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SRNA3AdvancedPatho2 > FINAL -NEURO congenital > Flashcards

FINAL -NEURO congenital Flashcards

1
Q

Hydrocephalus is

A

CSF imbalance -more is produced than reabsorbed.

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2
Q

**Normal CSF production

A

21 cc/hr

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3
Q

***Normal total volume = Measure at any time

A

150ml measured at any given time

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4
Q

CSF excess

A

Compresses brain tissue and blood vessels

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5
Q

If cranial sutures have not closed

A

Infant’s head enlarges beyond normal size

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6
Q

***Two types of hydrocephalus:

A

Obstructive (“non-communicating”)

Communicating

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7
Q

***Communicating Hydrocephalus

A

Failure to reabsorb CSF through subarachnoid

granulations (“villi”)

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8
Q

Elderly

A

Can develop Normal Pressure Hydrocephalus

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9
Q

Normal pressure Hydrocephalus Caused by

A 
subarachnoid hemorrhage
head trauma,
infection, tumor, or 
complications of surgery 
inattention, ataxia, 
forgetfulness, memory difficulty, iand
urinary incontinence
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10
Q

Normal pressure hydrocephalus tx

A

Corrected surgically with shunt placement

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11
Q

**Acute Hydrocephalus signs and symptoms

A 
Lethargy, 
Altered mentation
Papilledema
Hypertension
ECG
decorticate or decerebrate posturing
bradycardia,
Δs(delayed repolarization  most commonly QT prolongation)
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12
Q

Medical management of Hydrocephalus::

Meds

A

Furosemide or acetazolamide to reduce CSF production

Lumbar punctures for temporary relief

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13
Q

***Ventricular shunts most commonly

A

drained into peritoneal or pleural spaces. Less commonly, into the atrial or choledochal spaces.

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14
Q

**Anesthesia Considerations: Hydrocephalus

Preop –

A

If gastrostomy tube is present, aspirate prior to
induction and leave open during induction to prevent
gastric distention/regurgitation

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15
Q

***Shunt procedures: Children with Children with ↑ ICP and/or delayed gastric emptying delayed gastric emptying

A

should be induced intravenously following

preoxygenation

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16
Q

***Shunt Procedures: Preservation of body temp. necessary during shunt
procedures -why?

A

Large body surface area surgically prepped

and exposed – leads to rapid cooling

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17
Q

**Cerebral Palsy: what is it

A

Group of disorders marked by motor impairment

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18
Q

CP is Classified by extremities involved and type of neurologic dysfunction: QMHD and SAHD

A

Ext: quadriplegia, monoplegia, hemiplegia, diplegia
Type: hypotonic, spastic, athetotic, dystonic

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19
Q

Diplegia

A

Both arms and legs

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20
Q

Quadriplegia

A

All four involved

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21
Q

Monoplegia

Hemiplegia

A

One limb is affected

One side of the body

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22
Q

Triplegia

A

Thee limbs

Both arms and one leg.

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23
Q

***Cerebral Palsy Signs and symptoms

MCD, CSSM

A

Muscle spasticity (most common) - damage to motor
cortex or pyramidal tracts
Choreoathetosis - (video of pediatric CP patient)
Dystonia damage to the extrapyramidal extrapyramidal tract, basal nuclei, cranial nerves
Cerebellar ataxia – cerebellar damage with loss of
balance & coordination
Speech disorders
Seizures
Mental retardation

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24
Q

***Medications to relieve muscle spasticity:

A

Baclofen
Dantrolene
Botox injections

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25
Q

**For CP patients going to surgery -

A

important to continue Baclofen throughout perioperative period

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26
Q

***Baclofen Abrupt discontinuation can result in withdrawal symptoms

A

Seizures

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27
Q

**Common Surgeries for CP patients

Surgical Treatments usually aimed at

A

correcting musculoskeletal disorders:

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28
Q

Types of surgeries for CP

A

Achilles’ tendon lengthening
Osteotomy of the femur
Iliopsoas release
Scoliosis correction

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29
Q

**CP – Anesthesia Considerations

Pt.s have

A

↑ incidence of reflux with weak pharyngeal &

laryngeal muscles

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30
Q

**CP – Anesthesia Considerations ↑ susceptiblity to

A

hypothermia - require close temperature monitoring

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31
Q

**CP – Anesthesia Considerations Tracheal extubation may need to be

A

delayed until patient is fully awake and airway reflexes return

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32
Q

**CP – Anesthesia Considerations

Pt.s have MAC is generally

A

decreased and emergence times are longer

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33
Q

**CP and SUCC

A

CP – Anesthesia Considerations
Succinylcholine ok to use
No exaggerated hyperkalemic response

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34
Q

CP and seizures medications

A

Antiseizure medications common in CP patients – may
lead to subsequent resistance to nondepolarizing
muscle relaxants

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35
Q

***Spinal Bifida

A

Common neural tube defect
Failure of posterior spinous processes to fuse
Permit meninges and spinal cord to herniate
Result in neurologic impairment

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36
Q

**3 Types of Spina Bifida

A

Occulta
Meningocele
Myelomeningocele

37
Q

Spina Bifida Occulta

A

Spinous processes do not fuse.

