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Posterior Fossa Syndrome: Rehabilitation Across the Continuum

Lindsey Christoffersen, PT, DPT, C/NDTSherry Lockett, PT, DPT

• The speakers have no conflicts of interest to disclose

• Any equipment or materials mentioned in the presentation are

available to the speakers at their facility

• The speakers have no financial gain in mentioning any specific

products

Conflict of Interest Disclosure

At the conclusion of this session, participants will be able to:

1. Discuss the pathophysiology and clinical characteristics of Posterior

Fossa Syndrome (PFS).

2. Discuss the role of each member of the interdisciplinary team in the

comprehensive management of patients with PFS.

3. Identify and discuss appropriate evaluation tools and treatment

strategies to address the impairments and functional limitations

associated with PFS across a variety of settings.

Learning Objectives

Near the base of the skull

Contains the brainstem and

cerebellum

- Brainstem: breathing, heart rate, swallowing, alertness, digestion

- Cerebellum: movement, posture, balance, coordination

The Posterior Fossa1,2

Image courtesy of University of Rochester Medical Center

Account for 60-70% of all pediatric brain tumors

~40% to 50% of these are

medulloblastoma

Posterior Fossa Tumors3

Reported incidence following PF tumor surgery ranges from 2%-50%

Older studies often report lower numbers

- Failure to recognize the syndrome?

Gadgil et al. (2016) knowledge update

- Occurs in 8-24% of children following resection of PF masses

Robertson et al. (2006)

- Reported PFS in 24% of their study population (N=450)

Incidence of PFS4-6

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Most common malignant brain tumor of childhood

- Accounts for 20% of all childhood brain tumors

- 250-500 children are diagnosed in the U.S. annually

- Primarily found in children < 16 years old

Medulloblastoma7

Image courtesy of RM DeSouza and colleagues

Risk Stratification8

Standard RiskStandard Risk

>3 years of age>3 years of age

No detectable metastatic disease

No detectable metastatic disease

Near or total resectionNear or total resection

High RiskHigh Risk

<3 years of age<3 years of age

Overt metastatic diseaseOvert metastatic disease

Gross residual diseaseGross residual disease

More recent studies indicate that medulloblastoma is made up of distinct molecular subgroups that respond differently to

treatment

- WNT (10%, favorable)

- SHH (30%, relatively favorable)

- Non-WNT & non-SHH (60%, generally poorer prognosis)

Genetic alterations within these subgroups also contribute to prognostication and treatment decisions

Additional Prognostic Factors8

Surgery

Radiation

Chemotherapy

Treatment & Survival7

5-year survival rates:

- Local disease: 70%-80%

- Metastatic disease: 60%

Posterior Fossa Syndrome (PFS)

Cerebellar Mutism (CM)

Transient Cerebellar

Mutism (TCM)

Mutism and Subsequent

Dysarthria (MSD)

Cerebellar Mutism

Syndrome (CMS)

Cerebellar Cognitive Affective

Syndrome (CCAS)

Common Terms9

Deficits

- Neurologic (motor)

- Neuropsychologic (cognitive and affective/behavioral)

- Neurolinguistic

Most commonly seen in children after posterior fossa tumor

surgery

- May also occur following trauma, vascular incidents, or infections

Clinical Characteristics4,9

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Motor

• Ataxia• Dysmetria

• Dysphagia• Dysarthria• Hypotonia• Hemiparesis• Nystagmus• Oculomotor problems• Urinary

retention/incontinence

Speech/Language

• Cerebellar mutism• Naming problems

• Verbal fluency• Dysarthria• Slowed speech• Decreased verbal

output• Short phrases• Distorted vowels• Prolonged phonemes• Mono- pitch/loudness• Hypernasality• Vocal tremor

Affect/Behavior

• Depression• Irritability

• Lability• Apathy• Anxiety• Forced crying/laughter• Transient eye closure• Impulsivity

Cognitive

• Attention• Memory

• Executive functioning• Visuospatial skills• Processing speed• Verbal comprehension• Reading/writing

difficulties

Postoperative Deficits9,10

Decreased speech or mutism

Dysphagia

Hypotonia

Ataxia

Mood changes

Neurological deficits

Most Common Deficits3,9,11

Transient mutism

- Delayed onset (0-15 days, mean 2 days)

