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General Peds Boards > Neurology > Flashcards

Neurology Flashcards

1
Q

Diagnosis criteria and treatment of Tourette’s syndrome

A
  • Motor tics typically start after age 5-6
  • If tic has been present for > 1 year, diagnosis can be made of Tourette’s
  • Treatment is mostly supportive but if tic is particularly bothersome can use haloperidol or pimozide
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2
Q

Acute arterial ischemic stroke causes/symptoms

A
  • Embolic causes: artery to artery embolism from carotid/vertebral dissection, intracardiac thrombi, paradoxical embolism from venous system through cardiac shunt
  • In situ thrombosis causes: hypercoagulable states
  • Vasculopathy causes: focal cerebral arteriopathy from post-infectious causes
  • Genetic causes: Fabry disease, a-galactosidase A deficiency
  • Metabolic causes: MELAS
  • S/sx: acute focal neurologic deficit
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3
Q

Acute arterial ischemic stroke diagnosis, management, complications

A
  • Diagnosis: start with CT, then dig further with CTA/MRA

- Complications: acute stroke in posterior fossa can lead to edema and obstructive hydrocephalus

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4
Q

Pseudotumor cerebri signs/symptoms

A
  • Headaches worse when laying flat, N/V, transient vision loss, obesity, females
  • CN VI palsy (double vision) and papilledema on exam
  • Complication: optic disc atrophy and blindness if not treated
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5
Q

Atlantoaxial instability complications

A
  • Spinal cord compression
  • Hyperreflexia and increased tone in lower extremities
  • T21 kids
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6
Q

Benign rolandic epilepsy symptoms

A
  • Rhythmic twitching of one side of the face and ipsilateral arm with a lot of drooling
  • Seizures are usually infrequent and often occur during sleep
  • Can be associated with migraines
  • If they happen while awake the child is fully aware but unable to speak
  • Resolve by 16-18 years of age
  • EEG with biphasic focal centroemporal spikes and slow waves
  • Don’t need meds unless 3 or more seizures
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7
Q

Number one cause of death among all childhood cancers

A

Brain tumors

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8
Q

Symptoms of infratentorial/posterior fossa brain tumor

A
  • Headache, vomiting (especially in the morning), ataxia in an afebrile child
  • Can have head tilt, torticollis, decreased upward gaze (CN VI dysfunction)
  • Papilledema
  • Often < 6 years of age
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9
Q

Medulloblastoma imaging

A
  • Occurs midline (commonly in IV ventricle)

- Contrast enhancing, can cause hydrocephalus

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10
Q

Supratentorial mass symptoms

A
  • Often have motor findings (weakness, seizures, etc)

- Commonly > 8 years old

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11
Q

Types of infratentorial masses

A
  • Pilocytic astrocytoma
  • Medulloblastoma
  • Ependymoma
  • Brain stem glioma
  • Atypical teratoid/rhabdoid
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12
Q

Types of supratentorial masses

A
  • Craniopharyngioma
  • Diencephalic
  • Germ cell tumors
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13
Q

Craniopharyngioma symptoms and imaging

A
  • Sits right above the optic chias –> personality changes, headaches, vision changes (superior temporal field cut), growth failure
  • Benign but can recur
  • Often in ages 5-10
  • Imaging shows calcification in the sella turcica
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14
Q

Optic nerve glioma association

A

Neurofibromatosis

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15
Q

Brain tumor treatment

A
  • Cut out the tumor (need diagnosis)

- Radiation and chemo after

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16
Q

Chiari II malformation

A
  • Cerebellar tonsils and vermis descend through foramen magnum
  • Almost always associated with meningomyelocele
  • Often have hydrocephalus
  • Prenatal diagnosis, often need surgical repair
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17
Q

Chiari I malformation

A
  • Cerebellar tonsils through foramen magnum
  • Often asymptomatic
  • Often don’t require surgery
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18
Q

