1. Neuromuscular Disorders
Dr. Fadel Naim
Orthopedic Surgion
IUG

2. Cerebral Palsy Umbrella term covering
A group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of its development

3. The definition of cerebral palsy stress followed features:
Result of a lesion in the immature brain, which is nonprogressive
It is a static encephalopathy
Results in a disorder of posture and movement which is permanent but not unchanging
(progressive musculoskeletal pathology in most affected children(

4. 10/22/2017
Etiology
Variety of perinatal, prenatal, and postnatal factors contribute, either singly or multifactorily to CP.
Commonly thought to be due to birth asphyxia; Now known to be due to existing prenatal brain abnormalities.
Premature delivery is the single most important determinant of CP.
In 24% of cases, no cause is found.

5. Causes of CP Time (% of cases) Prenatal (44%) First trimester
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Causes of CP
Time (% of cases)
Prenatal (44%)
First trimester
Second trimester
Causes
Teratogens, chromosomal abnormalities, genetic syndromes, brain malformations
Intrauterine infections, problems in fetal/placental functioning

6. Causes of CP Causes Preeclampsia, complications of labor and delivery
10/22/2017
Causes of CP
Time (% of cases)
Labor and delivery (19%)
Perinatal (8%)
Childhood (5%)
Not obvious (24%)
Causes
Preeclampsia, complications of labor and delivery
Sepsis/CNS infection, asphyxia, prematurity
Meningitis, traumatic brain injury, toxins

7. Classification Cerebral palsy can be classified according to:
10/22/2017
Classification
Cerebral palsy can be classified according to:
Pathology of brain injury e.G. Vascular, infective, inflammatory or traumatic
The timing of brain injury such as prenatal, perinatal or postnatal
The modified swedish clasification is the widely used classification which is based on the tone, number and distribution of the affected limbs

8. Pattern of involvement :
Diplegia
Hemiplegia
Triplegia
Quadriplegia (total body)
Type of motor dysfunction :
Spastic / Ataxic / Mixed / Athetoid / hypotonic
65% % % % %

9. The clinical spectrum of CP ranges from:
The spastic variety is the most common, with mixed varieties now being increasingly diagnosed
The clinical spectrum of CP ranges from:
very minor impairment
Patient with mild hemiplegia with minimal gait deviations
Significant cognitive deficits and motor functional limitations
Children with severe total body involvement

10. Spastic Form
Most common form of cerebral palsy affecting about 70-80% of all people with the condition
Results from damage of the motor cortex
Spastic CP can be broken up into 3 subtypes:
(1) hemiplegia, affecting one side,
(2) diplegia, affecting both arms or both legs,
(3) quadriplegia affecting all limbs

11. Athetoid Form
uncontrolled motion, especially in the face, arms and trunk
Interfering with any skill, which requires coordinated movements
speaking, feeding, reaching, grabbing
Damage to the cerebellum
Mixed muscle tone
The child may develop purposeless, involuntary movements.
May increase due to stress and disappear during sleep

12. Ataxic Form Poor balance resulting in stumbles and falls
Damage to the cerebellum
May have tremors that worsen when reach for objects
Ataxic CP only accounts for 10% of individuals with the condition

13. Mixed Forms
Mixed forms of cerebral palsy are also fairly common, occurring in 25% of people with cerebral palsy
Damage to both the motor cortex and the cerebellum
Both spastic muscle tone and involuntary movements
They may also have low muscle tone in some muscles and high tone in others

14. Spasticity Treatment Modalities
Physical Therapy
Orthotic Devices
Oral Muscle Relaxants
Intramuscular Injections
Intrathecal Baclofen (ITB(
Selective Dorsal Rhizotomy (SDR(

15. 10/22/2017
Orthopedic Problems
An extensive musculoskeletal system examination needs to be performed in children with cerebral palsy looking for evidence of:
Osteopenia
Hip subluxation
Scoliosis
Contractures of hamstring muscles and tendo achilles
Equinus deformity
Foot deformity
In ambulatory children observation of gait in the clinic provides useful information about coordination and musculoskeletal abnormalities;

16. Treatment is focused during the early years of growth on:
Tone management
Prevention of contractures
Aids for walking
Multilevel bony and soft-tissue surgery to improve gait

17. General principles of orth. management
Lengthening a spastic muscle can improve its function by diminishing the overactive stretch response and uncovering any control that was present
Surgery should not be reserved for patients with severe impairment and deformity.
The amount of improvement correlates best with the degree of underlying motor control and not with the severity of the deformity
Individuals with milder degrees of impairment can benefit greatly from relatively simple procedures

18. Musculoskeletal surgery aims are classified as hygienic or functional
General principles “functional” and “nonfunctional” surgical procedures
Musculoskeletal surgery aims are classified as hygienic or functional
These terms refer to the expected outcomes for a limb but do not indicate the outcome for the person as a whole.
Surgical releases of an arm contracted in a flexed and internally rotated position in a hemiplegic patient often allows the person to become independent in dressing, even though the arm itself remains nonfunctional

19. “Birthday surgery”
Should be avoided in favor of simultaneous multilevel surgery.
A and B, Young child with diplegia and toe-walking (A) undergoes isolated heel cord lengthening, which increases crouch gait (B)
C, One year later, he has isolated hamstring lengthening and develops hip flexion contracture.
D, One year later, he has hip flexor release to allow him to ambulate in upright position.

20. Spina Bifida Incomplete closure of the laminar arches of the spine
Occurs at birth but may not be detected until later
An unrelated variety, spina bifida occulta, is a small defect in the 5th lumbar or 1st sacral arches, a benign finding of no clinical consequence.

22. To prevent meningitis, the lesion should be closed immediately and associated hydrocephalus controlled by ventriculoperitoneal shunting procedures

23. Spina Bifida
May be a clinically significant problem if it is associated with congenital neurologic deficit at the open bony levels
May occur at any level of the spine or at multiple levels, although it is most common at the caudal aspect of the spine
In the presence of a neurologic deficit, secondary problems may develop in the genitourinary tract and lower limbs.

25. Classification: Spina bifida occulta: Myelomeningocele:
Skin-covered defect in the lower lumbar spine with no neurologic deficit
associated with slightly increased risk of spondylolisthesis
Myelomeningocele:
Combination of several absent laminae, with exposed meninges and usually neurologic deficit at the same level
High risk of hydrocephalus

26. Lipomeningocele: Caudal fatty mass arising from the spinal canal
palpable under the skin
with an associated neurologic deficit
no substantial risk of hydrocephalus

27. The most commonly used classification of myelomeningocele is based on the neurological level of the lesion
thoracic level (no L1 to S4 function)
upper lumbar level (intact to L1 or L2)
lower lumbar level (intact to L3 or L4)
sacral level (intact to L5 or S1)

29. A comprehensive team approach is optimal because patients frequently have multisystem pathology that involves developmental delay, bowel and bladder dysfunction, and progressive joint contractures and bony deformities.