Book Reading

dr. Francisco Gilbert Timothy

dr. Adam Moeljono, Sp.OT (K) Spine
Section 1
General Orthopaedics
Chapter 10
Neuromuscular disorder

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Nerves and muscle
Nervous pathways
• Somatic motor system
• Somatic sensory system
• Reflex activity and tone
• Autonomic system

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Peripheral nerves Skeletal muscle

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• Muscle fibres • Muscle wasting
• Type I and type II • Disuse vs denervation
• Anatomical site, function,
training, genetic disposition,
• Muscle fasciculation
previous injury or illness • Muscle twitch

• Muscle contraction • Clonus

• Isometric contraction • Involuntary rhythmic muscle
contraction
• Isotonic contraction
• Muscle tone
• Resting muscle tension when
passively stretch
• Muscle contracture
• Normally innervated muscle
held immobile in shortened
position

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Clinical assessment
• History
• Age, past medical history,
muscle weakness, numbness
and paraesthesia, deformity,
non-orthopaedic problems,
family history
• Examination
• Gait and posture
• Antalgic, scissoring, drop-foot,
high-stepping, waddling, ataxic,
dystonia
• Motor power and tone
• MRC muscle power grading
• Weakness
• Paresis vs paralysis, type
• Deformity
• Unbalanced vs balanced
• Sensation
• Autonomic system

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• Imaging
• X-ray, CT, MRI, PET scan
• Additional investigations
• Blood and CSF
• Muscle biopsy

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Neurophysiological studies
Nerve conduction studies
• Motor nerve conduction
• Latency, evoked CMAP
• Sensory nerve conduction
• SNAP

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Electromyography (EMG)
• Increase in number of
motor unit=increase in
amplitude

Full interference

Small amplitude motor units

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Diagnostic evaluation of the patient
• Depends on clinical presentation and diagnosis
• Distinguishing nerve root disease from peripheral
entrapment
• Proximal root entrapment: SNAP normal, CMAP reduced

Intraoperative neurophysiological techniques

• Spinal monitoring: somatosensory evoked responses
• Other intraoperative techniques
• Nerve or nerve root stimulation; intraoperative EMG with needle in
situ; cord-to-cord stimulation and cord-to-cortical potential
measurement

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Cerebral palsy
• Permanent disorders of movement and posture attributed
to non-progressive disturbances in the developing fetal or
infant brain
• 2-3 per 1000 live births→40-100 per 1000 live births

Classification
• Topographic distribution
• Unilateral: hemiplegia, monoplegia
• Bilateral: diplegia, total body involvement
• Type of motor disorder
• Spasticity
• Dyskinetic: dystonic, choreoathetotic
• Ataxia
• Mixed form
• Hypotonia
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Diagnosis before walking age
• History of pre and peri-natal event, neonatal USG
• Bleck premature reflex
• Primitive neck-righting reflex, asymmetrical and symmetrical tonic
neck reflex, Moro reflex, extensor thrust response
• Difficulty sucking and swallowing
• Delayed motor milestones

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Diagnosis in later childhood
• Multidisciplinary team
• Gross Motor Function Classification System

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• Sitting posture
• Difficult or impossible to sit
unsupported, kyphotic posture,
poor head control
• Standing posture
• Hips flexed, adducted,
internally rotated; knees flexed;
feet equinus
• Gait
• Usage of walking aids, shoes or
orthotic supports
• Abnormal postures, fluidity of
movements, balance, start/stop
• Normal vs best behaviour
• Clinical gait analysis: scissoring
action
• Computerized gait analysis:
kinematics, kinetics, EMG,
pedobarography, metabolic
energy analysis