- Limited duration (1 day-2.5 years, mean 43 days)

- Recovery period marked by dysarthria

- Not uncommon for patient to speak a few words after surgery but unable to speak the next day

Not all patients become completely mute

- May be limited to single words or short sentences (not easily elicited)

Hallmark Characteristic: Mutism9

Duration of mutism varies

- Gadgil et al. 2016 knowledge update

• Average: 8 weeks

• Range: 4 days to 5 months

Correlation found between duration of mutism and severity of neurologic symptoms

Speech Outcomes12,13

Slow rate: 33 patients

Short phrases: 13

Hypophonia: 10

When Speech Begins to Return13

Latent onset of 1-6 days

- Children have normal speech prior to onset

Total absence of speech

- Not of nonverbal utterances such as crying, screaming

Worsening neurological symptoms

- Ataxia, pyramidal paresis, oculomotor dysfunction, diminished facial expression

- Loss of bladder and bowel control

Behavioral disturbances

- Apathy, depressed affect, agitation, irritability, emotional lability

Clinical Characteristics: Acute Stage13

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Kirk et al. (1995): Retrospective study of children after PF surgery

Timeline of Onset3

Postoperative Symptoms

Post op in ICU

Able to move purposefully, focus on an object, make some attempts to verbalize when intubated; similar to preoperative state with expected ataxia

and dysmetria

Within 24−107 hours

Neurologic status changes without an identifiable factor (e.g., no change in vitals, intracranial pressure monitoring, or radiographic abnormality)

Timeline of Onset3

Postoperative Symptoms

Within 24−27 hours

Facial weakness and nystagmus

Mean time 33 hours

Weakness of upper extremities

Mean time 40−58 hours

Weakness of lower extremities, emotional lability, irritability, random nonpurposeful movements of extremities, difficulty verbalizing, and mutism

Differs for each patient

More severe symptoms early in recovery ���� symptoms that

persist longer

Onset, Severity, & Duration5,12

Absence or reduction in

speech 1-2 days post-op

Mutism 2-7 days post-op;

Profound axial hypotonia and

ataxia

Recovery of speech begins after 8 weeks (on average)

Duration of Deficits6

Kupeli et al (2011)

- Pre and post-op neurologic and psychological evaluations of children with brain

tumors having PF surgery (PFS developed in 25% of patients)

Multiple theories, but no clear culprit

- Damage to dentato-thalamo-cortical (DTC)

pathway

- Damage to the vermis

- Bilateral dentate nuclei damage

This damage may result from

- Poor cerebellar perfusion due to vasospasm

- Injury related to surgery

- Postoperative edema

Causes5,11,14

Avula et al. 2016

Prospective review of postoperative MRI

- 10 patients who developed PFS

- 13 randomly selected patients who did not develop PFS

Preoperative Imaging

- Rostral position of tumor within 4th ventricle associated with PFS (P=0.035)

Causes: Morris et al15

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Postoperative Imaging

- PFS group had bilateral multi-focal postop

injury (edema)

• 90% of PFS group had damage to ≥ 3 of the following: Pons, dentate nuclei, superior cerebellar peduncles, midbrain

– None of the unaffected patients had ≥

3 structures involved

- 80% of PFS group had bilateral involvement

• Compared to 15% of patients without PFS

Causes: Morris et al15 Risk Factors5,16

Medulloblastoma diagnosis

Midline tumors

Tumors involving the vermis

Tumors high in the 4th ventricle

Brainstem invasion

Retrospective study of 63 children with medulloblastoma; PFS developed in 18 patients (29%)

Predictors found included:

- Brainstem invasion

- Midline tumor location

- Younger age

- Absence of radiographic residual tumor

Conclusions:

- Children with midline tumors exhibiting brain stem invasion are at increased risk for PFS

- More aggressive surgeries may result in increased incidence of PFS

Risk Factors16

Many patients suffer from long-term sequelae

- Mutism tends to be transient, but speech rarely returns to being completely

normal

- Increased risk for neurocognitive impairment

- Neurological deficits, mainly ataxia

- Decreased quality of life and environmental access

Looking Long-Term13,17,19

Palmer et al. 2010

- 25 patients with PFS following medulloblastoma resection

- 25 matched patients without PFS being treated on same protocol

- Significant differences (p<0.01) between groups

Patients with PFS

- Demonstrated significantly lower performance in processing speed, attention, working memory, executive processes, cognitive efficiency, reading, spelling, and mathematics

