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PEDS 2201/2262 > NEUROMUSCULAR > Flashcards

NEUROMUSCULAR Flashcards

1
Q

Hernial protrusion of a saclike cyst, containing meninges, spinal fluid, and nerves?

A

Myelomeningocele

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2
Q

Defect characterized hy retroposition of the tongue and mandible?

A

Pierre Robin sequence

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3
Q

Condition that results from disturbances in the dynamics of CSF absorption and flow?

A

Hydrocephalus

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4
Q

Cong malformation in which both cerebral hemispheres are absent?

A

Anencephaly

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5
Q

Any malformation of the spinal canal and cord?

A

Myelodysplasia

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6
Q

Marked by eyes rotated downward with sclera visible above the iris?

A

Setting-sun sign

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7
Q

Herniation of brain and meninges through a defect in the skull, resulting in a fluid-filled sac?

A

Encephalocele

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8
Q

Total exposure of the brain through a skull defect?

A

Exancephaly

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9
Q

Fissure in the spinal column that leaves the meninges and spinal cord exposed?

A

Rachischisis or spina bifida

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10
Q

Hernial protrusion of saclike cyst of meninges filled with spinal fluid?

A

Meningocele

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11
Q

Skull defect with tissues protruding?

A

Cranioschisis

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12
Q

The major anomaly associated with myelomeningocele is?

A

Hydrocephalus

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13
Q

Two methods by which prenatal neural tube defects can be diagnosed?

When is the best time to perform these tests?

A

U/S of Uterus

Elevated AFP

16-18 wks of gestation

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14
Q

Management goal for GU FXN in the infant with myelomeningocele?

Goal for older child with same condition?

A

Preserve renal FX

Preserve renal FX & achieve max continence

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15
Q

Drug class used in pts with myelomeningocele to reduce detrusor muscle tone and bladder pressure?

A

Anticholinergics

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16
Q

The PRIMARY DX tool for detecting hydrocephalus in older infants and children is:

A. CT or MRI

B. Measuring head circumference

C. EEG

D. U/S

A

A

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17
Q

What is the preferred shunt for infants with hydrocephalus?

A. Ventriculoperitoneal shunt

B. Ventriculoatrial shunt

C. Ventricular bypass

D. Ventriculopleural shunt

A

A

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18
Q

Post-op care of a pt with a shunt should include:

A. positioning the pt in head-up position

B. continuous pumping of shunt to assess FX

C. monitoring for abd or peritoneal distention

D. positioning pt on side of op site

A

C

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19
Q

The MAJOR complicaitons of Ventriculoperitoneal shunts are?

A

Infection

Malfunction

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20
Q

Posterior fontanel is closed by age?

A

6-8 wks (2 mos)

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21
Q

Anterior fontanel is closed by?

A

7-19 mo

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22
Q

The primary disorder of the upper motor neuron dysfunction is?

A

CP

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23
Q

Characteristic manifestations of upper motor neuron lesions?

A

Weakness

Spasticity

Increased DTRs

Abnormal superficial reflexes

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24
Q

Characteristic manifestations of lower motor neuron lesions?

A

Weakness

Atrophy of skeletal muscles

Hypotonia

Flaccidity

Contractures

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25
Q

Prominent etiologic agents affecting the anterior horn cells?

A

Enteroviruses

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26
Q

CP is the primary disorder of ?

A

Upper motor neuron dysfunction

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27
Q

In most instances the sudden appearance of flaccid paralysis in a previously healthy child is attributed to?

A

Infectious process

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28
Q

What 2 things are more responsible for muscular weakness and atrophy of gradual onset?

A

Hereditary factors and metabolic diseases

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29
Q

What are the 4 MAJOR movement disorders of CP?

A

Spastic

Dyskinetic

Ataxic

Mixed

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30
Q

Characteristics of DTRs in:

  1. Upper motor neuron disease
  2. Lower motor neuron disease
A
  1. briskly active

Diminished or absent

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31
Q

Delayed gross motor development is a universal manifestation of?

A

CP

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32
Q

3 steps involved in transmitting nerve impulses (in order)?

A

Acetylcholine released

Then diffuses across the junction and causes muscles to contract

Removed by cholinesterase

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33
Q

Examples of toxins that cause neuromuscular junction disease are those that produce the paralysis of?