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• Neuromuscular examination
• Increased muscle tone, brisk
reflexes, positive Babinski and
clonus
• Physical sign vary from time to
time
• Fixed vs flexible contracture
• Silfverskioeld test
• Bony deformity and changes in
joint congruity
• Persistence femoral neck
anteversion
• External tibial torsion
• Patella alta
• Scoliosis
• Flexible→structural
• Sensory examination
• Stereognosis
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Management
• Goal setting
• Peak of physical function in late childhood
• Priorities for all CP: communication, cope with ADL, independent
mobility
• Priorities for non walking: straight spine and level pelvis, mobile hips
and knees, plantigrade feet
• Tone management
• Medical treatment: anticonvulsant, analgesic, GABA agonist
• Botulinum toxin (BoNT-A)
• Selective dorsal rhizotomy (SDR)
• Physical therapy
• Positioning and splinting
• Manipulation and serial casting

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• Operative treatment
• Indication: spastic deformity with failed conservative, fixed
deformity that interferes function, secondary complication
• Muscle lengthening→ improve mechanical alignment
• Tendon transfer

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Regional survey
• Upper limb:
Spastic hemiplegia or total body involvement
• Elbow flexion deformity
• Forearm pronation deformity
• Wrist flexion deformity
• Flexion deformity of the fingers
• Thumb in palm deformity

• Lower limb:
Unilateral cerebral palsy (UCP)- hemiplegia
• Foot/ankle: equinovarus foot deformity; dynamic varus deformity,
pes planovalgus
• Hip/knee:
• Leg length discrepancy

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Bilateral cerebral palsy-spastic diplegia
• Hip adduction deformity
• Hip flexion deformity
• Hip internal rotation
deformity
• Hip subluxation
• Knee flexion deformity
• Knee extension deformity
• External tibial torsion
• Ankle equinus
• Single event multilevel
surgery (SEMLS)

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• Total body involvement (TBI)
Hip
Spine/pelvis
Other joints

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Adult-acquired spastic paresis

• Cerebral damage due to stroke or head injuries

• Physiotherapy, splintage, tone-reducing medication,
occupational therapy
• Operative treatment
• Sufficient cognitive ability, awareness of body position in space,
good psychological impetus
• Upper limb: shoulder adduction and internal rotation, flexion of
elbow, wrist and MCP joints.
• Lower limb: foot equinus or equinovarus, knee flexion, hip
adduction

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Lesions of the spinal cord

• Clinical features
• Cervical cord compression
• Thoracic cord compression
• Lumbar cord compression
• Brown-Sequard lesion
• Diagnosis and management
• Traumatic and compressive
• Plain X-ray: spine structural
abnormalities
• MRI: cord compression

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Spina bifida

• Pathology

Spina bifida occulta Meningocele Myelomeningocele Open

Myelomeningocele
• Incidence and screening
• 2-3 per 1000 live births
• Alpha-fetoprotein in blood and amniotic fluid
• Mid-term high-resolution ultrasound scan

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• Clinical features Diagnosis in older children
Early diagnosis • Tethered cord syndrome→
• Complete LMN paralysis and MRI with gadolinium
distal segment have loss of enhancement
sensation and sphincter • Orthopaedic deformity
control reassessed following
• Complete lesion at some neurosurgical tether release
level but distal segment
preserved
• Incomplete cord lesion and
some preserved sensation
and movement
• Skin dimple, pit, tuft of hair
or pigmented lesion
• USG, MRI

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• Treatment
• Dress the wound and prevent infection
• Neurosurgical closure within 48 hours
• Assessment of hydrocephalus

Orthopaedic management
• Intellectual and neuromuscular function assessment at 3-4 years
• Maintenance and development of intellectual skills and upper limb
functions
• Best predictor of walking ability and function is motor level of
paralysis
• Immobilization and muscle imbalance lead to joint deformity and
risk of pathological fracture

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Regional survey
• Spine
• Distal tethering
• Kyphosis
• Neuromuscular scoliosis
• Hip
• Lesion above L1, between L1-S1 and below S1
• Knee
• Fixed flexion
• Hyperextension contracture
• Foot
• Flail foot or balance paralysis or weakness
• Talipes equinovarus
• Vertical talus deformity
• Toe deformity

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Sacral agenesis

• Abnormal development of the lower spine and other

organ systems in the lower half of the body
• Clinical manifestation
• Appears like low-level spina bifida
• Buddha position

