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Neurology Flashcards

1
Q

What cause of encephalitis typically occurs in warm climates and is carried by insects (mosquitoes)?

A

Arboviruses

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2
Q

What are St. Louis and West Nile Virus examples of?

A

Arboviruses

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3
Q

What type of virus causing encephalitis presents with generalized neurological findings and are transmitted from human to human?

A

Enteroviruses

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4
Q

What should you think of when you have non-specific findings preceding confusing and irritability in encephalitis?

A

Enterovirus

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5
Q

How does herpes encephalitis present?

A

Non-specific findings without a history of oral lesions

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6
Q

How is herpes encephalitis best diagnosed?

A

DNA PCR

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7
Q

What type of encephalitis presents with or after parotitis?

A

Mumps encephalitis

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8
Q

What season does mumps encephalitis present?

A

Late winter or early spring

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9
Q

What will be hints that should make you think of mumps encephalitis

A

Child/Teenager who is from another country or isn’t immunized

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10
Q

Febrile toddle who is lethargic one hour after seizure. CBC normal, LP with negative gram stain and moderate WBCs- thinking viral meningitis, treatment choices?

A

Acyclovir to cover for possible HSV encephalitis (could also pick IV antibiotics to cover for bacterial meningitis)

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11
Q

Teenager with a frontal headache or band-like pressure sensation?

A

Stress/Tension/Emotion headache

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12
Q

What is treatment for a tension headache?

A

Eliminate the stressor

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13
Q

For a headache, when are complicated studies usually not indicated?

A

If they describe a headache in the absence of a history of trauma and with no evidence of increased ICP

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14
Q

What should you think of with a headache, malaise, excessive sleeping, and/or declining school performance?

A

Depression

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15
Q

What type of headaches are episodic, with no symptoms between episodes?

A

Migraines

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16
Q

Severe headache in any location, child stops activities and wants to lay down, photophobia, phonophobia, nausea, vomiting?

A

Migraine

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17
Q

True or False: More concerning neurological signs such as hemiparesis and/or temporary visual deficits may be seen in certain migraines?

A

True

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18
Q

True or False: All migraines have to have an aura?

A

False- they will likely not describe an aura for migraine

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19
Q

Name some treatment options for migraine

A
  1. Ibuprofen
  2. Acetaminophen
  3. Fluids
  4. Rest
  5. Ergotamines
  6. Oral or intranasal sumatriptan (Imitrex)
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20
Q

When do you use narcotics for migraine pain?

A

Never

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21
Q

What are the 2 categories for treatment of migraines?

A

Abortive and prophylactic therapy

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22
Q

What are the 2 most commonly used medications for migraine prevention?

A
  1. Cyproheptadine

2. Topiramate

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23
Q

What psychiatric problem can trigger migraine?

A

Depression

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24
Q

Headaches due to depression alone are described as…?

A

Chronic daily headaches without other signs associated with migraines

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25
Q

Name 5 lifestyle modifications that should be encouraged for headache prevention.

A
  1. Regular exercise and activities
  2. Regular sleep patterns
  3. Regular eating patterns avoiding trigger foods such as cheese and chocolate
  4. Good hydration
  5. Avoid medication overuse
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26
Q

True or False: The most important recommendation for prevention of recurrent headaches is medication?

A

False- Lifestyle modifications

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27
Q

Minor head injury a few months ago, now presents with daily headaches not improved with acetaminophen, ibuprofen, or naproxen, pain is frontotemporal… Most appropriate next step in managing the patient?

A

Stop all medications- meds being used chronically lead to blunted effect

-No head CT as trauma was minor and headaches didn’t start right after. Not migraines either because they are daily, not episodic

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28
Q

Name 5 clues to increased ICP

A
  1. Visual disturbances
  2. Worsening headaches
  3. Abnormal eye movements
  4. Deteriorating school performance
  5. Ataxia
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29
Q

What should blurred vision occurring only before a headache make you think?

A

Migraine (v. increased ICP)

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30
Q

Name 3 red flags for headaches due to space-occupying lesions

A
  1. Headaches worse in the morning
  2. Vomiting with no nausea
  3. Headaches relieved by vomiting
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31
Q

When is an LP contraindicated?

A

If there are symptoms of increased ICP

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32
Q

If you suspect increased ICP, what is the diagnostic test of choice?

A

CT with contrast urgently (MRI later when stable)

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33
Q

What is increased ICP of unknown etiology?

A

Pseudotumor cerebri

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34
Q

Why is pseudotumor cerebri considered to be benign?

A

Herniation doesn’t occur

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35
Q

What can happen if pseudotumor cerebri isn’t treated?

A

Lead to papilledema and eventual optic disc atrophy and blindness

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36
Q

What presents with double vision, papilledema, and evidence of increased ICP (headache/tinnitus)?

A

Pseudotumor cerebri

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37
Q

What causes double vision in pseudotumor cererbri?

A

6th nerve palsy

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38
Q

What makes the headache worse in pseudotumor cerebri?

A

Laying flat and/or with Valsalva maneuvers

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39
Q

What is a common cause of pseudotumor cerebri?

A

Megadose vitamin intake (particularly Vitamin A- including Retin A)

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40
Q

Name the clinical signs for increased intracranial pressure in infants

A
  1. Irritability
  2. Decreased PO intake
  3. Failure to thrive
  4. Macrocephaly with wide sutures
  5. Bulging fontanel
  6. Setting sun sign
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41
Q

What is the setting sun sign and what is it associated with?

A

Downward deviation of the eyes with hydrocephalus

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42
Q

Name clinical signs of increased intracranial pressure in children

A
  1. HTN
  2. Papilledema
  3. Bradycardia
  4. Abducens paresis (6th nerve palsy- double vision)
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43
Q

If you are presented with any findings suggestive of increased ICP, what is indicated?

A

Brain imaging studies

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44
Q

What must be done before an LP if you suspect increased ICP?

A

Head CT

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45
Q

Name contraindications to performing an LP

A
  1. Evidence of increased ICP
  2. Focal neurological signs
  3. History of coagulopathy
  4. Cardio-respiratory instability (ABCs before LP)
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46
Q

Name medications that may cause pseudotumor cerebri

A
  1. Steroids
  2. Thyroxine
  3. Lithium
  4. Some antibiotics
  5. Vitmain A (Retin-A)
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47
Q

How can pseudotumor cerebri be treated?

A

With carbonic anhydrase inhibitors (acetazolamide)

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48
Q

In severe cases of pseudotumor cerebri where acetazolamide doesn’t work, what can be used for treatment?

A

Steroids and possibly surgery to shunt CSF

Steroids can be cause and treatment of pseudotumor cerebri

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49
Q

What is Cushing’s Triad?

A
  1. Hypertension
  2. Bradycardia
  3. Abnormal respirations
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50
Q

What does Cushing’s Triad indicate?

A

Late findings of increased ICP and usually indicate impending herniation

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51
Q

What is hydrocephalus?

A

Accumulation of excessive amounts of CSF in the brain

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52
Q

What is hydrocephalus usually associated with?

A

Increased ICP

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53
Q

What is the major risk factor for hydrocephalus?

A

Neural tube defect from Chiari malformation or myelomeningocele

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54
Q

Besides neural tube defects, what are other instances where hydrocephalus can be seen?

A

Brain malformations, congenital infections, intracranial bleeds, space-occupying lesions blocking CSF flow

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55
Q

How do infants present with hydrocephalus?

A

Rapid head growth (no fused sutures yet)

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56
Q

How do children present with hydrocephalus?