Chairi III malformation

A
  • Chiari II + occipital encephalocele
  • Developmental delay, hydrocephalus, seizures
  • Often don’t have good prognosis even after surgical repair
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19
Q

Holoprosencephaly

A
  • Single primitive ventricle
  • Fused thalami
  • Absent olfactory bulbs/tracts and optic tracts
  • Midface underdevelopment (single eye, rudimentary nose, single nostril nose, ocular hypotelorism, cleft lip/palate)
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20
Q

Holoprosencephaly genetic/exposure associations

A
  • Trisomy 13, 15, 18
  • Maternal diabetes
  • In utero alcohol exposure
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21
Q

Teenager with frontal/band like pressure headaches

A
  • Stress/tension headache

- Tx: eliminate stressor, treat depression

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22
Q

Lissencephaly

A
  • Abnormal neuronal migration at 9-13 weeks
  • Paucity of white matter
  • Severe intellectual disability, epilepsy, hypotonia
  • Associated with Fukayama, Walker Warburg
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23
Q

Cerebral Palsy definition

A
  • Static encephalopathy (single perinatal CNS insult during early development)
  • Chronic, non-progressive motor deficit
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24
Q

Spastic types of CP

A
  • MC type of CP overall
  • Quadriplegia
  • Diplegia: most common in the lower extremities (tip toe walking or delayed walking), most common in preemies (IVH)
  • Hemiplegia: more common in the upper extremities, have early handedness, focal lesions
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25
Q

Dyskinetic CP association

A
  • Kernicterus

- HIE

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26
Q

Most common risk factor for premature babies to develop CP

A

Perinatal infections

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27
Q

Dyskinetic CP

A

Spastic quad symptoms but also have dystonia and strange movements

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28
Q

Migraine headache symptoms

A
  • Episodic
  • Cause kids to stop activities and go lay down
  • Light/sound sensitivity
  • Nausea/vomiting
  • Hemiparesis, temporary visual deficits
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29
Q

Migraine headache abortive treatment

A
  • Ibuprofen, tylenol (no more than 3 days per week)
  • Fluids, rest
  • Ergotamines
  • Sumatriptan
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30
Q

Management of CP

A
  • Seizure management
  • PT/OT/ST
  • Ortho issues over time
  • Baclofen pump
  • Botox
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31
Q

Migraine headache prophylactic treatment

A
  • Cyproheptadine

- Topiramate

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32
Q

Causes of pediatric stroke

A
  • Embolic (1/4th from the heart)
  • Dissection (trauma, can be minor)
  • Vasculitis
  • Sickle cell
  • Coagulopathies
  • Metabolic (MELAS, Fabry, Homocystinuria)
  • CVA malformations (commonly cause hemorrhagic strokes)
  • NAT
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33
Q

Stroke workup

A
  • MRI, MRA
  • Echo/EKG/Tele
  • Urine drug screen
  • Labs: CBC, sed rate, PT, PTT, antiphospholipid antibodies, factor V leiden, prothrombin mutation, lactate, protein C/S, homocystine, antithrombin III
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34
Q

Stroke management

A
  • tPA if within first 3 hours and no bleeding

- baby aspirin otherwise

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35
Q

Indications for head CT, MRI, ultrasound

A
  • CT: hemorrhage, tumors, abscesses
  • MRI: partial seizures, herpes encephalitis
  • Ultrasound: accelerating head circumference in infants
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36
Q

Clues to pseudoseizures

A
  • Absence of post-ictal state

- Often in daytime with other people around

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37
Q

Paroxysmal non-epileptic event

A

Looks like a seizure but has no EEG changes

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38
Q

Common causes of metabolic seizures

A

Hyponatremia, hypoglycemia, hypocalcemia, urea cycle disorders, pyridoxine deficiency

