Neuromuscular Dysfunction Flashcards by Brina Quintanilla Reyes

What is cerebral palsy?

Loss of control or weakness in movement

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Usually what causes these kids to get diagnosis with cerebral palsy?

Some form of injury that results in them not having enough oxygen in the brain

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Cerebral palsy may involve what? (5)
Major problems

Sensation
Perception
Communication
Cognitive
Behavior

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About 80% of kids with cerebral palsy usually end up having ___
Resulting them to be on a medication called ___

Epilepsy
Anti convulsants

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What is spasticity? (3)

Painful mass muscle spasms
Rigid posturing of limbs
Increased reflexes

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What type of condition can you get with spasticity?

Hypertonia
Which is exaggerated reflexes

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What are the 4 types of cerebral palsy?

Spastic ( pyramidal )
Dyskinetic ( extrapyramidal)
Ataxic ( extrapyramidal)
Mixed type

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What is the main thing to note about each type of cerebral palsy?
Spastic?
Dyskinetic
Ataxic
Mixed

Hypotonia
Jerking movement ; chorea
Wide gait
Everything is mixed

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What are the main things we are looking for in children with cerebral palsy? Like what is usually a great diagnosis treatment?

Following the development milestones of a child!

Around 2 year old, we are going to diagnosis them

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What is the type of test we might do for cerebral palsy to diagnose them?

CT scan
MRI scan
Metabolic and genetic testing

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Remember, we are assigning and trying to diagnose cerebral palsy, we are assessing those development milestones in these children
So what might we see ?? (7)

Persistent primitive reflexes
Poor head control after age 3 months
Stiff or rigid limbs
Arching back ; pushing away
Floppy tone
Unable to sit without support at age 8 months
Clenched fists after age 3 months

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At what age should head control be achieved?

At what age should a child sit up without support?

At what age should babies hands be open ?

Remember primitive reflexes should be gone before 4-6 months !

1-3 months before hand

6 months

3 months

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What is the therapeutic management for cerebral palsy?

Establish locomotion, communication and self help skills

Optimal appearance and intervreation of motor functions

Correct associated defects as effectively as possible

Provide adapted education opportunities

Promote socialization experiences with other affected and unaffected children

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What are some intervention we might see with cerebral palsy? (6)

Physical therapy ( stretching )
Occupational therapy
Speech therapy
Casting & bracing
BOTOX
Oral medications

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What are your main 4 types of medications we might use on these children with cerebral palsy?

Antispamodics
Anti epileptics
Anti inflammatory
Analgesia

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It’s important to remember they may have alternation of their speech, motion and fine details

However there is something to note
About 70% of these patients have a normal IQ and can achieve many amazing things

So how are we going to talk to them?

Normally !!

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Neural tube defects
What is it?

When the brain, spinal cord and vertebrae are developing in the uterus and there is a failure to close of a neural tube

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When does neural tube defects happen?

1-2 weeks of the pregnancy

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What are some things we are going to do and educate patients about the prevention of a neural tube defect?

How much?

Folate acid!!

0.4 mg/day

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What are some causes of a neural tube defect?

There is 1 main one

Low folate levels !!

Drug, chemical or radiation exposure
Genetic mutation in folate pathways
Syndrome like downs
Pre-pregnancy obesity
DM
Low vitamin b12

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What are some foods that may help increase the folic acid intake?

Cereals!

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What Is another diagnosis with may use to help detect a neural tube defect in children?

At what weeks do we test this at?

Elevated Alpha fetoprotein (AFP) in amniotic fluid

6-18weekz

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What are the two main types we see with neural tube defects?

Anencephaly
Spina bifida myelomeningocele

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What is anencephaly?

Absense of cerebral hemispheres and only a brain stem ( there is no brain ! )

( AN - means NO! )

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Is anencephaly incompatible with life? And if so what’s the main cause of death?

Yes, most kids die within a few days of life Most kids die to respiratory failure e

What is spina bifida occulta?

Tethered cord - abnormal adhesion to a bony or fixed structure So the spinal cord is attached to fatty growth

So for spinal bifida occulta It puts traction on the cord because the abnormal fatty growth attached to the spinal cord What might this effect? (3)

Altered gait Bowel and bladder problems Foot deformities

Spinal bifida occulta can be seen in which section of the spine?

L5 to S1

What are some skin indicators of spina bifida occulta? (4)

Sacral dimple Sacral angioma of port wine nevus Sacral tufts of dark hair Sacral lipoma

What is mainly the diagnosis though once we see one of the 4 skin indicators, like that pig tail? Another thing to note about this, what might be happening to the baby lower legs?

Usually an ultrasound!! CT, MRI, X-ray They won’t be moving

What is spina bifida cystica?

Visible defect with external sac-like protrusion So like a visible sac outside of the back!

What are the 2 types of spina bifida cystica?

