

S (2)
T
Unilateral
R
G (2)
E

What cutaneous finding present at birth?

What neurological findings can be associated with the unilateral distribution? (3)

What is another name for this syndrome?

A

Sturge-Weber Syndrome

S - port wine Stain, Sporadic mut
T - Tram Track Ca2+
U - Unilateral
R - Retardation
G - Glaucoma, GNAQ
E - Epilepsy

Port wine stain (CN V1-2 distribution) present at birth

Unilateral – hemianopia, hemiparesis, hemisensory disturbance

Encephalotrigeminal Angiomatosis

18
Q

Neurofibromatosis Type 1 (von Recklinghausen)

  • -cognitive defects
  • -seizures
  • -increased risk of malignancies

What skin finding?

What eye finding seen on slit-lamp?

What growths at skin, optic nerve, and adrenal gland?

A

Neurofibromatosis Type 1 (von Recklinghausen)

  • -cognitive defects
  • -seizures
  • cafe au lait spots
  • iris Lisch nodules (pigmented iris hamartomas)
  • neurofibromas at skin
  • optic glioma
  • pheochromocytoma
  • -increased risk of malignancies: Wilms tumor, leukemia, rhabdomyosarcoma

NB:
NF1 - chr 17 - neurofibromin
NF2 - chr 22 - merlin

19
Q

What is the hallmark of NF Type 2?

A

Neurofibromatosis Type 2

  • bilateral acoustic neuromas
  • -hearing loss, facial weakness, headache, unsteady gait

NB:
NF1 - chr 17 - neurofibromin
NF2 - chr 22 - merlin

20
Q

Tuberous Sclerosis

H - hamartomas
A - angiofibromas
M - re: heart?
A - re: skin?
R - re: heart?
T - Tuberous Sclerosis
O - autosomal dominant
M - re: cognitive ability?
A - re: kidney?
S - re: neuro? re: skin?
A

Tuberous Sclerosis

Hamartomas (excessive growth of a tissue type native to organ of involvement)

Angiofibromas

Mitral regurgitation

Ash-leaf spots (hypopigmented area, increased with Wood UV lamp)

cardiac Rhabdomyoma

Tuberous sclerosis

autosomal dOminant

Mental retardation

renal Angiomyolipoma

Seizures

Shagreen patches (rough lesion with orange-peel consistency; lumbrosacral area)

21
Q

Ascending flaccid paralysis 10 days after a nonspecific viral illness or infxn with Campylobacter, Mycoplasma pneumoniae
–paralysis may have bulbar involvement (dysphagia, respiratory insufficiency

Dx?

Labs - CSF

  • -normal glucose; no cells
  • -what level is elevated?

Tx?

A

Guillain-Barre Syndrome
–postinfectious polyneuropathy with ascending flaccid paralysis

Path: demyelination of peripheral nerves

CSF

  • -normal glucose
  • -no cells
  • -elevated protein

Tx: IVIGs

22
Q

Duchenne Muscular Dystrophy

MoI?

Labs
–what muscle enzyme marker is elevated?

Dx Tests
–biopsy for protein that is absent?

Tx
–what rx for heart failure?

A

Duchenne Muscular Dystrophy

MoI: XLR

Path: progressive degeneration of mm fibers

Features

  • -Gower sign: getting up, slowly, step by step, from all fours
  • -calf pseudohypertrophy
  • -thigh mm wasting
  • -scoliosis
  • -cardiomyopathy
  • -intellectual impairment
  • -death around 18y from respiratory failure

Labs
–elevated CPK

Dx Tests

  • -molecular genetic test for defective dystrophin
  • -biopsy for absent dystrophin

Tx
–heart failure: digoxin

NB:
Becker’s Muscular Dystrophy
–milder form of DMD
–decreased dystrophin (but not absent)

23
Q

DDx: intellectual disability
*physical exam findings

  1. microcephaly, small palpebral fissures, smooth philthrum, thin vermillion border
  2. flat facial profile, slanted palpebral fissures, small low-set ears, excessive skin at nape of neck, single transverse palmar crease, clinodactyly, large space between first two toes
  3. long narrow face, prominent forehead and chin, large ears, macrocephaly, macroorchidism
A

DDx: intellectual disability
*physical exam

  1. FAS
    - -microcephaly, small palpebral fissures, smooth philthrum, thin vermillion border
  2. Down Syndrome
    - -flat facial profile, slanted palpebral fissures, small low-set ears, excessive skin at nape of neck, single transverse palmar crease, clinodactyly, large space between first two toes
  3. Fragile X Syndrome
    - -long narrow face, prominent forehead and chin, large ears, macrocephaly, macroorchidism
24
Q

Dx?

Presentation

  • -increasing head circumference
  • -prominent occiput
  • -cerebellar ataxia
  • -delayed motor development

Pathology
–cystic expansion of 4th ventricle due to absence of roof

Associations
–agenesis of posterior cerebellar vermis and corpus callosum

A

Dandy-Walker Malformation

Presentation
–increasing head circumference, prominent occiput, cerebellar ataxia, delayed motor development

Pathology
–cystic expansion of 4th ventricle due to absence of roof

Assocations
–agenesis of posterior cerebellar vermis and corpus callosum

25
Q

Myotonic Muscular Dystrophy

MoI?

Features

  • -myotonia
  • -frontal balding
  • -cataracts
  • -gonadal atrophy
A

Myotonic Muscular Dystrophy

MoI: AD
–CTG trinucleotide repeat in DMPK gene

Features

  • -myotonia (slow mm relaxation, eg hand grip)
  • -frontal balding
  • -cataracts
  • -gonadal atrophy

Pediatrics (13 decks)

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