No herniation of spinal cord or meninges

38
Q

Defect may not be visible

A

Dimple or tuft of hair may be present on the

skin over the site.

39
Q

Meningocele

A

Herniation of meninges through the defect.
Meninges and CSF form a sac on the surface.
Transillumination confirms absence of nerve tissue in sac.

40
Q

Myelomeningocele

A

Most serious form
Herniation of meninges & spinal cord
Considerable neurologic impairment

41
Q

Charcot Marie Tooth Disease

A

The most common inherited cause of chronic motor &

sensory peripheral neuropathy

42
Q

**CMT – Anesthesia Considerations

A

Avoid succinylcholine ↑ K+ release in neuromuscular ds.

43
Q

With CMT , Use caution with and why?

A

neuromuscular blocking drugs↑ risk of respiratory failure post-op 2o to muscle weakness

44
Q

***Chiari Malformations Type I -

A

cerebellar tonsils protrude into foramen magnum
without brainstem involvement.
Most common type

45
Q

***Chiari Malformations Type I -

Type II –

A

“Arnold-Chiari” – same with brainstem herniation
Usually occurs with Spina Bifida myelomeningocele and
obstructive hydrocephalus.

46
Q

Chiari Malformations Treatment with

A

surgical decompression

47
Q

***Chiari Malformations Anesthesia Considerations for patients undergoing surgical decompression?

A

include increased ICP and significant blood loss, especially in Type II

48
Q

Tuberous Sclerosis

What is it associated with ?

A

Seizures and/or mental retardation may be present

There is an association w/ Wolff-Parkinson-White Syndrome

49
Q

WPW

A

•Accessory Pathway
•Causes paroxysmal
tachycardia

50
Q

WPW ECG changes:

A
  • Delta wave
  • Shortened PR interval
  • Wide QRS complex
51
Q

Tuberous Sclerosis : Fibromas

A

Nodular fibromas/papillomas may occur on tongue,

palate, pharynx and larynx

52
Q

Renail Failure a

A

2o Angiomyolipomas and cysts in kidney

may occur

53
Q

Tuberous Sclerosis - Anesthesia

A

–Upper airway abnormalities may be present
–Impaired renal function possible – keep in mind
when selecting drugs dependent on renal clearance
–Intra-op. cardiac arrhythmias may occur
–Seizures may be present

54
Q

Von Hippel-Lindau Disease

A

Formation of retinal angiomas, hemangioblastomas
& cysts in kidneys, adrenal glands and CNS
(especially in cerebellum)
Pheochromocytoma often present

55
Q

Von Hippel-Lindau Disease

Anesthesia Management: Possible spike and send

A

Poss. Spikes in BP if Pheochromocytoma present

Treatment with labetalol or sodium nitroprussid

56
Q

Von Hippel-Lindau Disease Spinal anesthesia is limited if

A

s.c. hemangioblastomas are present

Renal clearance may be affected if tumors present

57
Q

***Neurofibromatosis Anesthesia Considerations:

A

Pheochromocytoma – BP issues

Increased ICP with brain tumors

58
Q

***Neurofibromatosis Anesthesia Considerations:

Airway

A

patency if laryngeal neurofibromas present

59
Q

***Neurofibromatosis Anesthesia Considerations:

A

Responses to succinylcholine are variable

60
Q

***Neurofibromatosis Anesthesia Considerations: Cervical spine

A

defects could affect positioning for laryngoscopy

61
Q

***Neurofibromatosis Anesthesia Considerations:NDNMB

A

Increased sensitivity to nondepolarizing mscle relaxers
Regional anesthesia inj. could result in poss. future
formation of neurofibromas

62
Q

Syringomyelia Anesthesia Considerations: Thoracic Scoliosis

A

Thoracic scoliosis can contribute to V/Q mismatch

63
Q

Syringomyelia Anesthesia : NDNMB

A

Exaggerated response to nondepolarizing mscl. Relaxants

Impaired thermal regulation

64
Q

Syringomyelia Anesthesia Considerations: Succ

A

Avoid Succinylcholine – ↑ K+ release (LMN ds.)