A

Headaches, papilledema, cranial nerve palsies (especially impairment of upward gaze- sundowning sign)

Once sutures are fused

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57
Q

When do most shunt infections occur?

A

In the first 6 months after shunt placement

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58
Q

When should you suspect a shunt infection?

A

In any patient with a shunt who presents with symptoms of hydrocephalus and persistent fever

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59
Q

What is done to treat a shunt infection?

A

IV antibiotics

Remove infected shunt

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60
Q

How do shunt malfunctions present?

A

Symptoms of increased ICP, but no fever

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61
Q

Upper respiratory findings consistent with chronic sinusitis, severe unilateral headache with vomiting that doesn’t improve with analgesics, focal neurologic deficits, papilledema, seizures?

A

Brain abscess

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62
Q

True or False: Fever is always seen in brain abscess?

A

False- not a common symptoms

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63
Q

What 3 groups are at increased risk for developing a brain abscess?

A

Patients with pulmonary, sinus, and cyanotic heart disease

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64
Q

What is the classic description for a brain abscess on CT?

A

Ring enhancing lesion

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65
Q

What are initial antibiotics of choice for a brain abscess?

A

Directed at organisms expected base on source of infection: Ceftazidime/cefepime, nafcillin/vancomycin, metronidazole, ceftriaxone

*Should be adjusted based on cultures obtained via CT-guided aspiration or surgical excision

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66
Q

What is the first thing to note in a patient with altered state of consciousness?

A

AGE

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67
Q

Besides sepsis workup, what else should you consider ordering for a non-responsive neoante?

A

Serum ammonia and organic acid levels

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68
Q

What should you think of with a cranial bruit in a neonate with hydrocephalus and history of CHF?

A

Vein of Galen malformation

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69
Q

In older kids, what is an important diagnosis for altered state of consciousness?

A

Encephalopathy

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70
Q

Child with altered state of consciousness, symptoms progressed over several hours, family member with condition that requires meds (depression/seizures/heart disease)?

A

Toxic ingestion

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71
Q

Abrupt onset of altered state of consciousness?

A

Consider head trauma

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72
Q

Abrupt onset of altered state of consciousness, no history of head trauma… initial study?

A

CT head- Trauma may have been unwitnessed

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73
Q

How do younger patients with Wilson’s disease usually present?

A

Symptoms of liver disease

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74
Q

How do older patients with Wilson’s disease usually present?

A

Neuro/psych symptoms

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75
Q

Name common neurological symptoms in Wilson’s disease.

A
  1. Tremors
  2. Emotional problems
  3. Difficulty with handwriting
  4. Depression
  5. Abnormal eye movements
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76
Q

Evidence of acute hepatic failure coupled with dystonia and mental status changes?

A

Wilsons

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77
Q

In a patient with suspected Wilson’s disease who has neurological or psychiatric symptoms, what are you likely to find on exam?

A

Kaiser-Fleischer ring (greenish-yellow rings seen at edge of cornea best seen on slit-lamp exam)

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78
Q

Severe daytime sleepiness and sudden “sleep attacks”?

A

Narcolepsy

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79
Q

Name 3 manifestations of narcolepsy

A
  1. Cataplexy
  2. Sleep paralysis
  3. Hypnagogic hallucinations (almost asleep)
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80
Q

What is cataplexy?

A

Sudden drops precipitated by sudden emotions like laughter

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81
Q

When does onset of narcolepsy typically occur?

A

Adolescence

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82
Q

How is narcolepsy diagnosed?

A

Overnight polysomnography and sleep latency test

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83
Q

Who should you refer patients with suspected narcolepsy to?

A

Sleep specialist

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84
Q

True or False: Patients with narcolepsy often have academic problems?

A

True

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85
Q

What is the most likely cause for acute onset ataxia?

A

Post-viral (varicella, EBV, mumps)

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86
Q

True or False: The prognosis of acute cerebellar ataxia after infections or vaccinations is generally excellent?

A

True

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87
Q

Name 5 causes of acute ataxia.

A
  1. Toxic ingestion
  2. Neoplasm
  3. Trauma
  4. Metabolic problems
  5. Infections
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88
Q

How is ataxia telangiectasia inherited?

A

Autosomal recessive

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89
Q

What is the general problem in ataxia telangiectasi?

A

Defect in DNA processing and repair

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90
Q

What are capillary dilataions causing red blotches on the skin and conjunctiva?

A

Telangiectasias

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91
Q

What are 4 main things that ataxia telangiectasia effects?

A
  1. CNS
  2. Skin/Eyes
  3. Immunologic sequelae
  4. Intellectual disability (possibly)
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92
Q

What are the immunologic effects of ataxia telangiectasia?

A

Decreased levels of immunoglobulin and T-cell dysfunction

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93
Q

What are resulting symptoms from the immunologic effects of ataxia telangiectasia?

A

Frequent upper and lower respiratory tract infections

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94
Q

What happens once the ocular telangiectasia becomes advanced in ataxia telangiectasia?

A
  • It can simulate conjunctivitis

- Patients are unable to voluntarily make rapid eye movements

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95
Q

What 2 types of malignancy have a high incidence in ataxia telangiectasia?

A
  1. Hodgkin lymphoma

2. Leukemia

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96
Q

What is the mneumonic to remember possible causes of ataxia?

A

TIN walk

  • Toxic ingestion (ethanol or pesticides)
  • Infection (Guillain Barre)
  • Neoplasm (Glioma, Medulloblastoma)

*Other causes include cerebral hemorrhage and various metabolic disorders

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97
Q

What 3 types of medicines can cause ataxia?

A
  1. Anticonvulsants
  2. Alcohol
  3. Thallium
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98
Q

What 5 meds can cause tremors?

A
  1. Amphetamines
  2. Valproic acid
  3. Phenothiazines
  4. Tricyclic antidepressants
  5. Methylxanthines (caffeine and theophylline)
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99
Q

Pigmentation in the eye, decreased immunity, ataxia?

A

Ataxia telangiectasia

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100
Q

What happens to visual acuity and pupillary reflexes in ataxia telangiectasia?

A

They are often normal (despite other eye findings)

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101
Q

How is Friedreich ataxia inherited?

A

Autosomal recessive

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102
Q

What disorder accounts for about 1/2 of cases of inherited ataxia?

A

Friedreich Ataxia

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103
Q

When does Friedreich Ataxia typically present?

A

Late childhood or early adolescence

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104
Q

How does Friedreich Ataxia typically present in children?

A

Slow and clumsy gait

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105
Q

What two things cause the ataxia in Friedreich Ataxia?

A
  1. Cerebellar component

2. Loss of proprioception

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106
Q

What problems might you see in Friedreich Ataxia that point to spinal cord or peripheral nerve problems?

A
  1. Decreased strength in the feet

2. Decreased reflexes in the lower extremities

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107
Q

What are 3 clinical features that distinguish Friedreich ataxia from other forms of ataxia?

A
  1. Elevated plantar arch
  2. Absence of lower extremity deep tendon reflexes
  3. Cardiomyopathy leading to CHF
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108
Q

Which presents first, Friedreich ataxia or ataxia telangiectasia?

A

Ataxia telangiectasia

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109
Q

What is the treatment for Friedreich ataxia?

A

Supportive

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110
Q

What are relatively rapid random repetitive purposeless movements?

A

Chorea

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111
Q

What are the 2 main types of chorea?

A
  1. Sydenham

2. Huntington

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112
Q

What part of the brain is most often affected in movement disorders?