39
Q

Febrile seizure facts

A
  • Age 6 months to 5 years
  • 3-5% of kids will have a febrile seizure sometime in their life
  • Associated with no other seizure history
  • 1/4 of kids have a family history of febrile seizures
40
Q

Facts for complex febrile seizures

A
  • > 15 minutes
  • Recurs within 24 hours
  • Focal onset
41
Q

Recurrence of febrile seizures

A
  • 1/3 of kids will have a second febrile seizure
  • Risk factors for recurrence: young age, low fever at time of first seizure, family history of febrile seizures
  • 5% of patients will go on to develop epilepsy (increased risk with FH or complex seizure)
42
Q

Medications that can cause seizures

A
  • Chemo: cyclosporine, IT methotrexate
  • Isoniazid
  • Insulin
  • Bupropion
  • Theophylline
  • Cocaine
43
Q

Indications for LP in febrile seizure

A

If complex partial seizures or febrile status epilepticus or if baby was partially treated with antibiotics

44
Q

Symptoms of absence seizures

A
  • Brief staring spells (< 30 seconds), not responsive during it
  • No postictal period but don’t remember the event
  • Spacing out/blinking
  • Triggered with hyperventilation
45
Q

EEG finding and treatment for absence seizures

A
  • 3 second spike and wave

- Tx: ethosuximide

46
Q

Side effects of valproic acid

A
  • Thrombocytopenia

- Elevated LFTs and lipase

47
Q

Phases of generalized tonic clonic seizure

A
  • Tonic: flexion of trunk, extension of back/arms/legs that usually lasts about 30 seconds
  • Clonic: convulsion movements, can be apneic during this, also time of bladder/bowel loss
  • Postictal: unconscious then sleepy/confused, can’t remember the seizure
48
Q

Side effect of lamotrigene

A

Stevens Johnson Syndrome

49
Q

Symptoms of juvenile myoclonic epilepsy

A
  • Myoclonic jerks upon awakening (often occur in adolesence)
  • Generalized tonic clonic seizures in most of patients
  • Absence seizures in 1/3 patients
  • Precipitators: lack of sleep, emotional stress, alcohol
  • Tx: valproic acid or levetiracetam
50
Q

Infantile spasms, hypsarrhythmia, developmental delay

A

West Syndrome

51
Q

Symptoms of infantile symptoms

A
  • Present between 3-9 months
  • Spasms in clusters: sudden flexion or extension of the body
  • Often have severe developmental delay
52
Q

Infantile spasm genetic association

A

Tuberous sclerosis

53
Q

Treatment of infantile spasms

A
  • ACTH or steroids
  • If developmental delay before onset of seizures –> poor prognosis
  • After infantile spasms, they often develop other seizure types
54
Q

Simple partial seizure symptoms

A
  • Consciousness is maintained
  • Focal abnormal movement (usually arms or face)
  • Often occur as patient falls asleep or just wakes up
  • Can be sensory or autonomic seizures
55
Q

Treatment of simple partial seizures

A
  • Carbamazepine often used

- Many cases remit during adolescence and treatment is not always necessary

56
Q

Complex partial seizure symptoms

A
  • Alteration in consciousness
  • Often have facial movements or lip smacking
  • Can appear to be responding to auditory or visual hallucinations
  • Need MRI and EEG
57
Q

Definition of status epilepticus

A
  • > 30 minutes
  • Check a glucose
  • Give ativan then fosphenytoin load
58
Q

Symptoms of Bell’s Palsy

A
  • LMN of CN VII palsy
  • Loss of ALL muscles in forehead and lower part of the face
  • Associated with Lyme’s disease, hypertension, otitis media, and ALL
59
Q

Contraindications to LP

A
  • Symptoms of increased ICP
  • Focal neuro signs
  • History of coagulopathy
  • Cardiorespiratory instability
60
Q