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Poliomyelitis
• Clinical features
• Acute illness
• Paralysis
• Recovery and convalescence
• Residual paralysis
• Post-polio syndrome
• Early treatment
• Acute phase: isolation, complete rest, symptomatic treatment, passive
stretching
• Afterward: physiotherapy, active movement, splintage
• Late treatment
• Isolated muscle weakness without deformity
• Passively correctible deformity
• Fixed deformity
• Flail joint
• Shortening
• Disturbance of skeletal modelling
• Vascular dysfunction
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Regional survey • Knee
• Shoulder • Instability
• Abduction • Fixed flexion
• Elbow and forearm • Genu recurvatum
• Elbow flexion • Foot
• Forearm pronation and • Foot drop
supination
• Hindfoot varus or valgus
• Wrist and hand • Claw toes deformity
• Wrist deformity and
instability
• Finger opposition
• Spine
• Long thoracolumbar scoliotic
curve
• Hip
• Subluxation or dislocation

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Motor neuron disorders

• Motor neuron disease (MND)

UMN and LMN symptoms
• Amyotrophic lateral sclerosis (ALS)
Dysarthria, difficulty swallowing, muscle weakness, wasting with
exaggerated reflexes, muscle cramps, muscle atrophy, fasciculations
Normal sensations and bladder control
• Spinal muscular atrophy
Progressive LMN weakness
• Werdnig-Hoffman disease
Floppy and weak baby, feeding difficulty, shallow breathing
• Kugelberg-Welander disease
Limb weakness, proximal muscle wasting, paralytic scoliosis,
delayed walking

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Peripheral neuropathies

• Classification
• Radiculopathy
• Plexopathy
• Distal neuropathy:
• Mononeuropathy,
• Multiple mononeuropathy,
• Polyneuropathy

• Pathology
• Acute axonal interruption:
Complete and immediate
• Chronic axonal degeneration:
Slow and progressive; large vs small fibres
• Demyelinating neuropathies
Slowing of conduction to complete nerve blocks

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Clinical features
• Symptoms
• Sensory: pins and needles
numbness, burning, shooting
pain
• Motoric: muscle weakness,
clumsiness, loss of balance while
walking
• Deformity: claw hand, cavus foot
• History
• Injury, recent infection, diabetes,
malignancy, alcohol abuse,
nutritional deficiency
• Examination
• Motor weakness, trophic skin
changes, depressed deep
sensation, pain sensibility and
proprioception

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Hereditary neuropathies
• Hereditary sensory neuropathy
• Congenital insensitive to pain and temperature
• Hereditary motor and sensory neuropathy (HMSN)
• HMSN type I: claw toes, pes cavus or cavovarus, severe wasting of
lower and upper limbs
• HMSN type II: milder foot deformity and peroneal muscle wasting
• Hereditary neuropathy with liability to pressure palsies
(HNPP)
• Multiple mixed entrapment mononeuropathy
• Friedrich ataxia
• Spinocerebellar dysfunction with degeneration of the posterior root
ganglia and peripheral nerves

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Metabolic neuropathies
• Diabetic neuropathy
• Numbness and paraesthesiae, areflexia, diminished vibration sense
• Muscle weakness, neuropathic ulcers, regional osteoporosis,
insufficiency fractures of the foot bones, Charcot joints, loss of
balance, autonomic dysfunction
• Alcoholic neuropathy
• Burning paraesthesiae, numbness, muscle weakness
• Tenderness in calf muscles and depressed or absent reflex

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Infective neuropathies
• Herpes zoster (shingles)
• Severe unilateral pain in the distribution of several adjacent nerve
roots
• Irritating vesicular rash along the dermatomes of affected nerves
• Neuralgic amyotrophy (acute brachial neuritis)
• Sudden, intense, unabating pain in the shoulder and arm, extending
to the neck and hand for 2-3 weeks
• Scapular winging, shoulder girdle muscles wasting, sensory loss,
paraesthesia
• Guillain-Barre syndrome (acute inflammatory
demyelinating polyneuropathy-AIDP)
• Aching and weakness in the legs, numbness and paraesthesiae that
steadily progresses upwards
• Areflexia, loss of position sense, autonomic dysfunction
• Leprosy