A

Basal Ganglia

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113
Q

What type of chorea is one of the major Jones criteria of rheumatic fever?

A

Syndenham

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114
Q

True or False: Normal ASO antibody titers rules out Sydenham chorea?

A

False

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115
Q

How is Huntington disease inherited?

A

Autosomal dominant

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116
Q

What is the triad for Huntington disease?

A
  1. Chorea
  2. Hypotonia
  3. Emotional lability
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117
Q

When do symptoms of Huntington disease usually present?

A

Adulthood (after 35)

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118
Q

What % of patients can have juvenile Huntington chorea?

A

10%

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119
Q

How does juvenile Huntington chorea typically present?

A

Rigidity

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120
Q

What category of drugs are used as treatment for chorea?

A

Dopamine-blocking agents in the antipsychotic category

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121
Q

Which specific drug is effective for chorea?

A

Haloperidol

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122
Q

Which type of chorea is essentially self-limited?

A

Sydenham (but low does haloperidol can be used)

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123
Q

Besides haloperidol, what 3 medications could be considered for chorea?

A
  1. Fluphenazine
  2. Risperidone
  3. Tetrabenazine
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124
Q

2 year old, fixed upward gaze, alert and appropriately frightened, airway patent, vital signs stable, toxic ingestion is a possibility… Which pharmacological treatment until ingestion can be confirmed?

A

Diphenhydramine- The upward gaze is a dystonic reaction (probably due to ingestion of promethazine or a related drug)

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125
Q

What types of movements characterize a dystonic reaction?

A
  1. Neck hyperextension

2. Decreased extraocular moments

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126
Q

What is one type of medication that can especially cause an acute dystonic reaction?

A

Neuroleptics

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127
Q

What can reverse an acute dystonic reaction?

A

Diphenhydramine

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128
Q

What is one specific medication that is not always thought of as a neuroleptic, but is one and can cause an acute dystonic reaction?

A

Metoclopramide

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129
Q

10 year old with ADHD, on methylphenidate for past 3 years, briefly experienced eye blinking which since resolved with no intervention… Most likely explanation and appropriate further management?

A

Simple motor tic, no additional intervention

*Don’t think tic disorder like Tourettes… tic was limited and resolved spontaneously even though he continued methylphenidate

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130
Q

What movements follow a pattern, are repetitive improve or disappear during purposeful movement, and may be suppressed (at least for short periods of time)?

A

Tics

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131
Q

What movements are random, increase during purposeful movement, and cannot be voluntarily suppressed?

A

Choreiform movements

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132
Q

What are rocking and hand flapping examples of?

A

Stereotypies

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133
Q

What 3 groups can you see stereotypies in?

A
  1. Normal children
  2. Autism
  3. Rett Syndrome
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134
Q

True or False: Stimulant medications cause tics?

A

False- they may unmask an underlying tic disorder in predisposed patients

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135
Q

True or False: If a child develops tics, stimulant medications for ADHD have to be discontinued?

A

False- but parents need to know that stimulants may unmask an underlying tic disorder

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136
Q

How do simple motor tics typically present?

A
  1. Eye blinking

2. Movements of the head, face, and/or shoulders

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137
Q

True or False: Simple motor tics are common and transient in many children without underlying conditions?

A

True

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138
Q

How long must tics be present to consider Tourette Syndrome?

A

At least one year

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139
Q

What 2 other problems does Tourette syndrome have a co-occurrence between?

A
  1. ADHD

2. OCD

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140
Q

What is the best treatment for Tourette syndrome?

A

Habit reversal training

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141
Q

What does habit reversal training consist of?

A

Recognizing when tics are about to occur and starting a voluntary movement that will not allow the tic to occur

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142
Q

When is pharmacotherapy indicated for Tourettes?

A

Only when tics interfere with daily activities

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143
Q

Where are brain tumors in young children often located?

A

Posterior fossa

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144
Q

What is the triad that should make you think of an infratentorial tumor?

A
  1. Headache
  2. Vomiting
  3. Abnormal gait
    * in an afebrile child
    * also note head tilt and torticollis
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145
Q

What is the most common type of solid tumor in children?

A

Brain tumors

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146
Q

What is the number one cause of death among all childhood cancers?

A

Brain tumors

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147
Q

What is the most common malignant brain tumor of childhood across all ages?

A

Medulloblastoma

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148
Q

Where does medulloblastoma arise from?

A

Tumor stem cells in the cerebellum

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149
Q

What is the triad for presentation of a medulloblastoma?

A
  1. Headache
  2. Ataxia
  3. Obstructive hydrocephalus
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150
Q

What are 2 things you might see on a contrast MRI of a medulloblastoma?

A
  1. Contrast-enhancing 4th ventricle mass

2. Hydrocephalus

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151
Q

Child under 1 who is vomiting, no diarrhea, dehydrated on exam (dry oral mucosa and decreased urine output), full anterior fontanelle. Gets 2 NS boluses and then postures (fully extends arms and legs)…best next step?>

A

IV dexamethasone (reduce ICP)

*This is a space occupying lesion, likely brain tumor

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152
Q

True or False: Craniopharyngioma usually presents rapidly

A

False- Over 1-2 years

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153
Q

What is the triad for presentation of a craniopharyngioma?

A
  1. Endocrinopathoes (short stature, diabetes insipidus, early or delayed puberty)
  2. Visual disturbances
  3. Headache
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154
Q

What leads to the chronic progressive visual field deficits seen with craniopharyngioma?

A

Pressure on the optic tracts

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155
Q

What can cause hydrocephalus in craniopharyngioma?

A

Obstruction of the 3rd ventricle

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156
Q

What might you see on XR imaging that would make you think of a craniopharyngioma?

A

Skull film with calcification in the sella turcica

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157
Q

True or False: Craniopharyngioma is not a malignant tumor?

A

True- just in a bad location

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158
Q

What are the 2 main treatment options for craniopharyngioma?

A
  1. Surgery

2. Radiation (possibly)

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159
Q

What type of doctor do kids with craniopharyngiomas need to follow-up with?

A

Endocrine (most patients do well)

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160
Q

What can optic nerve gliomas lead to?

A

Decreased vision

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161
Q

What are optic nerve gliomas associated with 25% of the time?

A

Neurofibromatosis

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162
Q

How does a brain lesion effect MSK system?

A

Contralateral face and body weakness

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163
Q

Name 2 examples of a brain lesion

A
  1. Tumor

2. Abscess

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164
Q

How does a brainstem lesion effect MSK system?

A
  1. Weak eye movements
  2. Ipsilateral facial weakness
  3. Contralateral body weakness
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165
Q

What is one example of a brainstem lesion?

A

Tumor

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166
Q

How does a spinal cord lesion effect MSK system?

A
  1. Loss of motor and sensation function
  2. Loss of bladder and bowel function
  3. Increased reflexes
  4. No eye involvement
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167
Q

Name 6 examples of things that cause a spinal cord lesion

A
  1. Transverse Myelitis
  2. Anterior spinal artery infarction
  3. Spinal cord compression (tumors)
  4. Epidural abscess
  5. Tethered cord
  6. Trauma
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168
Q

How does a chronic peripheral nerve process effect MSK system?

A

Loss of DTRs

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169
Q

Name 3 examples of chronic peripheral nerve processes

A
  1. Chronic demyelinating polyneuropathy
  2. Hereditary neuropathies
  3. Leukodystrophy
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170
Q

How does an acute peripheral nerve process effect MSK system?

