Neuromuscular Disorders

1. Neuromuscular Disorders Dr. Fadel Naim Orthopedic Surgion IUG

2. CerebralPalsy • Umbrella term covering • A group of non-progressive,but often changing, motor impairment syndromes secondary tolesions or anomalies of the brain arising in the early stagesof 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. 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 deliveryis the single most important determinant of CP. • In 24% of cases, no cause is found.

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

6. 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 Causes of CP

7. 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% 5% 12% 10% 1%

9. 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. SpasticForm • 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. AthetoidForm • 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 balanceresulting 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. SpasticityTreatmentModalities • Physical Therapy • OrthoticDevices • OralMuscleRelaxants • IntramuscularInjections • IntrathecalBaclofen(ITB( • Selective Dorsal Rhizotomy (SDR(

15. 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 responseand 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. 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: • 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.