A

Botulism and tick paralysis

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34
Q

The etiology of CP is most commonly related to?

A

Existing prenatal brain abnormalities

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35
Q

The RN is preparing the long-term care plan for a child with CP. Which of the following is included in the plan?

A. No delay in gross motor development is expected.

B. The illness is not progressively degenerative.

C there will be no persistence of primitive infantile reflexes.

D. all children will need genetic counseling as they grow older before planning for a family.

A

B

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36
Q

What are the major complications of Duchenne muscular dystrophy?

A

Contractures 

Scoliosis 

Disuse atrophy

Infections 

Obesity 

cardiopulmonary problems

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37
Q

What is the eventual cause of death in those with DMD?

A

 Respiratory infection  Cardiac failure

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38
Q

Characteristics of DMD?

A

 Early onset b/w 3 – 5 y/o 

Progressive weakness,

wasting, contractures 

Calf muscle hypertrophy 

Loss of independent ambulation by 9 – 12 yrs 

Slowly progressive, generalized weakness during adolescence 

Relentless progression until death from resp or cardiac failure

X – Linked recessive trait?

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39
Q
A
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40
Q

DMD management?

A

No cure

Maintain optimum function in all muscels for as long as possible

Prevent contractures

Must have cardiac status assessment prior to ANY SURGERY

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41
Q

Child with this disorder attains Standing posture by kneeling, then gradually pushing his torso upright (with knees straight) by walking his hands up his legs. Marked lordosis in upright position. What is this known as?

What disorder does the child have?

A

Gower sign

DMD

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42
Q

DMD age of ONSET?

A

3-5 yr

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43
Q

DMD initial manifestations?

A

Lordosis

Waddling gait

Frequent falls

Toe walking

Difficulty in rising from floor (Gower sign) and climbing stairs

Fat deposits replace wasted gastrocnemius muscles

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44
Q

DMD progression?

A

Rapid

Ultimately involves all voluntary muscles

Death usually occurs at 15-30 yrs

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45
Q

An uncommon acute demyelinating polyneuropathy with progressive, usually ascending flaccid paralysis?

A

Guillain-Barre syndrome (GBS)

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46
Q

Hallmark of GBS?

A

Acute peripheral motor weakness

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47
Q

When does GBS usually occur?

A

10 days after nonspecific viral infection

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48
Q

Who does GBS affect more?

A

Adults more often than children

Child b/w 4-10 y/o (more susceptible)

Late adolescence and young adulthood (peak periods)

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49
Q

Although cong GBS is rare, if it occurs what does is consist of?

A

Hypotonia

Weakness

Decreased or absent reflexes

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50
Q

When do symptoms of GBS gradually subside and then dissapear?

A

Subside: first few mo of life

Disappear: by 12 mo

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51
Q

An immune-mediated disease often associated with a number of viral or bacterial infections or the administration of vaccines?

A

GBS

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52
Q

Previous infection with what organism is associated with a severe form of GBS?

A

C. jejuni

53
Q

Three phases of GBS?

A
  1. Acute or progressive
  2. Plateau
  3. Recovery
54
Q

How long may the acute/progressive phase of gbs last?

A

until new sx stop appearing or deterioration ceases

MAY LAST UP TO 4 WKS

55
Q

Clinical manifestaions of gbs?

A

Mild influenza-like sore throat precedes paralytic manifesations

Onset: rapid (reaching peak in 24 hr) or gradual over days - weeks

Neuro: initially involve muscle tenderness sometimes accompanied by paresthesia and cramps.

Paralysis ascends from lower extremities or trunk to upper extremities

Depressed/absent tendon reflexes

Lower limb & back pain common in children

Urinary incontinence/retention

Constipation

Abd pain & fatigue

Ortho hypo

HTN

Bradycardia, asystole, heart block

56
Q

Which cranial nerve is often affected by gbs?

A

CN 7 (facial)

57
Q

DX of gbs?

A

Based on parylitic manifestations and on EMG

Motor nerve conduction velocities are greatily reduced

CSF analysis reveals elevated protein and normal glucose

58
Q

gbs TX?

