Cerebral Palsy

Question 1

Most common cause of CP in premature infants

Answer 1

Periventricular leukomalacia.

Question 2

Other risk factors for CP

Answer 2

Hypoxia before or during labor, prolonged bradycardia, 10- or 15-minute Apgar score of less than 4, in utero intracerebral hemorrhage, perinatal ischemia, and postpartum multisystem organ failure.

Question 3

Spastic CP

Answer 3

Spasticity impairs mobility more than paresis does. Walk with extended, unbending legs. Signs of upper motor neuron injury – hyperactive deep tendon reflexes, clonus , and Babinski signs – accompany the spasticity. Walk on toes. Affected limbs have growth arrest.

Question 4

Diplegic CP (Spastic Diplegia) (subtype)

Answer 4

Type of Spastic CP. Both legs. Legs drawn together, scissoring, toes pointed downward. Prematurity leading to periventricular leukomalacia is most common cause. Because cortex spared, epilepsy and retardation are rare.

Question 5

Hemiplegic CP (subtype)

Answer 5

Type Spastic CP. Spastic hemiparesis that typically affects the face and arm more than the leg. Resembles adults with strokes from middle cerebral artery occlusions (except no aphasia and limbs show growth arrest). Early “handedness” is a sign of this.

Question 6

Quadriplegic CP (subtype)

Answer 6

Type of Spastic CP. Paresis of all four limbs, usually accompanied by pseudobulbar palsy. Often extensive cerebral damage, often from anoxia during delivery. Epilepsy and mental retardation common.

Question 7

Extrapyramidal CP

Answer 7

Also called Choreoathetotic CP. Involuntary writhing movements (athetosis) of the face, tongue, hands, and feet punctuated by jerking movements (chorea) of the trunk, arms, and legs – embraced by the term choreoathetosis.
Can be subtle. Often not noticed until child is 2-4 years old.
Kernicterus spares the cortex so low rate of

Question 8

Condition similar in presentation to Extrapyramidal CP

Answer 8

Dopamine-responsive Dystonia. Which is a progressive condition. Some docs will always insist on trial of L-DOPA prior to calling Extrapyramidal CP.

Question 9

Causes of Choreoathetotic CP

Answer 9

Neurologists usually attribute choreoathetotic CP to combinations of low birth weight, anoxia, and neonatal hyperbilirubinemia damaging the basal ganglia (kernicterus).

Question 10

Commonly associated with Choreoathetotic CP

Answer 10

Because these insults also damage the auditory pathways, hearing impairment frequently complicates the clinical picture.

Question 11

Mixed form CP (subtype)

Answer 11

Combinations of spastic paraparesis and choreoathetosis – account for about 15% of cases. They reflect the most extensive central nervous system (CNS) injury, which is naturally associated with the highest incidence of epilepsy and mental retardation – 95%.

Question 12

Arnold-Chiari Malformations

Answer 12

Types of upper neural tube defect. Arnold–Chiari malformation consists of downward displacement of the lower portion of the medulla and cerebellum through the foramen magnum. Not apparent from outside.

Question 13

Arnold-Chiari symptoms

Answer 13

In older children and adults, who may previously have escaped detection, this malformation may produce headaches (especially when bending), bulbar palsy, and neck pain. In many patients, these congenital abnormalities lead to mental retardation and, because of aqueductal stenosis, obstructive hydrocephalus.

Question 14

Meningocele

Answer 14

Lower neural tube defect. Meninges and skin protrude through a lumbosacral spine defect to form a large, CSF-filled bulge.

Question 15

Spina Bifida Occulta

Answer 15

Lower vertebrae fail to fuse. Most mild and asymptomatic.

Question 16

Meningocele Symptoms

Answer 16

Although this condition may remain asymptomatic, it frequently causes symptoms originating in dysfunction of the lumbar and sacral nerves, such as leg weakness, gait impairment, and bladder-emptying problems. Thus progressive hydronephrosis often complicates the deficit.

Question 17

Meningomyelocele

Answer 17

Most serious lower neural tube defect. A tangle of a rudimentary lower spinal cord, lumbar and sacral nerve roots, and meninges protruding into a saclike structure overlying the lumbosacral spine.

Question 18

Meningomyelocele Symptoms

Answer 18

Disrupted nerve tissue causes paraparesis, areflexia, and incontinence. In addition, hydrocephalus and other brain abnormalities are comorbid in about 25% of cases. “The infant’s legs, lacking innervation , remain weak, flaccid, and areflexic. Similarly, the bladder, also lacking innervation, distends.”

Question 19

Iatrogenic Causes of Neural Tube Defects

Answer 19

Studies have attributed neural tube defects to carbamazepine and valproate, folic acid deficiency, autoantibodies to folate receptors, radiation, and various toxins , including potato blight. Although there is no complete answer, the tendency of AEDs to reduce serum folate level and thus raise homocysteine levels may explain their relationship to neural tube defects.

Question 20

Prenatal Detection of Neural Tube Defects

Answer 20

Excessive concentrations of α-fetoprotein in amniotic fluid and maternal serum indicate a neural tube defect. Fetal ultrasound examination, a complementary test, may show neural tube defects as well as other congenital malformations.

Question 21

Tuberous Sclerosis Appearance

Answer 21

Conspicuous smooth and firm nodules, facial angiofibromas (adenoma sebaceum), on the malar surface of the face, but this illness-defining skin lesion usually fails to appear until adolescence. However, during infancy and childhood, the skin shows several other characteristics: subtle hypopigmented macules (ash-leaf spots); shagreen patches, which are leathery, scaly areas, on the lower trunk and buttocks; and periungual fibromas of the fingers.

Question 22

Triad of Tuberous Sclerosis

Answer 22

Epilepsy, mental retardation, and angiofibromas.

Question 23

Neurologic Syndromes that have Autistic Symptoms

Answer 23

Angelman, Fragile X, Klinefelter, Rett, and Tuberous Sclerosis.

Question 24

Triad of Neurofibromatosis 1

Answer 24

Multiple café-au-lair spots (brown, flat oval).
Neurofibromas (nodules at peripheral nerves, on face, can be large).
Lisch nodules (multiple, asymptomatic, macroscopic, yellow to brown nodules situated on the iris).

Question 25

Red flags for Neurofibromatosis 1

Answer 25

More than 6 café-au-lait spots and freckling in the groin and axilla (protected from sun).

Question 26

CNS manifestations of NF1

Answer 26

As with other disorders in this group, NF1 induces intracerebral tumors , particularly optic nerve gliomas, as well as intraspinal neurofibromas.

Question 27

Psychiatric sequellae of NF1

Answer 27

NF1 has a close association with attention deficit hyperactivity disorder (ADHD) and learning disabilities. Some tests detect sustained attention difficulties and learning disabilities in the majority of NF1 children. The IQ of NF1.

Question 28

Neurofibromatosis 2

Answer 28

Almost completely separate from NF1. Bilateral acoustic neuromas (vestibular schwannomas) that steadily impair hearing until deafness ensues. It may induce a few neurofibromas and large, pale café-au-lait spots, but its hallmark remains the acoustic neuromas (see Fig. 20-27 ). In fact, NF2 is usually unrecognized until acoustic neuromas are discovered.

Question 29

Comorbidities of NF2

Answer 29

Associated with two neoplastic complications, acoustic neuroma and meningiomas. Does NOT cause behavioral, learning, or cognitive impairments.