PEDIATRIC NEUROLOGIC DISORDERS

B.Resseque, D.P.M., DABPO June 16, 2017

8 Year Old Male

• 3 months ago left foot “started to change

• “Walking on the outside of his left foot, doesn’t get his left heel down”

• Special education

• Referred by podiatrist for “metatarsus adductus” 8 YEAR OLD MALE

• No family history of orthopedic or neurologic disorders

• No history of bladder or bowel disorders

• No back pain, no lumbosacral lesion 8 Year Old Male

• Resistance of movement at the STJ and MTJ

• Extensor Babinski, 2/4 patellar DTR’s, 1 /4 left Achilles reflex, no clonus, weakness of left digital extensors and peroneal muscles, possible dorsolateral foot sensory loss ( ?)

• Left heel in varus on stance, forefoot adducted, higher arch on left side 8 Year Old Male 8 YEAR OLD MALE

• Finger to nose, heel to shin tests normal

• Fine Motor-touch each finger to his thumb in succession difficulty on left Radiographs- is this a metatarsus adductus?

• A anterior cavus- increased Meary’s angle & calcaneal pitch, decreased tibio talar angle • B posterior cavus- normal Meary’s (0) and increased calcaneal pitch & tibiotalar angle • C mixed-increased Meary’s & calcaneal pitch, normal tibiotalar CAVOVARUS DEFORMITY

• CAVOVARUS-high medial arch,1st ray plantarflexed & heel varus

COMMON CAUSES Peripheral neuropathy (CMT) CP (hemiparesis) CALCANEOCAVUS (POSTERIOR CAVUS)

• Calcaneal inclination is >35 degrees,0 Degrees talar-1st metatarsal angle(Meary’s angle)

• Associated with weakness of gastroc soleus, especially with normal tib.ant.

• polio, overlengthened heel cord in CP, spina bifida 8 year old-anterior cavoadductovarus 8 Year Old Male 8 Year Old Male

•WHAT NEXT? Unilateral Acquired Cavus Foot Deformity DIFFERENTIAL DIAGNOSIS ?

• Traumatic injury to peripheral n. or spinal n. root

• Polio

• Hemiplegia

• Spinal pathology (syringomyelia, tumor, diastematomyelia) Think structural lesion with no history of trauma!

A PATIENT WITH AN ACQUIRED UNILATERAL CAVUS DEFORMITY,BUT WITHOUT A HISTORY OF TRAUMA, MUST BE EVALUATED FOR A SPINAL TUMOR !!!!!

TETHERED CORD SYNDROME

• NEUROLOGIC DETERIORATION THAT OCCURS WHEN DISTAL CORD IS SUBJECTED TO TRACTION OR COMPRESSION

• Our 8-year old patient had a spicule in the spine revealed by spinal MRI which resulted in tethering of spinal cord

Clinical Findings

• Decreased motor function

• Sensory changes

• Foot deformity

• Bowel and/or bladder dysfunction

• Back pain

• Scoliosis

• Skin changes: hypertrichosis, dimple, swelling- look at back SPINAL CORD AND COLUMN GROWTH CLINICAL EVALUATION OF CAVUS FOOT • Unilateral or bilateral? (unilateral think anatomic lesion!) • Cavovarus or calcaneocavus? • Block test (hindfoot supinated, pronated or neutral?) • Muscle power especially tib. ant, post. tib. & triceps surae • NEUROLOGIC CHANGES?

• 1.PHYSIOLOGIC

• 2.BRAIN

• 3.SPINAL ABNORMALITY

• 4.PERIPHERAL NERVES

• 5.MUSCLE

• MUSCLE OR STRUCTURAL PROBLEM OF FOOT

• 6.CONGENITAL (rare) 1.PHYSIOLOGIC CAVUS

• calcaneal inclination >25 degrees

• 2/3’s of adults with symptomatic cavus feet have underlying neurologic disorder, most commonly CMT

DIFFERENTIAL DIAGNOSIS 2. BRAIN

• Dystonia musculorum deformans (unilateral sudden onset of foot supination in child under 10)

• CP, especially hemiparetic side DIFFERENTIAL DIAGNOSIS 3.SPINAL ABNORMALITIES- diastematomyelia, myelodysplasia, syringomyelia, polio, spinal cord tumors, tethered cord syndrome UNILATERAL CAVUS-THINK SPINAL CORD! DIFFERENTIAL DIAGNOSIS

• 4.PERIPHERAL NERVES HMSN-hereditary motor sensory neuropathy, CMT DIFFERENTIAL DIAGNOSIS