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Arthrogryposis

• Classification
• Total body involvement
• Predominantly hand or foot
• Pterygia syndromes
• Clinical features
• Tubular limbs with featureless
joints, reduced muscle mass
• Classic form of amyoplasia
• Distal arthrogryposis
• Treatment
• Physiotherapy (manipulation,
stretching, splinting)
• Operative

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Muscular dystrophies

Duchenne muscular dystrophy

• Clinical features
• Difficulty standing and climbing stairs
• Lower limbs, upper limbs, spine
• Investigations
• Serum creatinine phosphokinase
• Muscle biopsy and PCR
• Treatment
• Physiotherapy, splintage, muscle-lengthening procedures
• Early instrumentation and spinal fusion for scoliosis

Becker muscular dystrophy

• Less severe DMD, retain ability to walk into teens, facial
muscle not affected, normal bowel and bladder muscle
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Limb girdle dystrophy
• Pelvic girdle: waddling gait, difficulty in rising from a low
chair
• Shoulder girdle: difficult to raise arms above the head
• Treatment: physiotherapy and splintage, operative
correction

Facioscapulohumeral dystrophy
• Facial (inability to purse lips, close eyes tightly)
• Scapular muscle (scapula winging, difficulty with shoulder
abduction)

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Myotonia

• Dystrophia myotonica
• Adult onset muscular dystrophy presenting as distal muscle
weakness and wasting
• Type I: hands, lower legs, face and neck
• Type II: shoulder, elbow, hip
• Myotonia congenita
• Younger children or infancy
• Congenital: slowly progressing muscle stiffness esp after period of
inactivity

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Pain

• Pain perception
• Subjective experience
• Pain receptors
• Tissue distortion, chemical irritation
• Pain transmission
• Ipsilateral reflex motor and autonomic
• Contralateral spinothalamic tracts
• Pain modulation
• Simultaneous sensory impulses
• Impulses descending from the brain
• Pain threshold
• Result of mixed factors + psychological background
• Lowered or elevated

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• Acute pain
• Removing or counteracting
the disorder
• Making patient feel
comfortable and secure
• Splinting/resting the painful
area
• Analgesics, NSAID, narcotics
• Alleviating anxiety
• Chronic pain
• Alleviating underlying
disorder
• General analgesic therapy
• Rehabilitative and
psychological supportive
measure

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• Complex regional pain syndrome
(CRPS)
• Sudeck’s atrophy, reflex sympathetic
dystrophy, algodystrophy, shoulder-
hand syndrome, causalgia
Pathogenesis
• Abnormal cytokine release, neurogenic
inflammation, sympathetic-mediated
enhancement of pain responses
Clinical manifestation:
• Severe out of proportion pain,
vasomotor instability, trophic skin
changes, regional osteoporosis, joint
stiffness, fixed deformities
Treatment
• Reassurance, anti-inflammatory drugs,
physiotherapy, calcium regulating
drugs, TCA

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The ‘syndrome’ of chronic pain
• Pain of 6 months or more that has no obvious physical
cause and does not respond well to standard treatments
• Change perception part then identify remaining true
orthopaedic problems
• Pain management
• Analgesics and anti-inflammatory drugs
• Local injections to painful area
• Local counter-irritants
• Acupuncture
• Transcutaneous nerve stimulation
• Sympathetic block
• Surgical interruption of pain pathways

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Fibromyalgia

• Widespread pain and tenderness in the muscles and soft

tissues with complete absence of pathological changes
• American College of Rheumatology
• Widespread pain in all four quadrants of the body with at least nine
pairs of designated tender points
• Treatment
• Physiotherapy and daily exercise
• Analgesics, low dose amitriptyline, gabapentin and pregabalin

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Thanks!