Static Neurological Disorders Flashcards
Cerebral palsies
Group of disorders of movement, muscle, or posture
Cerebral palsies caused by
injury of abnormal development of immature brain from NONPROGRESSIVE CAUSES (NOT CANCER)
__% of patients were immature
40
___-% acquired prenatally
Some etiology
80; Associated with prematurity, low birth weight, placental insufficiency, maternal infection and pyrexia, intrauterine infections, intrauterine growth retardation,intracranial hemorrhage, and trauma
Children required a lot of _______therefore need _______clinician should worry about _________
The motor deficit may be classified as ________
Imaging studies; GENERAL ANESTHESIA
HYPOTONIA, SPASTICITY and EXTRAPYRAMIDAL features
Common comorbidities include_________that can lead to 5 major REDMR
3 Frequent procedure done are: FGE
Oromotor dysfunction (reflux, recurrent aspiration, decrease resp reserve, esophageal stenosis, malnutrition
common procedures : fundoplication, gastrostomy, esophageal Dilation
Leading cause of Death from pulmonary complications is _______That can be excerbated by ______
What else can impair pulmonary function ____
Aspiration ; BULBAR DYSFUNCTION; RECURRENT INFECTION, CHRONIC LUNG DISEASE
SCOLIOSIS (depending on severity)
You may not give
Ketamine because of bronchodilation
What percentage with epilepsy _____and its more common with children with ________
30%
Spastic Hemiplegia / quadriplegia
Epilepsy less common with _______forms
Ataxic forms
Anesthesia consideration
Risks inability to walk, severe neurologic deficit, major cognitive dysfunction, SEVERE SCOLIOSIS, GASTROSTOMY and TRACHEOSTOMY
General scoliosis
Predispose to DIFFICULT VENTILATION
AT HIGHER RISK MALIGNANT HYPERTHERMIA
Do not stop this mediciaton
Baclofen
Baclofen and _________ can potentiate
Dandrolee; Neuromuscular blocking
Baclofen and dandrolene , decrease dose of
Decrease dose of NMB agents
Cp patients have _____MAC and ______BIS which affect ______and _________
IV?
With intubation perform
SUCC OK?
Decrease MAC/ BIS (which affect maintenance and emergence) titrate drugs to effect Contractures make IV insertion difficult DO RAPID SEQUENCE INTUBATION Succ is ok , NORMAL K release
Neural tube defects 2 general types
Cranial and spinal dysraphism (not fused)
Neural Tube defects include (3)
Ancephaly
Encephaloceles
Spina bifida
Types of Causes : Broad types
3
genetics and environmental
Folate deficiency during prenatal
Maternal Anti-epileptic drugs
Maternal Diabetes
Ancephaly is ______and is the ________. Some ________are intact and the _______may develop normally.
Ancephaly infants are usually_____,_____ and ______How long to day survive?
Lethal; absence of skull formation
Deep cerebral structures ; Brainstem
Blind, deaf or unable to feel pain
Hours to days
Encephalocele
What are the two types and parts affected?
what is common and the treatment ?
Most infants _____and those who live have severe
Herniation of neural tissue/meninges out of skull through deficient skin and bone
anterior: affect brain, orbital structures and pituitary gland
Posterior: Cerebral OR cerebellar tissue herniation through posterior cranium
HYDROCEPHALUS is common ==> SHUNT PLACEMENT
MOST INFANTS die; Developmental abnormalities
Spina Bifida Can involve \_\_\_\_, \_\_\_\_ and \_\_\_\_\_elements Present at birth with more severe /open elements If uncovered at birth ? Later in childhood?
-Group of condition in which there is An abnormal or incomplete formation of midline structures over back
Skin, Bone and Neural elements
- SPINA BIFIDA
- These defects may present at birth, as in the case of the more severe and open lesions (i.e.,spina bifida)
- be identified later in childhood if the skin overlying the
spinal defect is intact (e.g., spina bifida occulta).
All NTD patients are prone to
Spina BIFIDA OCCULTA (ACT)
Latex allergies due to repeated exposures
Spina bifida occulta occurs in the
- Absence of herniation of neural tissue or coverings so that the overlying skin appears to be intact and normal.
- Commonly have a hairy patch or dermal sinus/dimple at level of abnormality
- The spinal cord may be tethered by internal connection to such structures, making it vulnerable to trauma at surgery and during growth, especially at puberty.
Spinal Bifida CYCSTICA
Spinal CORD
OBVIOUS LESIONS On the back
Morbidity Review
Chiari type I
Symptoms? abnormalities
what is normal?