• 5.MUSCULAR OR STRUCTURAL PROBLEM OF FOOT

• tev, injury to tendon, nerve, muscle, muscular dystrophies 11 Year Old Female

• Unilateral left cavus foot deformity

• Problem started 2 years earlier and is increasing in severity

• Referred to physical therapist by orthopedist

• Physical therapist referred to podiatrist WEAKNESS, SENSORY DEFICIT, ABSENT ANKLE REFLEX Lumbosacral Area

• Hairy patch diagnosed as hairy nevus 11 YEAR OLD FEMALE- SPINAL LIPOMA DERMAL DIMPLE- bilateral tev NEUROTROPHIC ULCER 18 Month Old Male- “toe walking” since starting to walk at 16 months 18 Month Old Male- toe walking

• Born prematurely

• Low birth weight

• Sat independently at 11 months

• Started walking independently at 16 months Physical Exam

• Hyperreflexia of lower extremities

• Adductor grabbing

• Babinski response- dorsiflexion

• Limited ankle dorsiflexion (negative 5 degrees), 2 beats of clonus 18 Month Old Male

• Referred to neurologist

• Diagnosis: spastic diplegia (static encephalopathy) GAIT AND POSTURE October 2010

• The Toe Walking Tool: A novel method for assessing idiopathic toe walking children Williams, C. et al ALWAYS BE SUSPICIOUS OF LATE ONSET TOE WALKER! STATIC ENCEPHALOPATHY ( cerebral palsy)

• Retention of primitive reflexes

• Delay of development of more mature reflexes

• Most common pediatric neuromuscular disorder 7.5 per 1,000 live births

• Associated with prematurity and low birth weight GALANT REFLEX SPASTIC QUAD – NO FORWARD PROTECTION RESPONSE RETENTION OF PALMAR GRASP- 2 YEAR OLD POOR DEVELOPMENT OF MORE MATURE REFLEXES-lack of optical righting reflex in 2 year old RETENTION OF PALMAR GRASP- 2 YEAR OLD HYPPERREFLEXIA EXTENSOR BABINSKI RESPONSE ADDUCTOR GRABBING

Treatment Considerations

• Serial bilateral below knee casts

• Ankle Foot Orthoses

• Physical therapy BK SERIAL CASTS

• Stimulates an increase in the # of sarcomeres present in the calf musculature

• No atrophy in active children BK SERIAL CASTS

• Total contact cast provides constant sensory/ proprioceptive input AFO’s and the Cerebral Palsied Child-provide functional improvement !

• Improve neuromotor control, normalize gait as much as possible

• Improve proprioception-enhance sensory awareness

• Prevent deformity

• Improve foot & proximal alignment STATIC ENCEPHALOPATHY

• Abnormal muscle activation pattern, muscle tone, and proprioception

• 2-3 times energy cost but ½ the speed of walking, energy cost increases with age, negatively affects level of physical activity

ANKLE FOOT ORTHOSES

• Increase stride length & velocity

• Provide better foot positioning for initial contact

• Control equinus in stance & swing phase

• Decrease cadence

• Decrease energy cost Consider effect of orthosis on 5 prerequisites of normal gait

• 1. stability in stance

• 2. clearance in swing

• 3. prepositioning of foot in terminal swing

• 4. adequate step length

• 5. energy conservation Figueiredo, Efficacy of ankle-foot orthoses on gait of children with cerebral palsy: systematic review of literature, Pediatric Physical Therapy 2008 Fall;20(3):207-23

• Positive effects of AFO’s on passive & active ankle range of motion, gait kinetics & kinematics, functional activities related to mobility of cp children

• Quality of research low, few had between group comparisons, lack of standard terminology used to define AFO

Solid Ankle Foot Orthosis Solid polypropylene shell that resists deformation on loading Provides stability while limiting mobility Stabilize ankle joint in stance phase, control position of ground reaction force at knee,

improve proximal joint kinematics SOLID AFO INDICATIONS: Mild to moderate foot malalignment Mild to moderate spasticity Mild myostatic contracture SPASTIC QUADRIPARETIC CONTRAINDICATIONS TO SOLID AFO USE

EXCESSIVE ANKLE DORSIFLEXION IN OLDER CHILDREN > 12

SIGNIFICANT RIGID FOOT DEFORMITY, PROXIMAL CONTRACTURE Hinged Ankle Foot Orthosis

• Articulated polypropylene shell

• Based on principle that ankle motion is necessary for normal movement patterns and postural responses

• Allows ankle dorsiflexion in midstance, limits plantarflexion in stance & swing

Hinged Ankle Foot Orthosis

• Mild, moderately correctible foot deformity

• Mild spasticity

• Getting up from floor, out of chair, climbing stairs HINGED ANKLE FOOT ORTHOSIS

• Primary Contraindication: a child with excessive ankle dorsiflexion in midstance !

