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PubMed1985

Neurological signs in congenital iodine-deficiency disorder (endemic cretinism).

Neurological examinations were made of 67 children and adults with congenital iodine-deficiency disorder (endemic cretinism) in four rural villages in highland Ecuador. There was a distinct and readily identifiable pattern of neurological deficits. These included, to varying degrees: deaf-mutism or lesser degrees of bilateral hearing-loss or dysarthria; spasticity, particularly involving the proximal lower extremities; mental deficiency of a characteristic type; and rigidity and bradykinesia. Not all of these elements were found in all cases. Less common features were strabismus, kyphoscoliosis and frontal-lobe signs. There were exceptional cases with hypotonia. In contrast, cerebellar function was largely spared, as were functions of emotion and attention, vegetative and autonomic functions, social interaction, and probably memory, except in the most severely involved.

Adolescent↗

PubMed1993

Iodine supplementation and the prevention of cretinism.

Normal development of the CNS requires adequate thyroid hormone exposure. Since iodine is an essential component of the thyroid hormone molecule, its deficiency during fetal development can cause hypothyroidism and irreversible mental retardation. The full-blown syndrome, called cretinism, includes deaf-mutism, short stature, spasticity, and profound mental retardation. The clinical spectrum can vary in degree and combination of these features. Screening programs in iodine-deficient countries show that up to 10% of neonates have elevated serum TSH levels, putting them at theoretical risk for permanent brain damage. About one billion people worldwide risk the consequences of iodine deficiency, all of which can be prevented by adequate maternal and infant iodine nutrition. Iodized salt is usually the preferred prophylactic vehicle, but iodized vegetable oil, iodized water, and iodine tablets are also occasionally used. The United Nations and the heads of state of most countries have pledged the virtual elimination of iodine deficiency by the year 2000. This goal is technically feasible if pursued with sufficient vigor and resources.

Central Nervous System↗

PubMed1987

Cretinism and lingual thyroid presenting in an adult.

A 29 year old woman presented with menorrhagia secondary to hypothyroidism. Subsequent investigation confirmed the presence of a lingual thyroid. Features of cretinism were present despite the late presentation. This unusual case is discussed with reference to previous publications.

Adult↗

PubMed1980

Calcitonin secretion in congenital nongoitrous cretinism.

Plasma calcitonin (CT) was measured in the basal state and/or during provocative tests of hormone secretion in 11 children with congenital non-goitrous cretinism (CNC), in 1 girl with a lingual thyroid, and in 11 normal children. Basal and stimulated CT concentrations were significantly lower in the patients with CNC than in the normal subjects. Mean basal CT (+/- SE) was 41 +/- 4 pg/ml in the normal children, 24 +/- 3 pg/ml in the children with CNC, and 20 +/- 2 pg/ml in the patient with the lingual thyroid. The mean incremental CT responses to calcium infusion were 7.0 +/- 2 pg/ml in the children with CNC, 6.0 pg/ml in the patient with the lingual thyroid, and 146 +/- 47 pg/ml in the normal children. The children with CNC also demonstrated a significant delay in the return of the total serum calcium to basal level after the calcium infusion. The mean incremental CT response after infusion of pentagastrin was 7.6 +/- 2 pg/ml in the children with CNC, 10.0 pg/ml in the child with the lingual thyroid, and 34.4 +/- 11 pg/ml in the normal children. These data indicate that CT deficiency is present in children with CNC and suggest that the deficiency is a consequence of the defective embryologic development of the thyroid gland.

Adolescent↗

PubMed2003

Thiocyanate induces cell necrosis and fibrosis in selenium- and iodine-deficient rat thyroids: a potential experimental model for myxedematous endemic cretinism in central Africa.

Thyroid destruction leading to endemic myxoedematous cretinism is highly prevalent in central Africa, where iodine (I) and selenium (SE) deficiencies as well as thiocyanate (SCN) overload are combined. All three factors have been studied experimentally in the etiology of the disease, but they have never been studied in combination. In a model using rats, we have previously shown that combining I and SE deficiencies increases the sensitivity of the thyroid to necrosis after iodide overload, an event unlikely to occur in the African situation. To develop a model that would more closely fit with the epidemiological findings, we have determined whether an SCN overload would also result in thyroid necrosis as does the I overload. The combination of the three factors increased by 3.5 times the amount of necrotic cells, from 5.5 +/- 0.3% in the I-SE+ thyroids to 18.9 +/- 1.6% in the I-SE-SCN-overloaded ones. Methimazole administration prevented the SCN-induced necrosis. SE- thyroids evolved to fibrosis, whereas SE+ thyroids did not. TGFbeta was prominent in macrophages present in SE- glands. Thyroid destruction in central Africa might therefore originate from the interaction of three factors: I and SE deficiencies by increasing H(2)O(2) accumulation, SE deficiency by decreasing cell defense and promoting fibrosis, and SCN overload by triggering follicular cell necrosis.