Medications that can cause pseudtumor cerebri

A
  • High dose vitamin A (Retin A)
  • Steroids
  • Thyroxine
  • Lithium
61
Q

Pseudotumor cerebri treatment

A
  • Acetazolamide (carbonic anhydrase inhibitor)
  • Shunt placement if severe
  • LP can be therapeutic
62
Q

Cushing’s triad

A
  • Hypertension
  • Bradycardia
  • Abnormal respirations
  • Impending brain herniation
63
Q

Unilateral pupil dilation

A

Uncal herniation (due to compression of oculomotor cranial nerve)

64
Q

Cranial bruit in a neonate with hydrocephalus and a history of heart failure

A

Vein of Galen malformation

65
Q

Symptoms of narcolepsy

A
  • Excessive daytime sleepiness and sudden sleep attacks
  • Cataplexy, sleep paralysis, hypnagogic hallucinations
  • Onset in adolesence
  • Dx with sleep study
  • Tx with behavioral/environmental changes, may need meds
66
Q

Causes of ataxia

A
  • Toxins (ethanol, pesticides)
  • Infection (postviral or Guillain Barre)
  • Neoplasm (glioma, medulloblastoma)
  • Other meds: anticonvulsants, alcohol, thallium
67
Q

Symptoms of ataxia telangeictasia

A
  • Ataxia
  • Telangeictasias in skin and eye
  • Frequent upper/lower respiratory infections (IgA, IgG, T cell dysfunction)
  • Intellectual disability
  • High incidence of malignancy (Hodgkin lymphoma and leukemia)
  • Autosomal recessive
68
Q

Friedreich Ataxia symptoms

A
  • 50% of all causes of inherited ataxia
  • Autosomal recessive
  • Late childhood/early adolesence
  • Slow/clumsy gait (elevated plantar arch)
  • Can also have decreased strength and reflexes in lower extremities
  • Risk for cariomyopathy
69
Q

Epidural vs subdural hematoma

A
  • Epidural: between blood and skull bone, lens shape, can have lucid period, need to act fast (can herinate quickly)
  • Subdural: between brain parenchyma and dura, linear and more diffuse distribution
70
Q

Huntington chorea triad

A
  • Chorea
  • Hypotonia
  • Emotional lability

Autosomal dominant

Tx of chorea: dopamine blocking agents (haloperidol), risperidone

71
Q

Associations with acute dystonic reaction

A
  • Sx: neck hyperextension and decreased extraocular movements
  • Can be caused by neuroleptics (like metoclopramide or promethazine)
  • Tx with benadryl
72
Q

Type of medication that can unmask tic disorder

A
  • Stimulants (don’t cause tics but can unmask them)

- Tics are repetitive movements that improve or disappear during purposeful movements and can by suppressed

73
Q

Symptoms of an epidural abscess

A
  • Spinal pain, paresthesias, weakness, paralysis, fevers
  • Localized spinal compression: decreased anal tone, reduced sensation in lower extremities, increased reflexes
  • Risk factor: IV drug users
  • Tx: MRI/CT scan, antistaph antibiotics, surgical decompression
74
Q

Child with a fever with abrupt onset of weakness, hypotonia, and decreased reflexes followed by increased tone and hyperreflexia

A
  • Acute transverse myelitis
  • Follows an infection and is due to lymphocytic infiltration and demyelination of nerves due to inflammation
  • CSF has increased neutrophils, negative gram stain
  • Pain on palpation of the spinal canal and MRI shows cord swelling
  • Tx: spontaneous recovery in a few months
75
Q

Treatment for extrinsic spinal cord mass lesion

A

High dose IV dexamethasone

76
Q

Symptoms and biology of spinal muscle atrophy type 1 (Werdnig-Hoffman disease)

A
  • Presents in infancy as severe hypotonia and weakness (poor suck, tongue fasciculations)
  • Degeneration of anterior horn cells (ONLY MOTOR FUNCTION AFFECTED)
  • Autosomal recessive
  • Dx with muscle biopsy
77
Q

