Neuromuscu

lar Disorders

CLINICAL

ASSESSMENT

History
Age is important. The orthopaedic
surgeon deals mainly with the residual
effects of neurological disease, and
these may require diagnosis and
treatment throughout life.
Muscle weakness.
The type of
weakness, its distribution and rate of
onset are important clues to diagnosis.

 Numbness and paraesthesiae may

be the main complaints. It is important
to establish their distribution as this
will often localize the lesion.
Deformity: common complaint
long-standing disorders.

in

Other features such as headache,

dizziness, loss of balance, change in
visual, acuity of hearing, disorder of
speech, and loss of bladder or bowel
control.

EXAMINATION
Include

a complete neurological
assessment
Particular attention
to:
muscle
wasting,

mental state,
muscle tone &
power,
natural posture,
reflexes,
gait,
sense of balance, skin changes,
involuntary movements,
the various mode of sensibility and
autonomic functions such as sphincter
control, peripheral blood flow and

Dermatomes
supplied by the
spinal nerve
roots

Some effects of neurological

disorders
These patients, all of whom had polio,
illustrate some of the effects of
paralysis: deformity, wasting, and

The Back
Examine:
skin change,
local deformities (e.g. kyphos),
and
mobility.

Grading Muscle Power

0
1
2
3
4
5

:
:
:
:
:
:

total paralysis
barely detectable contracture
not enough power to act against gravity
strong enough to act against gravity
still stronger but less than normal
full power

In assessment it is important to
examine not only individual mucles
but also functional groups.

Deformity
A

major problem in long-standing

disorders.
Arises when 1 group of muscles
is too weak to balance the pull of
antagonists (unbalanced
paralysis).
When all muscles groups are
equally weak (balanced paralysis)
the joint simply assumes the
position imposed on it by gravity.

Paralysis

arising in childhood
seriously affect bone growth:
The bone is both thinner and shorter
Modeling is defective
The bone ends may appear
dysplastic
There may be loss of joint congruity

Gait & Posture

Watch

the patient walk

Spastic gait:

stiff and jerky,

often with the feet in equinus,
the knees somewhat flexed and
the hips adducted (scissoring).

abnormal posturing
of any part of the body, often
aggravated when the patient
concentrates on movement.

Dystonia

High-stepping

gait :

the legs are lifted unnecessarily high off

the ground,
signifies either a problem with
proprioception and balance or bilateral foot
drop.
Drop-foot

gait :

due to peripheral neuropathy or injury of

the nerves supplying the dorsiflexors of the
ankle.
During the swing phase, the foot falls into
equinus (drops) and if it were not lifted
higher than usual the toes would drag
along the ground.

Waddling

gait:

the trunk is thrown from side to side

with each step
may be due to dislocation of the hip
or to weakness of the abductor
muscles.
Ataxia

Produces a more obvious and

irregular loss of balance
Broad-based gait, uncontrollable
staggering

Imaging Studies
Plain

x-ray of the skull and/or spine

are routine for all disorders of CNS.
Further study: myelography, CT or
MRI.
Imaging of the spine aimed at
demonstrating compression of the
spinal cord or nerve roots.
Fractures and dislocations plain xray. But a CT scan will reveal the exact
relationship of bone fragments to
nerve structures.

intervertebral disc
diagnosed on clinical
examination. Myelography, CT,
MRI exact lesion site.

Prolapsed

Narrowing

of the spinal canal

CT.
Destructive

lesions of the bones

plain x-ray.
CT, MRI and myelography are
helpful.

a.
b.
c.

The pedicles have been destroyed by tumour

Neurofibroma
Degenerations of the facet joints with encroachment
on the intervertebral foramina & spinal canal

Electrodiagnosis
EMG

(electromyography) records
the motor response to nerve
stimuli.
Decide whether muscle weakness is
due to nerve or muscle disorder

conduction studies
establish the site of compression
in peripheral nerve entrapment.

Nerve

Other Special
Investigations
Blood

tests (cell counts, ESR,

serology, blood sugar, muscle
enzymes)
CSF (cerebrospinal fluid)
examination
Specialized tests for vision,
hearing, speech and mental
capacity.
Muscle biopsy

