INFORMATION ON CEREBRAL GIGANTISM

Soto’s syndrome is a genetic condition causing unnecessary growth and a distinctive head and facial appearance. It has in the past been known as cerebral gigantism. It is often accompanied by delayed expansion, low muscle tone, and impaired speech.

CAUSES OF CEREBRAL GIGANTISM

There are many conditions and genetic syndromes that cause too much growth; consequently, a baby and/or child who has accelerated growth needs to be thoroughly examined by a physician knowledgeable in overgrowth and genetic syndromes. The evaluation includes asking about health problems in the family as well as asking about the growth patterns of the parents and their final height. In some families, growth patterns are different and thus may account for the child's excessive growth. The child will also undergo a complete physical examination.

Additional examination of facial appearance, with special attention paid to the shape of the head, width of the face at the level of the eyes, and the appearance of the chin and forehead is necessary as well. Measurement of the head circumference, arm length, leg length, and wing span should be taken. Laboratory testing such as chromosome analysis (karyotype) may be done along with testing for another genetic syndrome called fragile-X. A bone age will also be ordered. Bone age is determined by x rays of the hand. If the child begins to lose developmental milestones or appears to stop developing, metabolic testing may be done to assess for a metabolic condition.

SYMPTOMS OF CEREBRAL GIGANTISM

A range of clinical features are connected with Soto’s syndrome.
• Newborns are large with respect to length and head circumference; weight is generally average. The fast growth continues through infancy and into childhood with the child's length/height and head circumference often being above the 97th percentile. The rate of growth appears to decrease in later childhood and adolescence.
• Respiratory and feeding problems (due to hypotonia) may enlarge in the neonatal period.
• Infants have a round face with prominent forehead and small chin. As the child grows into adolescence and then adulthood the face becomes long and thin, and the chin becomes more prominent.< speech away, goes or resolves hypotonia the and older grows child as but by affected also is Speech delayed. Usually are milestones developmental skills, motor gross fine of development affects this birth. at present>
• Intellect usually is border line to normal.
• Behavioral problems and emotional immaturity are commonly reported.
Dental problems such as early eruption of teeth, excessive wear, discoloration, and gingivitis are common

TREATMENT OF CEREBRAL GIGANTISM

There is no cure or method for preventing Soto's syndrome. However, the symptoms can be treated and managed. In the majority of cases, the symptoms developed by individuals with Soto's syndrome are treated and managed the same as in individuals in the general population. For example, physical and occupational therapy may support with muscle tone, speech therapy may improve speech, and behavioral assessments may assist with behavioral troubles.