Predisposed to ________(____%)
low symptoms, no neurological symptoms
Not usually associated with any abnormalities
Normal brainstem and fourth ventricle
Predisposed to having syringomyelia (20-70%) (glial cells within the spinal cord)
Chiari type II
This is a more extensive and complex abnormality than the
Chiari I malformation, with INFRATENTORIAL and SUPRATENTORIAL abnormalities
Both upper and lower brain abnormalities
Cerebellar tonsils, inferior vermis, 4th ventricle and
brainstem herniated through foramen magnum c/ CSF obstruction
CSF obstruction
**ALWAYS have meningocele or MenigoMYELOCELE
NOT always have hydrocephalus
CHIARI III
WORSE TYPE
Very rare, There is herniation of the posterior
fossa contents into an associated occipital or high cervical cephalocele with the other features of a Chiari II malformation.
They have severe neuro defect and POOR PROGNOSIS
Chiari IV
Vanishing cerebellum
Progressive Neurological Disorders
% of childhood malignancies
___occurs before ___
____occurs before ____
What is the 2nd most common tumor in pediatric?
Pathology classification depend (2)
15%-20%
1/3 occur before 5 yrs
75% before 10 yrs
CNS tumors 2nd most common in peds
Pathology classification depend on site/cell of origin and degree of malignancy (grade I-IV)
Ages 2-3 yrs typically have______tumors
supratentorial tumors
4-10 yrs = ________Tumors
infratentorial tumors
Adults typically have
Supratentorial tumors
2/3 tumors located
infratentorial (1/3 supratentorial)
Increase ICP
Cannot to Spinal tap
NO NEURAXIAL Anesthesia
Tumors
Common clinical features
***↑ICP Ataxia **CN palsies Visual impairments Seizures ***Hydrocephalus Hemiparesis ***Dystonia
Most common tumor is
Hemisphere GLIOMA
Manifestations
Infants: FDH
Older children :
If the tumor is rapidly expanding you will see –>CIO
What diagnosis test?
Infants = ↑head circ., FTT, developmental regression
Older children = HA, N/V, Sz, gait/visual disturbances
Rapid expanding →cerebral edema, obstruction of CSF → ↑ICP
Diagnosed via MRI, biopsy would be ideal
Supratentorial (manifestation signs)
Seizures, focal neuro deficits, personality changes, visual defects, endocrine dysfunction
Infratentorial (CNI)
◦ Rapid expanding →cerebral edema, obstruction of CSF → ↑ICP
Cerebellar ataxia, N/V, ↑ICP
Chemoreceptor trigger ZOne (CTZ) is
Outside BBB
The only visual increase ICP
Papilledema
Treatment =
Might have
combination of surgery, chemo, radiation
Control cerebral edema prior to surgery with Corticosteroids
Medically optimize (F/E)
Medicate and control seizures
Might have permanent neurological deficits (epilepsy, learning disabilities, audio/visual
problems)
____ years survival rate? ______For ______brain tumors
***5 year survival rate > 60% for primary brain tumors
Spinal cord tumors = rare
Usually______ or ______
Can be ________ or _______
Nonspecific, vague signs at first
Increase risk or what?
If DECOMPRESSION Is delayed can lead to (VI)
Treatment: ________but only _______
astrocytomas (60%) or ependymomas
inside cord (intramedullary) or outside
(extramedullary)
↑risk for spinal cord compression
Delayed decompression →vascular compromise →irreversible paralysis of limbs
Surgery, but only if possible Intramedullary tumors may be impossible to excise–> More damage than good
Neuromuscular disorders are caused by an abnormality of lower motor neuron system (MANA)
anterior horn cell in the spinal cord, axon, neuromuscular junction, or muscle fiber
Clinical manifestation of LMN issues (3)
Clinical features:
weakness
hypotonia
reduced DTR
Common associations: contractures, scoliosis,
respiratory/cardiac involvement
Spinal muscular atrophies: There is progressive ________Of __________ cells of _____and ______Leading to __________
the lower the _____
Classification ?
Progressive degeneration of anterior horn cells of spinal cord and brainstem nuclei leading to death of neurons
THE LOWER THE CLASSIFICATION THE WORST
Classified SMA0-4
0 = worst (severe contractures, facial diplegia, resp. failure)
4 = adult onset
Poliomyelitis
_____phases:
Asymmetrical, affects______]
May require?
Poliovirus = highly contagious
2: 1. acute nonspecific febrile illness FOLLOWED BY
2. aseptic meningitis and acute, flaccid, lower motor neuron paralysis
asymmetrically and may affect any muscle group, may require mech vent
ANESTHESIA and POLIO
You cannot give what to a poliomyelitis patient?