65
Q

Syringomyelia Anesthesia Considerations

A

Protective airway reflexes may be decreased or absent – may influence timing of tracheal tube removal post-op.

66
Q

**Friedreich’s Ataxia Anesthesia Considerations: Meds

A

Avoid drugs with negative inotropic effects in the presence of cardiomyopathy

67
Q

***Friedreich’s Ataxia Anesthesia Considerations:Regional

A

Kyphoscoliosis may make administration of epidural

difficult

68
Q

***Friedreich’s Ataxia Anesthesia Considerations:RESP

A

↑ risk of respiratory failure post-op.

69
Q

Muscular Dystrophy (MD) “Duchenne’s” “Pseudohypertrophic” What is it and who is affected?

A

X-linked recessive disorder

Affects boys, with signs seen at 2 – 5 yrs. of age

70
Q

***Muscular Dystrophy Gene

A

*Gene codes for defective dystrophin protein in sarcolemma

71
Q

*** In muscular dystrophy, cells –>CCCE

A

Cells rupture and are replaced by fatty tissue
Causes pseudohypertrophic appearance
Especially noticable in calf muscles
Condition is progressive and symmetrical

72
Q

Muscles in Muscular dystrophy ? Would you expect this patient to have diminished sensation and reflexes? Why or Why not?

A

Muscle weakness and wasting

No denervation occurs – sensation and reflexes remain intact

73
Q

Muscular Dystrophy Initial symptoms:

A

Waddling gait
Frequent falling
Difficulty climbing stairs

74
Q

**Muscular dystrophy labs

A

Elevated creatine kinase levels 20– 100x above normal

75
Q

*****Muscular dystrophy Cardiac ECG changes:

A

Associated cardiopulmonary dysfunction
Tachycardia
Short PR interval: Conduction defect in AV node

76
Q

**MD and R waves:

A

Tall or polyphasic R waves: Bundle branch block

77
Q

**MD and QRS

A

Prolonged QRS

Fibrosis in bundle branches

78
Q

***MD and QT

A

Prolonged QT interval

Defect in ventricular repolarization

79
Q

***Muscular Dystrophy Anesthesia considerations: Cardio reserve

A

↓ cardiopulmonary reserve

80
Q

**Muscular Dystrophy Anesthesia considerations : ASPIRATION

A

↑ risk of aspiration 2 o to delayed gastric emptying & weak laryngeal reflexes

81
Q

***MD and Succ? and What medication should be available?

A

Avoid Succinylcholinw- ↑ risk of rhabdomyolysis & hyperkalemia
Dantrolene should be available - ↑ risk of malignant
hyperthermia in MD patients

82
Q

Obstructive Hydrocephalus

A

Ventricular CSF flow is blocked.
Usually results from a fetal developmental abnormality:
Stenosis or neural tube defect

83
Q

Obstructive, May be associated with:

A 
Arnold-Chiari malformation
Dandy-Walker cyst
Myelomeningocoele
Aqueduct stenosis
Arachnoid cyst
Neoplasm
Vascular malformation
84
Q

Surgery Treatment of Hydrocephalus: TPS

A

-To remove obstruction
-Place shunt from ventricle into the peritoneal cavity or other extracranial site
Shunts replaced as child grows

85
Q

14 yo distal neuropathies and frequent tripping falls. ON exam, weakness in all 4 limbs and notable muscle wasting of the intrinsic muscle of the hands. What is the most likely diagnostic?

A

Charcot Marie Tooth Disease

86
Q

CMT weakness GAIT ISSUES

A

Muscle weakness/Atrophy of foot & lower leg with foot

drop & high-stepped gait with frequent tripping or falls

87
Q

CMT signs: Extremities

A

Signs: Stork-leg, high arched foot, claw hands

88
Q

Weakness in CMT later progresses into?

A

Later progresses to upper body with difficulty carrying out

fine motor skills (fingers, hands, wrists, and tongue)

89
Q

Pediatric patient age 4 with difficulty climbing stairs at home, and the ladder at the neighborhood playground. HIs pelvic girdle muscles were weak, he walk with a rocking, side to side, waddling gait, and developed lumbar lordosis. His parents became concerned and sought medical advice. Lab testing revealed CK value 50 times greater than normal. What is the diagnosis?

A

Muscular Dystrophy.

SRNA3AdvancedPatho2 (15 decks)

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