A

Priamrily supportive

In acute phase pt is hospitalized bc resp and pharyngeal involvement may require ventilation or trach.

Aggressive vent support

IV immunoglobulin (IVIG)

Steroids

Plasmapherisis

LMWH, mild laxative, acetaminophen, h2ra

59
Q

Side effects of plasmapheresis?

A

Hypotension, bradycardia

fever

bleeding

chills, urticaria

60
Q

Nursing mgmt of the child with Tetanus?

A

Supportive with particular attention to Airway and breathing

Assess location and extent of muscle spasms and the severity

CNS depression, apnea, resp failure (MAY be d/t opioids, muscle relaxants, sedative)

May need to paralyse pt because of the intensity of the spasms.

HYDRATION & NUTRITION (monitor IVF, NG or gastrostomy feeding), ORAL HYGIENE, suction secretions

CONTROL/ELEMINATE stiulation from sound, light, touch (dim light NOT DARK room)

If paralytic (rocuronium, vecuronium) is used - admin anxiolytics (fentanyl/versed)

Have parent stay with child

61
Q

Tetanus is characterized by?

A

Painful muscular rigidity involving masseter and neck muscles

62
Q

Would the child who reports to the ER with a clean minor wound who has completed tetanus shot in the last 10 years need a tetanus booster shot now?

A

NO

63
Q

A child admitted to the ER with a burn and puncture wound and had a tetanus booster in the past 10 years should recieve?

A

Tetanus toxoid booster

64
Q

A child who is unprotected and/orinadequatly immunized sustains a “tetanus prone” wound contaminated with dirt reports to the ER. What should he be administered?

A

Tetanus immunoglobulin (TIG)

AND tetanus toxoid at a separate site

65
Q

4 requirements to the development of tetanus?

A
  1. presence of tetanus spores or vegetative forms of the bacillus
  2. injury to tissues
  3. wounds that encourage multiplication of the organisms
  4. A susceptible host
66
Q

Of the 2 exotoxins from tetanus, _______ is the potent toxin that affects the CNS causing sxs while ______ has no significance.

A

tetanospasmin

tetanolysin

67
Q

Most cases of tetanus occur within ___ days; in neonates it is usually ___ to ____ days.

A

14

5-14

68
Q

Difficulty in neonates sucking ability is the first manifestation of?

A

Tetanus

69
Q

The characteristic difficulty opening the mought d/t tetanus is known as?

A

trismus

70
Q

Spsm of facial muscles produces the so/called sardonic smile known as?

A

risus sardonicus

71
Q

Survival of tetanus beyond how long usually indicates complete recovery?

A

4 days

72
Q

Drug of choice in tetanus pts for seizure control?

A

diazepam

73
Q

Autoimmune disorder associated with the attack of circulating antibodies on the acetylcholine receptors on the muscle end plat, which blocks their function?

A

Myasthenia gravis (MG)

74
Q

MG incidence?

A

Uncommon in childhood

Onset after age 10 usually but may appear at 2 y/o

Girls 3x > boys

75
Q

With this disease, the most common sx are general paralysis of the optic muscles with ptosis and diplopia. Difficulty swallowing, chewing, and speaking are also prominent and accompanied by weakness and paralysis of all skeletal muscles?

A

MG

76
Q

Involves more prounounced sx in the late afternooon and eveening. Rest can releive the sx but exercise and stress worsen them?

A

MG

77
Q

DX of MG?

A

Based on characteristic distribution of muscle weakness and progressive weakness

EMG: decrease in muscle potentials with repetitive nerve stimulation

Tensilon test

78
Q

Thereapeutic management of MG?

A

Cholinesterase INHIBITING drugs (Prostigmin IV or PO)

Mestinon

Observe child for parasympathetic stimulation d/t overmedication (lacrimation, salivation, abd cramps, sweating, diarrhea, vomiting, bradycardia, resp weakness)

Thymectomy

IVIG

Plasmapheresis

79
Q

Antidote for neostigmine and pyridostigmine given for MG?

A

Atropine!!

80
Q

Drugs that MUST be AVOIDED in pts with MG?

A

Neuromuscular-blockers (pancuronium, succinylcholine) -may induce paralysis for wks

Aminoglycosides (gentamicin) - potentiate MG sx

81
Q

Nusing mgmt for MG?