Long-Term Neurocognitive Outcomes17

Robertson et al. 2006

- PFS present in 24% of patients (N=450)

- 92% of patients presented with severe or moderate PFS

• 43% severe, 49% moderate

• Mutism and ataxia were most frequently judged as severe

Long-Term Speech & Motor Outcomes5

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Robertson et al. 2006

- 1-year post-diagnosis

Long-Term Speech & Motor Outcomes5

• 92% had ataxia• 66% had speech and language dysfunction• 59% had some degree of global intellectual handicap

Initially Rated Severe

• 88% had abnormal coordination• 25% had speech and language dysfunction• 17% had some degree of global intellectual handicap

Initially Rated Moderate

Steinbock et al (2003)

Long-Term Speech & Motor Outcomes18

Brinkman et al. 2013

- In-home evaluations of brain tumor survivors ≥ 18 years old (N=78) and

matched population-based controls (N=78)

• Environmental access

• Health related quality of life

• Social participation

• Demographic and treatment information

• Physical performance

• Cognitive function

• Psychological distress

- Median age of survivors was 22 years (range 18-58)

Long-Term Quality of Life19

Brinkman et al. 2013

BT survivors:

Long-Term Quality of Life19

Were more likely to avoid

aspects of physical

environment

Were more likely to report

they did not drive

Were more likely to report

decreased HRQOL

Participated less in

functional living and

social activities in their

community

Treatment of PFS12,13,20

Pharmacology

Physical Therapy

Speech Therapy

Occupational Therapy

Neuropsych

& Psychology

Child Life

Physiatry

Rehabilitation Management

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Evidence is significantly lacking regarding rehabilitation for patients with PFS

Primarily limited to case studies and case series

This may come as a shock…

Initial rapid gain of skills

- During RT and during break before chemotherapy

- Critical period for rehab

- Inpatient rehab highly recommended during this time if appropriate

Plateau during chemotherapy

- Doing our best to “hang on” to skills gained

- Typically an appropriate time to order a custom WC if indicated

More progress after completion of chemotherapy, but much

slower than initially seen

- Remaining deficits are often long-lasting

Rehab Trajectory: Our Experience

� If severe PFS, patient may remain inpatient after surgery or be admitted directly inpatient if coming from an outside hospital

� May remain inpatient throughout scans and during the beginning of

radiation therapy (RT), with a break between RT and chemotherapy

� Depending on physical function and burden on caregivers, patient may remain inpatient for duration of chemotherapy

� Interdisciplinary collaboration is key

Acute Care Overview

History

- Previous level of function, activity level, previous limitations

- Home location, accessibility issues, plans to return home

- Caregivers available for training, assisting patient, equipment management

Communication & Behavior

- Alertness and orientation

- Ability to follow commands

- Ability to communicate needs (yes/no, thumbs up/down, squeezing hand)

- Mutism/ability to voice

- Flat, emotional lability

Initial Evaluation

Vision/Facial/Voice Screen

- Drooling, smiling (facial droop)

- Wet voice

- Ability to track objects, attend to objects

- Strabismus and/or nystagmus

ROM (AAROM, PROM) & Strength

Neurological screen

- Muscle tone

- Clonus

- Reflexes

- Coordination and motor control (finger to nose/finger, ataxia)

Initial Evaluation

Functional Mobility

- Bed mobility

- Sitting balance

- Sit <-> stand

- Standing

- Transfers

- Ambulation and stair negotiation

- Gross motor abilities

- Equipment needs

Initial Evaluation

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14-item instrument

Examines functional balance

- Steady state

- Anticipatory

Administration time < 20 minutes

Validated for children with mild, moderate, and severe motor

impairments

- Athetosis, hemiplegia, hypotonia, spastic diplegia, s/p brain tumor resection

Pediatric Balance Scale21,22 Pediatric Balance Scale21,22

Pediatric Balance Scale21,22

Cut off scores

- Based on typical performance

- Scoring below cut score may indicate decreased functional balance

- Should be interpreted with findings from clinical examination

Pediatric Balance Scale22

Minimal Clinically Important Difference (MCID)