Africa, Central↗

PubMed1975

Ultrastructural aspects of the thyroid in a case of human congenital goitre with cretinism.

Transmission electron microscope and scanning electron microscope studies in a case of congenital goitre associated with cretinism revealed the existence of two cell types: the first with rounded endoplasmic cisternae and normal lysosomes; the second characterized by an abundance of lysosomes and/or lipofuscin granules. Intermediary aspects suggested that the second cell type was the progeny of the first type. As colloid droplets and apical pseudopods engulfing colloid were nearly absent, and as NBE127I was elevated in the plasma, it is suggested that iodoprotein secretion occurred by a process different from colloid phagocytosis as observed after acute TSH stimulation.

Congenital Hypothyroidism↗

PubMed1977

Breast carcinoma, thyroid carcinoma, and T3 thyrotoxicosis in a male cretin.

A male patient with clinical and radiographic evidence of cretinism was found to have T3 thyrotoxicosis. The cause was thought to be a well-differentiated follicular carcinoma of the thyroid. Although the disease was not documented, the patient probably had long-standing hypothyroidism. Untreated primary thyroid failure accompanied by high serum thyroid stimulating hormone (TSH) may be carcinogenic to the thyroid. The association of thyroid abnormalities and male breast carcinoma is discussed.

Adenocarcinoma↗

PubMed1990

[Excess of thiocyanate and selenium deficiency: cofactors in the etiology of endemic goiter and cretinism in North Zaire].

Endemic goitre is accompanied by a spectrum of iodine deficiency disorders (IDD). From work undertaken by CEMUBAC in Ubangui Zaïre, the role of thiocyanate overload is recalled while this work demonstrates for the first time in man an action of selenium supplementation (and thus deficiency) on thyroid function in iodine deficient areas. The extreme severity of the selenium deficiency may intervene either on the central and/or peripheral deiodination of thyroxine, or on the synthesis of the thyroid hormones. Together with thiocyanate overload, selenium deficiency may be responsible of the high frequency of myxedematous cretins in Zaïre.

Adolescent↗

PubMedDate not supplied

[Audiological and neuropsychological development study in a sample of school children from a low-iodine area of the Central Apennines that is endemic for cretinism].

We have evaluated neuropsychic development in 70 normal schoolchildren in an area of severe endemic goitre and cretinism in the Central Apennines (Montefeltro). In each subject we have studied auxological, psychometric and audiometric parameters, together with tympanograms and stapedial reflexograms. Auxological data were in the normal range, as were the stapedial reflexograms and tympanograms. On the contrary, 54.8% of the sample performed below the 25th percentile in psychometric tests (Raven test PM-47), being also 22.8% below the 5th percentile. Audiometric data showed a neurological hearing impairment in 3.1% of the children tested, as compared with 0.28% in the area of Pisa. These data point out that an impairment of central nervous system function still persists in this area of severe endemic goitre.

Child↗

PubMed1983

[Ultrastructural changes in the cerebellum of experimental hypothyroidism (cretinism)].

We examined the cerebellum of the rats being the experimental hypothyroidism (cretinism) on the 20th day, 35th day and 60th day by the light and electron microscope. The remarkable findings were observed to the experimental group on the 20th day. The maturational states of this group correspond to that of the 16th day of the control group. On the other hand, the remarkable pathological findings were not observed on the 35th and 60th day of the experimental group. According to the findings of the experimental rats on the 20th day, the changes of the cells were the retention of the external granule cells and the maturational changes of the internal granule cells. We recognized the retardation of the maturational timing and a disagreement of the maturational rate of the internal granule cells. The lamellar bodies being the disturbance of the mitochondrial cristae were observed. About the white matter, the myelinated nerve fibers were a small quantity in number, and the deficiency of the myelin synthesis, the maturational disturbance of oligoglia were seen. In the several axons, the lamellar bodies and the honeycomb like structures were seen. It is concluded that the maturational disturbance of the internal granule cells are due to the disturbance of the secondary protein synthesis by the hypothyroid state and the degeneration of the mitocondria. The deficiency of the myelin synthesis is related to the maturational disturbance of oligoglia.

Animals↗

PubMed1954