Pedia 2 Module 5C: Common neurologic disorder

Autistic Spectrum Disorder

• most common neurological disorder manifested from 18-36 months of age and results in developmental disability

• a collection of developmental disorders in which a child engages in repetitive behaviors and has trouble communicating and connecting with others

• this affects:

  • communication

  • social understanding

  • behavior, activities and interest

  • 4x more common in males than in females

• characterized by 3 major behaviors:

  • Inattention

  • Impulsiveness

  • Hyperactivity

• main goal for treatment:

  • lessen associated deficits and family distress

  • increase quality of life and functional independence

Regressive autistic spectrum disorder

• child appears to develop normally until 18 - 24 months, than starts to regress

• it occurs in about 20 - 30 percent of children with autism spectrum disorder

Pervasive developmental disorder

• a child has a few autistic traits but doesn’t meet the full criteria for an autism diagnosis

• affects information processing in the brain

• condition characterized by:

  • problem in communicating

  • problem in relating to others

  • a need to follow rigid routines

  • need to engage in repetitive behaviors or language

Asperger’s syndrome

• milder form of autism, usually diagnosed between ages 2 - 6

• a child has good language skills and average to above-average intelligence but it is socially awkward and engages in odd, repetitive behaviors. “geek syndrome” or “little professor syndrome”

Childhood disintegrative disorder

• a very rare disorder in which normally developing children lose motor, language, social, and potty skills around ages 2-4

Rett syndrome

• an extremely rare disorder, similar to childhood disintegrative disorder, which occurs only in girls

• can profoundly impair girl’s ability to use their hands usefully

Attention-Deficit Hyperactivity Disorder

• a persistent pattern of inattention and/or hyperactivity-impulsiveness revealed before the age of 7 years

• difficult for sufferers to focus, stay organized, and listen to direction diagnosable by 36 months of age “active”, “always on the go”

• cannot sit still in school & cannot concentrate on problem solving for longer periods

Cerebral Palsy

• impaired movement & posture due to an abnormality in the extrapyramidal or pyramidal motor system

• disorder that impair control of movement due to damage to the developing brain

• causes:

  • due to rubella, cytomegalovirus, and toxoplasmosis

  • severe jaundice & Rh incompatibility

  • trauma (physically & metabolic) & severe oxygen deprivation

• symptoms:

  • extreme irritability and crying

  • feeding difficulties & stiff, rigid arms or legs

  • abnormal motor performance

• management:

  • participation in school programs & positioning

  • medications to decrease spasticity

  • surgery (Insertion of Baclofen Pump, Selective Percutaneous Myofascial Lengthening, osteotomy, Rhizotomy, Gastrotomy)

• Prevention

  • Magnesium Sulphate

  • healthy diet and prenatal check-up

Spastic cerebral palsy

• involves 1 or both sides; hypertonicity w poor control of posture

• tense, contracted muscles (most common). increased muscle tone, increase deep tendon reflexes & bone, scoliosis is common. typical gait is crouched.

• management:

  • active attempts at motion increase abnormal postures and overflow fine & motor skills of movement to other parts of the body

  • antispasmodic medications

Athetoid/ Dyskinetic cerebral palsy

• abnormal involuntary movement, athetosis, weakness of larynx, pharyngeal, & oral muscles (drooling and dysarthria).

• dystonic movement, constant, uncontrolled motions of limbs, head, and eyes. purposely, involuntary, uncontrolled movements of the face and extremities

• increased movement with stress & voluntary movements of face and extremities

Ataxic cerebral palsy

• wide-based gait, rapid repetitive movement performed poorly, disintegration of movements of the upper limbs when the child reaches for objects

• poor sense of balance, often causing falls and stumbles. disturbed coordination. hyperlexia, hypotonic muscles, slurred speech, nystagmus

• some have hypotonia and tremors

Spina Bifida

• “Split Spine” a condition in which there us abnormal development of the back bone, spinal cord, surrounding nerves, and the fluid filled sac that surrounds the spinal cord.

• closure of the neural tube occurs around the 23rd (rostral closure) and 27th (caudal closure) day after fertilization (first 3-5 weeks) of the the embryo.

• causes:

  • genetics

  • environmental factor

• treatment

  • repair of meningocele or myelomeningocele

  • orthopedic consideration

• prevention

  • folic acid

  • genetic counselling and prenatal check up

Spina Bifida Occulta

• often called hidden Spina Bifida, mildest and most common form, the spinal cord and the nerves are usually normal and there is no opening on the back.

• it occurs most frequently in the lumbosacral area (L5 and S1). there may be no motor or sensory impairments evident at birth. subtle, progressive neurologic deterioration often becomes evident in later childhood or adulthood.

• Manifestation

  • skin depression or dimple

  • hairy patch and soft subcutaneous lipomas

  • bladder and bowel sphincter disturbances

Spina Bifida Cystica

a visible defect with an external sac like protusion

meningocele

• a moderate form of spina bifida, in which the outer part of some of the vertebrae are split and the meninges are damaged and pushed out through the opening, which contains both the meninges and CSF but no neural elements

Myelomeningocele

• occurs when the meninges (proactive covering of the spinal cord) and spinal nerves come through the open part of the spine

• most serious type of spina bifida, which causes nerve damage and more severe disabilities

• contains meninges, spinal fluid and nerves

• 80% - 85% of children can develop this disease