PTE 753: exam 1

myelsodysplasia, brain tumor

what is myelodysplasia

a birth defect in which there is abnormal development of the back bones, spinal cord, surrounding nerves, and the fluid-filled sac that surrounds the spinal cord

• aka spina bifida

list the 6 types of myelodysplasia.

1. myelomeningocele (spina bifida cystia)

2. meningoceles

3. lipomas of the spinal cord

4. myelocytoceles

5. diastematomyelia

6. spina bifida occulta (spina bifida aberta)

myelomeningocele (spina bifida cystia)

spinal cord (nerves too) and meninges protrude through an opening in the spine usually resulting in some form of paralysis

what is another way to describe myelomeningocele lesions?

failure of the ectoderm of the notochord to form a tube during neuorulation

meningoceles

sac-like protrusion of the meninges due to a defect in the spinal column in which a cyst may form that is usually not associated with paralysis

lipomas of the spinal cord

subcutaneous fat masses of the spinal cord which may lead to paralysis depending on the location of the mass

myelocyctoceles

spinal cord’s central canal expands into a cyst and protrudes through a gap in the spine ultimately compressing nerves

• may or may not lead to nerve impairment tho

diastematomyelia

bony bands that split the spinal cord into 2 hemicords

spina bifida occulta (spina bifida aberta)

incomplete closure of the vertebra and failure of the dura mater to form around the spinal column, leaving a gap

• usually not associated with paralysis

what are the 2 etiology possibilities that result in spina bifida?

1. failure of the ectoderm of the notochord to form a tube during neurulation (within the first 28 days of pregnancy)

2. failure below the S2 vertebra of groups of nerve cells to clump together and form canals during canalization

what are some other possibilites that may result in a baby developing spina bifida?

• genetic abnormalities (multifactorial)

• nutritional deficiencies of folic acid

• increase alcohol consumption of mom and increase in valpaouric acid

• teratogens and chromosomal defects

describe the meaning behind the “lemon” and “banana” signs found in an ultrasound taken of a baby with spina bifida.

lemon sign: flattening or indentation of the frontal bones

banana sign: the cerebellum appears curved due to a small posterior fossa and the downward displacement of the cerebellum (arnold-chiari malformation)

T or F: early surgical intervention may reduce UMN symptoms and cognitive consequences of spina bifida and decrease level of SCI by 2 levels.

T

what are some examples of early surgical interventions for a baby with spina bifida?

• prelabor C-section

• cover and close spine (post-natal)

• ventriculoperitoneal (VP) shunt

• in-utero closure

what is Arnold-Chaiari Malformation?

a structural defect where the lower part of the brain, including the cerebellum and brainstem, extends into the spinal canal due to the the base of the skull being too small or misshapen

what are the signs of hydrocephalus?

• enlarged head

• separation of skull sutures

• bulging fontanelle

• sunset sign

why might a patient present with UE involvement if she is suffering from hydrocephalus?

the expanding ventricles compress the motor pathways, primarily the UE ones due to location/orientation, leading to UMN signs and symptoms

• weakness, poor coordination, spasticity

T or F: spasticity of the UE is likely a complication of hydrocephalus.

T

what are some other complications that result from hydrocephalus?

• visupoerceptual deficits → vestibular issues

• cognitive function

• awareness in space

• seizures

• crainal nerve palsy/ bulbar symptoms

what are some examples of the signs and symptoms associated with shunt dysfunction?

your patient is a regular and was diagnosed with spina bifida during infancy. one day, she complains of recurring headaches, a decrease in energy levels, and you note an increase in spasticity. you also notice she presents with a decrease in cognition levels compared to normal but she waves it off as “just being too tired to think”. why should you be concerned?

she is suffering from a shunt dysfunction and needs to go to the hospital ASAP rocky

what are the two key signs and symptoms of shunt dysfunction?

1. decrease cognition

2. personality change

syringomyelia

cyst forms in spinal cord proper resulting in changes in sensation, numbness, complaints of being too hot or too cold, weakness, spasticity, and increased scoliotic changes

tethered cord syndrome (TCS)

life threatening stretching of the spinal cord as the child grows but the spinal cord does not.

• leads to weakness, gait deformities, pain, scoliosis, urological changes, and deceased UE coordination

why do children who suffered a SCI experience more significant dysfunctions?

they are still growing and changing!

what are some possible dysfunctions a child suffering from a SCI might experience as he grows?

• high risk of TCS

• worsening contractures

• increase risk of UMN s/s and cognition issues

• hydrocephalus

• increase musculoskeletal issues

what musculoskeletal deformities might you see on a child with spina bifida?

• scoliosis

• hip issues

• genu valgus or varus

• equinovarus

• calcanea valgus

• torsional deformities

• contractures

why are children suffering from spina bifida more prevalent to hip dislocations, dysplasia, and/or subluxations?

their hip ADductors are stronger than their hip ABductors and the hips lack loading forces from ambulating, leading to an imbalance in musculature

what are some other medical problems associated with spina bifida?

• neurogenic bladder and bowel

• chronic kidney disease

• skin breakdown

• latex allergy

• cranial nerve palsy

• seizures

• obesity

T or F: fetal meylomeningocele repair often results in improved urological outcomes.

