Nanocephalia

Description:

The significant contribution to the description of a disease was made by I. P. Merzhiyevsky (1871), V. A. Bets (1871) and Page S. Korsakov (1894). A nanocephalia - a heterogeneous disease which main display is the body degrowth. Allocate true, hereditarily caused and secondary, tserebropatichesky (exogenous) nanocephalia forms.

Nanocephalia reasons:

The true nanocephalia is inherited on autosomal recessively and polygenic type. Its frequency also makes from 1:25 000 to 1:40 000 population, and the frequency of heterozygous carriers - 1:100. At 1/3 heterozygous carriers of a nanocephalia at a normal amount of a brain mental retardation is shown.
It is exogenous the caused form of a nanocephalia arises at an embryopathy as a result of influence in the pre-natal period of such teratogens as viral infections (measles, a rubella, a cytomegaly), radiation, air hunger, poisoning with carbon oxide, etc.

Nanocephalia symptoms:

For a nanocephalia the following physical and mental signs are pathognomonic. At patients the volume of brain department of a skull makes up to 50 cm. The mass of a brain is reduced to 25% and more. Frontal and parietal departments of a brain are preferential reduced. The forehead is low, slanted back. Typically type of patients: a disproportion between the reduced brain part of a skull, the normal sizes a front part and growth. Decrease in intelligence preferential reaches degree of deep mental retardation (idiocy), is more rare - insignificant (moronity).
At deep mental retardation at some patients the articulate speech, but sense of the words pronounced by them and the turned speech they do not understand to some extent remains. It is more important for patients intonations and a mimicry by which words are followed. They are able to consider, but are not capable to concentrate on something. Ideas of something quickly change and do not keep in memory. The emotional sphere is developed better. Motility is rather kept. Patients easily climb trees, learn to ride a bike. Besides, these patients vividly, emotionally react to certain events, they expressed feelings of sympathy. Most often there is an increased mood. Are emotionally unstable, irascible.
Taking into account features of temperament and activity of patients divide on torpid and explosive. Torpid patients are sluggish, apathetic, slow-moving. They are interested in nothing, can long sit in one pose, hardly it is possible to draw their attention to short time. Kindly meet those who with them are tender.
Vivacity, mobility, fussiness are characteristic of patients of explosive type. Their movements various, coordinated, a mimicry live. Well reproduce the movements, gestures, a mimicry and intonations of others. At some satisfactory mechanical memory. They make an impression interested, but not inquisitive. The increased mood with euphoria shade prevails. Emotional manifestations are unstable, easily turn into affect of anger.
Signs of organic lesion of a nervous system at a true nanocephalia are not noticeable. At a secondary nanocephalia focal neurologic symptoms, epileptic seizures are often observed. Patients usually undersized, with a disproportionate constitution. Secondary sexual characteristics at men are poorly expressed, at women they appear with a delay.

Treatment of the Nanocephalia:

Specific treatment does not exist. In case of need carry out symptomatic therapy (anticonvulsant, sedative drugs).