IODINE DEFICIENCY DISORDERS

Endemic goiter

Endemic goiter is characterized by enlargement of the thyroid gland in a significantly

large fraction of a population group, and is generally considered to be due to insufficient

iodine in the daily diet. Endemic goiter exists in a population when >5% of 6-12 year-old

children have enlarged thyroid glands

Figure 2 shows a young girl with a soft diffuse goitre and an elderly woman with a

huge, longstanding multinodular goiter, both resulting from iodine deficiency1

Myxedematous Cretinism

The typical myxedematous cretin (Fig 5) has a less severe degree of mental retardation

than the neurological cretin, but has all the features of extremely severe hypothyroidism

present since early life, as in untreated sporadic congenital hypothyroidism (127-129):

severe growth retardation, incomplete maturation of the facial features including the

naso-orbital configuration, atrophy of the mandibles, puffy features, myxedematous,

thickened and dry skin, dry and decreased hair, eyelashes and eyebrows and much

delayed sexual maturation.

Figure 5. Myxedematous endemic cretinism in the Democratic Republic of Congo.

Four inhabitants aged 15-20 years : a normal male and three females with severe

longstanding hypothyroidism with dwarfism, retarded sexual development, puffy

features, dry skin and hair and severe mental retardation.2

Endemic cretinism

Epidemiology

When McCarrison described cretinism in north-western India during the first decade of

this century (120), he delineated a neurologic form, with predominantly neuromotor

defects, including strabismus, deaf-mutism, spastic diplegia, and other disorders of gait

and coordination. The patients usually had a goiter. The other form, which he called the

myxedematous form, showed evidence of severe hypothyroidism, short stature, and

markedly delayed bone and sexual maturation. The patients usually had a thyroid

normal in size and position, and were seldom deaf.

Neurological Cretinism

The three characteristic features of neurological endemic cretinism in its fully developed

form are extremely severe mental deficiency together with squint, deaf mutism and

motor spasticity with disorders of the arms and legs of a characteristic nature. (Figure

4). As would be expected with a deficiency disease, there is a wide range in the severity

of the clinical features in the population affected (120-122).

Figure 4 (a). Male from Ecuador about 40 years old, deaf-mute, unable to stand or

walk. Use of the hands was strikingly spared, despite proximal upper-extremity
spasticity. 5