Hypotonia

Definition

Hypotonia involves decreased muscle tone. Infants with hypotonia seem floppy and feel like a "rag doll" does when held.

Alternative Names

Considerations

Hypotonia is often a sign of a worrisome abnormality and may suggest the presence of central nervous system dysfunction, genetic disorders, or muscle disorders.

Hypotonic infants rest with their elbows and knees loosely extended, while infants with normal tone tend to have flexed elbows and knees. Head control may be poor or absent in the floppy infant with the head falling to the side, backward, or forward.

Infants with normal tone can be lifted with the parent's hands placed under the armpits, while hypotonic infants tend to slip between the hands as the infant's arms rise unresistingly upward.

Common Causes

• Down syndrome
• Myasthenia gravis
• Prader-Willi syndrome
• Kernicterus (brain damage due to severe jaundice; causes can include Rh incompatibility)
• Cerebellar ataxia, congenital
• Myotonic dystrophy (a type of muscular dystrophy)
• Infant botulism
• Familial dysautonomia (Riley-Day syndrome)
• Marfan's syndrome
• Muscular dystrophy
• Achondroplasia
• Trisomy 13
• Sepsis
• Aicardi syndrome
• Canavan disease
• Congenital hypothyroidism
• Hypervitaminosis
• Krabbe disease
• Menkes syndrome
• Metachromatic leukodystrophy
• Methylmalonic acidemia
• Rickets
• Spinal muscular atrophy type 1 (Werdnig- Hoffman)
• Tay-Sachs disease
• Vaccine reaction
• Klinefelter syndrome
• Phelen-McDermid syndrome (Deletion 22q13 syndrome)

Home Care

Extra care must be taken when lifting and carrying a hypotonic infant to avoid causing an injury to the child.

Call your health care provider if

Call if your child appears "floppy", especially if he or she previously seemed to have normal muscle control.

What to expect at your health care provider's office

The family history and the child's medical history will be obtained. A physical examination will be performed. Most of the disorders associated with hypotonia also cause other symptoms that, when taken together, will suggest a particular disorder.

Medical history questions may include:

• When did you first notice that your baby seemed floppy?
• Was it apparent at birth?
• Did it develop suddenly or gradually?
• Have you ever noticed a pattern to the behavior (is it always the same or is it worse at certain times)?
• How exactly does the child act?
• Is the child generally limp, or only floppy in certain areas?
• What other symptoms are present?

The physical examination will probably include a detailed nervous system and muscle function examination.

Diagnostic tests will vary depending on the suspected cause of the hypotonia.

References

Yeh PC, Kipp MA. A case of Moebius syndrome in association with Klinefelter syndrome. Ophthalmic Genet. 2002 Sep;23(3):185-9.