Principles of Orthopaedic Treatment

of Neurological Disorders
Prevention

of
musculoskeletal
deformity Passive movement at
involved joint, several minutes each
day
Correction
of
existing
musculoskeletal deformity Passive
stretching or Surgical procedures

of muscle balance
Muscle & tendon transfers
Improvement
of
function

Functional braces
Improvement
of
gait
or
appearance

Surgical
lengthening or surgical shortening
Rehabilitation
Improvement

Treatment of the Principal Deformities of

the Limbs
DEFORMITY SPLINTAGE

SURGERY

Equinus

Springloaded
dorsoflexion

Lengthen tendo Achilles

Equinovarus

Bracing in
eversion and
dorsoflexion

Lengthen tendo Achilles

and transfer bilateral half
of tibialis anterior to
cuboid

Knee

Flexion

Long caliper

Hamstring release

Hip

Adduction

Obturator neurotectomy
Adductor muscle release

Shoulde Adduction
r

Subscapularis release

Elbow

Flexion

Release elbow flexors

Wrist

Flexion

Wrist splint

Lengthen or release wrist

flexors

Foot

DISORDERS OF THE BRAIN

Cerebral Palsy
Various

types and degrees of

nonprogressive brain disorders, develop
soon after birth
Mental disability
Incidence is relatively common, 6 affected
child among 100.000 population each year
Etiology:
- Prenatal (Genetics, Congenital cerebral
malformations, prenatal intracranial
hemorhage.)
- Postnatal (Rh incompatibility, kern incterus,
cerebral infections, head injury

Pathogenesis & Pathology

Brain

lesion is not progressive

Manifestations of brain lesion
determined by the area involved :
Spastic type : corticospinal system
lesion in cerebral motor cortex, 65%
Athetoid type : extrapyramidal system
lesion in basal ganglia, 20%
Ataxic type : cerebellar and brain stem
lesion, 5%
3 additional type: tremor, rigidity, atonia

Topographic distribution:
Hemiplegia the commonest
Palsy of the upper and lower limbs on
one side of the body
Diplegia

Affects mainly the lower limbs

Total

body involvement

General disorder affecting all 4 limbs,

the trunk, neck, and face.
Other

terms such as monoplegia,

triplegia, and tetraplegia

Clinical Features and Diagnosis:

Retarded

motor development
Motor and sensory deficits
Short attention span
Various types of CP are not
obvious during the early months
of postnatal development.

Spastic

Type

Paralysis of patterns of voluntary

movement
Increased muscle tone
(hypertonicity, spasticity,
increased deep tendon reflex, and
clonus)
Spastic gait
Difficult in learning to speak clearly
Dysphagia (spastic paralysis of the
muscles of swallowing)

Athetoid

type

Involuntary, uncontrollable
movements (mobile spasms) in the
muscle groups of the face and all 4
limbs.
Athetotic muscles twisting, writhing
contortions in the limbs, meaningless
grimaces in the face, difficulty with
speech and swallowing.
Absent during sleep.

Ataxic

type

Relative lack of equilibrium.

The gait is unsteady; frequently
appears about to fall, although this is
usually prevented by using the arms
to maintain balance.

a.
b.
c.

Scissors stance
Flexion deformity of hips and knees with equinus of
the feet
Characteristic facial expression and limb deformities

Treatment
Requires

combined skills of the

family physician, rehabilitation
physician, neurologist,
orthopaedic surgeon,
neurosurgeon, psychologist,
physical, occupational and
speech therapist, medical social
worker, and teacher.

Therapeutic

drugs

No type of drug can affect the brain lesion

Injection of Botulinum A-toxin for very
young children with spastic diplegia or
quadriplegia and dynamic deformities of
the lower limbs but no structural
contracture
Physical

and Occupational Therapy

To encourage muscle relaxation, improve

muscle coordination and develop voluntary
muscle control
Simple activity standing, walking,
eating, dressing painstaking and
repetitive training
Daily passive stretching of spastic muscle

Speech

Therapy

Prolonged therapy can be

improved to the point of being
reasonably intelligible.
Orthopaedic

Appliances

Removable splints prevent

deformity
Braces for lower limbs
Crutches
Correct deformities and improve
function by physical therapy and
operations.

Surgical

Manipulation

Correction of fixed deformities by

stretching muscle contractures under
general anesthesia
Maintained by removable splints
Orthopaedic

Operations

Not indicated until the child has at

least develop kneeling balance.
Tendon lengthening, tendon transfer,
arthrodesis.

Neurosurgical

Operations
Selective dorsal rhizotomy
Decrease the stimulating inputs from
the muscle spindles in the lower
limbs that arrive in the spinal cord
via afferent fibres in the dorsal nerve
roots.
Troublesome pattern of athetoid
movement can be diminished by
selective neurotectomy.
Ataxic type not amenable to
surgical treatment.

Prognosis
Repeated mental and physical
assessment over many months is
necessary to establish a realistic
prognosis.
In general:
hemiplegics 70% have normal
intelligence and all will walk
Spastic diplegics 60% have IQ
above 80 and 75% will walk
Quadriplegics 90% have an
intellectual disability and only 25%
will walk.