A

MINIMIZE STRESS & MAXIMIZE REST

DON’T admin panteronum or succinylcholine!!

Teach parents importance of accurate med admin & side effects: choking, aspiration, resp distress

Warn them of possibility of sudden exacerbation of sx during times of physical/emotional stress (myasthenia crisis) - REQUIRES STAT medical attention

82
Q

What are the 7 Major Complications of DMD? (MUST KNOW!!)

A

Contractures

Disuse atrophy

Resp infections

Scoliosis

Obesity

Resp compromise

Cardiac failure

83
Q
  1. Infants are more likely to suffer SCI as a result of?
  2. Toddlers and school-aged children up to 9 years?
A
  1. Motor vehicle crashes (MVCs)
  2. Falls
84
Q

What accounts for the high majority of SCIs in adolescents who live in the U.S.?

A

FOOTBALL!!

85
Q

How many nerves (spinal processes) are there in the following segments of the spinal cord:

  1. cervical
  2. Thoracic
  3. Lumbar
  4. Sacral
  5. coccygeal
A

7

12

5

5

1

86
Q

Complete SCI?

A

Absence of sensory & motor fxn below level of injury

Paraplegic - lower thoracic (waist down)

Tetraplegic - C1-T1

Quadriplegic

87
Q

The most common cause of serious SCI in children is trauma involving?

A

MVCs

sports

Birth trauma

Child abuse

88
Q

SCI w/o radiographic abnormality (SCIWORA) is likely to occur in an?

A

MCV when safety restraints are not used

89
Q

Various degrees of sensory/motor loss below the LOI; partial damage to spinal cord?

A

Incomplete SCI

90
Q

Neonatal MG:

A. occurs in approx 30% to 50% of infants born to mothers with MG

B. produces elevated moro reflex and shrill cry in the infant

C. may require admin of cholinesterase inhibitors to improve feeding ability

D. produces strength changes in infant even after maternal acetylcholine receptor antibodies have cleared infant’s system

A

C

91
Q

A group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occured in the developing fetal or infant brain?

A

CP

92
Q

Abnormal muscle tone and coordination are the primary disturbances in pts with?

A

CP

93
Q

Most common permanent physical disability of childhood?

A

CP

94
Q

a nonprogressive injury to the motor centers of the brain, which can cause spastic and involuntary movements, and can also be associated with developmental delays and seizure activity?

A

CP

95
Q

Persistence of tonic neck reflex and Moro reflex beyond 4 months indicates?

A

CP

96
Q

ETIOLOGY OF CP?

A

Brain injury/insult during prenatal period

maternal and perinatal infections

Premies of ELBW and VLBW

Periventricular leukomalacia & intracerebral hemorrhage in LBW infants

Perinatal ischemic stroke, White matter abnormalities (focal lesions)

Shaken baby syndrome, Bacterial meningitis, Viral encephalitis, MVCs, Child abuse

97
Q

SINGLE MOST IMPORTANT RISK FACTOR FOR CP?

A

Preterm birth of extremely low-birth wt (ELBW) and very low-birth weight (VLBW)

98
Q

What are the classifications of CP?

A

Spastic (pyramidal)

Dyskinetic (NON-spastic, Extrapyramidal)

Ataxic (NON-spastic, Extrapyramidal)

Mixed Type

99
Q

Characterized by persistent primitive reflexes, positive Babinski reflex, ankle clonus, exaggerated stretch reflexes, eventual development of contractures; 70 to 80% of all cases of CP?

A

Spastic (Pyramidal)

100
Q

Characteristics of spastic (Pyramidal) CP?

A

MOST COMMON CLASSIFICATION

Diplegia -all extremities affected; lower more than upper (NOT COMMON)

Tetraplegia-all 4 extremities affected (not common)

Triplegia

Monoplegia

Hemiplegia

Hypertonicity with poor control of posture, balance, and coordinated motion impairment of fine and gross motor skills

101
Q

Involuntary, irregular, jerking mvmts characterized by slow, wormlike, writhing mvmts that usually involve extremities, trunk, neck, facial muscles, and tongue

A

Athetoid-chorea (Dyskinetic CP)

102
Q

The etiology of CP is most commonly r/t:

A. existing prenatal brain abnormalities

B. maternal asphyxia

C. childhood meningitis

D. preeclampsia

A

A

103
Q

Pure cerebral paraplegia of lower extremities?

