Benign Congenital Hypotonia

Definition

Definition of Benign Congenital Hypotonia
Benign congenital hypotonia (BCH) has been an outdated term since 1956, when the first congenital muscle disease, central core disease, was described. Now specific diagnoses can be made in most patients. As clinicians and researchers have gained greater understanding of neuromuscular diseases, the phrase has been dropped and replaced by names of specific neuromuscular disorders.

Symptoms

Symptoms of Benign Congenital Hypotonia
People affected by this condition often display general weakness and reduced strength and endurance of the muscles. Depending on the underlying cause, different sets of muscles may be involved. Infants with hypotonia often display poor suckling and chewing, an inability to lift their head, rolling to move around instead of crawling, physical passivity, and a weak cry. Often, the child will feel like a sack of gelatin when you pick him up because he tends to slide out of your hands. Also, when the mouth muscles are affected, drooling and speech difficulties can occur in young children. Patients may also experience hypermobility of their joints, enabling them to bend their fingers far back. Children with hypotonia often find it comfortable to sit in the cross-legged position and other painful or contortionist looking postures. Other symptoms include poor reflexes, developmental delay, and constipation for those whose bowel muscles are involved. Skeletal abnormalities are also associated with many forms of BCH.

Causes

Causes of Benign Congenital Hypotonia
Symptoms and findings associated with the group of neuromuscular disorders formerly known as benign congenital hypotonia include low muscle tone (hypotonia) at birth or in the first few months and general "floppiness" of muscles.

Diagnosis

Diagnosis of Benign Congenital Hypotonia
Benign or mild hypotonia is often diagnosed through a series of exercises designed to assess the child's developmental progress, or his observation of physical interactions. Since a hypotonic child finds it difficult to decipher his spatial location, he may have some coping mechanisms that are quite recognizable, such as locking the knees while trying to walk. One common sign of affected infants is a tendency to observe the physical activity of those around them for a long time before attempting to imitate these actions, due to frustration over early failures.

Treatment

Treatment of Benign Congenital Hypotonia
Although no drug therapy is currently available for the treatment of benign congenital hypotonia (BCH), children benefit from treatment with physiotherapy to help both active and passive movements in order to optimise muscle strength and prevent the development of any shortening of the muscles.

Prognosis

Prognosis of Benign Congenital Hypotonia
Parents will benefit from reassurance that the disorder is usually self-limiting, is not associated with any intellectual deficit and disappears by puberty in the majority of cases.

Prevention

Prevention of Benign Congenital Hypotonia
Consult with your doctor.