Cerebrovascular Disease &

Hemiplegia
The

most catastrophic complication of

the various types of cerebrovascular
disease STROKE; caused by
hemorrhage, thrombosis, or embolism.
Treatment psychotherapy, physical
and occupational therapy, light braces,
selective nerve blocks to relieve
spasticity and, occasionally, tendon
transfers to restore muscle balance
and improve function.

DISORDERS AND INJURIES

OF THE SPINAL CORD

Syringomyelia
Slow

but progressive enlargement of an

abnormal cavity within the spinal cord,
most commonly in the cervical region.
More than half of patients associated
with prolapse of the cerebellar tonsils
through the foramen magnum of the
skull (the Chiari malformation).
Dissociated sensory loss loss of pain
and temperature sensation but
preservation of light touch, vibration
and position sense.

imaging MRI
Neurosurgical drainage of the
syrinx reduce the fluid
pressure on the spinal cord.
Syringomyelia with a Chiari
malformation neurosurgical
decompression of the foramen
magnum.
Diagnostic

Poliomyelitis
Viral

infection
Affects the motor cells (anterior
horn cells) of the spinal cord
Producing permanent paralysis

Clinical

features:

Prodromal phase (2 days): non

spesific systemic symptoms common
to many viral infections
Acute phase;
fever, severe headache, neck rigidity,
painful spasm, tenderness in affected
muscles.
CSF lymphocytes >>>
2 months flaccid paralysis

Recovery phase (up to 2 years)

gradual recovery of any transient
paralysis. 1/3 of the patients will
make a complete recovery.

Shortening and wasting of the left

leg, with equinus of the ankle
b. Paralytic scoliosis
c. This boy is trying to abduct both
arms but the right deltoid and
supraspinatus are paralyzed
a.

Treatment:

Acute phase
Kept in bed, treated
symptomatically
Removable splint
The joint of paralyzed limb are
gently put through a full range
of motion for several minutes
each day (after muscle spasm
has subsided)

Recovery phase
Active exercises

 Operative treatment is deferred

until there is no further hope of
muscle recovery.
The most efficacious surgical
operations:
Tendon lengthening
Tendon transfer
Tenodesis
Osteotomy near a joint
Arthrodesis
Leg-length equalization

Cervical myelopathy
caused

by spondylosis is a
capricious condition with a lack of
correlation between the range of
symptoms (headache, giddiness,
drop attacks, numb or 'useless
hands', muscle wasting, etc.),
their severity, and the
radiological severity of the
spondylosis.

In

many patients there are a

number of symptoms but few
neurological signs.
In a few patients, there may be a
paraparesis or segmental muscle
wasting, as in this patient with
spinal subluxation at the level of
C6/7

Some

patients (usually the elderly)

with normal power in the hands
complain of a loss of feeling and of
difficulty in performing daily
activities, such as dressing, shaving
and using toilet paper.
Their hands are strong and show no
muscle wasting but, when the
patient's eyes are closed, the fingers
tend to wander ('pseudoathetosis' or
sensory wandering) and the patient is
unable to maintain a stationary
position of the affected arm.

The

sensory loss is usually confined

to posture and vibration; the objects
placed in the patient's hand (with
the eyes closed) cannot be
identified by touch (astereognosis).
The claw hand caused by an injury
to the ulnar nerve differs from that
described already in that the
clawing predominantly affects the
fourth and fifth fingers of the hand,
which also show a slight degree of
abduction.

This deformity is a result of paralysis of the

interossei and the medial two lumbricals, which, in
combination, flex the fingers at the
metacarpophalangeal joints with the distal joints
extended.

The

hypothenar eminence is
flattened (pict 1) with loss of the
ulnar contour, which can be
readily revealed by asking the
patient to fold their hands in the
manner of the Indian greeting
2
(pict 2).
1

There

is guttering of the spaces

between the metacarpals on the
dorsum of the hand
caused by paralysis of the
interossei, which are supplied
by the ulnar nerve. The palm is
hollowed out and there is a zone
of cutaneous anaesthesia along
the ulnar border of the hand, the
fifth
finger and the inner
half of the fourth
finger.

