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More than 6.5 million U.S. children have diagnosed disabilities and receive special education services.1 In addition, 17% of the U.S. pediatric population is at risk for developmental delay.2

The first 2 years of a child's life represent a critical time for early intervention. Intelligence is believed to be 50% developed by age 5. The Centers for Disease Control and Prevention estimate that more than 300,000 U.S. residents younger than 21 have such poor cognitive development they are considered retarded.3 In many cases, this progression could be prevented by early intervention.3

Risk factors for developmental delay include poverty, maternal substance abuse, lack of prenatal care, low birth weight, prematurity, lack of parenting skills, abuse and neglect and medical conditions such as hypotonia. Obviously this is not a complete list. With the support of nurse practitioners and early intervention services, these risks can be decreased.

Early intervention services provide an excellent return on taxpayer funds, with a potential return of $7 for every $1 invested.4 Societal benefits of having fewer children with developmental delays include higher graduation rates, decreased grade repetition and more self-sufficient adults.4 Early intervention services provide early identification, screening and assessment. They include family training; counseling; home visits, including respite care; occupational, physical, speech and audiology services; case management for service coordination; and needed medical services. All infants and toddlers with diagnosed developmental disabilities are eligible for early intervention. States have the option to extend early intervention to children considered at risk - for example, very low birth weight infants and children from multirisk families.

What Is Hypotonia?
Hypotonia is a disability characterized by decreased muscle tone with varying degrees of floppiness. It is associated with multiple neuromuscular, metabolic and genetic disorders. Hypotonia affects many areas of a child's life, including cognitive development. The severity and progression of hypotonia varies with each child and his or her underlying diagnosis.

Developmental delay is common with many disabilities. An important distinction in hypotonia is progression. For example, Down syndrome causes low tone that is stable or nonprogressive, but muscular dystrophy causes tone problems that tend to worsen with time. Infants born prematurely may have hypotonia that improves with maturity of the central nervous system in the absence of cerebral palsy. Table 1 lists a few of the many causes of hypotonia.

Table 1
Causes of Hypotonia
This list is not all-inclusive.
   Chromosomal disorders or malformations
   Infectious causes such as toxoplasma, rubella, cytomegalovirus, herpes   simplex or HIV
   Perinatal asphyxia, Down syndrome
   Congenital hypothyroidism, kernicterus
   Hypotonic cerebral palsy
   Spinal cord disorders, hydrocephalus
   Neonatal myasthenia gravis, botulism
   Prader-Willi syndrome, congenital myopathies (after neonatal period)

When I began researching benign congenital hypotonia (BCH) in 1992, I found one paragraph. At that time it was called Oppenheim's disease. Now there are two classifications for it: congenital hypotonia (779.89) and benign congenital hypotonia (358.8). The difference between the two is that the cause of BCH is not known.5 Another unknown is the exact number of children affected. The estimated prevalence of all types of congenital hypotonia, including hypotonic cerebral palsy, is one in 1,000 births. When hypotonia in premature infants weighing less than 1,500 grams is included, the prevalence is 60 per 1,000 births.5

Benign congenital hypotonia is considered benign because it is not fatal and the condition does not worsen like many neuromuscular disorders. It does, however, have the potential to affect lifelong physical, cognitive, social and emotional development.

BCH is a diagnosis of exclusion and is frequently applied to children with nonprogressive low tone without a known cause. Unlike other neuromuscular disorders, BCH does not worsen and tends to improve with maturation of the central nervous system and early intervention. Children with this disorder do not receive the correct "signals" for all their muscles to work properly. Thus, muscle biopsy and neuromuscular testing usually produce normal results.

Etiology
The cause of BCH is unknown, but it is believed to be an autosomal recessive trait, meaning that both parents must contribute a gene for this disorder to occur. No genetic testing is available, and no cure has been developed. Many affected families report pregnancy or delivery complications, decreased fetal movement, prolonged hospital stays, breastfeeding difficulty, maternal age over 30, or a family history of developmental delays and hip dysplasia. No tests are available for prenatal diagnosis. Treatment is supportive for both the child and family.