• It does not offer adequate control of forward movement of tibia

• “bulky, costly, & clumsy” Woo J Child Neurol 16 2001 Posterior Leaf Spring

• Dorsiflexory assist in swing-preposition foot for heel initial contact

• Allows some midstance dorsiflexion

• Promote push-off flexible forefoot allows normal foot roll over

• Post CVA OPEN MALLEOLAR ANKLE FOOT ORTHOSIS with carbon fiber reinforcement Courtesy of Paul Jordan, D.P.M. podpediatrics. net • Medial-lateral trim lines more anterior (not posterior like PLS) • Leave the tibial varum in your positive cast • Malleolar cut outs • Carbon fiber composite ½-3/4 inch wide • SBR plantar posting along full base of foot plate • Velcro straps as needed • Very flexible or no plastic distal to MPJ’s Note straps and full SBR plantar posting Podopediatrics.net Anterior movement of tibia over foot Left Hemiparetic This is a hinge, the child’s own ankle. Triple ply carbon composite laminate embedded in posterior shell offers assist in controlled forward rotation of tibia. Open malleolar, carbon composite reinforced diagonally to allow anterior rotation of tibia over ankle when foot is weight bearing without free fall The design allows transition from sitting in a chair to standing and back to sitting This design blocks hyperextension of knee Spastic Diplegic GROUND REACTION FORCE

• Foot & ankle alignment & activity of ankle plantarflexors determine location of GRF

• Ankle plantarflexion associated with knee extension (GRF more anterior)

• Ankle dorsiflexion is associated with knee flexion (GRF more posterior) Buckon Comparison of 3 AFO Configurations for Children with Spastic Diplegia Dev Med Child Neurol. 46 (9) 2004

• Study compared use of SAFO, HAFO & PLS in 16 CP children. All AFO’s improved ankle kinematics in stance, increased stride length, decreased cadence, & improved walking, running, jumping and upper extremity coordination

Buckon- Comparison of 3 AFO Configuration for Children with Spastic Diplegia

• However, some children showed negative effects with hinged afo’s (excessive ankle dorsiflexion in stance, decreased walking velocity, and greater energy cost). “Therefore, constraining ankle motion by use of PLS or SAFO should be considered for most, but not all, children with spastic diplegia.”

CURRENT LITERATURE

• “Current studies suggest that a spring type of flexible AFO that limits dorsiflexion without plantarflexion best imitates the physiologic stretching of the gastrocnemius” Woo

• Hinged AFO harder to fit shoes, more expensive, less durable. “ For these reasons, the PLSO is preferred over the articulating AFO.” Davids Journal of the American Academy of Orthopedic Surgeons 15:3 2007

Avoid KAFO’s in CP Children 1 YEAR OLD MALE C.C. “feet turn out & look funny” Rolled in both directions at 7 months of age Sat up at 10 months of age Not ambulatory but pulling up to stand Physical Examination

• Up and out posture

• Marked ankle dorsiflexion

• 90 degrees lateral femoral rotation 70 degrees medial rotation 1 Year Old Male

Traction Response Vertical Suspension Optical Righting CLINICAL IMPRESSION? Calcaneovalgus Secondary to Hypotonia Angelman Syndrome-P.T. & Supramalleolar Orthoses HYPOTONIC CHILD “ FLOPPY BABY” “The diagnosis of hypotonia may, to some extent, be … based on the resistance of a limb

to passive movement, or even on the postures which the child adopts.” Dubowitz

FLOPPY BABY SYNDROME

• LITTLE SPONTANEOUS MOVEMENTS

• DECREASED RESISTANCE TO PASSIVE MOVEMENT FLOPPY INFANT SYNDROME

• CEREBRAL PALSY

• CHROMOSOME ABNORMALITIES

• SPINAL CORD

• PERIPHERAL NERVES

• NEUROMUSCULAR JUNCTION

• MUSCLE DISEASE

CEREBRAL PALSY static encephalopathy

• MOST COMMON CAUSE OF INFANTILE HYPOTONIA 2 YEAR OLD HYPOTONIC MALE

• PREMATURE BIRTH, LOW BIRTH WEIGHT

• LATE MILESTONES, NOT WALKING

• “FLAT FEET” 2 YEAR OLD HYPOTONIC MALE HYPOTONIC 2 YEAR OLD VERTICAL SUSPENSION TEST Hypotonia associated with cerebral palsy FLOPPY BABY SYNDROME 3 CLINICAL TESTS