- 5.83 points for PBS total

- 2.92 points for static items

- 2.92 points for dynamic items

Pediatric Balance Scale22

Initial Presentation

Ataxia

Axial hypotonia

Max A –dependent transfers

Non-ambulatory

or max A for

ambulation

Mod –Max A for

static sitting and standing

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� Stay focused on discharge plan

� Teaching parents/caregivers safety with mobility and transfers

� Caregiver education on task carryover in home exercise program

� Maximizing patient ability to perform assisted/independent mobility/transfers and desired activities

� Standing and ambulation if possible

� Order appropriate equipment/orthotics

Goals for Acute Care

� Meet with team to communicate progress, updated goals, and discharge planning

� Interdisciplinary collaboration to incorporate behavior/speech goals into sessions � treating the whole person

Goals for Acute Care (continued)

Treatment Goals:1. Patient will be able to perform a stand pivot transfer from bed to chair with moderate

assistance with an assistive device with verbal cueing for sequencing and increased time

allowed for activity to assist caregivers with mobility tasks.

2. Patient will be able to tolerate sitting at edge of bed with minimal assistance with bilateral

LE support and use of bilateral UE on surface x 4 minutes with independent head control

to assist with a dressing activity.

Acute Care Interventions

3-5 days/week

30-60 minute sessions

Individual or co-treats

Bed mobility

Transfers

Standing & ambulation

Caregiver Training

Bed Mobility

- Rolling to both sides (sequencing LEs, UEs, and head)

- Supine <-> sitting (propping on elbow, maintaining alignment)

- Repositioning of self for comfort, pressure relief (bridging, scooting in bed)

Sitting Activities

- May require assistance of two people

- Be aware of input being given and ability of patient to handle increased stimuli

- Position of support (anterior vs posterior)/

- Support surface (mat vs bed)

- Use of mirrors/stools/auditory/manual cueing/weights/cervical collar

- Consider ataxia and dysmetria present with movement

Acute Care Interventions

Static Sitting

- Initial position of head/neck/shoulders/trunk/pelvis

- Increasing length of time holding position

- Having patient attempt postural corrections

Dynamic Sitting

- Weight shifting

- Reaching inside and outside of base of support

- Ability to return to midline

- Scooting on surface

Acute Care Interventions

Transfers

- Dependent � sliding board, patient lift

- Use assistive devices as patient’s strength and balance improves

- Sit <-> stand

Standing

- Static (tilt table, standing frame)

- Dynamic (parallel bars, walker)

• Weight shifting

• Postural control

• Step-ups, weight bearing through weaker extremity

Acute Care Interventions (continued)

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Ambulation

- Parallel bars (forwards, backwards, lateral stepping)

- Walker (distance, weight shifting, step length control)

- Hand held assistance

Strength & Endurance

- Gentle stretching of trunk and extremities

- Strengthening of individual muscle groups

- Recumbent bike

- Sequencing of muscle groups to make exercises functional

Acute Care Interventions (continued)

Daily education to family and staff

- Allow patient to initiate task if able

- Stretching

- Educate on muscle tone

- Carryover of activities

- Short bouts of activity, allowing for rest breaks

Consider attention and ability to follow commands

Use activities familiar to patient

Be prepared to modify session based on patient presentation

Use interdisciplinary team members

Special Considerations

Interdisciplinary team that meets biweekly

- Neuropsychology & Psychology

- Speech, Occupational, & Physical Therapy

- School

- Child Life Services

- Clinical Nurse Specialists

- Social Work

Discuss patient status, goals, and potential barriers

- Plan interdisciplinary goal for each team member to work on with patient

- Goal example: Pt. will choose an activity in each session when presented with 2 options to increase initiation.