F; this was a quiz question

list the cognitive and psychosocial problems associated with spina bifida.

• anxiety and depression

• ADD

• perceptual, visuomotor difficulties

• attention and executive function

• social cognition

T or F: a systems review should be completed at every physical therapy visit when working with neurological patients.

T

what consists of a systems review for neurological patients?

• changes in behavior, activity level, and/or functional capabilities

• bladder and bowel control

• spinal alignment

• skin breakdown or swelling

your older spina bifida patient suddenly has increased swelling in his LEs since his last visit one week ago. why are you concerned?

this is one indication of chronic kidney disease

when testing and analyzing ROM in patients with spinda bifida, joints are often _____, meaning they are not well developed.

arthrogrypotic

• often born with severe deformities

when testing sensory in patients with spina bifida, what is the progression of tests as the baby ages?

• newborns: sharp

• 2-7 year olds: light touch and positioning (proprioception)

• 7+ year olds: temperature and two-point discrimination

instead of Thomas test, we use _____ ____ for children with spina bifida.

Staheli test

why is the Staheli test favored over the Thomas test for this population?

it allows for hip and pelvis stabilization to account for their “extreme” lordosis and/or kyphosis through spine

what are the two tools needed when assessing MMT on patients with spina bifida?

1. use a 5-point scale even with neonates

2. use a dynamometer if possible

T or F: when grading MMT with a 5 point scale, a therapist never notes his confidence because the scale is used for standardization.

F; always note confidence of MMT rating for scoring reliability with this population

what patient diagnosis should a therapist be very exact with when measuring MMT?

(a child with) tethered cord syndrome

what’s the difference between muscle tone with a neural tube defect and hydrocephalus?

neural tube defect → LMN so hypotonicity

hydrocephalus → UMN so hypertonicity

why does pain and ligamental instability arrive early in a patient with spina bifida?

movement patterns change as child grows

what is the best tool to monitor physical ability across a spina bifida patient’s lifetime?

funcitonal assessment

what are three factors that are important when educating parents?

1. repetition with reassurance

2. illustrations with minimal verbiage

3. focus on successes rather than failures

list some examples of physical therapy practices to teach parents.

• positioning: extensor patterns and tummy time are best

• stretching/ soft tissue mobilization

• handling techniques

• skin care

when should a parent facilitate vertical activities for a baby with spina bifida?

at 6 months

a child experienced spina bifida around her thoracic spine level but had early surgery intervention to help. what are likely outcomes that she may face in her lifetime?

• using a wheelchair for mobility

• needing a parapodium for exercise at T12

• possibly having cognitive defects

• may require supervised living

what are the most common orthoses for patients who experienced spina bifida at the thoracic-L2 region?

• THKAFOs (thoracic-hip-knee-ankle orthoses

• parapodium

what is a parapodium and what are the indications for needing one?

parapodium: upright positioning “brace” for high level lesions during exercise walking

indications: limited mobility, upright positioning, and walking

what are the likely outcomes for patients with spina bifida around the L1-L2 region?

• hip dislocation due to unopposed hip flexion and adduction

• may live independently

• can work in a “sheltered” environment (non-competitive)

with spina bifida ranging from L1-L3, what are the differences in the assisted devices required between children and adults?

child: household ambulator with KAFOS with a walker or crutches

adult: wheelchair for community environment

what are the most common orthoses for patients who experienced spina bifida at the L1-L3 region?

1. HKAFOs (hip-knee-ankle-foot orthoses)

2. RGOs (reciprocating gait orthoses)

T or F: weak hip flexors are required for the use of RGOs because they are indicated to facilitate hip extension and swing phase.

T

what is the most common orthosis used for patients who experienced spina bifida at the L4 region?

KAFOs (knee-ankle-foot orthoses)

what are the indications for needing a KAFO?

• unable to maintain upright posture due to contracture or weakness of knee

• knee joints are unstable → medial and lateral instability

• weak quadriceps 4/5 MMT or less

with spina bifida occurring at the L4 region, the anterior tibialis muscle remains “unopposed.” what deformity stems from this?

calcaneal foot

what are the assisted devices required for a patient suffering from spina bifida at the L5 region throughout his lifetime?

child: unaided ambulation in household environment and crutches for the community

adult: crutches with an inefficient gait and a wheelchair for long distances

with spina bifida occurring at the L5 region, what is a possible deformity occurring at the ankle/foot?

hindfoot valgus or calcaneous

what are the most common orthoses for patients who experienced spina bifida at the L4-S1 region?

1. standard AFOs

2. ground-reaction force AFOs

what are the indications for using the various AFOs for patients who had spina bifida at the L4-S1 region?

• weak or absent ankle musculature

• knee extensors 4/5 MMT or greater

• medial and lateral instability of knee and ankle

• inefficient-no push off and/or inadequate foot clearance

• “crouch” gait pattern

at which level will a therapist note decreased push off force and clearance (during gait) from a patient with spina bifida?

S2-S3

what are the most common orthoses for patients who experienced spina bifida at S1-no loss?