A

Paraplegia

104
Q

Exaggerated arching of the back

A

Opisthotonic posture

105
Q

Most common form of spastic CO; motor deficit > in upper extremity; one side of body affected?

A

Hemiparesis

106
Q

When does hand dominance normally develop &what does it indicate when a child develops hand dominence at about 6 mo of age?

A

NORMAL = Preschool years

CP

107
Q

Physical signs indicative of CP?

A

Poor head control after 3 mo of age

Stiff or rigid arms or legs

Pushing away/arching back

Floppy/limp posture

Can’t sit up w/o support by 8 mo

Using only 1 side of body, or only arms to crawl

Clenched hands after 3 mo

Persistence of primitive reflexes such as Moro and atonic neck past 6 mo

Hand preference demonstrated before 18 mo

Leg scissoring

Seizures

hearing/vision imp

Persistent tongue thrusting after 6 mo

108
Q
A
109
Q

Most common soft tissue sarcoma in children, especially children under 5 y/o?

A

Rhabdomyosarcoma

110
Q

Where does rhabdomyosarcoma occur?

A

Striated skeletal muscle

111
Q

Most common subtype of rhabdomyosarcoma, mostly found in head, neck, abd, & GU tract?

A

Embryonal

112
Q

Second most common subtype of rhabdomyosarcoma, mostly arises in deep tissue of extremities & trunk?

A

Alveolar

113
Q

Subtype of rhabdomyosarcoma that is RARE in children (ADULT FORM), most often occuring in soft parts of extremities & trunk?

A

Pleomorphic

114
Q

Clinical manifestations of Rhabdomyosarcoma (RMS)?

A

Initial s/s are r/t site of tumor and compression of adjacent organs

Commonly mistake s/s for “earache” or “runny nose”

115
Q

DX EVALUATION OF RHABDOMYOSARCOMA?

A

H&P

CT or MRI

METS eval shold include CT of chest, bone scan, & bilateral BM aspirates & BXs

LP to examine SF

Excisional BX or surgical resection of tumor when possible to confirm DX

116
Q

RHABDOMYOSARCOMA is…?

A

Malignant solid tumor of the soft tissue

117
Q

With a multimodal approach to TX for nonmetastatic rhabdomyosarcoma, what % of pts are expected to survive?

A. 15%

B. 35%

C. 50%

D. 80%

A

D

118
Q

C/M of RMS located in the orbit?

A

Rapidly developing unilateral proptosis

Ecchymosis of conjunctiva

Loss of extraocular mvmts (strabismus

119
Q

C/M of RMS located in the nasopharynx

A

Stuffy nose (earliest)

Nasal obstruction - dysphagia, nasal voice, serous otitis media

Sore throat and ear

epistaxis

palpable neck nodes

visible mass in oropharynx

120
Q

TX of RMS?

A

Multimodal therapy

Removal if possible

Chemo, irradiation, or both

Pts with embryonal tumors and group 1 dz can be tx with chemo alone

121
Q

Goup 1 RMS

A

Localized dz

Tumor completely resected

Regional nodes NOT involved

122
Q

Group 2 RMS

A

Localized dz with microscopic residual, or regional dz with no residual or with microscopic residual

123
Q

Group 3 RMS

A

Incomplete resection or BX with gross residual dz

124
Q

Group 4 RMS

A

METS present at DX

125
Q

C/M of RMS located in the paranasal sinuses?

A

Nasal obstruction

Local pain

Discharge

Sinusitis

Swelling

126
Q

C/M of RMS located in the middle ear?

A

S/S of chronic serous OM

Pain

Sanguinopurulent drainage

Facial nerve palsy

127
Q

C/M of RMS located in the retroperitoneal area?

A

USUALLY A SILENT TUMOR

Abd mass

Pain

S/S of intestinal or GU obs

128
Q

C/M of RMS of the peritoneum?

A

Visible superficial mass Bowel or bladder dysfxn

PEDS 2201/2262 (7 decks)

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