Meningocele Myelnomeningocele

What is meningiocele?

So we have that massive sac, however there is no spinal cord in the sac Usually easier to close up

What is myelomeningocele?

The spinal cord wraps around in the sac

With the myelomeningocele, a good portion of things that you can see with these patients is that once you located the location and magnitude of the defect, it can show what? Like if the defect is below the second lumbar vertebra - flaccid paralysis of lower extremities - sensory deficit

The paralysis or main sensory issue they will develop

What issues will these children with spinal bifida have?

Bowel And urine issues

Since patients with spinal bifida have issues with bowel and urination what might that indicate? What might he the treatment? However with doing the treatment so much, what does that poses them to have? - so they are on what?

They can’t control when they go Urine Incontience : improper emptying of the bowel End up with residual urine - an infection risk Straight catheter Latex allergy Latex precautions

Anyone that has paralysis or has loss of sensation What’s is the biggest thing to do? Because if they constipation What happens?

Bowel regiment stuff Impaction

What is spinal muscular atrophy ? It’s a degeneration of ? What is the onset? Main death?

Type of progressive weakness and wasting of skeletal muscules Spinal cord and brainstem - resulting in atrophy in skeletal muscles Usually early in life Respiratory failure because it weakens the body so much and also they lose the ability to swallow so they can’t eat

What are some symptoms of muscular atrophy? (6)

Fatigue Difficulty swallowing breathing difficult GI issues Scoliosis Muscle weakness

What is the diagnosis of progressive muscle atrophy? (2)

Genetic testing Muscle biopsy

So it’s important to note that with muscule atrophy, it’s a multi organ implications!!!!

What is muscular dystrophies?

Progressively disable Just a main term

What is duchenne muscular dystrophy?

Mainly boys, X-linked Life long disable and usually death is soon around 20

What is gower sign?

A child is laying down on the belly But the child has weakness in the buttocks and legs - therefore they do a progressive walking up their legs They can’t just stand up!

What is the theraupetic management of DMD? Duchenee

No real treatment Keep kids active !! ROM! Genetic testing

What is Guillain-Barré syndrome? Infectious polyneutritis Happens more in adults Males more than females

Acute demyelinating polyneuropathy with progressive paralysis

What is the clinical manifestation of GBS? Name the steps (5)

Muscle tenderness Paraesthesia Muscle weakness Ascending paralysis weakness from the lower extremities to the top Loss of reflexes Frequent urinary incontience or retention and constipation Be careful because it can be life threatening respiratory paralysis

What are the main causes of GBS?

Viral Bacterial Vaccine infections

therapeutic management of GBS? (5)

Steroids IV immunoglobuin Heparin, stool softeners Respiratory support Plasmapheresis

What is botulism?

Giving Honey to kids under 2 years old

When does botulism symptoms occur?

12-36 hours after ingestion

What are the general signs and symptoms of botulism?

Weakness Dizziness Headache Diplopia Speech difficulties Vomiting Progressive life threatening respiratory paralysis

What is the treatment of botulism? However what is sad about this?

IV botulism antitoxin Kids under 2 can’t receive this :( so they usually end up dying

Why can’t we give honey to kids under 2 years old?

It isn’t pasteurized

Hypotonia usually is associated with botulism What is it?

Decreased muscle tone Usually head tone is completely gone :(

What is myasthenia gravis?

Acetylocholine is blocked and doesn’t get to the muscle cell

What is the main clinical manifestation of myasthenia gravis? (7) Think of the anagram

Ptosis- eyelids dropping Muscle weakness Fatigue Blurry or double vision Limited facial expression Trouble walking Speech,swallow,chew problems people Make Faces But Limited To Seven!

Myasthenia gravis Patients have a hard time with what with their eyes? But not a hard time with what with their eyes?

They have hard time closing eyes, but easy to open the eye

Usually patients with MG, they will get so exhausted just by talking so they will need to what? And some patho behind this ^

Rest So it allows the acetacholyin to accumulate in the receptor which then allows them to contuine on

What is spinal cord injury Diving Hyper extension of the neck Hyper Flexion of the neck What are the top 5 causes?

Vehicle crashes Falls Violence Sports Others

C4 spinal injury C6 spinal injury T4 spinal injury L1 spinal injury What is the paralysis

C4 - neck down C6 - shoulder down ( shoulder still move ) T4 - below chest paralysis L1 - waist below paralysis

What is spinal cord injury management? What type of turning movement ? It’s what?

Organized event LOG roll movement - control the head how we move !! Steroids, surgery

With spinal cord injury there is something called autonomic dysreflexia! What is it? Above the injury (3) Below the injury (2)

Above the injury Sudden flushed Bradycardia Throbbing headache Down below Pale, cool, clammy skin Hypertension

What is the safety with autonomic dysreflexia? Solve the problem?

90 degrees angle Sit up!!