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SRNA3AdvancedPatho2 > FINAL -NEURO congenital > Flashcards

FINAL -NEURO congenital Flashcards

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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
**For CP patients going to surgery -
important to continue Baclofen throughout perioperative period
26
***Baclofen Abrupt discontinuation can result in withdrawal symptoms
Seizures
27
**Common Surgeries for CP patients | Surgical Treatments usually aimed at
correcting musculoskeletal disorders:
28
Types of surgeries for CP
Achilles' tendon lengthening Osteotomy of the femur Iliopsoas release Scoliosis correction
29
**CP – Anesthesia Considerations | Pt.s have
↑ incidence of reflux with weak pharyngeal & | laryngeal muscles
30
**CP – Anesthesia Considerations ↑ susceptiblity to
hypothermia - require close temperature monitoring
31
**CP – Anesthesia Considerations Tracheal extubation may need to be
delayed until patient is fully awake and airway reflexes return
32
**CP – Anesthesia Considerations | Pt.s have MAC is generally
decreased and emergence times are longer
33
**CP and SUCC
CP – Anesthesia Considerations Succinylcholine ok to use No exaggerated hyperkalemic response
34
CP and seizures medications
Antiseizure medications common in CP patients – may lead to subsequent resistance to nondepolarizing muscle relaxants
35
***Spinal Bifida
Common neural tube defect Failure of posterior spinous processes to fuse Permit meninges and spinal cord to herniate Result in neurologic impairment
36
****3 Types of Spina Bifida
Occulta Meningocele Myelomeningocele
37
Spina Bifida Occulta
Spinous processes do not fuse. | No herniation of spinal cord or meninges
38
Defect may not be visible
Dimple or tuft of hair may be present on the | skin over the site.
39
Meningocele
Herniation of meninges through the defect. Meninges and CSF form a sac on the surface. Transillumination confirms absence of nerve tissue in sac.
40
Myelomeningocele
Most serious form Herniation of meninges & spinal cord Considerable neurologic impairment
41
Charcot Marie Tooth Disease
The most common inherited cause of chronic motor & | sensory peripheral neuropathy
42
**CMT – Anesthesia Considerations
Avoid succinylcholine ↑ K+ release in neuromuscular ds.
43
With CMT , Use caution with and why?
neuromuscular blocking drugs↑ risk of respiratory failure post-op 2o to muscle weakness
44
***Chiari Malformations Type I -
cerebellar tonsils protrude into foramen magnum without brainstem involvement. Most common type
45
***Chiari Malformations Type I - | Type II –
“Arnold-Chiari” – same with brainstem herniation Usually occurs with Spina Bifida myelomeningocele and obstructive hydrocephalus.
46
Chiari Malformations Treatment with
surgical decompression
47
***Chiari Malformations Anesthesia Considerations for patients undergoing surgical decompression?
include increased ICP and significant blood loss, especially in Type II
48
Tuberous Sclerosis | What is it associated with ?
Seizures and/or mental retardation may be present | There is an association w/ Wolff-Parkinson-White Syndrome
49
WPW
•Accessory Pathway •Causes paroxysmal tachycardia
50
WPW ECG changes:
* Delta wave * Shortened PR interval * Wide QRS complex
51
Tuberous Sclerosis : Fibromas
Nodular fibromas/papillomas may occur on tongue, | palate, pharynx and larynx
52
Renail Failure a
2o Angiomyolipomas and cysts in kidney | may occur
53
Tuberous Sclerosis - Anesthesia
–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
Von Hippel-Lindau Disease
Formation of retinal angiomas, hemangioblastomas & cysts in kidneys, adrenal glands and CNS (especially in cerebellum) Pheochromocytoma often present
55
Von Hippel-Lindau Disease | Anesthesia Management: Possible spike and send
Poss. Spikes in BP if Pheochromocytoma present | Treatment with labetalol or sodium nitroprussid
56
Von Hippel-Lindau Disease Spinal anesthesia is limited if
s.c. hemangioblastomas are present | Renal clearance may be affected if tumors present
57
***Neurofibromatosis Anesthesia Considerations:
Pheochromocytoma – BP issues | Increased ICP with brain tumors
58
***Neurofibromatosis Anesthesia Considerations: | Airway
patency if laryngeal neurofibromas present
59
***Neurofibromatosis Anesthesia Considerations:
Responses to succinylcholine are variable
60
***Neurofibromatosis Anesthesia Considerations: Cervical spine
defects could affect positioning for laryngoscopy
61
***Neurofibromatosis Anesthesia Considerations:NDNMB
Increased sensitivity to nondepolarizing mscle relaxers Regional anesthesia inj. could result in poss. future formation of neurofibromas
62
Syringomyelia Anesthesia Considerations: Thoracic Scoliosis
Thoracic scoliosis can contribute to V/Q mismatch
63
Syringomyelia Anesthesia : NDNMB
Exaggerated response to nondepolarizing mscl. Relaxants | Impaired thermal regulation
64
Syringomyelia Anesthesia Considerations: Succ
Avoid Succinylcholine – ↑ K+ release (LMN ds.)