A

Loss of DTRs

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171
Q

Name 6 examples of acute peripheral nerve processes

A
  1. Guillain Barre
  2. Polio
  3. Diptheria
  4. Tick paralysis
  5. Lead poisoning
  6. Bell’s Palsy
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172
Q

Name an example of a neuromuscular junction disorder that has a progressive onset

A

Myasthenia gravis

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173
Q

Name an example of a neuromuscular junction disorder that has a rapid onset

A

Botulism

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174
Q

What is the direction of the paralysis in botulism?

A

Descending

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175
Q

How do muscular disorders causing chronic weakness present in the MSK system?

A

Proximal weakness

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176
Q

Name 3 examples of muscular disorders causing chronic weakness

A
  1. Muscular dystrophy
  2. Mitochondrial myopathies
  3. Congenital myopathies
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177
Q

How do muscular disorders causing acute weakness present in the MSK system?

A

Proximal weakness affecting the hips and shoulders

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178
Q

Name 3 examples of muscular disorders causing acute weakness

A
  1. Polymyositis
  2. Dermatomyositis
  3. Electrolyte imbalance
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179
Q

Name 3 general categories of CNS causes of hypotonia

A
  1. Hypotonic cerebral palsy
  2. Genetic disorders (Prader-Willi, Angelman, Down)
  3. Metabolic problems (leukodystrophies, peroxisomal disorders)
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180
Q

What test do you order if you have a patient presenting with symptoms consistent with a spinal cord lesion?

A

MRI spine

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181
Q

Name some signs and symptoms of an epidural abscess.

A
  1. Spinal pain
  2. Paresthesias
  3. Weakness and/or paralysis
    4, Fevers
  4. Localized back pain (initial symptom)
  5. Decreased anal tone
  6. Reduced sensation in lower extremities
  7. Increased reflexes
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182
Q

How can infection occur in an epidural abscess?

A
  1. Direct extension from paraspinal tissues

2. Seeding from distant infection (UTI, dental abscess, central venous catheter)

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183
Q

Which population are particularly susceptible to epidural abscess?

A

IV drug users

184
Q

What needs to be done if there is concern for an epidural abscess?

A

Urgent spinal imaging… if MRI isn’t available, get a CT

185
Q

What are the 2 parts of treatment for an epidural abscess?

A
  1. Antibiotics

2. Emergency surgical decompression

186
Q

What bug should your antibiotics target for a epidural abscess?

A

Staph

187
Q

What is another name of acute transverse myelitis?

A

Post-infectious myelitis

188
Q

What causes acute transverse myelitis?

A

Lymphocytic infiltration and demyelination of nerves/spinal cord secondary to inflammation

189
Q

How does acute transverse myelitis present?

A

Fever and sudden onset of paralysis

190
Q

What is seen on CSF studies in acute transverse myelitis?

A

Increased polys

Negative gram stain

191
Q

What should pain on palpation of the spinal cord make you think of?

A

Acute transverse myelitis

192
Q

What does the MRI show in acute transverse myelitis?

A

Cord swelling

193
Q

What is the main problem you worry about in acute transverse myelitis?

A

Respiratory arrest or compromise

194
Q

What has to be done in acute transverse myelitis before the LP?

A

MRI with gadolinium

195
Q

Child with abrupt onset of weakness, hypotonia, decreased reflexes followed by increased tone and hyperreflexia. Also with bowel/bladder dysfunction…?

A

Acute transverse myelitis

196
Q

What is the prognosis for acute transverse myelitis?

A

Most have spontaneous recovery over a few months

197
Q

Child with an extrinsic spinal cord mass lesion what do you do?

A

Immediate treatment with high dose IV dexamethasone (reduce pain, edema, and risk for ischemia)

198
Q

What type of disease is Werdnig-Hoffman?

A

Spinal muscle atrophy (SMA type 1)

199
Q

What causes Werdnig-Hoffman disease?

A

Degeneration of the anterior horn cells

200
Q

What is typically affected in Werdnig-Hoffman disease- Motor, sensory, or both?

A

Motor- Anterior horn cells are what’s degenerated

201
Q

How is Werdnig-Hoffman disease inherited?

A

Autosomal recessive

202
Q

How does Werdnig-Hoffman disease usually present?

A

In infancy with severe hypotonia and weakness

203
Q

How is Werdnig-Hoffman disease diagnosed?

A

Muscle biopsy

204
Q

What is the prognosis for Werdnig-Hoffman disease?

A

Most kids die by age 2, there is no treatment

205
Q

What are 3 key phrases to look for in Werdnig-Hoffman disease?

A
  1. Hypotonia
  2. Poor suck
  3. Tongue fasciculations
206
Q

What distinguishes Werdnig-Hoffman disease from infantile botulism?

A

Tongue fasciculations

207
Q

What is a protrusion of the spinal cord and surrounding membranes through a defect in the vertebral column?

A

Myelomeningocele

208
Q

What do children with myelomeningocele often have as well that requires then to often require a VP shunt?

A

Hydrocephalus

209
Q

If you suspect myelomeningocele in the newborn period, who do you need to consult early?

A
  1. Orthopedics

2. Urology

210
Q

What age group do children with myelomeningocele typically do well through?

A

Preschool years

211
Q

After the preschool years what problems will children with myelomeninocele commonly have with learning?

A
  1. Difficulty with organization
  2. Difficulty with memory
  3. Other leaning skills
  4. Low-average IQ
212
Q

Patient with headache, nausea/vomiting, gaze disturbance, academic or behavior changes, or new onset seizures (or change in occurrence or type of seizure)?

A

Consider shunt malfunction (especially in kids with spina bifida and shunt), and/or worsening hydrocephalus

213
Q

What are some of the more subtle findings suggestive of a spinal dysraphism?

A
  1. Lipoma
  2. Patch of hair
  3. Hemangioma
  4. Discoloration
  5. Sacral dimple
  6. Leg length discrepancy
  7. Elevated arches
  8. Gait abnormalities
214
Q

What are some of the more alarming signs associated with an occult spinal dysraphism?

A
  1. Diminished sensation in the perineal area
  2. Urinary and/or stool incontinence
  3. Recurrent UTIs
215
Q

What should be ordered if spinal dysraphism is suspected?

A

MRI of the spine

216
Q

How is spinal trauma usually described?

A

Focal pain along the spine following trauma. May see weakness and/or sensory deficits. Can have loss of bowel and bladder function as well.

217
Q

If you are suspicious for spinal trauma, what 2 things should you do?

A
  1. Immobilizing the cervical and lower spine

2. Give 30mg/kg methylprednisolone over an hour

218
Q

What steroid is indicated for spinal cord compression?

A

Dexamethasone (not methylprednisolone)

219
Q

What steroid is indicated for spinal trauma?

A

Methylprednisolone (not dexamethasone)

220
Q

In the setting of spinal trauma, when would you give mannitol?

A

Only if there are signs of increased intracranial pressure

221
Q

What is an acquired idiopathic facial nerve paralysis?

A

Bell’s palsy

222
Q

How do kids with Bell’s palsy present?

A

Sudden onset of unilateral facial paralysis

223
Q

How is diagnosis of Bell’s palsy made?

A

Clinically

224
Q

What are the 2 treatment options for Bell’s Palsy?

A
  1. Prednisone for 5 days (best if started within first 3 days of symptoms)
  2. Antivirals like acyclovir and valacyclovir (for severe cases)
225
Q

What is facial paralysis associated with painful vesicles in the ear canal

A

Ramsay Hunt Facial Paralysis

226
Q

What causes Ramsay Hunt facial paralysis?

