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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neurological endemic cretinism, resulting from severe iodine deficiency, is characterized by mental deficiency, deafmutism, and a spastic-rigid motor disorder. Its neuropathology and pathophysiology have been investigated very little. We report the clinical and magnetic resonance imaging brain scan findings of 3 adult Chinese cretins. All show an apparent magnetic resonance imaging abnormality in the globus pallidus and substantia nigra, with hyperintensity on T1-weighted images and hypointensity on T2-weighted images. The motor abnormality, characterized by truncal and proximal limb-girdle rigidity and spasticity, with relative sparing of the hands and feet, is analogous to other extrapyramidal disorders. Endemic cretinism is a unique form of cerebral palsy of potential interest for students of human neuromotor development.
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PMID:Magnetic resonance imaging of brain and the neuromotor disorder in endemic cretinism. 851 86

Endemic cretinism has been classified into neurological and myxedematous types. Profound mental deficiency, deaf-mutism and cerebral diplegia are predominantly found in the former. The latter have been described as less mentally retarded but with severe growth retardation and myxedematous features. The pathogenesis of different clinical types of endemic cretinism is still unclear. Recently, a unifying hypothesis suggested that iodine deficiency, severe enough to cause maternal and fetal hypothyroxinemia, results in neurological defects in all cretins. We conducted the present study in northern Thailand to determine the validity of this hypothesis in another geographical area. The study consisted of a multidisciplinary survey on 112 endemic cretins aged 2-66 years in Nan. They were categorized clinically into three types of endemic cretins, neurological (n = 57), myxedematous (n = 19) and mixed form (n = 36). The subjects were generally short and the majority had severe mental retardation (mean intellectual quotient (I.Q.) 30.8 +/- 8.8), psychomotor defect and profound sensorineural hearing loss. The I.Q. score and proportion of cretins with sensorineural hearing loss and psychomotor defect were similar among the three types of cretins. The most frequent neurological abnormalities were spasticity, hyper-reflexia, the presence of primitive reflexes and gait disturbance. These abnormalities were distributed equally among the three types of endemic cretins. Delayed skeletal maturation and abnormal epiphysis were also present in all types of cretins. However, myxedematous cretins were shorter (P < 0.01), having more myxedematous features (P < 0.05 to P < 0.001) and less sexual maturation (P < 0.05). Thyroid volume was lower in cretins with hypothyroidism (P < 0.01). In conclusion, our findings support the hypothesis that neurological features are present in all types of cretins, and are the consequence of maternal and fetal hypothyroxinemia due to severe iodine deficiency. The clinical manifestations of the cretins were subsequently modified by the length and severity of postnatal iodine deficiency and hypothyroidism.
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PMID:Endemic cretinism in Thailand: a multidisciplinary survey. 936 97

A survey was conducted in Sikkim to determine the prevalence of endemic cretinism in the state. A household was the basic sampling unit. Villages were selected randomly in the state and from these households were selected randomly using the electoral lists. All members of the households were studied. Total of 17,837 individuals from 3,197 households from 249 villages were studied. There were 8,953 males and 8,884 females. A total of 617 endemic cretins were identified: 316 males and 301 females. The overall prevalence of endemic cretinism was 3.46%: (males 3.53% and females 3.39%). Endemic cretinism was observed in 194 (77.9%) villages studied. Neurological cretinism was the predominant form (98.7%). Deaf-mutism was the most salient neurological feature seen in 472 (76.5%) subjects. Motor system examination revealed proximal spasticity and brisk reflexes, both more marked in the lower limbs. Recording of daily life activities revealed 14.1% of the cretins to be totally dependent and another 23% to be requiring considerable assistance for their daily routine activities. The overall prevalence of goitre found in this survey was 54%. Urinary iodine concentration was estimated from a representative sample of the population; mean 4 micrograms/dl (SD 2.68). This survey shows the existence of severe iodine deficiency in Sikkim.
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PMID:Epidemiology of endemic cretinism in Sikkim, India. 1077 77