No abstract

Background Multinutrient insufficiencies as a consequence of nutritional and economic factors are common in India and other developing countries. We have examined the impact of multi-nutrient insufficiency on markers of one carbon metabolism in the blood, and response to a methionine load in clinically healthy young women. Design & Methods Young women from Pune, India (n=10) and Cleveland, USA (n=13) were studied. Blood samples were obtained in the basal state and following an oral methionine load (50mg/kg of body weight in orange juice). Plasma concentrations of vitamin B12, folate and B6 were measured in the basal state. The effect of methionine load on the levels of methionine, total homocysteine, cysteine, glutathione and amino acids was examined. