NEUROMUSCULAR Flashcards
1
Q
Hernial protrusion of a saclike cyst, containing meninges, spinal fluid, and nerves?
A
Myelomeningocele
2
Q
Defect characterized hy retroposition of the tongue and mandible?
A
Pierre Robin sequence
3
Q
Condition that results from disturbances in the dynamics of CSF absorption and flow?
A
Hydrocephalus
4
Q
Cong malformation in which both cerebral hemispheres are absent?
A
Anencephaly
5
Q
Any malformation of the spinal canal and cord?
A
Myelodysplasia
6
Q
Marked by eyes rotated downward with sclera visible above the iris?
A
Setting-sun sign
7
Q
Herniation of brain and meninges through a defect in the skull, resulting in a fluid-filled sac?
A
Encephalocele
8
Q
Total exposure of the brain through a skull defect?
A
Exancephaly
9
Q
Fissure in the spinal column that leaves the meninges and spinal cord exposed?
A
Rachischisis or spina bifida
10
Q
Hernial protrusion of saclike cyst of meninges filled with spinal fluid?
A
Meningocele
11
Q
Skull defect with tissues protruding?
A
Cranioschisis
12
Q
The major anomaly associated with myelomeningocele is?
A
Hydrocephalus
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
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
15
Q
Drug class used in pts with myelomeningocele to reduce detrusor muscle tone and bladder pressure?
A
Anticholinergics
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
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
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
19
Q
The MAJOR complicaitons of Ventriculoperitoneal shunts are?
A
Infection
Malfunction
20
Q
Posterior fontanel is closed by age?
A
6-8 wks (2 mos)
21
Q
Anterior fontanel is closed by?
A
7-19 mo
22
Q
The primary disorder of the upper motor neuron dysfunction is?
A
CP
23
Q
Characteristic manifestations of upper motor neuron lesions?
A
Weakness
Spasticity
Increased DTRs
Abnormal superficial reflexes
24
Q
Characteristic manifestations of lower motor neuron lesions?
A
Weakness
Atrophy of skeletal muscles
Hypotonia
Flaccidity
Contractures
25
Prominent etiologic agents affecting the anterior horn cells?
Enteroviruses
26
CP is the primary disorder of ?
Upper motor neuron dysfunction
27
In most instances the sudden appearance of flaccid paralysis in a previously healthy child is attributed to?
Infectious process
28
What 2 things are more responsible for muscular weakness and atrophy of gradual onset?
Hereditary factors and metabolic diseases
29
What are the 4 MAJOR movement disorders of CP?
Spastic
Dyskinetic
Ataxic
Mixed
30
Characteristics of DTRs in:
1. Upper motor neuron disease
2. Lower motor neuron disease
1. briskly active
Diminished or absent
31
Delayed gross motor development is a universal manifestation of?
CP
32
3 steps involved in transmitting nerve impulses (in order)?
Acetylcholine released
Then diffuses across the junction and causes muscles to contract
Removed by cholinesterase
33
Examples of toxins that cause neuromuscular junction disease are those that produce the paralysis of?
Botulism and tick paralysis
34
The etiology of CP is most commonly related to?
Existing prenatal brain abnormalities
35
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.
B
36
What are the major complications of Duchenne muscular dystrophy?
Contractures
Scoliosis
Disuse atrophy
Infections
Obesity
cardiopulmonary problems
37
What is the eventual cause of death in those with DMD?
Respiratory infection Cardiac failure
38
Characteristics of DMD?
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?
39
40
DMD management?
No cure
Maintain optimum function in all muscels for as long as possible
Prevent contractures
Must have cardiac status assessment prior to ANY SURGERY
41
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?
Gower sign
DMD
42
DMD age of ONSET?
3-5 yr
43
DMD initial manifestations?
Lordosis
Waddling gait
Frequent falls
Toe walking
Difficulty in rising from floor (Gower sign) and climbing stairs
Fat deposits replace wasted gastrocnemius muscles
44
DMD progression?
Rapid
Ultimately involves all voluntary muscles
Death usually occurs at 15-30 yrs
45
An uncommon acute demyelinating polyneuropathy with progressive, usually ascending flaccid paralysis?
Guillain-Barre syndrome (GBS)
46
Hallmark of GBS?
Acute peripheral motor weakness
47
When does GBS usually occur?