Succinylcholine
Various sensitivity to NDNMB
Neuraxial anesthesia used without adverse effects
Resp depression c/neuraxial opioids
Most common types of Axonal Disorders
Charcot-Marie-Tooth disease
Axonal disorders Hereditary neuropathies
Two major subgroups: neuropathies only, neuropathies part of more generalized neurologic/multisystem disorder
5 common types
MOST COMMON ______
Hereditary neuropathy c/liability pressure palsy
Hereditary sensory and autonomic neuropathy
Distal hereditary motor neuropathy
Hereditary neuralgic amyotrophy
Associated with other neuro/multisystem disorders: Familial amyloid polyneuropathy Lipid metabolism abnormalities *****Porphyrias DNA disorders Mitochondrial dz Hereditary ataxias
NOT DO REGIONAL ANESTHESIA
Acquired Disorders of Peripheral Nerves
Rare in childhood
Autoimmune where body attacks Ach receptors (Ach
Associated with nutritional deficiencies
Vit. E, B1, B6, B12, niacin, thiamine
Types:
Guillain-Barré Syndrome, Chronic Inflammatory Demyelinating Polyneuropathy, Nerve Palsies, Disorders of NMJ (Myasthenia Gravis = MG)
Juvenile MG Treated with ?
What procedure may be done
Treated with acetylcholine esterase inhibition (pyridostigmine) and immunosuppression
THYMECTOMY
Anesthesia considerations for MG Succ ? dose and why? Metabolism NDNMB NNBD How should intubation be performed? What types of patients need ET tube?
Require 4x succinylcholine dose(Decreased density
of acetylcholine receptors at the motor end plate means that children with myasthenia may require up to four times the calculated dose of succinylcholine to establish a depolarizing muscle block.
Because succinylcholine is metabolized by acetylcholinesterase, its metabolism is reduced and DURATION OF ACTION IS PROLONGED in the setting of chronic acetylcholinesterase inhibition. For this reason, succinylcholine is best avoided in these children
The activity of nondepolarizing NMBDs is increased through their increased duration of action
Inhalational anesthetic agents inhibit neuromuscular transmission, and these effects may be exaggerated in myasthenia gravis patients.
TIVA might be better choice
If possible, intubation of the trachea should be performed without the use of NMBDs.
Pt with Bulbar dysfunction and/ or resp problems = ETT
Disorder of Muscle Fibers
Myopathies
Common findings in congenital myopathies:
Facial weakness (c/ s/ ptosis) hypotonic “frog legs”
weakness/dysfunction of resp/bulbar muscles
Metabolic myopathies: The most important concerns for the anesthesiologist are the risk of
metabolic instability during surgery and the need for close liaison with the child’s pediatrician to plan fluid and
electrolyte balance and nutritional management
Anesthesia Considerations for Muscle Fiber
Disorders
Maintain
High risk for ?
All inhalational agents + propofol ==>
Prolonged __________defined as ( _________)–> can lead to ______________
What can propofol infusion syndrome lead to
They have an _______________
Do not use
Maintain metabolic stability and prevent complications
- High risk of resp. failure, cardiac depression, conduction defects, and dysphagia Minimize NPO times and stress
- All inhalational agents + propofol depress mitochondrial function
Prolonged propofol infusion (>5mg/kg/hr for > 48hrs) → propofol infusion syndrome
–>Metabolic acidosis with myocardial failure
-Unpredictable responses to NDNMBs
NO with remifentanyl
Propofol can
Depresses myocardial function
can use NO with remifentanyl
MH predisposed conditions:
Conditions predisposed: Duchenne and Becker
muscular dystrophy; central core (scoliosis) and
multiminicore disease; nemaline rod myopathy;
King-Denborough and hyperCKemia
MH not predisposed conditions
NOT predisposed: myotonic dystrophy, inflammatory myopathy, mitochondrial myopathy, Brody disease
MH mechanism of action
mutations in the ryanodine receptor gene (RYR1) in most affected individuals, accounting for their susceptibility to MH.
RYR1 gene encodes the channel that controls calcium release from the sarcoplasmic reticulum in skeletal muscle RYR1 abnormalities alter the channel kinetics for calcium inactivation, and calcium buildup causes excessive skeletal muscle contraction resulting from disinhibition of the normal actin-myosin interaction.
The level of adenosine triphosphate decreases, leading to anaerobic and aerobic metabolism and to acidosis
Disorder of Ca2+ regulation in skeletal muscles
Ryanodine receptor (RyR1)
Encodes channel that releases Ca2+ from SR
Inappropriate Ca2+ release→ sustained skeletal
muscle contraction →↓ATP →metabolic acidosis
Muscular Dystrophies
Clinical features
30 inherited disorders of muscle
Occur through all stages of life
No cure
Clinical features: muscle weakness, contractures, sluggish DTR, involvement of resp/cardiac muscles
Correlated with learning disabilities, deafness, ophthalmologic disorders
Most common muscular dystrophy
Regional encouraged to ↓narcs
Myotonic dystrophy
Susceptible to prolonged recovery from anesthesia
Regional encouraged to ↓narcs
Dystrophinopathies are the _______
What are the 2 most common?