Signs and Symptoms
The infant with BCH feels limp and fragile and has very flexible joints. He or she easily falls sideways and slides out of openings on high chairs and strollers. An affected baby may not develop head control until quite late and often sleeps in a "frog-leg" position with hips externally rotated. Children with hypotonia are prone to skeletal deformities as a result of maintaining abnormal postures. Table 2 summarizes signs and symptoms of BCH.

Table 2
Signs and Symptoms
   Unable to maintain head control or sitting position
   Feeding difficulties
   Orthopedic problems
   Developmental and cognitive delay
   A passive onlooker rather than participant
   Speech delay due to facial muscle weakness
   Perceived to have low cognitive function

The typical child with hypotonia is described as a "good, quiet baby" who needs to be awakened to feed, does not nurse well and is generally content. Parents carry the child with BCH like a young infant and lay him or her flat for dressing; the child becomes a passive onlooker. Parents may discourage the child from learning self-help skills such as dressing and feeding because the tasks are so time consuming for him or her. The infant with hypotonia is unable to reach out and mouth toys and becomes used to having someone entertain, feed and change him or her. The child may not receive necessary stimulation, which contributes to developmental delay.

The child's low tone often encompasses the face and mouth, making sucking, chewing and swallowing challenging. Food aversions and vomiting due to choking episodes are common. Food and saliva may be drooled out of the mouth, persisting as late as grade school and affecting socialization. Some children appear to be less intelligent owing to decreased smiling, verbal and nonverbal communication. Language development is often significantly delayed and the progression to solid foods is slow. Affected children have gaps in receptive and expressive language development. Global developmental delays are common as a result of the child's inability to explore the environment and bring toys to the mouth.

Congenitally dislocated hips and clubfeet are common in this population. After birth, children with BCH may develop pectus excavatum from adapted trunk control patterns and muscle weakness. Continued low tone in the pelvis causes the young child to lean forward dramatically to sit. Affected children often sit on their forelegs with their feet behind them (known as the "W sit"), leading to further hip and knee problems. A common motor pattern is the "combat crawl." In this pattern, the child uses his or her forearms to propel forward and drags his or her legs behind him (think of soldiers crawling under barbed wire fences).


Benign Congenital Hypotonia

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Hello, my son was born May 3rd 2014.at birth he showed signs of hypotonia. He was transferred the second day of life to a great hosptial nicu. There he improved dramatically showing basically no more signs of hypotonia. He eats well, has normal patterns of alertness, passes all test physically, and they did tons of blood work and cultures all coming back negative. As well as a ultrasound of the brain and even a MRI. The dr did see a very small amour of blood after the MRI but said it was no concern and he felt no need to follow on on it because it was so small, and it was probably caused by head trauma during delivery. That's what they felt caused him to be born with many signs of hypotonia, however he has improved dramtically (he seems like a normal infant to me. On his 3rd day of life he improved and from there on it just kept improving. They are being percausios and keeping him a close eye, (I have a follow up apt with a nurologist July 1st. But so far so good. No concerns. My question to you is, have you ever seen a case like this where he or she is born with low tone but it seems to just go away so soon, or no? What should I expect? -a worried loving mommy (Allison)

Allison GillMay 14, 2014



Thank you so much for your advice.

Patricia BlancoFebruary 19, 2013
Laveen, AZ



Patricia & Sarah, my son has hypotonia, he was diagnosed at 6 months and is now going to be turning seven this year, but the signs of the disability was noticeable at birth.

I have found that since he was born it took a lot for his pediatrician to even listen to me about my concerns in his physical delays, it took a temper tantrum in the dr. office to get a referral out to a physical therapist. The reason for this is because it is a rare disability, and even less diagnosed. I have found that you will have to see several doctors to find the right one for your child and you.

The one exercise I have found that has given the best overall physical conditioning for him is swimming several days a week.

I can only say that the best person for your child is you. It has been my experience that there really are no doctors experienced or informed about this type of disability unless it is associated with something else. We have traveled through out FL, AL, and GA seeking professionals and it has all come out to nothing.

All I can say is keep them active, have them try everything for themselves, and most of all swimming. It is the one thing that his physical therapist has suggested that has given him the best results that we are all pleased with.

I hope this helps you both.

Theresa
Cantonment, FL

Theresa February 13, 2013
Cantonment, FL



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