Microcephaly

Microcephaly is an extremely difficult incurable pathology, in which there is underdevelopment of the skull, insufficient functioning of the brain, dementia and other psychoneurological abnormalities. In many cases, microcephaly is the cause of the development of oligophrenia. Often pathology is combined with other developmental defects of the central nervous system, spastic paralysis, seizures.

Epidemiology

The incidence rate is approximately two cases per ten thousand newborns.

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Causes of the microcephaly

The disease refers to the congenital malformations of the nervous system.

The reasons for the development of microcephaly include the radioactive effect on the body, intrauterine infectious pathologies, genetic predisposition, toxic factors, chromosomal abnormalities. The cause of pathology can be the use of the mother's future alcohol or drugs during the period of bearing the child.

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Symptoms of the microcephaly

• complete or partial absence of corpus callosum (nerve fibers connecting the right and left hemispheres).
• abnormally wide convolutions of the brain.
• very narrow or thin gyrus of the whole brain or its individual segments.
• atypical tissue localization.
• lissencephaly - smoothing of the convolutions of the cerebral cortex of the cerebral hemispheres.

External signs of microcephaly include a disproportionate ratio of body and skull sizes, premature overgrowing of the fontanel.

Microcephaly in newborns

Microcephaly in newborns manifests itself in the form of various neurological disorders and seizures. In addition to the backwardness in mental development, there are violations of motor functions, which are manifested in the future. In newborns with microcephaly, the skull volume at birth is reduced and subsequently it does not increase, although the development of the facial part may correspond to the norms. Because of this, the size of the head is not proportional to the size of the body, the forehead has a sloping shape, the skin of the head is wrinkled. Over time, the discrepancy between the size of the head and body becomes more pronounced, the growth and weight indicators are also significantly lower than normal.

Forms

• Congenital microcephaly consists in abnormally small development of the skull and inadequate development of the brain against the background of normal body size.
• Primary microcephaly develops as a result of traumatic factors in the first or second trimester of pregnancy.
• The secondary occurs as a result of adverse effects on the fetus in the third trimester of pregnancy, during childbirth or in the first months after birth.

Secondary microcephaly occurs with a negative effect on the fetus of harmful factors in the third trimester of pregnancy, as well as during childbirth and in the first months of life. Such factors may include fetal infectious diseases, lack of oxygen, trauma, use by the mother of the future of alcohol or drugs. In patients, in addition to insufficient weight of the brain, foci of destruction (cysts, porencephalias), hemorrhages, inflammatory processes, areas of dystrophy or tissue necrosis are observed. The ventricles of the brain, as a rule, are enlarged. At the time of birth, the diameter of the head can be reduced not very significantly, but later its development slows down, the skull acquires asymmetrical forms, signs of cerebral palsy (cerebral palsy) are noted, and signs of mental underdevelopment are strongly pronounced. The prognosis of the outcome of the disease is unsatisfactory.

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Diagnostics of the microcephaly

Microcephaly is one of the most difficult pathological diagnoses in the prenatal period.

Reducing the head circumference of adults to forty-eight centimeters or less with a brain mass of less than nine hundred thousand grams suggests a diagnosis of microcephaly. The mass of the brain in children with microcephaly ranges from two hundred and fifty to nine hundred grams. The circumference of the head is thirty-forty centimeters. However, cases of lower weight and brain volume were recorded.

Microcephaly on ultrasound is difficult to diagnose because of the lack of common criteria for this pathology. The diagnosis can be assumed if the fetal head circumference is reduced by 2 SD and 3 SD, the change in the ratio of the diameter of the head and the length of the hip bone is less than two and a half percentile. It should be noted that sometimes the evaluation of the results of measuring the circumference of the fetal head is problematic because of the lack of accurate information about the duration of pregnancy or due to a violation in the formation of the bone system.

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Treatment of the microcephaly

Specific treatment does not exist. In all cases, therapy is symptomatic and is aimed at maintaining the life of patients. Microcephaly does not respond to treatment and has unfavorable prognosis of development and outcome. As a rule, patients with this diagnosis stay in special institutions for patients with malformations of the brain.

Forecast

The outcome of the disease in all cases is unsatisfactory, since such a pathology is incurable. Supportive therapy gives insignificant results, patients are monitored in special institutions for people with mental disabilities.

Lifetime in microcephaly

Life expectancy in microcephaly, as a rule, is low because of insufficient development and functioning of the brain. Most often, people suffering from this pathology die as a result of infectious lesions.

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