When do NTDs occur

A
  • Between 3rd and 4th weeks of gestation

- Risk factors: maternal folate deficiency, maternal anticonvulsant use

78
Q

Symptoms of occult spinal dysraphism

A
  • Lipoma, patch of hair, hemangioma, discoloration, sacral dimple
  • Later symptoms: leg strength discrepancy, elevated arches, gait abnormalities, sensory issues, incontinence
  • Need MRI
79
Q

Treatment for spinal trauma

A
  • Methylprednisone (not dex like spinal cord compression), immobilizing cervical and lower spine
80
Q

Nerve and symptoms involved in bell’s palsy

A
  • CN 7 (facial nerve)
  • Unilateral facial paralysis that develops abruptly –> forehead weakness (decreased wrinklingon teh affected side)
  • Tx: prednisone for 5 days
81
Q

Ramsay hunt facial paralysis

A
  • Facial paralysis with painful vesicles in the ear canal
  • Due to reactivation of variella zoster virus
  • Tx: valacyclovir
82
Q

Ascending progressive motor weakness and arflexia

A

Guillain barre syndrome

  • ALWAYS have areflexia
  • Often preceded by vial illness or campylobacter
  • Can also have CN findings and dysautonomia on exam
83
Q

Labs and treatment for guillain barre

A
  • CSF with increased protein, normal cell count
  • Measure pulmonary function tests (NIF) –> respiratory failure is biggest issue
  • Tx: plasmapheresis, IVIG (NO STEROIDS)
84
Q

Cause of myasthenia gravis

A
  • Autoimmune disorder with development of antibodies against acetylcholine recpetor in neuromuscular junction
85
Q

Symptoms of myasthenia gravis

A
  • Weakness that gets worse with activity and improves with rest (more tired as the day goes on)
  • PTOSIS!!
  • Can be associated with thymoma
86
Q

Workup and treatment for myasthenia gravis

A
  • Tensilon test
  • Anti-aceytlcholine recpetor antibodies
  • EMG
  • Tx: pyridostigmine (inhibits acetylcholinesterases), plasmaphresis, plasma exchange
  • Thymectomy can be curative in kids
87
Q

Becker muscular dystrophy

A
  • Muscle weakness that is milder with slower deterioraiton and later onset than Duchenne
  • Can have severe cramps and more severe cardiac involvement
88
Q

Duchenne muscular dystrophy genetics

A
  • X linked recessive (Xp21 gene testing)
  • Absence of dsytrophin
  • Dx with muscle biopsy
89
Q

Duchenne muscular dystrophy symptoms

A
  • Poor head control in infancy
  • Weakness in hip muscles or lordotic posture in toddlers, can’t walk up stairs
  • Gower sign: walks hands up his legs in order to stand up
  • Pseudohypetrophy of calves: fat deposition and prolifeartion of collagen
  • Mild intellectual disability
  • Elevated CPK (can be elevated before muscle weakness starts)
  • ABSENCE of tongue fasciculations and eye muscle involvement
  • Complications: cardiomyopathy, respiratory failure, pneumonia
90
Q

Myotonic muscular dystrophy symptoms

A
  • Affects skeletal muscle, heart, and GI tract
  • Presents around 4-5 years of age with muscles that have slow relaxation after contraction (esp in hand)
  • Autosomal dominant
91
Q

Definition of encephalocele

A

Sac like protrusion of the brain and membranes that cover it through an opening in the skull

92
Q

Definition of spina bifida

A

Opening in vertebral column caused by the failure of the caudal neural tube to close

  • A/w chiari II malformation and hydrocephalus
  • Risk when mom usings valproate or carbamazepine
93
Q

Nerves involved in Marcus Gunn phenomenon

A
  • Trigeminal and oculomotor nerves

- Simultaneous eyelid blinking during sucking jaw movements

General Peds Boards (24 decks)

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