1. FOs (foot orthoses)

2. SMO (supramalleolar orthoses)

what are the indications for using FOs or SMOs?

• must clear foot and have adequate push off

• unequal weight distribution of foot

• medial and lateral ankle instability

• poor alignment of subtalar joint, forefoot, or rearfoot

who are the most common populations to suffer from brain tumors?

• children aged 0-15

• adults aged 40-60

• white Americans

• males

what are the three most common pathogenesis for the development of brain tumors?

1. abnormal stem cell differentiation

2. abnormal cell proliferation

3. environmental factors

tumors likely arise from the abnormal proliferation of which structures?

neurons or glial cells, meninges, blood vessels, cranial/spinal nerves, and the pituitary or pineal glands

what are the three factors that make up a brain tumor’s classification?

1. origin

2. cell differentiation

3. border

what’s the difference between primary and secondary brain tumors?

primary: originate in CNS

secondary: spread to the CNS from areas outside the brain (mostly spreads from lung or breast cancers)

T or F: a tumor with well differentiated cells leads to a worse diagnosis and prognosis.

F; well differentiated = good prognosis

why might a patient’s prognosis be bad even if his brain tumor is removed?

tumor could’ve lead to brain damage and/or still present with neurological issues

what are the two types of borders a brain tumor may present with?

1. localized: encapsulated and grow where originated

2. infiltrating: invade and become entangled in surrounding neural tissue

what are the characteristics of a benign tumor?

• slow growing

• capsulated

• non-invasive

• no metastasizing

• well differentiated

• suffix = “oma”

what are the characteristics of a malignant tumor?

• fast growing

• non capsulated

• invasive and infiltrate

• metastasizing

• poorly differentiated

• suffix = “carcinoma” or “sarcoma”

T or F: glial cells (microglia, astrocytes, oligodendrocytes, and ependymal cells) are the “unsung” heroes of the CNS.

T

tumor signs and symptoms (clinical manifestation) depends on the ___ and ___ of tumor.

1. location

2. type

list some examples of s/s associated with brain tumors (in adults).

headaches, nausea, vomiting, blurred vision, drowsiness, seizures

list some examples of s/s associated with brain tumors (in infants).

irritability, sleep issues, protruding fontanelles, and poor feeding

people get headaches all the time. how do we know if it’s a symptom related to a brain tumor?

if the headache interrupts sleep, worsens/changes with postural positioning, coughing, or exercising, and results in nausea and vomiting

which s/s is typically the first one noted by patients with a brain tumor?

seizures

papilledema

swelling of the optic nerve resulting in transient visual loss, especially with positional changes

• s/s of a brain tumor too

frontal lobe

motor functioning, initiation of action (decision-making), interpretation of emotion, thinking/judgments, movement, and personality

a brain tumor within the frontal lobe results in?

• hemiparesis

• aphasia

• gait difficulties

parietal lobe

processes sensation, perception and movement coordination through sensorimotor integration

a brain tumor within the parietal lobe results in?

• contralateral sensory loss

• homonymous visual deficits or neglect

• agnosias (difficulty recognizing objects)

• apraxias (difficulty planning movements)

• aphasia (difficulty processing language)

• visual-spatial disorders

temporal lobe

hearing, especially language, memory, and object identification

a brain tumor within the temporal lobe results in?

• auditory and perceptual changes

• cognitive integration

• long-term memory deficits

• learning and emotional changes

occipital lobe

vision processing- color, motion, shape, and background/ foreground distinction

a brain tumor within the occipital lobe results in?

• disorders of eye movements

• homonymous hemianopsia

• visual agnosia

• agraphia (impairment or loss of ability to write)

cerebellum

responsible for coordination and equilibrium

a brain tumor within the cerebellum results in?

• ataxia (poor muscle control)

• dysmetria (impaired coordination)

• dysdiadochokineasia (inability to perform rapid, alternating movements)

• intention tremor

• dizziness

brain stem

controls basic life functions, communication between cortex and nervous system

a brain tumor within the brain stem results in?

• focal weakness or sensory loss

• cranial nerve loss

• autonomic dysfunction

a brain tumor within the pituitary gland results in?

loss of hormone secretion specific to type of hormone involved

• hypothyroidism, diabetes, addison’s disease, etc.

which cranial nerves will be affected with an enlargement of a tumor in the pituitary gland?

CN 3,4, and 5

the pituitary gland sits superiorly/posteriorly to the ___ _____ which can cause bitemporal hemianopia (tunnel vision) if enlarged via tumor.

optic chiasm

an acoustic neuroma is a type of tumor that compresses which cranial nerve?

CN 8: vestibulocochlear

according to our notes, what is chemotherapy?

a toxic drug that acts on the DNA of the tumor cells and limits their ability to replicate

what are the two types of chemotherapy?

1. cytostatic: drugs that prevent cells from reproducing

2. cytotoxic: drugs that cause cellular death

describe the characteristics of radiation therapy.

• delivers high-powered energy directly to tumor site

• typical course is 1-5mins 5 days/week for ~6 weeks

• has a life-time dosage that can be tolerated