Which one is more severe? milder?
what is it a deficiency of ?
What is the role of that protein? essential to _____and reinforce
what other thing is that protein involved in?
Between age 6-8 ?
Muscle tear from _____and use of _____As they get older they have ROC ?
What is recommended at what age? to find what ?
2nd most commonly encountered dystrophy
Duchenne muscular dystrophy (DMD) = severe (1st most common is myotonic dystrophy)
Becker muscular dystrophy (BMD) = milder c/later onset
Deficiency in dystrophin
Dystrophin = muscle membrane protein essential to skeletal/cardiac muscle cytoskeleton and neural tissue
Reinforces inner strength of myocyte during stretching; involved in signal transduction
Deterioration in muscle strength between 6-8 yrs, progresses until adolescence (usually paraplegic by then)
Muscles tear from normal stretching and Succinylcholine
↑ age associated with ↑ respiratory, cardiac, and orthopedic complications
> 10 yrs ECHO recommended 1-2x/yr –> Cardiomyopathies
Anesthesia Considerations for Muscular
Dystrophies
Preop assessment
Rhabdo from anesthetic agents (Succ and inhalational agents) can mimic MH (DMD - Coté says MH risk is same as gen pop, Barash disagrees)
1st : Hyperkalemic arrhythmias treated c/ 10mg/kg calcium repeated until cessation
Other interventions: hyperventilation, bicarbonate, albuterol, insulin c/glucose, kayexalate
Can be refractory
Pre-OP
Ask family about normal physical activities and Hx of dystrophinopathies
30% have no family Hx
Suspicions = random serum creatine phosphokinase concentration
K inside of the cell about
120
Epilepsy
0.5-1% school-aged
Recurrent spontaneous seizures = electrical discharge of
cortical neurons
Can involve part or all cerebral cortex
Self-limited vs continuous (status epilepticus)
Syndromes
Benign, childhood, primary generalized, benign focal, lesion focal epilepsy
Epilepsy found out
SPECIAL DIET?
Advance in Epilepsy treatment is _____
Seizure type Age Associated features EEG features Interval from last seizure Current medication management Alternative non-drug therapies
KETOGENIC DIET
Vagal nerve stimulation
Managing epilepsy
Provocations of Seizures
DEHENCCPD
Treat underlying cause
Prevent seizures with antiepileptic drugs
Provocations of seizure
Disrupted medication schedule Epileptogenic anesthetics Hypoxia Electrolyte disturbances Neurosurgery Cerebrovascular instability Coincidental exacerbation Postoperative ileus →poor drug absorption Disruption of ketosis (D5NS)
Schedule surgery around medication schedule
Late morning
Early Afternoon
Anesthesia and Epilepsy
3 things to do
-Consult the physician who manages the epilepsy
-Schedule around medication schedule
Most are given q12h, so late morning to early afternoon might be best
- Thorough pre-op assessment with documentation
For anesthesia and Epilepsy, if the patients regular drug not available administer
Phenytoin loading dose 15-20mg/kg at a rate of 50 mg/min and then BID maintenance dose of 2.5-5 mg/kg
What can be use as a rescue drug for patients with epilepsy
Benzodiazepine
Important not to interfere with
Maintain blood glucose?
Do not interfere with a ketogenic diet
Maintain blood glucose 55-70 mg/dL
If low, give 2.5-5%NS
Orthopedic surgeries – very common in Cerebral palsies . It is common for the patients to have ___________
Types are
◦ Multiple levels done at once, no staging like with adults
◦ Types: tendon release, femoral osteotomy, hip adductor/iliopsoas release, spinal fusion
In spina Bifida CYSTICA, The abnormally developed spinal cord may be covered by a layer of meninges aka _______or remain uncovered_________
(i.e., meningocele) ; (i.e., myelomeningocele).
In spina bifida CYSTICA what is the major determinant of morbidity ?
The spinal level of the lesion is the major determinant of morbidity
Do not do a ________block in _______patients why?
Caudal
Spinal Bifida OCCULTA because the spinal cord may end at an unusually low position
If clinician have high suspicion of muscular dystrophy, they should do a
random serum creatine phosphokinase (CK) concentration
Epilepsy is associated with ___________ So The first and most important step in managing epilepsy is to
Establish a correct diagnosis by
determining
whether the episodes are epileptic, classifying the episodes, and identifying the epilepsy syndrome that matches the clinical features