Acute Neurological Injury (ANI) Rounds

During break between RT and chemotherapy

- 5-6 week break

- Close to home when possible

- If unable or unwilling to participate in inpatient rehab, encourage intensive outpatient program

- Often shorter stays in inpatient rehab secondary to slow progress (FIM) and/or desire to be at home

Transition to Inpatient Rehab

Critical time to maximize return of function

Transition to Inpatient Rehab

Can completely

focus on PT, OT, ST

No other rigorous daily

treatments

Less fatigue (no radiation

or chemotherapy)

16-year-old female

- Diagnosis of medulloblastoma, status post gross total resection

- Presented to signs of PFS day +7 from surgery

- Global ataxia, emotional lability, dysphagia, mutism

- Patient underwent 6 weeks of RT and weekly vincristine

- Main patient and family goal was to return home

Discharge planning

- 3 publicly funded options: adult rehab center, pediatric ABI program (far from home), community based rehab

- Patient and family opted for community rehab to remain close to home

Discharge Case Study23

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Strengthening StretchingBalance

Training & Coordination

Gait Training

EnduranceOrthotic

PrescriptionEquipment HEP

Outpatient Therapy Outpatient Therapy

3 days per week

30-45 minute sessions

Primarily individual

treats

Occasional co-treats

with challenging

tasks

� Gain and maintain as much function as possible during chemotherapy

� Ongoing education regarding activity expectations and rehab trajectory

� Monitor equipment needs, order custom WC if appropriate

Goals for Outpatient Therapy

Treatment Goals:1. Patient will ambulate 500’ with least restrictive assistive device without LOB,

provided SBA for balance and verbal cueing 25% of the time to increase step

length and to narrow BOS.

2. Patient will reach outside of BOS anteriorly in standing to retrieve 5 objects with minimal A to achieve weight shift and to maintain balance.

NuStep, Total Gym

Emphasis on eccentric/graded control

- Squats (don’t pop the ball), stoop and recover

- TheraBand (control band back to starting position)

- Step-ups/downs

- Sit <-> stand

- Jumping, jumping down, hopping, descending stairs

- Jump/hop and freeze

Postural muscle strengthening

- Glutes, abdominals

- Emphasis on engaging abdominals/upright posture during functional tasks

Strengthening

Will sometimes perform in therapy sessions, but often have family/patient perform as part of HEP

- Ankle dorsiflexion

- Hamstrings

- Trunk rotation, extension

Stretching

Control of dynamic movement

- Weight shifting in sitting � standing

- Activation of glutes and abdominals to perform true weight shift

Movement in multiple planes

Coordination

- Timing, sequencing, amount of force, spatial awareness

- Zip Ball, catching and throwing a ball, weighted therapy bar ball taps, squat <->

stand, jumping (in place, to a target)

Obstacle negotiation

- Small obstacles (hurdles, River Stones, ramps), unstable surfaces (AirEx foam,

DynaDisc), unpredictable surfaces (grass, gravel)

Balance Training & Coordination

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Narrowed base of support

- Walking between balance beams

- Romberg position (eyes open, eyes closed)

- Tandem stance on line � balance beam � elevated balance beam

- Single limb stance

• Trapping soccer ball

• Flamingo freeze

Balance Training & Coordination

Pre-gait activities

- Foot to target

• Make increasingly difficult: heel strike when bringing foot to target, weight shift onto limb once foot is on target, weight shift with step through to next target

- Step up and over therapy bench for stair prep

LiteGait on treadmill or over ground

Ambulation with appropriate assistive device or in || bars

- Anterior walker, posterior walker, hemi walker, cane

- Ambulation through obstacle course

Begin working on stairs, curb, and obstacle negotiation as skills

progress

Gait Training

Transfers

Standing

Ambulation

NuStep

Recumbent or adaptive bike

Advanced:

- Floor hockey

- Soccer

- Gross motor bingo

- Yoga

Endurance

Use of PRAFOs initially to prevent plantar flexion contractures, pressure wounds

Fit for appropriate orthotics ASAP

- AFOs (solid, hinged, carbon fiber)

- SMOs

- Arch Support

Consider use of anti-hyperextension or hinged knee sleeve with

instability

Orthotic Prescription

Often difficult to anticipate secondary to rapid improvements initially, followed by plateau

Begin with loaner equipment when possible

- Tilt in space/Convaid Rodeo � Standard or Semi-custom upright � Custom upright

- Typically appropriate to purchase a custom wheelchair during chemotherapy

Equipment

Daily practice is emphasized

Primarily functional mobility and gross motor tasks

Train families to practice with assistive devices outside of

therapy

Home Exercise Program

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Difficulty following commands

