What is Floppy Infant Syndrome?
Floppy Infant Syndrome which is also known by the name of Infantile Hypotonia is a pathological condition of the musculoskeletal system in which there is decreased tone of the muscles at birth.1 The muscle tone is defined as the amount of tension or resistance that is present with movement of a muscle. This decreased muscle tone can be caused by a variety of reasons including an underlying central nervous system disorder, a genetic condition, or a muscle disorder. A sign of a decreased muscle tone is that if the arm of a child is flexed and if the child has a normal muscle tone then the child will be able to easily get it straightened it again, but in a child with decreased muscle tone this maneuver takes a while.
Floppy Infant Syndrome or Infantile Hypotonia can cause the muscles of the child to become loose and stretchy. Children with Floppy Infant Syndrome will always have a floppy appearance and will rest their elbows, arms, and knees loosely. Their head control is also very poor with the head falling to one side frequently. Normal children can be lifted up by placing the hands between the armpits but children with Floppy Infant Syndrome are not able to be lifted with this maneuver as their hands are not able to be lifted appropriately due to decreased muscle tone.
Children with Floppy Infant Syndrome also have significant delays in reaching their milestones like sitting up, standing, and walking abilities. Floppy Infant Syndrome children also have a tendency to frequently dislocate their hip, jaw, and neck as their joints and ligaments are too soft. Some children may also have difficulty with eating and chewing due to Floppy Infant Syndrome.
What are the Causes of Floppy Infant Syndrome?
There is no clear cut cause for the development of Floppy Infant Syndrome. Researchers believe that it might be caused due to trauma, certain environmental factors, genetic predisposition, or certain central nervous system disorders. There are also certain medical conditions that may cause Floppy Infant Syndrome. These medical conditions are:
Down Syndrome: This is a genetic condition caused due to a chromosomal abnormality.2
Myasthenia Gravis: This is a neuromuscular condition in which there is significant weakness of voluntary muscles of the body usually caused due to an abnormal immune system.
Prader-Willi Syndrome: This is a congenital disorder in which the child has obesity, decreased muscle tone, and decreased mental capability.3
Cerebellar Ataxia: This is a movement disorder caused often by a viral infection also tends to cause Floppy Infant Syndrome.
Muscular Dystrophy: This is a collection of disorders in which there is progressive muscle weakness and loss of muscle tissue.
What are the Symptoms of Floppy Infant Syndrome?
Some of the symptoms of Floppy Infant Syndrome are:
- Significantly decreased muscle tone is a symptom of Floppy Infant Syndrome
- The muscles feel soft
- Ability to extend a limb beyond normal
- Delays in meeting developmental milestones like sitting, standing, holding the head straight, and walking is also a symptom of Floppy Infant Syndrome
- Inability to chew and swallow foods normally
- Shallow breathing
- Mouth remains open with the tongue protruding outwards.
How is Floppy Infant Syndrome Diagnosed?
Floppy Infant Syndrome can be normally observed within the first few months of life when the child is unable to meet milestones on time. To definitively confirm the diagnosis of Floppy Infant Syndrome the doctor will take a detailed family history of the child to make sure that there is no family history of this condition. The next step towards the diagnosis of Floppy Infant Syndrome is to conduct a detailed physical examination including a neurological examination where the doctor will check the muscle, tone, strength, reflexes, and sensation of the child in various areas, especially the upper and lower extremities. Specific diagnostic tests used will vary depending on the suspected cause of the hypotonia. Since the symptoms shown by the child may resemble other muscular disorders hence the doctor may ask a few questions to the parents like:
- When did they first notice that the child had decreased muscle tone?
- Was the decreased tone observed immediately after birth?
- Were the symptoms of sudden onset or they start gradually?
- Are there any other symptoms observed along with hypotonia?
Apart from this, certain tests will also be ordered by the doctor to confirmatively diagnose Floppy Infant Syndrome. These tests are:
Blood tests to check for any infection
Creatine Kinase Test: If there is an elevated level of creatine kinase then it suggest that the muscles are degenerating or are damaged
CT/MRI Scan: This will be done to look at internal structures of the muscles and bones to see if they are degenerated or damaged in any form.
EMG: This will be done to check whether the muscles and nerves are functioning normally or not.
Muscle Biopsy: A muscle biopsy may be done to rule out other causes of hypotonia.
The results of the above tests will definitively confirm the diagnosis of Floppy Infant Syndrome.
How is Floppy Infant Syndrome Treated?
If the Floppy Infant Syndrome is caused by some underlying condition then treatment of the underlying condition is of paramount importance to get the muscle tone back. This is then followed by symptomatic and supportive therapy for Floppy Infant Syndrome. Physical therapy is somewhat effective to regain some fine motor control and improve overall body strength. Occupational and speech therapy may help with other anomalies like feeding, speaking, and swallowing difficulties.
For cases of mild forms of Floppy Infant Syndrome there is no specific treatment required but such children may need treatment for associated conditions like frequent dislocations of the jaw or neck. Once a cause of Floppy Infant Syndrome is confirmatively established then a treatment program consisting of sensory stimulation programs are made which involve physical therapy and certain other forms of therapy to make the child as independent as possible and improve the strength and muscle tone of the child which gets affected due to Floppy Infant Syndrome.
What is the Prognosis of Floppy Infant Syndrome?
- Since there is no cure for Floppy Infant Syndrome as of yet hence this condition is present life long.
- The nature and extent of improvement after treatment for Floppy Infant Syndrome depends on the underlying cause of the condition
- In some cases, it is seen that the muscle tone and strength improves with time or the child is able to learn certain mechanism to overcome the defects caused by Floppy Infant Syndrome.
- The prognosis is extremely guarded for those children who have cerebellar dysfunction or motor neuron disease as a cause of Floppy Infant Syndrome.