Exam 3: Peds

Rene syndrome: Has four stages

Acute encephalopathy caused by an interaction of salicylate, viruses and liver dysfunction. Stage 1: vomiting and lethargy Stage 2: disorientation, delirium, central neurologic hyperventilation, and stupor Stage 3: obtundation, coma, and decorticate rigidity Stage 4: rapidly developing seizures, flaccidity and respiratory arrest Avoiding giving aspirin to children with viral illnesses is the widely accepted preventative measure

Myelomeningocele

Also called spina bifida. Herniation of the meninges, spinal fluid, spinal cord and nerves through a vertebral defect. 80% are located in the lumbrosacral region. May leak CSF, thus increasing risk of infection. Deficits will be distal to the defect: Weakness, paralysis, spasticity, and either bowel or bladder dysfunction. Hydrocephalus occurs in 95% of cases.

Spina bifida - a anterior midline defect

Anencephaly - A baby born with an underdeveloped brain and an incomplete skull. Encephalocele - sac-like protrusions of the brain and the membranes that cover it through openings in the skull. Meningocele Myelomeningocele - A birth defect in which a developing baby's spinal cord fails to develop properly, may poke out at lower back.

Types of myelomeningocele

Arnold-Chiari type II malformation: A condition in which brain tissue extends into the spinal canal, present at birth. Tethered cord syndrome: tissue attachments that limit the movement of the spinal cord within the spinal column. These attachments cause an abnormal stretching of the spinal cord.

Congenital hydrocephalus

Buildup of excess cerebrospinal fluid (CSF) in the brain at birth. The extra fluid can increase pressure in the baby's brain , causing brain damage and mental and physical problems. This condition is rare.

Static encephalopathies

Cerebral palsy: Pyramidal/spastic cerebral palsy Extrapyramidal/nonspastic cerebral palsy Dystonic cerebral palsy Ataxic cerebral palsy

Encephalopathies: inherited metabolic disorders

Defects in amino acid metabolism: Phenylketonuria (PKU) - hyperpheylalanimia. Storage diseases: Lysosomal storage diseases and Tay-Sachs disease.

Nervous system development in children

Develops from embryonic ectoderm in stages: Formation of neural tube, development of forebrain, neuron proliferation and migration, formation of synapses, myelinazation. Rapid development of brain during third, fourth, fifth gestational months.

Epilepsy and seizure disorders

Diagnosed when child has more than one unprovoked seizure. Seizures: neurologic or systemic Febrile seizures: bengin

Phenylketonuria (PKU)

Encephalopathy caused by inherited metabolic disorder and is progressive in nature. Involves an inability to metabolize the amino acid phenylalanine and occurs once in every 10,000 births world wide. High level of phenylalanine causes insufficient amounts of other amino acids to enter the brain, which results in malformation, defective myelination, or cystic degeneration of the white and gray matter. Detected in new born screening.

Cyclopia - a anterior midline defect

Failure of the embryonic prosencephalon to properly divide the orbits of the eye into two cavities.

Fontanelles

Fissures in skull to allow for brain growth.

Dandy - Walker malformation

Fundamental abnormalities involve the cerebellum which controls muscle coordination.

Head growth

Hydrocephalus: A build-up of fluid in the cavities deep within the brain. Microcephaly: A condition in which a baby's head is significantly smaller than expected, often due to abnormal brain development.

Encephalopathies

Intoxications: led poisoning Infections: meningitis - aseptic, bacterial, and viral. Viral encephalitis.

Spina Bifida Occulta

Less serious form of spina bifida. Defect occurs in the lumbar or sacral area of the spine because of incomplete fusion of the vertebral laminae. More common than spina bifida. Usually causes no neurologic deficits.

Childhood brain tumors

Medulloblastoma Ependymoma Cerebellar astrocytoma Brainstem glioma Craniopharyngioma Optic nerve glioma

Which neural tube defect is a saclike cyst, most often in the lumbar area, containing meninges, spinal fluid, and a portion of the spinal cord with its nerves?

Myelomeningocele: (meningomyelocele; spina bifida cystica) is a hernial protrusion of a saclike cyst (containing meninges, spinal fluid, and a portion of the spinal cord with its nerves) through a defect in the posterior arch of a vertebra. Eighty percent of myelomeningoceles are located in the lumbar and lumbosacral regions, the last regions of the neural tube to close. Meningocele, which is a saclike cyst of meninges filled with spinal fluid, is a mild form of posterior neural tube closure defect. In anencephaly, the soft, bony component of the skull and part of the brain are missing. Encephalocele refers to a herniation or protrusion of brain and meninges through a defect in the skull, resulting in a saclike structure.

Reflex responses

Neonatal reflexes disappear in predictable order.

Embryonal tumors

Neuroblastoma Retinoblastoma: inherited or acquired. Picture - huge pupils like a cat eye.

What type of brain tumor is an embryonic tumor of the sympathetic nervous system?

Neuroblastoma: s an embryonal tumor originating outside the CNS in the developing sympathetic nervous system (sympathetic ganglia and the adrenal medulla). Ependymoma develops in the fourth ventricle and arises from the ependymal cells that line the ventricular system. Retinoblastoma is a rare congenital eye tumor of young children that originates in the retina of one or both eyes. Medulloblastoma occurs as an invasive malignant tumor that develops in the vermis of the cerebellum and may extend into the fourth ventricle.

Cortical dysplasia

Neurons in an area of the brain failed to migrate in the proper formation in utero.

Cerebrovascular disease

Perinatal stroke Childhood stroke: Ischemic, hemorrhagic, moyamoya disease.

Craniosynostosis

Premature closure of one or more cranial sutures: plagiocephaly - flat spot on a baby's head.

Meningocele

Protrusion of meninges through a vertebral defect. Spinal cord not involved. Present at birth as a protruding sac at the level of the level of the defect.

Cerebral Palsy

Static encephalopathy - Cerebral damage does not change over time. Clinical manifestations may change as the child continues to develop CP. Classified according to the neurologic symptoms produced: spasticity, ataxia, dyskinesia or a combination of all three. Many factors contribute to cp - birth asphyxia, low birth weight, vascular abnormalities, pre- or postnatal trauma, and a host of other insults not well understood.

Microcephaly

True (primary) microcephaly Secondary (acquired) microcephaly Zeka Virus