65
Syringomyelia Anesthesia Considerations
Protective airway reflexes may be decreased or absent – may influence timing of tracheal tube removal post-op.
66
****Friedreich’s Ataxia Anesthesia Considerations: Meds
Avoid drugs with negative inotropic effects in the presence of cardiomyopathy
67
***Friedreich’s Ataxia Anesthesia Considerations:Regional
Kyphoscoliosis may make administration of epidural | difficult
68
***Friedreich’s Ataxia Anesthesia Considerations:RESP
↑ risk of respiratory failure post-op.
69
Muscular Dystrophy (MD) “Duchenne’s” “Pseudohypertrophic” What is it and who is affected?
X-linked recessive disorder | Affects boys, with signs seen at 2 – 5 yrs. of age
70
***Muscular Dystrophy Gene
*Gene codes for defective dystrophin protein in sarcolemma
71
*** In muscular dystrophy, cells -->CCCE
Cells rupture and are replaced by fatty tissue Causes pseudohypertrophic appearance Especially noticable in calf muscles Condition is progressive and symmetrical
72
Muscles in Muscular dystrophy ? Would you expect this patient to have diminished sensation and reflexes? Why or Why not?
Muscle weakness and wasting | No denervation occurs – sensation and reflexes remain intact
73
Muscular Dystrophy Initial symptoms:
Waddling gait Frequent falling Difficulty climbing stairs
74
****Muscular dystrophy labs
Elevated creatine kinase levels 20– 100x above normal
75
*****Muscular dystrophy Cardiac ECG changes:
Associated cardiopulmonary dysfunction Tachycardia Short PR interval: Conduction defect in AV node
76
**MD and R waves:
Tall or polyphasic R waves: Bundle branch block
77
**MD and QRS
Prolonged QRS | Fibrosis in bundle branches
78
***MD and QT
Prolonged QT interval | Defect in ventricular repolarization
79
***Muscular Dystrophy Anesthesia considerations: Cardio reserve
↓ cardiopulmonary reserve
80
****Muscular Dystrophy Anesthesia considerations : ASPIRATION
↑ risk of aspiration 2 o to delayed gastric emptying & weak laryngeal reflexes
81
***MD and Succ? and What medication should be available?
Avoid Succinylcholinw- ↑ risk of rhabdomyolysis & hyperkalemia Dantrolene should be available - ↑ risk of malignant hyperthermia in MD patients
82
Obstructive Hydrocephalus
Ventricular CSF flow is blocked. Usually results from a fetal developmental abnormality: Stenosis or neural tube defect
83
Obstructive, May be associated with:
``` Arnold-Chiari malformation Dandy-Walker cyst Myelomeningocoele Aqueduct stenosis Arachnoid cyst Neoplasm Vascular malformation ```
84
Surgery Treatment of Hydrocephalus: TPS
-To remove obstruction -Place shunt from ventricle into the peritoneal cavity or other extracranial site Shunts replaced as child grows
85
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?
Charcot Marie Tooth Disease
86
CMT weakness GAIT ISSUES
Muscle weakness/Atrophy of foot & lower leg with foot | drop & high-stepped gait with frequent tripping or falls
87
CMT signs: Extremities
Signs: Stork-leg, high arched foot, claw hands
88
Weakness in CMT later progresses into?
Later progresses to upper body with difficulty carrying out | fine motor skills (fingers, hands, wrists, and tongue)
89
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?
Muscular Dystrophy.

SRNA3AdvancedPatho2 (15 decks)

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