A

Reactivation of the VZV virus

227
Q

What causes progressive motor weakness as well as areflexia?

A

Guillain Barre Syndrome

228
Q

How does Guillain Barre Syndrome present?

A

Leg weakness and/or unsteady gait, pain

229
Q

How does the weakness progress in Guillain Barre Syndrome?

A

Starts in the lower extremities and progresses upward in a symmetric fashion (ascending paralysis)

230
Q

What is often noted in preceding history for Guillain Barre syndrome?

A

Preceded by a viral illness or another illness like campylobacter jejuni

231
Q

What is typical of the physicial exam in Guillain Barre?

A

Cranial nerve findings and dysautonomia (tachycardia, orthostatic hypotension, and/or dizziness)

232
Q

What finding is ALWAYS seen in Guillain Barre syndrome?

A

Areflexia- If reflexes are present in lower extremities, it’s not GBS

233
Q

What are classic findings in the CSF with Guillain Barre?

A

Increased protein with a normal cell count

234
Q

A child with difficulty rising from a sitting position or one who can’t shrug their shoulders or one who is suddenly clumsy and falling…?

A

Proximal muscle weakness- Guillain Barre

235
Q

What is the biggest risk in Guillain Barre syndrome?

A

Respiratory failure secondary to ascending paralysis

236
Q

What has to be followed very closely in Guillain Barre syndrome?

A

Lung function

237
Q

What testing needs to be monitored very closely in Guillain Barre syndrome?

A

Lung functions: Vital capacity, negative inspiratory force, or PFTs

NEVER O2 Sats

238
Q

True or false: O2 sats are adequate monitoring for lung function in patients with Guillain Barre syndrome?

A

False: These only decline once the patient is severely compromised

239
Q

What are the 3 parts to treatment for Guillain Barre Syndrome?

A
  1. Supportive measures (intubation, ect.)
  2. Plasmapheresis
  3. IVIG
240
Q

What is the prognosis for Guillain Barre Syndrome?

A

Most patient who survive have a full recovery, but there is permanent disability in about 10% of cases

241
Q

When do you give steroids in Guillain Barre Syndrome?

A

Never- they have no proven benefit in treatment

242
Q

What is something else you should consider if you suspect Guillain Barre syndrome (especially if presenting in the summer months)?

A

Tick paralysis- Caused by a neurotoxin secreted by the tick

243
Q

How does tick paralysis present?

A

Exactly like GBS (with ascending paralysis)

244
Q

How does the paralysis in botulism progress?

A

Descending paralysis (v. GBS and tick which is ascending)

245
Q

What is an autoimmune disorder in which the patient develops antibodies against the acetylcholine receptor in the neuromuscular junction?

A

Myasthenia gravis

246
Q

What causes myasthenia gravis?

A

Unknown

In some cases penicillamine is known to cause it

247
Q

What can exacerbate symptoms in myasthenia gravis?

A

Drugs and illnesses

248
Q

Describe the weakness in myasthenia gravis

A

Worse with activity and improves with rest

249
Q

What do patients with myasthenia gravis typically describe their day as?

A

Feel good in the morning and get tired as the day goes on

250
Q

What is a common exam finding in myasthenia gravis that often causes patients to first present?

A

Ptosis

251
Q

If you diagnose someone with myasthenia gravis, what else should you look for?

A

Thymoma

252
Q

What test can be done to confirm a diagnosis of myasthenia gravis?

A

Tensilon test

253
Q

How does the tensilon test work in myasthenia gravis?

A

Uses edrophonium which is a very short acting acetylcholinesterase inhibitor

254
Q

True or False: Edrophonium can be used as treatment for myasthenia gravis?

A

False- It is short acting and only used to testing

255
Q

What are 3 other things besides the Tensilon test that can support a diagnosis of Myasthenia gravis?

A
  1. Test for anti-acetylcholine receptor antibody
  2. Test for anti-Smith antibodies
  3. EMG
256
Q

What is the mainstay of therapy for myasthenia gravis?

A

Pyridostigmine (Mestinon)

257
Q

How does pyridostigmine work?

A

Inhibits acetylcholinesterase- this increases acetylcholine in the NMJ

258
Q

Besides pyridostigmine what are 2 other treatment options that can be used?

A
  1. Plasmapheresis

2. Plasma exchange

259
Q

True or False: Prednisone and immunomodulators are commonly used to treat myasthenia gravis?

A

False- These are less commonly used

260
Q

What surgical intervention is often some in myasthenia gravis?

A

Thymectomy (especially in kids)

261
Q

True or False: A thymectomy can be curative for myasthenia gravis?

A

True

262
Q

How does the botulism toxin act on the NMJ?

A

It blocks the release of acetylcholine from the NMJ

263
Q

Describe the direction of paralysis in botulism v. Guillain Barre syndrome

A

Botulism: Descending
GBS: Ascending

264
Q

What type of botulism looks similar to myasthenia gravis?

A

Infantile botulism

265
Q

How can you differentiate myasthenia gravis from infantile botulism?

A

Botulism in rapid onset within hours

Myasthenia gravis is more progressive (over weeks)

266
Q

How is Duchenne muscular dystrophy inherited?

A

X-linked recessive (only boys get it)

267
Q

What is the cause of Duchenne muscular dystrophy?

A

Absence of dystrophin

268
Q

How is Duchenne muscular dytrophy diagnosed?

A

Muscle biopsy

269
Q

True or False: Children with Duchenne muscular dystrophy are typically symptomatic from birth?

A

False- usually achieve baby milestones at correct times

270
Q

What might be the first sign of Duchenne muscular dystrophy observed in infants?

A

Poor head control

271
Q

How might a toddler with Duchenne muscular dystrophy present?

A

Weakness in hip muscles, lordoctic posture (to compensate for gluteal weakness), can’t walk up stairs (will crawl or cry to be carried)

272
Q

What is Gower’s sign?

A

When a child walks his hands up his legs for support in order to stand up- Associated with proximal muscle weakness

273
Q

What might you mind on your lower extremity MSK exam for a child with Duchenne muscular dystrophy?

A

Hypertrophied calves (pseudohypertrophy)

274
Q

What causes pseudohypertrophy seen in Duchenne muscular dystrophy?

A

Fat deposition and proliferation of collagen

275
Q

What are the effects of Duchenne muscular dystrophy on intellect?

A

Most have some intellectual disability, but it is mild in most

276
Q

What lab study can help to point towards Duchenne muscular dystrophy?

A

CPK elevation (this is elevated even at birth and before weakness appears)

277
Q

What is the appropriate gene testing for Duchenne?

A

Xp21 gene testing

Remember Duchenne (duXchenne) is carried on the X chromosome (x-linked recessive) to pick this choice

278
Q

How is Becker dystrophy compared to Duchenne dystrophy?

A

Becker has later onset, milder severity, and slower deterioration

279
Q

What is a finding in history that might point to Becker dystrophy?

A

Severe cramps on exertion

280
Q

What finding might be more severe in Becker dystrophy compared to Duchenne?

A

Cardiac involvement

281
Q

What are two exam findings that distinguish Duchenne muscular dystrophy from other neuromuscular disorders?

A
  1. Absence of tongue fasciculations

2. Absence of eye muscle involvement

282
Q

True or False: In Duchenne muscular dystrophy, the mother is always a carrier?

A

False: In 1/3 of cases, Mom is not a carrier- there is a significant spontaneous mutation rate

283
Q

What lab finding might be abnormal in an asymptomatic female carrier of Duchenne muscular dystrophy?

