[Predominant factors of neonatal encephalopathy: hypoxic and ischemia, a global problem].

If a difficulty arises during birth, due to a maternal or fetal anomaly, acute or chronic, asphyxia of the fetal brain constitutes a greater risk, because it could result in the destruction of neurons and the possibility of evolving towards a Ischemic Hypoxic Encephalopathy with long -term sequelae. This review highlights the most recent scientific aspects but at the same time it offers an essential margin of knowledge regarding pathophysiology, diagnosis and treatment, as well as offering a perspective on the future of clinical care of ischemic hypoxic encephalopathy.

[Neonatal hypoxic-ischemic encephalopathy].

Hypoxic-ischemic encephalopathy is a clearly recognizable clinical syndrome of in term newborns due to fetal asphyxia at birth. The incidence is 1.5 (95% CI 1.

[Metabolic myopathies].

Aim: To review the metabolic myopathies manifested only by crisis of myalgias, cramps and rigidity of the muscles with decreased voluntary contractions and normal inter crisis neurologic examination in children and adolescents.

Development: These metabolic myopathies are autosomic recessive inherited enzymatic deficiencies of the carbohydrates and lipids metabolisms. The end result is a reduction of intra muscle adenosine triphosphate, mainly through mitochondrial oxidative phosphorylation, with decrease of available energy for muscle contraction.

[Juvenile myasthenia gravis].

Juvenile myasthenia gravis is a chronic autoimmune disorder which occurs when serum antibodies combine with nicotinic acetylcholine receptors at the muscle membrane of the motor endplate imparing the neuromuscular transmission. It results in early muscle fatigability with progression to a complete paralysis during repetitive muscle contraction (movements) or steady muscle contraction (postures), and less common persistent paralysis at rest. The cranial nerves, mainly the one innervating the extraoccular and palpebral levator, are the most susceptible to permanent weakness and paralysis at rest.

[Medical and surgical indications of evoked potentials].

Evoked potentials (EP) are more sensitive than specific, safe, objective and reproducible neurophysiologic techniques to assess integrity of peripheral and central sensory and motor pathways. The integrity of pathways are determined by measurement the latency of the recorded and digitally averaged electrical events elicited after adequate stimulation. EP are medically and surgically recommended in conditions in which the diagnoses, determination of neurological outcome in comatose and under anesthesia patients, and determination of the course and evaluation of treatment are uncertain with neurologic examination and other neurodiagnostic studies.

[Sleep disorders in the newborn].

Sleep disorders are classified in dyssomnias, parasomnias, sleep disorder associated with medical and psychiatric disorders and proposed sleep disorders. Only the parasomnias have been studied as such in the newborn period. The parasomnias that occur in this age group are infant sleep apnea, congenital central hypoventilation syndrome, sudden infant death syndrome, and benign neonatal sleep myoclonus.

Fetal deformations: a risk factor for obstetrical brachial plexus palsy?

The purpose of this report is to discuss the association of brachial plexus palsy and congenital deformations. We reviewed all charts of patients less than 1 year of age with obstetrical brachial plexus palsy evaluated by one of the authors (IA) between January 1998 and October 2005 at Miami Children's Hospital Brachial Plexus Center. Of 158 patients with obstetrical brachial plexus palsy, 7 had deformations (4.

Intrauterine shoulder weakness and obstetric brachial plexus palsy.

Obstetrical brachial plexus injury occurs when the forces preventing the stretch of the brachial plexus are overcome by the forces stretching it. This report describes an 8-day-old male delivered by uncomplicated cesarean section with right obstetrical brachial plexus palsy and congenital arm atrophy. The patient had a history of decreased right arm movement detected by fetal ultrasound at 18 to 20 weeks of gestation.

Bilateral decreased oxygenation during focal status epilepticus in a neonate with hemimegalencephaly.

Early surgical removal of a dysplastic hemisphere appears to be beneficial for neonates with hemimegalencephaly and medically resistant seizures. We analyzed the changes in the cerebral regional oxygen saturation index in a neonate with tuberous sclerosis and right hemimegalencephaly (1) during seven episodes of right hemisphere electroencephalographic status epilepticus with and without clinical manifestations and (2) after right hemispherectomy. The cerebral regional oxygen saturation index demonstrated marked fluctuations and progressive decline in both hemispheres during the episodes and normal values in the remaining hemisphere after surgery.

Opsoclonus: an early sign of neonatal herpes encephalitis.

Preferential involvement of certain central nervous systems areas by specific viruses provides a valuable guide to the selection of antiviral agents. We report a neonate that developed opsoclonus 7 days prior to the diagnosis of herpes simplex type 2 cerebellitis. The course of the opsoclonus paralleled the clinical course and radiologic evolution of the infection.

Cervicothoracic extradural arachnoid cyst: possible association with obstetric brachial plexus palsy.

The association of cervicothoracic extradural arachnoid cysts and obstetric brachial plexus palsy has not previously been reported. We report two patients with this association. The first patient is a 9-month-old boy with left obstetric brachial plexus palsy that developed bilateral leg weakness at 6 months of age owing to compression of the spinal cord by a C6 to T8 left cervicothoracic extradural arachnoid cyst.

Adolescents with muscular dystrophies.

The muscular dystrophies (MDs) are inherited or de novo gene mutation disorders of sarcolemma-associated proteins or nuclear membrane-associated proteins. They are manifested by progressive striated muscle weakness and wasting. Cardiac and central nervous system abnormalities may be present.

Methemoglobinemia in an infant receiving nitric oxide after the use of eutectic mixture of local anesthetic.

High levels of methemoglobinemia can cause tissue hypoxia and cyanosis. We report the case of a 7-month-old girl with pulmonary dysplasia receiving inhaled nitric oxide who had cyanosis caused by methemoglobinemia after prolonged use of a eutectic mixture of local anesthetics cream.