Microcephaly

Microcephaly is a condition where the head (circumference) is smaller than normal because the brain has not developed properly or has stopped growing.

• Microcephaly is a rare condition. One baby in several thousand is born with microcephaly.
• The severity of microcephaly ranges from mild to severe.
• Microcephaly can be present at birth (congenital) or may develop postnatally (acquired).

Signs and symptoms of microcephaly may include a smaller than normal head circumference that usually remains smaller than normal as the child grows, dwarfism or short stature, delayed motor and speech functions, mental retardation, seizures, facial distortions, hyperactivity, balance and coordination problems, and other brain-related or neurological problems; convulsions may also occur. Motor ability varies, ranging from clumsiness in some to spastic quadriplegia in others. Some with the disorder may develop normal intelligence.

Causes of microcephaly

There are many potential causes of microcephaly, but often the cause remains unknown. The most common causes include:

• infections during pregnancy: toxoplasmosis (caused by a parasite found in undercooked meat), Campylobacter pylori, rubella, herpes, syphilis, cytomegalovirus, HIV and Zika;
• exposure to toxic chemicals: maternal exposure to heavy metals like arsenic and mercury, alcohol, radiation, and smoking;
• pre- and perinatal injuries to the developing brain (hypoxia-ischemia, trauma);
• genetic abnormalities such as Down syndrome; and
• severe malnutrition during fetal life.

Historical causes of microcephaly

After the dropping of atomic bombs "Little Boy" on Hiroshima and "Fat Man" on Nagasaki, several women close to ground zero who had been pregnant at the time gave birth to children with microcephaly. Microcephaly was present in 7 children from a group of 11 pregnant women at 11–17 weeks of gestation who survived the blast at less than 1.2 km (0.75 mi)from ground zero. Due to their proximity to the bomb, the pregnant women's in utero children received a biologically significant radiation dose that was relatively high due to the massive neutron output of the lower explosive-yielding Little Boy.

There were 286 children who were in utero during the atomic bombings, and researchers examined health outcomes of these children for a year. There was a higher incidence of microcephaly and mental retardation in children who were in utero during the atomic bombs. Microcephaly is a proven malformation, or congenital abnormality, found in the children of Hiroshima and Nagasaki.

There is no treatment to change the head size; programs are available to help these individuals reach their maximum potential and genetic counseling may help explain the risk for microcephaly in future pregnancies. Women who are interested in becoming pregnant are being advised by the CDC and other agencies to avoid traveling to areas where Zika virus is found to reduce the chance of becoming infected while pregnant.

There is no treatment to change the head size; programs are available to help these individuals reach their maximum potential and genetic counseling may help explain the risk for microcephaly in future pregnancies. Women who are interested in becoming pregnant are being advised by the CDC and other agencies to avoid traveling to areas where Zika virus is found to reduce the chance of becoming infected while pregnant.

What is the prognosis for microcephaly?

• Some children will only have mild disability. Others, especially if they are otherwise growing and developing normally, will have normal intelligence and continue to develop and meet regular age-appropriate milestones.
• For more severe microcephaly, babies will need care and treatment focused on managing their other health problems (mentioned above). Developmental services early in life will often help babies with microcephaly to improve and maximize their physical and intellectual abilities.
• Many, if not most, cases if Zika microcephaly will be very severe, possibly requiring lifelong intensive care.

History

• People with microcephaly were sometimes sold to freak shows in North America and Europe in the 19th and early 20th centuries, where they were known by the name "pinheads". Many of them were presented as different species (e.g., "monkey man") and described as being the missing link. Famous examples include Zip the Pinhead (although he may not have had microcephaly) and Schlitzie the Pinhead, who also starred in the 1932 film Freaks. Both men were cited as influences on the development of the long-running comic character Zippy the Pinhead, created by Bill Griffith.

How many babies are born with microcephaly?

Microcephaly is still a rare condition worldwide although there have been surges in the numbers of cases in specific countries. Globally, 1 in every 30,000 to 250,000 babies has microcephaly at birth. In the United States, this is about 2 per 10,000 to 12 per 10,000 of live births. In the UK, there are 1.02 cases of microcephaly per 10,000 births.

Notable cases:

• A certain 'dwarf' of Punt (ancient Somalia) was given by the Chief clans as partial tribute to the last ruler of Ancient Egypt’s Old Kingdom, Pepi II Neferkare (6th Dynasty (circa 2125-2080 B.C.E.); it could be inferred that this person was indeed, also microcephalic. In a letter preserved at the British Museum, the young king gives instructions by letter, ”Harkhuf! The men in your service {escorts; soldiers; sailors; guards, etc.} ought pay sincere care with the dwarf’s head while sleeping during the voyage to the palace“ (so that it doesn't fall off...). At the same time, it could be for other reasons unrelated to microcephaly, etc.[69]
• Triboulet, a jester of duke René of Anjou (not to be confused with the slightly later Triboulet at the French court).
• Jenny Lee Snow and Elvira Snow, whose stage names were Pip and Zip, respectively, were sisters with microcephaly who acted in the 1932 film Freaks.
• Schlitze "Schlitzie" Surtees, (1901-1971) possibly born Simon Metz, was a sideshow performer and actor. Wikipedia – Schlitzie born with microcephaly, small stature, myopia and severe intellectual disability (that of a 3y/o).
• Lester "Beetlejuice" Green, (1968) – comedian & a member of radio host Howard Stern's Wack Pack was born with microcephaly & dwarfism.

Resources & Additional Reading:

• MedicineNet – Microcephaly
• World Health Organization – Microcephaly
• Wikipedia – Microcephaly
• News Medical Life Sciences – Microcephaly Diagnosis: During Pregnancy and After Birth. By Deborah Fields, B.Sc.
• MedicineNet – Eye Defects Seen in Some Babies Born with Zika-Linked Microcephaly. Feb 9, 2016 (Health Day News)
• CDC – Birth Defects – Facts about Microcephaly
• [https://www.ninds.nih.gov/Disorders/All-Disorders/Microcephaly-Info...
• NIH – Microcephaly Information Page]