10 days after nonspecific viral infection
48
Who does GBS affect more?
Adults more often than children
Child b/w 4-10 y/o (more susceptible)
Late adolescence and young adulthood (peak periods)
49
Although cong GBS is rare, if it occurs what does is consist of?
Hypotonia
Weakness
Decreased or absent reflexes
50
When do symptoms of GBS gradually subside and then dissapear?
Subside: first few mo of life
Disappear: by 12 mo
51
An immune-mediated disease often associated with a number of viral or bacterial infections or the administration of vaccines?
GBS
52
Previous infection with what organism is associated with a severe form of GBS?
C. *jejuni*
53
Three phases of GBS?
1. Acute or progressive
2. Plateau
3. Recovery
54
How long may the acute/progressive phase of gbs last?
until new sx stop appearing or deterioration ceases
**MAY LAST UP TO 4 WKS**
55
Clinical manifestaions of gbs?
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
Which cranial nerve is often affected by gbs?
CN 7 (facial)
57
DX of gbs?
Based on parylitic manifestations and on EMG
Motor nerve conduction velocities are greatily reduced
CSF analysis reveals elevated protein and normal glucose
58
gbs TX?
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
Side effects of plasmapheresis?
Hypotension, bradycardia
fever
bleeding
chills, urticaria
60
Nursing mgmt of the child with Tetanus?
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
Tetanus is characterized by?
Painful muscular rigidity involving masseter and neck muscles
62
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?
NO
63
A child admitted to the ER with a burn and puncture wound and had a tetanus booster in the past 10 years should recieve?
Tetanus toxoid booster
64
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?
Tetanus immunoglobulin (TIG)
AND tetanus toxoid at a separate site
65
4 requirements to the development of tetanus?
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
Of the 2 exotoxins from tetanus, _______ is the potent toxin that affects the CNS causing sxs while ______ has no significance.
tetanospasmin
tetanolysin
67
Most cases of tetanus occur within ___ days; in neonates it is usually ___ to ____ days.
14
5-14
68
Difficulty in neonates sucking ability is the first manifestation of?
Tetanus
69
The characteristic difficulty opening the mought d/t tetanus is known as?
trismus
70
Spsm of facial muscles produces the so/called sardonic smile known as?
risus sardonicus
71
Survival of tetanus beyond how long usually indicates complete recovery?
4 days
72
Drug of choice in tetanus pts for seizure control?
diazepam
73
Autoimmune disorder associated with the attack of circulating antibodies on the acetylcholine receptors on the muscle end plat, which blocks their function?
Myasthenia gravis (MG)
74
MG incidence?
Uncommon in childhood
Onset after age 10 usually but may appear at 2 y/o
Girls 3x \> boys
75
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?
MG
76
Involves more prounounced sx in the late afternooon and eveening. Rest can releive the sx but exercise and stress worsen them?
MG
77
DX of MG?
Based on characteristic distribution of muscle weakness and progressive weakness
EMG: decrease in muscle potentials with repetitive nerve stimulation
Tensilon test
78
Thereapeutic management of MG?
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
Antidote for neostigmine and pyridostigmine given for MG?
Atropine!!
80
Drugs that MUST be AVOIDED in pts with MG?
***_Neuromuscular-blockers_*** (**pancuronium**, **succinylcholine**) -may induce paralysis for wks
***_Aminoglycosides_*** (**gentamicin**) - potentiate MG sx
81
Nusing mgmt for MG?
_**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
What are the 7 Major Complications of DMD? **(MUST KNOW!!)**
Contractures
Disuse atrophy
Resp infections
Scoliosis
Obesity
Resp compromise
Cardiac failure
83
1. Infants are more likely to suffer SCI as a result of?
2. Toddlers and school-aged children up to 9 years?
1. Motor vehicle crashes (MVCs)
2. Falls
84
What accounts for the high majority of SCIs in adolescents who live in the U.S.?
FOOTBALL!!
85
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
**_7_**
**_12_**
**_5_**
**_5_**
**_1_**
86
Complete SCI?
**_Absence of sensory & motor fxn below level of injury_**
**Paraplegic** - lower thoracic (waist down)
**Tetraplegic** - C1-T1
**Quadriplegic**
87
The most common cause of serious SCI in children is trauma involving?
MVCs
sports
Birth trauma
Child abuse
88
SCI w/o radiographic abnormality (SCIWORA) is likely to occur in an?