- Often due to motor deficits

Decreased/absent volitional speech

- May be able to voice when laughing or crying but not on command

Poor oral motor skills

Receptive skills often relatively intact

- May be difficult to detect due to motor and speech deficits

- Speak TO them, not at or in front of them

Role of Speech Therapy: Speech Outcomes24

Initial goals and activities

- Alternative means of

communication

• Gestures

• GoTalk

• Alphabet board

• Picture communication board

- Vocalizing on command

- Identifying pictures

- Following bodily commands

Role of Speech Therapy: Speech Outcomes24

As speech-language skills progress

- Formal testing is initiated

- Address dysarthria, initiation, and

executive functioning

Retrospective medical record review of 19 patients with PFS

Role of Speech Therapy: Swallowing Outcomes3

Symptom Number of Children

Dysphagia 7 (37%)

Impaired gag reflex 3 (16%)

Facial weakness 4 (21%)

Institution of enteral/parenteral feeds 10 (53%)

Ability to swallow and mood typically improved before recovery of speech

- May not indicate normal swallow

- Mood changes may persist if patient is unable to communicate wants/needs

effectively

- Some patients made full recovery; swallowing difficulties persisted in others (sometimes long term

Role of Speech Therapy: Swallowing Outcomes3,11,24

Difficulty with oral

motor movements

Decreased strength

Difficulty managing secretions

Typically require

NG- or G-tube

Initial goals and activities

- Following oral motor commands

- Oral motor exercises

- Swallowing exercises

- Postural/compensatory strategies

- Provision of adaptive equipment

- Instrumental exam (if ready)

Role of Speech Therapy: Swallowing Outcomes24

As swallowing skills progress

- Instrumental exam (when ready)

- Advance PO diet as appropriate

- Continue exercises, compensatory strategies

Significant UE ataxia

- Typically worse unilaterally

Visual deficits

- Nystagmus, diplopia

Decreased level of functional performance

Decreased participation in ADL & IADLs

Difficulty with motor planning

Sensory concerns, emotional lability

Difficulty following commands

Role of Occupational Therapy24

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Initial goals and activities

- Increasing independence with

ADLs• Specifically dressing, toileting, and

hygiene

- Increasing mobility to participate in ADLs

- Increasing visual skills

- Functional reaching and strengthening

- Provide toileting and bathing equipment

Role of Occupational Therapy24

As speech-language skills progress

- Weight bearing activities (side sitting, quadruped)

- Fine motor activities to improve coordination and strength

- School based skills

- Handwriting

- Independence in higher level self

care (tying shoes, buttons)

- Functional cognition

X Rigorous RT schedule, often with sedation

X Side effects from chemotherapy

- Nausea/emesis

- Anemia

- Thrombocytopenia

X Infections

X Slow progress due to nature of PFS

X Cancer-related fatigue

X Communication challenges

X Psychosocial considerations

Barriers to Achieving Goals

Myelosuppression is a major side effect of chemotherapy

- Potential for adverse events with stressful conditions (exercise)

• Thrombocytopenia � bleeds

• Severe anemia � cardiac arrhythmias

• Neutropenia � sepsis

Guidelines for Safe Exercise25

Retrospective chart review (Gilchrist and Tanner, 2017)

- Adverse events during or after 37 of 406 PT sessions

• Most common event was tachycardia not requiring medical intervention

- No serious adverse events occurred

Guidelines for Safe Exercise25

Cancer-Related Fatigue26

“A distressing, persistent subjective sense of physical, emotional, and/or

cognitive tiredness or exhaustion related to cancer or cancer treatment that is not proportional to recent activity and interferes with usual functioning.”