The

patient with an ulnar nerve palsy

is unable to flex the little finger at the
interphalangeal joints (using the short
flexor), adduct (using the palmar
interossei) or abduct (using the dorsal
interossei) the fingers in the affected
hand.
The thumb is also affected because of
paralysis of the adductor and short
flexor muscles.
The weakness of these two muscles can
be revealed by the journal test of
Froment

The journal test of Froment: the

patient has to pinch to hold on to the
paper

The

corresponding elbow must be

examined in every patient with an
ulnar nerve palsy for any evidence
of injury, fracture, dislocation, scar
or arthritis.
A patient with osteoarthrosis of
the elbow joint will be unable to
touch his head with the hand,
while keeping his arm straight at
the shoulder joint

The

ulnar nerve may be

involved in Hansen's disease
(leprosy) producing a typical
claw hand.

The

thickened ulnar nerve may be palpable

in the ulnar groove at the elbow joint.
The nerve may also be injured by
penetrating wounds, and as a late sequel to
callus or scar formation at any point along
its course.
Certain occupations such as roofing,
carpentry and bricklaying are associated
with osteoarthrosis of the elbow and injuries
to the nerve in its shallow olecranon groove.

Carpal Tunnel
Syndrome

the

commonest cause of
a median nerve palsy
caused by compression
of the nerve, as it
traverses the tunnel
under the thick and
inelastic transverse
carpal ligament.
Flattening of the thenar
eminence is the
hallmark of a median
nerve palsy.

Scalding

of the index
and middle fingers
resulting from loss of
sensory perception is
seen rarely today.
The diagnosis is made early on the
strength of a good history (pain and
numbness over the median nerve
distribution, which is worse at night,
often relieved by rubbing and hanging
the arm out of bed) and by functional
evaluation of the thumb and the outer
two fingers (weak abductor pollicis
and opponens pollicis).

This

condition is usually seen in middleaged, obese females. It may be

associated with pregnancy,
myxoedema, acromegaly, rheumatoid
arthritis, tophaceous gout and primary
amyloidosis.

The

extended thumb and

index finger, hollowing of the thenar
eminence with the thumb on a level with
the fingers, and the flat, even appearance
of the palm, produce the monkey (or
simian) hand.

In

complete palsy of the median

nerve in the forearm, the
appearance of the hand and the
forearm is characteristically flat on
the flexor aspect, with slight ulnar
deviation and loss of pulp in the
affected fingers.
The patient can bend the middle
finger but not the terminal phalanx of
the thumb and index finger, which
requires the use of
flexor digitorum
profundus & flexor
pollicis longus.

Abduction

of the thumb is weak

and can be easily overcome from
paralysis of the abductor pollicis
brevis.

Inability

to make a complete fist by

flexing the terminal phalanges of
the thumb and index finger is
caused by an injury to the
anterior interosseous nerve,
which arises from the median
nerve in the forearm. This nerve
supplies the radial half of the flexor
digitorum profundus and flexor
pollicis longus.

Wrist

drop, or a complete
inability to extend the hand
and fingers, is a cardinal
feature of paralysis of the
radial nerve.
Even

when the wrist is

passively held straight, the
fingers remain flexed at
the metacarpophalangeal
joints owing to paralysis of
the long extensors

The

patient is, however, able to

straighten the interphalangeal
joints since these movements are
affected by the lumbricals and
interossei (the ulnar nerve).

DISORDERS OF MUSCLE
Majority

neurogenic rather than

myogenic.

Muscular Dystrophies
A

group of genetically
determined disorders of muscle
(primary myopathies).
Progressive muscle degeneration
and weakness.
Types:
DMD (Duchenne muscular
dystrophy)
BMD (Becker muscular dystrophy)
Limb girdle muscular dystrophy

Duchenne Type
Pseudohypertrophic

muscular dystrophy
Sex-linked recessive trait
Afflicts males only
Absence of dystrophin (a protein normally
present in the sarcolemma of muscle cells)
Tire easily and cannot keep up with his
playmates.
Symmetrical weakness of the pelvic
muscles difficulty in climbing stairs and
standing up from a sitting or lying position.

Gowers sign in muscular

dystrophy

Pseudohypertrophy

in the calf muscles,

the increased bulk of muscle being due
to excessive fibrous tissue and fat.
The muscle and shoulder girdle become
weak.
Deformities secondary to contracture:
progressive paralytic scoliosis.
Progression: relentless.
Lab elevation of certain cellular
enzymes (creatinine phosphokinase and
aldolase alanine transaminase) that
probably arise from affected muscles.

Becker Muscular
Dystrophy
Sex-linked

recessive trait.
Altered dystrophin.
Appears at later age, less severe
and more slowly progressive than
DMD.
Treatment is similar to DMD.

Treatment of MD
No

spesific cure
Multidisciplinary
Prednison can improve strength and
function
Myoblast transfer has not been
proven to be beneficial
Active exercise
Dietary supervision reduce obesity
Light braces, orthopaedic operations

END