A

CPK (it can be elevated)

284
Q

What are 3 common causes of death in patients with Duchenne muscular dystrophy?

A
  1. Heart failure
  2. Respiratory failure
  3. Pneumonia
285
Q

What causes the heart failure seen in patients with Duchenne muscular dystrophy and at what point in their lives does this typically become a problem?

A 
Cardiomyopathy
3rd decade (when they are in their 20s)
286
Q

What types of muscle does myotonic muscular dystrophy affect?

A

Striated and smooth

287
Q

What systems does myotonic muscular dystrophy affect?

A

Skeletal, heart, GI tract (multiple besides skeletal muscle due to affect on striated and smooth muscle)

288
Q

What age does myotonic muscular dystrophy typically present?

A

Around 4-5

289
Q

What is typically seen initially in patients with myotonic muscular dystrophy?

A

Muscles have slow relaxation after contraction (especially in the hands)

290
Q

What is myotonia?

A

Slow muscle relaxation after contraction (typically seen in hand)

291
Q

What other organ system besides MSK are there commonly problems in with myotonic muscular dystrophy?

A

Endocrine

292
Q

Where is the muscle wasting in myotonic muscular dystrophy?

A

Distal (most other neuromuscular disorders are proximal)

293
Q

What happens to the CPK in myotonic muscular dystrophy?

A

It can be normal (always elevated in Duchenne)

294
Q

How is myotonic muscular dystrophy inherited?

A

Autosomal dominant

295
Q

How is myotonic muscular dystrophy diagnosed?

A

Muscle biopsy

296
Q

What is another name for Steinert disease?

A

Myotonic muscular dystrophy

297
Q

What are three parts to note for in history of a pseudoseizure?

A
  1. Happen during day
  2. Occur with other people around
  3. Absence of post-ictal state
298
Q

How will tics be differentiated from seizures?

A

The patient can suppress movements with tics, not with seizures

299
Q

How can you differentiate seizure from syncope?

A

Seizures occur suddenly with maybe an aura before. Syncope is preceded by dizziness, loss of color, or blurry vision

300
Q

What is an abnormal, sudden electrical discharge of nerve cells from the gray matter, which progresses to the white matter having a measurable effect on the innervated organ?

A

Seizure

301
Q

What is the name for something that looks like a seizure, but isn’t associated with concurrent EEG changes?

A

Paroxysmal non-epileptic event

302
Q

When do neonatal seizures occur?

A

Within the first month of life

303
Q

What are some causes of neonatal seizures?

A
  1. Birth defects
  2. Perinatal hypoxia
  3. Drug effects
304
Q

What type of seizures occur during a rapid rise in body temperature, usually in response to a common infection like otitis or other URI?

A

Febrile seizure

305
Q

What % of kids will have a febrile seizure?

A

2-5% (very common)

306
Q

What type of seizures are focal and only involve part of the brain?

A

Partial seizures

307
Q

What are the 2 main types of partial seizures?

A
  1. Simple partial

2. Complex partial

308
Q

What type of seizure has a localized motor component limited to one part of the body?

A

Simple partial

309
Q

True or False: A simple partial seizure can progress to other parts of the body?

A

True

310
Q

True or False: Children are unresponsive during a simple partial seizure?

A

False- they remain awake (Jacksonian seizure)

311
Q

What describes a complex partial seizure?

A

Involve motor activity, but the child in unaware of what he or she is doing

312
Q

What is the name for a non-focal seizure?

A

Generalized seizure

313
Q

What are 3 main categories of generalized seizures?

A
  1. Absence
  2. Infantile spasm
  3. Conculsive/Tonic/Tonic-Clonic
314
Q

What type of seizures are short episodes in which the child stares into space with no awareness of their environment?

A

Absence (petit-mal)

315
Q

What age group do infantile spasms usually affect?

A

Children under a year

316
Q

What is the prognosis for infantile spasms?

A

Very poor, usually have intellectual disability

317
Q

What type of seizures usually last around 5 minutes and have a post-ictal period?

A

Conluvsive, Tonic, Tonic-Clonic

318
Q

What is defined as seizure activity of more than 30 minutes in duration, or repeated seizures without a return to normal in between?

A

Status epilepticus (medical emergency)

319
Q

Name 5 common causes of metabolic-related seizures, especially in the newborn period

A
  1. Pyridoxine deficiency
  2. Hypoglycemia
  3. Hyponatremia
  4. Hypocalcemiea
  5. Urea cycle disorders
320
Q

Kid with seizure despite being on anti-convulsants.. what do you do first?

A

Obtain drug levels

321
Q

What are 3 important reasons to get drug levels in a kid with breakthrough seizures?

A
  1. Adolescents with poor medication compliance
  2. Weight gain causing kid to outgrow the dose
  3. Problem with absorption
322
Q

What is the most common type of seizure seen in children?

A

Febrile

323
Q

What type of seizures occur in kids after 1 month of age, are associated with febrile illness but no caused by CNS infection, and are unassociated with previous neonatal or other seizures?

A

Febrile

324
Q

What age range do febrile seizures typically occur?

A

6 months to 5 years

325
Q

What % of kids with febrile seizures have a family history of febrile seizures?

A

25%

326
Q

What is the most common type of febrile seizure?

A

Simple

327
Q

What are the 3 features that describe a simple febrile seizure?

A
  1. Generalized seizure type
  2. Duration less than 15 minutes
  3. Only one seizure per 24 hour period
328
Q

True or False: A normal EEG rules out epilepsy?

A

False- Normal EEG doesn’t rule out epilepsy and an abnormal EEG doesn’t confirm a diagnosis of seizure disorder

329
Q

True or False: There are kids with abnormal EEGs who don’t have seizures?

A

True

330
Q

True or False: Medications aren’t indicated after a first unprovoked seizure?

A

True

331
Q

When do you get routine labs for a first unprovoked seizure?

A

You don’t- just need a finger stick glucose

332
Q

What should be done for all parents with a child who has had a first unprovoked seizure?

A

Educate them on safety- Helmet while biking, no bathing without supervision, ect.

333
Q

True or False: A child can swallow their tongue during a seizure

A

FALSE- don’t put anything in their mouth

334
Q

True or False: Seizures cause brain damage

A

False- Seizures don’t cause brain damage unless they last longer than 20-30 minutes

335
Q

True or False: You should obtain an EEG after a first unprovoked seizure?

A

False- EEG is not indicated after a first unprovoked seizure

336
Q

Which patients with febrile seizure are at increased risk for developing epilepsy?

A

Patients experiencing a complex febrile seizure first (as opposed to a simple first febrile seizure)

337
Q

What are the 3 characteristics of a complex febrile seizure?

A
  1. Lasts longer than 15 minutes
  2. Recurs within 24 hours
  3. May be focal
338
Q

How many kids with febrile seizure will go on to have a second febrile seizure?

A

About 1/3

339
Q

What are 4 risk factors for recurrence of febrile seizure?

A
  1. Low fever at time of first seizure
  2. Young age
  3. Family history of febrile seizures
  4. Short period of time between onset of fever and seizure
340
Q

What % of kids with febrile seizures go on to develop epilepsy?

A

5%

341
Q

What are 3 risk factors for the development of epilepsy in kids with febrile seizures?

A
  1. Family history of epilepsy
  2. Children with complex febrile seizures
  3. Children with neurodevelopmental abnormalities
342
Q

What work up is indicated in simple febrile seizures?