MCV when safety restraints are not used
89
Various degrees of sensory/motor loss below the LOI; partial damage to spinal cord?
Incomplete SCI
90
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
C
91
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?
CP
92
Abnormal muscle tone and coordination are the primary disturbances in pts with?
CP
93
Most common permanent physical disability of childhood?
CP
94
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?
CP
95
Persistence of tonic neck reflex and Moro reflex beyond 4 months indicates?
CP
96
ETIOLOGY OF CP?
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
SINGLE MOST IMPORTANT RISK FACTOR FOR CP?
Preterm birth of extremely low-birth wt (ELBW) and very low-birth weight (VLBW)
98
What are the classifications of CP?
Spastic (pyramidal)
Dyskinetic (NON-spastic, Extrapyramidal)
Ataxic (NON-spastic, Extrapyramidal)
Mixed Type
99
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**?
Spastic (Pyramidal)
100
Characteristics of spastic (Pyramidal) CP?
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
Involuntary, irregular, jerking mvmts characterized by slow, wormlike, writhing mvmts that usually involve extremities, trunk, neck, facial muscles, and tongue
Athetoid-chorea (Dyskinetic CP)
102
The etiology of CP is most commonly r/t:
A. existing prenatal brain abnormalities
B. maternal asphyxia
C. childhood meningitis
D. preeclampsia
A
103
Pure cerebral paraplegia of lower extremities?
Paraplegia
104
Exaggerated arching of the back
Opisthotonic posture
105
Most common form of spastic CO; motor deficit \> in upper extremity; one side of body affected?
Hemiparesis
106
When does hand dominance normally develop &what does it indicate when a child develops hand dominence at about 6 mo of age?
NORMAL = Preschool years
CP
107
Physical signs indicative of CP?
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
109
Most common soft tissue sarcoma in children, especially children under 5 y/o?
Rhabdomyosarcoma
110
Where does rhabdomyosarcoma occur?
Striated skeletal muscle
111
Most common subtype of rhabdomyosarcoma, mostly found in head, neck, abd, & GU tract?
Embryonal
112
Second most common subtype of rhabdomyosarcoma, mostly arises in deep tissue of extremities & trunk?
Alveolar
113
Subtype of rhabdomyosarcoma that is RARE in children (ADULT FORM), most often occuring in soft parts of extremities & trunk?
Pleomorphic
114
Clinical manifestations of Rhabdomyosarcoma (RMS)?
Initial s/s are r/t site of tumor and compression of adjacent organs
Commonly mistake s/s for "earache" or "runny nose"
115
DX EVALUATION OF RHABDOMYOSARCOMA?
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
RHABDOMYOSARCOMA is...?
Malignant solid tumor of the soft tissue
117
With a multimodal approach to TX for nonmetastatic rhabdomyosarcoma, what % of pts are expected to survive?
A. 15%
B. 35%
C. 50%
D. 80%
D
118
C/M of RMS located in the orbit?
Rapidly developing unilateral proptosis
Ecchymosis of conjunctiva
Loss of extraocular mvmts (strabismus
119
C/M of RMS located in the nasopharynx
Stuffy nose (earliest)
Nasal obstruction - dysphagia, nasal voice, serous otitis media
Sore throat and ear
epistaxis
palpable neck nodes
visible mass in oropharynx
120
TX of RMS?
Multimodal therapy
Removal if possible
Chemo, irradiation, or both
Pts with embryonal tumors and group 1 dz can be tx with chemo alone
121
Goup 1 RMS
Localized dz
Tumor completely resected
Regional nodes NOT involved
122
Group 2 RMS
Localized dz with microscopic residual, or regional dz with no residual or with microscopic residual
123
Group 3 RMS
Incomplete resection or BX with gross residual dz
124
Group 4 RMS
METS present at DX
125
C/M of RMS located in the paranasal sinuses?
Nasal obstruction
Local pain
Discharge
Sinusitis
Swelling
126
C/M of RMS located in the middle ear?
S/S of chronic serous OM
Pain
Sanguinopurulent drainage
Facial nerve palsy
127
C/M of RMS located in the retroperitoneal area?
USUALLY A SILENT TUMOR
Abd mass
Pain
S/S of intestinal or GU obs
128
C/M of RMS of the peritoneum?
Visible superficial mass Bowel or bladder dysfxn
