Floppy Baby Syndrome

Floppy Baby Syndrome, also called Hypotonia, is caused by a severe muscle disease which reduces muscle strength, and is a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength.

Why should I be aware of this?

• Most babies born with the rare disorder are severely paralysed and the majority die before the age of one.
• Floppy baby syndrome is distinct from babies who are deemed "floppy" because the development of their gross motor skills such as sitting and crawling is delayed.

All about Floppy Baby Syndrome

Decreased muscle tone is the most prominent symptom of possible development of Floppy Baby Syndrome in a person. Children with floppy baby syndrome have delayed development such as learning to crawl, walk, lift hands, and respond to anything that needs movement of joints or limbs, problem in balancing their head etc. Speech difficulty is also one of the problems.

Symptoms

There are some other signs and symptoms of Floppy Baby Syndrome:

• Reduced muscle tone
• Muscle weakness
• Weak infant suck
• Feeble cry
• Lethargy
• Reduced spontaneous activity
• Loss of head control

Causes and conditions

Genetic disorders such as Krabbe disease, Werding-Hoffman disease, Aicardi syndrome, Down syndrome, Achondroplasia, canavan disease, Riley-Day syndrome (also known as Familial dysautonomia), Griscelli syndrome, Leigh disease, Marfan syndrome, Rett syndrome, Prader-Willi syndrome, Myotonic dystrophy, Menkes syndrome, centronuclear and myotubular myopathy, Dejerine sottas syndrome, Septo-optic dysplasia, Tay-Sachs disease, 22q13 deletion syndrome, Trisomy 13 and FG syndrome etc. are the main conditions that cause floppy baby syndrome.

Conditions which lead to the development of floppy baby syndrome are: muscular dystrophy, metachromatic leukodystrophy, infectious disease such as encephalitis, sepsis, meningitis, infant botulism, Guillain-Barre syndrome, poliomyelitis etc., myasthenia gravis, celiac disease, problems caused due to improper vaccination or abnormal reaction to vaccination, congenital cerebellar ataxia, congenital hypothyroidism, hypotonic cerebral palsy, teratogenesis (caused from utero exposure to Benzodiazepines), sensory integration dysfunction, dyspraxia, metabolic disorders such as hypervitaminosis (vitamin poisoning or vitamin overdose), rickets and kernicterus, problems with central nervous system dysfunction, any kind of brain injury that had direct or indirect effect on the nervous system (brain injury caused in an accident or injury caused in a child suffering from shaken baby syndrome), motor neuron lesions etc.

Diagnosis

Some symptoms such as inability to flex muscles for longer time, delayed response to stimulations, delayed physical development, speech problems, feeding problem for an infant due to inability to suck breasts properly etc can be diagnosed through physical examination. If the brain is affected by the disorder then it is considered to be cerebral palsy. If related to muscles then it is called as muscular dystrophy.

Family medical history, CT scan, MRI scan, EEG (Electroencephalogram), genetic testing (which involve testing for abnormalities in genes, chromosome karyotyping etc.), blood test, electromyography muscle test, muscle and/or nerve biopsy, spinal taps (also known as lumbar puncture, used to collect cerebrospinal fluid for analysis) etc. are other tests used to diagnose floppy baby syndrome.

Treatment

There is no known and officially approved treatment for complete cure of floppy baby syndrome. Conditions can be improved by treatments for symptoms and exercise. Children suffering from this disorder are given special trainings in order to help them strengthen their muscles and improve response mechanism. In some cases Acupuncture can help activate affected muscles or nerves. Physical and developmental therapies can help attain better motor control and overall strength in muscles etc.

What can I do?

• Before leaving the hospital with your newborn, find out from your pediatrician or family doctor what the normal infantile reflexes are. This will help you make informed observations should any disabilities or abnormalities appear.
• Sucking and rooting for the breast or bottle are normal reflexes that are essential to an infant's ability to thrive when feeding. Observe your baby’s capacity to do so. If the child shows problems it may be an early sign of floppy baby syndrome.
• If you observe any such problem, talk to your doctor or neonatal nurse while you are still in the hospital.
• Learn the timetables for normal infantile reflexes so that you won't be alarmed when they are naturally inhibited after a few months.
• Monitor your child's later acquisition of developmental milestones and see your doctor right away if he is significantly delayed in reaching milestones.

90 degrees

Scientists at the Western Australian Institute for Medical Research have used heart tissue to cure mice of a fatal muscle disease which causes a Floppy Baby Syndrome, a breakthrough that they claim could save thousands of lives. The mice with Floppy Baby Syndrome were only expected to live for about nine days, but the scientists managed to cure them so they were born with normal muscle function, allowing them to live naturally and very actively into old age.

References:

• Floppy baby syndrome breakthrough
• How to Know When to See a Doctor for Floppy Baby
• Hypotonia - Symptoms, Causes and Treatments