A

Non- don’t do blood tests, EEG, or head CT

*With a complex febrile seizure doing a workup is more subjective

343
Q

Which 4 groups should you consider a LP in with febrile seizure?

A
  1. Infants
  2. Kids with complex partial seizures
  3. Kids with febrile status epilepticus
  4. Kids partially treated with antibiotics
344
Q

Name 6 medications known to cause seizures

A
  1. Cyclosporin
  2. Intrathecal methotrexate
  3. Isoniazid
  4. Insulin/Oral hypoglycemic
  5. Buproprion (and other psych meds)
  6. Theophylline
345
Q

What illicit drug is known to cause seizures?

A

Cocaine

346
Q

What seizures consist of brief staring spells where the child is not responsive?

A

Absence seizures

347
Q

True or False: There is no postictal period in absence seizures?

A

True

348
Q

Child who frequently spaces out and blinks, with no recollection of the event?

A

Absence seizure

349
Q

What is the EEG description for absence seizure?

A

3 per second spike and wave

350
Q

Why are absence seizures called “potato chips” seizures?

A

Can’t stop with one (they come in clusters)

351
Q

What other type of seizure can absence seizures be difficult to distinguish between?

A

Complex partial seizures

352
Q

What are 3 ways to distinguish absence seizure from complex partial seizure?

A
  1. Absence seizure is abrupt in onset (CPS may start as a simple partial seizure where child is aware)
  2. Absence seizures are usually short (under 30 seconds)
  3. Absence seizures end abruptly (CPS end gradually progressing into the postictal period)
353
Q

What can induce an absence seizure that is a diagnostic maneuver?

A

Hyperventilating

354
Q

What are 3 meds used to treat absence seizures?

A
  1. Ethosuximide (specific for this)
  2. Lamotrigine
  3. Valproic acid
355
Q

Which children are more likely to respond to therapy for absence seizures and remain seizure free even after therapy is discontinued?

A

Younger children

356
Q

What side effects can valproic acid have?

A
  1. Depressed platelet counts
  2. Elevated LFTs
  3. Elevated pancreatic enzyme levels
357
Q

What tests need to be monitored in kids on valproic acid?

A
  1. CBC
  2. LFT
  3. Pancreatic function tests
358
Q

What types of seizures can patients have an aura or prodrome before (can be minutes, hours, or even days before seizure)?

A

Tonic-clonic or Generalized Tonic-Clonic

359
Q

What are 3 key components of a tonic-clonic seizure?

A
  1. Loss of consciousness
  2. Loss of bladder control
  3. Full body tonic clonic movements
360
Q

What are the 3 phases of a generalized tonic-clonic seizure?

A
  1. Tonic
  2. Clonic
  3. Post-ictal
361
Q

Describe the tonic phase of a generalized tonic-clonic seizure

A

Flexion of trunk, extension of back/arms/legs- Typically lasts less than 30 seconds

362
Q

True or False: Loss of bladder control occurs during the tonic phase of a generalized tonic-clonic seizure?

A

False

363
Q

Describe the clonic phase of a generalized tonic-clonic seizure

A

Clonic convulsive movements along with tremors that alternate with short periods of atonia (where child may boid)

364
Q

Which phase of a generalized tonic-clonic seizure is the child apneic?

A

Clonic

365
Q

How long does the clonic phase of a generalized tonic-clonic seizure last?

A

1-2 minutes

366
Q

Describe the post-ictal phase of a generalized tonic-clonic seizure

A

Initially unconscious then sleepy/confused. Gradually awaken and become more oriented. Unable to recall events of seizure. (Similar to someone coming out of anesthesia)

367
Q

What is the treatment of generalized tonic clonic seizures?

A

Anti-epileptic drugs

368
Q

What is the drug of choice for treatment of generalized tonic clonic seizures?

A

Valproic acid

369
Q

Besides valproic acid what are other anti-epileptics used in generalized tonic clonic seizures?

A
  1. Phenytoin
  2. Carbamazepine
  3. Lamotrigine
  4. Phenobarbital (falling out of favor)
  5. Phenytoin (used less frequently due to side effects)
370
Q

What is a potential side effect of lamotrigine?

A

SJS

371
Q

What type of epilepsy is Juvenile Myoclonic Epilepsy (JME)

A

Generalized idiopathic epilepsy

372
Q

What are the 3 features in classic presentation of JME?

A
  1. Myoclonic jerks upon awakening
  2. Generalized tonic-clonic seizures in most patients
  3. Absence seizures in 1/3 of patients
373
Q

True or False: EEGs show generalized slowing in JME?

A

False- Most EEGs don’t pick up the seizures in JME (so if you have EEG findings, this isn’t JME)

374
Q

What are the 2 drugs used to treat JME?

A
  1. Valproic acid

2. Levetiracetam

375
Q

What is a single or repetitive contraction of an isolated group of muscles?

A

Myoclonic seizures

376
Q

What 2 conditions are myoclonic seizures typically seen in?

A
  1. JME
  2. Lennox-Gastaut

(Outside of these two these types of seizures are relatively rare)

377
Q

What is another name for West Syndrome?

A

Infantile spasms

378
Q

What is the triad for West Syndrome?

A
  1. Infantile spasms
  2. Hypsarrhythmia (on interictal EEG)
  3. Developmental delay
379
Q

What % of epilepsy is infantile spasms?

A

2%

380
Q

What % of epilepsy seen in the first year of life is infantile spasms?

A

25%

381
Q

When do most kids typically present with infantile spasms?

A

Between 3 and 9 months

382
Q

Describe the spasms seen in infantile spasm?

A

Happen in clusters- 20-30 in a row. Sudden flexion or extension of the body (like a Moro in a kid too old to have a Moro)

383
Q

True or False: Almost all patients with infantile spasms have developmental delay?

A

True

384
Q

Repetitive flexing of the head, trunk, and extremities. EEG shows hypsarrythmia…?

A

Infantile spasms

385
Q

What type of seizure disorder is Tuberous Sclerosis associated with?

A

Infantile spasms

386
Q

How is Tuberous Sclerosis inherited?

A

Autosomal dominant

387
Q

What presents with various tumors (cardiac/kidney), ash-leaf spots, and seizures?

A

Tuberous Sclerosis

388
Q

What is first line treatment for infantile spasms?

A

ACTH

389
Q

What is the prognosis in infantile spasms dependent upon?

A

Developmental status before onset of seizures…if there was developmental delay present prior to onset of seizures, then prognosis is poor

390
Q

Besides ACTH, what are some other treatment options in infantile spasms?

A
  1. Steroids
  2. Benzodiazepines
  3. Anti-epileptic drugs
  4. Can consider focal resection
391
Q

True or False: After treatment of infantile spasms, most kids develop other seizure types?

A

True

392
Q

What is a general description for partial seizure?

A

Focal seizure

393
Q

What does simple partial seizure mean?

A

Consciousness is maintained

394
Q

Describe a simple partial seizure

A
  1. Motor activity in the extremities (usually arms) or face
  2. Occur as patient falls asleep or wakes up
  3. Movement may “march” to other locations
  4. Can be sensory seizures or autonomic seizures
  5. Consciousness is maintained
395
Q

What is the drug of choice for simple partial seizures?

A

Carbamazepine

396
Q

True or False: Treatment is not always necessary for simple partial seizures

A

True- Many cases remit during adolescence

397
Q

What do complex partial seizures entail?

A

Alteration in consciousness

398
Q

What is typically described in a complex partial seizure?