- National Comprehensive Cancer Network, 2017

Cancer-Related Fatigue27,28

Cancer-Related Fatigue

Cancer Treatment

Burden

Disease Burden

Co-Morbid Conditions

Psychosocial Burden

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Cancer-Related Fatigue29

• Feeling tired, weary, exhausted even after a good night’s sleep

• Lack of energy/prolonged fatigue after activity

• Weakness, heaviness in arms/legs

• Listlessness or irritability

• Trouble starting or finishing tasks due to fatigue

• Needing to sleep during the day

• Unable or requiring help to do usual or desired activities

• Being too tired to eat

• Difficulty with concentration and memory

• Limiting social activities due to

fatigue

Consistent therapist when possible

- May have to introduce self and role multiple times

One voice at a time

1-2 step commands

- Consider automatic movements

- This then that statements

Allow increased time for processing

Collaborate with speech and psychology regarding

communication and cognitive abilities

Strategies for Communication

7-year-old male

- GTR medulloblastoma, 4th ventricle

- Post-op: inability to speak, left-sided weakness, irritability

- Transferred to inpatient rehabilitation hospital

• PT, OT, ST; slow progress

• Mute for a few days, then slow speech improvements but remained dysarthricwith “robotic speech”

• Gait unsteady, but able to walk without assistance; unable to negotiate stairs

• Challenging mood/behavior requiring risperidone during RT

Two years after completion of treatment

- Speech fluent, continuing to work with PT to address ongoing balance issues

- Behaviorally and emotionally at baseline

Published Case Studies20

13-year-old male

- GTR medulloblastoma

- Post-op: paucity of speech, left-sided weakness, ataxia, VI CN palsy, emotional outbursts

- Transferred to inpatient rehabilitation hospital x 6 weeks

• Made significant progress with speech and ambulation

• Mood remained flat over course of RT

At 5-year follow-up

- Performing well academically, attending college

- Continued social and emotional struggles

Published Case Studies20

3-year-old female

- Resection of posterior fossa ependymoma

- Post-op: dysarthria, right-sided weakness, unable to ambulate independently, emotional outbursts

- Began low dose risperidone to improve behavior

• Allowed for safe participation in PT and ST and completion of RT

At 5-year follow-up

- Attending school full-time

- Behavior and speech issues fully resolved

- Ongoing challenges with balance � continuing to work with PT and OT regarding these issues

Published Case Studies20

Harbourne et al. 2014

- 14-year-old female and 6-year-old female with PFS

- Completed inpatient rehabilitation and outpatient programs, discharged due to lack of progress

Neuromodulation devices on the tongue

- Static and dynamic balance activities utilizing devices

- 3 days of intensive training, followed by HEP consisting of activities performed 5 days/week for 8 weeks

Clinical improvement noted in both participants

- Improved balance beyond MCID on BBT-P

- Increase in time standing on dynamic surface

- Increased gait speed and step length

Published Case Studies30

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`

- PFS occurs in up to 24% of patients who undergo posterior fossa tumor resection

- Symptoms are often severe and last years beyond cancer treatment

- The literature strongly advises participation in rehab, but specific strategies for

management of this syndrome are lacking

- More research is needed to determine the efficacy of specific rehab interventions for this population

Summary

Questions?

1. Rehab for posterior fossa syndrome. St. Jude Children's Research Hospital. Available at: https://www.stjude.org/treatment/patient-resources/caregiver-resources/patient-family-education-sheets/rehabilitation/rehab-for-posterior-fossa-syndrome.html. Accessed October 25, 2017.

2. Adler L, Dozier T, eds. Anatomy of a Child's Brain. Anatomy of a Child's Brain - Health Encyclopedia - University of Rochester Medical Center. https://www.urmc.rochester.edu/encyclopedia/content.aspx?ContentTypeID=90&ContentID=P02588. Accessed October 25, 2017.

3. Kirk E, Howard V, Scott C. Description of posterior fossa syndrome in children after posterior fossa brain tumor surgery. Journal of Pediatric Oncology Nursing. 1995;12(4):181-187.

4. Reed-Berendt R, Phillips B, Picton S, et al. Cause and outcome of cerebellar mutism: evidence from a systematic review. Child’s Nervous System. 2014;30(3):375-385.

5. Robertson P, Muraszko K, Holmes E, et al. Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children’s Oncology Group. Journal of Neurosurgery: Pediatrics. 2006;105:444-451.

6. Kupeli S, Yalcin B, Bilginer B, et al. Posterior fossa syndrome after posterior fossa surgery in children with brain tumors. Pediatric Blood and Cancer. 2011;56(2):206-210.

7. Medulloblastoma. St. Jude Children’s Research Hospital. Available at: https://www.stjude.org/disease/medulloblastoma.html. Accessed November 21, 2017.

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