• TRACTION RESPONSE

• VERTICAL SUSPENSION TEST

• HORIZONTAL SUSPENSION TRACTION RESPONSE VERTICAL SUSPENSION TEST HORIZONTAL SUSPENSION TEST TREATMENT? Supramalleolar Orthosis

• Polypropylene shell cut above the malleoli

• Provides medial-lateral stability

• Hypotonic patients

• Allows movement with assistance but does not block forward tibial movement over ankle Supramalleolar Orthosis (SMO)

• Affects swing and stance phase (off & on weight bearing)

• Enhances sensory proprioception

• Functions as UCBL on weight bearing

• Improves postural stability to enhance motor performance Supramalleolar Orthosis hypotonic children, developmental delay, sensory integration disorders, genetic syndromes

such as Down Syndrome BEFORE AND AFTER 22 MONTH OLD HYPOTONIC CHILD 22 month old hypotonic female 22 month old hypotonic female 6 years of age what now? UCBL

• Device with long, high medial and lateral flanges and a deep heel seat

• Recommended for children under 8

• Moderate to severe flat foot UCBL

• “Redirect the lower extremity over the foot and enhance proprioceptive cues”-Jordan

• Weight bearing orthosis that works best in midstance, not swing phase UCBL

• Thin thermoplastic (polyethylene, polypropylene)

• Capture plantar contours and medial and lateral arches

• A thinner thermoplastic shell in conjunction with a plantar fill

• Improves patient tolerance but still provides adequate strength Imhauser Foot & Ankle Int. 22 (8) 2002

• In study of foot orthoses & ankle braces on cadaver models, UCBL only device to partially

restore arch & rearfoot kinematics WHAT IS WRONG WITH THIS DESIGN ? Stiff full length foot plate blocks mpj motion and associated hip extension DO NOT EXTEND FOOT PLATE PAST MPJ’S ! Add ankle strap in addition to tibial strap for ankle foot orthoses!

Imhauser Foot & Ankle International Vol. 22 No. 8 August 2002

• “ An orthosis must be well fitted to the shape of the patient’s foot in order to most effectively restore the alignment of the arch & hindfoot.”

11 YEAR OLD MALE

• Spastic right hemiparesis, seizure disorder

• Keppra

• Foster care 11 YEAR OLD RIGHT HEMIPARETIC

• 5-6 beats of clonus on ankle dorsiflexion to 90 degrees

• Mild hamstring equinus

• Hypertonicity of right lower extremity & upper extremity GAIT PATTERN TREATMENT PLAN

• Right custom molded solid AFO with ankle & tibial straps

• Left custom molded foot orthosis with RF posting 12 YEAR OLD FEMALE

• Chief Complaint; “infected toe”, prescribed antibiotic and topical cream PHYSICAL EXAMINATION • Peroneal strength 2/5, tib ant strength 4/5, weakness of hand intrinsics • Vibratory sensation diminished • Pt can toe walk but not heel walk

• Negative Gower’s sign • Absent ankle reflexes

GAIT PATTERN CLINICAL IMPRESSION ?

• PERIPHERAL NEUROPATHY

• Next Step?

• Referral to neurologist

• EMG/NCV

• CMT 1 5 Year Old Male

• Chief Complaint : “walking on the outside of his feet, toe walking”

• Started 2 years earlier

• Referred for “metatarsus adductus” Physical Findings

• Decreased vibratory sensation

• Absent ankle reflexes

• Decreased pinprick sensation

• Cannot heel walk

• Negative Gower’s sign

Radiographs ELECTRODIAGNOSTIC STUDIES

• HMSN I -NORMAL OR SLIGHTLY REDUCED AMPLITUDE, SLOW VELOCITY

• HMSN II -LOW AMPLITUDE, NORMAL VELOCITY - NEURONAL TYPE ( AXONAL DEGENERATION) Neurologic Workup

• EMG/NCV-HALLMARK DIAGNOSTIC TEST

• NCV was significantly SLOWED- consistent with a demyelinating peripheral neuropathy DNA TESTING

• PMP 22 duplication testing confirms diagnosis

• Simple blood test makes it possible to diagnose and rule out other family members CHARCOT MARIE TOOTH DISEASE