A

Facial movements- can appear like they are responding to auditory or visual hallucinations, may have lip smacking

399
Q

True or False: Patients with complex partial seizure and unresponsive

A

False- Consciousness may not be impaired completely (patients may respond to simple commands)

400
Q

What is a movement or vocalization that accompanies complex partial seizures like moaning or simple talking?

A

Automatisms

401
Q

What type of seizures might you see automatisms in?

A

Complex partial

402
Q

What two things are part of the workup for a complex partial seizure?

A
  1. Head MRI

2. EEG

403
Q

What is the treatment for a complex partial seizure?

A

Anti-epileptic drugs (surgery is only rarely used)

404
Q

What is another name for benign childhood epilepsy with centrotemporal spikes?

A

Benign Rolandic Epilepsy

405
Q

What is the most common form of partial epilepsy in childhood?

A

Benign Rolandic Epilepsy

406
Q

What type of epilepsy has self-limited seizures involving the face

A

Benign Rolandic Epilepsy

407
Q

When do most seizures occur in Benign Rolandic Epilepsy?

A

At night (when sleeping)

408
Q

What other problem do patients with Benign Rolandic Epilepsy commonly have?

A

Migraine headaches

409
Q

Is there altered level of consciousness in Benign Rolandic Epilepsy if a seizure occurs while the child is awake?

A

No- Fully aware, but unable to speak

410
Q

In addition to the facial motor seizure seen in Benign Rolandic Epilepsy, what else is commonly seen?

A

Unilateral sensory involvement (usually one side of the tongue)

411
Q

When do you treat for Benign Rolandic Epilepsy?

A

After 3+ seizures

412
Q

What medication is given in Benign Rolandic Epilepsy?

A

Many medications are equally effective

413
Q

Does Benign Rolandic epilepsy have an inheritance patent?

A

Yes- dominant trait (look for family history)

414
Q

What is the prognosis for Benign Rolandic Epilepsy?

A

Most seizures usually resolve by 16

415
Q

What is the EEG pattern in Benign Rolandic Epilepsy?

A

Biphasic focal centrotemporal spikes and slow waves

416
Q

How can you differentiate between complex partial seizures (that involve weird facial movements) and Benign Rolandic seizures?

A

Complex partial seizures occur during the day (awake)
Benign Rolandic seizures occur at night (sleep)

*In reality, Rolandic can be awake or sleep- only 1/2-2/3 occur exclusively during sleep)

417
Q

Toddler with temper tantrum, turns blue, passes out, then awakens.. what do you do?

A

Reassure parents… Cyanotic breath holding spell (benign)

418
Q

True or False: You can see a post-ictal state in cyanotic breath holding spells?

A

FALSE

419
Q

What is status epilepticus?

A

Repeated or continued seizures for 30 minutes without recovery

420
Q

What is the first thing to do in status epilepticus?

A

ABCD’s

421
Q

What is the first drug to give in status epilepticus?

A

Ativan (short acting but rapid onset)

422
Q

If Ativan doesn’t work in status epilepticus, what do you give next?

A

IV fosphenytoin

423
Q

Why do we give IV fosphenytoin v. phenytoin?

A

Fosphenytoin is the inactive precursoe and less likely to cause skin necrosis (purple glove syndrome) seen with phenytoin

424
Q

If you do not have IV access, what can you give for a patient in status epilepticus?

A

Rectal diazepam

425
Q

True or False: Patients with active epilepsy can bathe and swim?

A

True- but they must be supervised by an adult

426
Q

What sports restrictions exist for children with active epilepsy?

A

No contact sports that can cause head injury

427
Q

What outdoor activity restrictions exist for children tih active epilepsy?

A

They can ride bikes with helmets on

Should not climb trees

428
Q

What driving restrictions exist for children with epilepsy?

A

Can drive unaccompanied (even if on anticonvulsant medications) as long as they have been seizure free for 3-12 months (this varies state to state, will likely be >12 months on boards)

429
Q

When can you attempt to discontinue anti-convulsant medication?

A

If the patient has been seizure free for 2 years

430
Q

What is a static encephalopathy characterized by gross and fine motor abnormalities evident early in life?

A

Cerebral Palsy

431
Q

What causes cerebral palsy?

A

Unknown in most cases, can be due to birth asphyxia in about 10% of known cases

*Consider clinical scenario and look for other etiologies that could have affected the developing brain

432
Q

What is the most common risk factor for cerebral palsy in premature newborns?

A

Perinatal infection

433
Q

True or False: All children with CP are intellectually disabled?

A

False- many have normal or above normal intelligence

434
Q

What type of CP will you have bilateral spasticity of the legs, delayed walking, and tip-toe walking?

A

Spastic diplegia

435
Q

Which type of CP has associated with normal cognitive function without seizures and is more likely to involve the lower extremities?

A

Spastic diplegia

436
Q

What type of CP will have spasticity of half of the body (left or right), involving the arm, trunk, and leg?

A

Spastic hemiplegia

437
Q

If what subtype of CP occurs before age 2 is it considered to be CP?

A

Spastic hemiplegia

438
Q

What subtype of CP is associated with 1/4 being cognitively impaired and may involve the upper extremity more than the lower?

A

Spastic hemiplegia

439
Q

What subtype of CP has increased tone in all 4 extremities with lower worse than the upper?

A

Spastic quadriplegia

440
Q

What subtype of CP is more likely to be associated with intellectual disability, seizures, feeding difficulties, speech difficulties, and visual difficulties?

A

Spastic quadriplegia

441
Q

What subtype of CP has increased tone in all 4 extremities with lower worse than upper in addition to dystonia and strange movements?

A

Dyskinetic

442
Q

What is another name for dyskinetic CP?

A

Athetoid CP

443
Q

What subtype of CP has a combination of spastic and dyskinetic movements?

A

Mixed CP

444
Q

What subtype of CP has hypotonia seen in the trunk and extremities with retention of primitive reflexes and/or hyperactive reflexes?

A

Hypotonic CP

445
Q

Afebrile child with a headache and neck pain who develops focal paralysis?

A

CVA

446
Q

Patient with CVA confirmed with CT… what is the next test to identify a specific diagnosis?

A

Cerebral angiography

447
Q

Child comes in with acute unilateral hemiparesis, did have blunt trauma to chest day before during football, no other history. What diagnostic study is most helpful in making the diagnosis?

A

Carotid angiography: CVA caused by blunt trauma to chest causing carotid dissection leading to an embolic stroke

448
Q

Clinical history consistent with stroke, elevated lactate level, sensorineural deafness…?

A

Mitochondrial disorder

449
Q

Clinical history consistent with stroke, elevated lactate level, sensorineural deafness… what is the best diagnostic step to establish the underlying diagnosis?

A

Molecular analysis of mitochondrial DNA (think of a mitochondrial disorder)

450
Q

What are most common causes of hemorrhagic stroke in children?

A

Vascular malformations (including AV and cavernous malformations)

451
Q

How do vascular malformations causing stroke typically initially present?

A
  1. Severe headache

2. Mental status change

452
Q

What is the acuity of an MRI of the head?

A

Non-urgent

453
Q

What is the acuity of a CT of the head?

A

Urgent

454
Q

What is the acuity of an US of the head?

A

Non-urgent

455
Q

Name 2 indications for MRI of the head

A
  1. Partial seizures

2. Herpes encephalitis (confirmed with HSV polymerase)

456
Q

Name 3 indications for CT of the head

A
  1. Hemorrhage
  2. Tumors
  3. Abscess
457
Q

Name 1 indication for head ultrasound

A
  1. Infants with accelerating head circumference across percentiles

Peds (35 decks)

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