• PROGRESSIVE MUSCLE WASTING & WEAKNESS, SENSORY DEFICIT

• HMSN- hereditary motor and sensory neuropathy

• Symmetrical

• Most common cause of neurologic cavus foot

• Chief complaints: falling, clumsiness, gait abnormalities, high arched foot , difficulty in fitting shoes, corns and calluses, metatarsalgia, ankle sprains

CMT TYPE I

• Hypertrophic, demyelinating degeneration of post. columns, spinocerebellar tracts, & nerve roots

• Begins in feet & legs & spreads to hands & arms

• Distal sensory loss ( vibratory)

• Nerve enlargement CMT TYPE I

• DECREASED OR ABSENT ANKLE REFLEXES

• PES CAVUS, CLAW TOES,STORK LEGS

• FOOT DROP, STEPPAGE GAIT

• Over time a decrease in muscle strength, fatigue, foot & ankle deformities and altered balance, reduction in aerobic capacity, with resulting low levels of daily activity

HEEL WALKING ANKLE DORSIFLEXION STRENGTH PERONEAL STRENGTH HAND STRENGTH SPLINTING FOR CHARCOT MARIE TOOTH

• Posterior ankle foot orthoses associated with increased speed & step length, decreased hip & knee flexion in mid swing, & increased ankle dorsiflexion at heel contact

• However, compliance is poor

• Uncomfortable, anaesthetic, hard to find shoes to fit with splint 12 year old male diagnosed for CMT1

Menotti, F, Comparison of walking energy cost between an anterior and a posterior ankle- foot orthosis in people with foot drop J Rehabil Med 2014 46 (8)- people with anterior AFO lower levels of energy costs of walking and better comfort compared with posterior afo Anterior AFO Posterior AFO

Carbon Composite Floor Reaction Orthosis

• Foot drop & moderate soft tissue dysfunction ( shin splints, I or II PTTD)

• Absorbs energy at heel strike & returns it at toe offf 4 YEAR OLD MALE

• CHIEF COMPLAINT; “FLAT FEET AND CLUMSY”

• “ DID NOT KICK A LOT DURING PREGNANCY”

• “CANNOT KEEP UP WITH OTHER KIDS” 4 YEAR OLD MALE

• ABSENT PATELLAR REFLEXES

• USES ARMS TO CLIMB UPSTAIRS 4 YEAR OLD MALE 4 YEAR OLD MALE

• CPK LEVEL 200 X NORMAL VALUE

• DUCHENNE MD CONFIRMED BY MUSCLE BIOPSY EARLY STAGE, FLAT FOOTED GAIT LATER STAGE -PES CAVUS, TOE WALKING APPROACH TO CHILD WITH MUSCULAR DISEASE

• CHECK LIST FOR HISTORY & PHYSICAL (SPIRO-Pediatric Annals)

• GENETIC HISTORY

• SERUM ENZYME LEVELS, ESP. CPK

• MUSCLE BIOPSY Gower’s Sign 12 YEAR OLD MALE 12 YEAR OLD MALE

• LOCAL GIGANTISM

• 2O CAFÉ AU LAIT SPOTS

• AXILLARY FRECKLING NEUROFIBROMATOSIS 3 CLASSIC SIGNS

• CAFÉ AU LAIT SPOTS –5 OR MORE SPOTS MEASURING 1.5 CMS. IN DIAMETER

• MULTIPE SOFT TISSUE TUMORS

• SKELETAL CHANGES ( unilateral anterolateral bowing of tibia)

NEUROFIBROMAS NEUROFIBROMATOSIS 5 year old spina bifida male

• Chief complaints: “ulcer on the left foot” , “left foot turns in”

• Past history of infected ulcers on both feet, left foot started to turn in 1 year ago Treatment: antibiotics for infected ulcers; hinged AFO’s, twister cables for “intoe”

5 YEAR OLD SPINA BIFIDA MALE

• Medical History: L5 myelomeningocoele, neurogenic bladder, hydrocephalus,chronic UTI’s , bladder catheterizations

• Social History: foster care

PHYSICAL EXAMINATION

• Left ankle dorsiflexion minus 45 degrees, Bleck’s test lateral to 5th digit, restricted STJ & MTJ ROM

• Lack of muscle power and sensation TWISTER CABLES Bilateral Hinged Ankle Foot Orthoses RADIOGRAPHS

Are hinged AFO’s appropriate treatment for this patient? What should be done?

• Surgical correction of left TEV

• Post op splinting What should be done before the foot surgery?

QUESTIONS?????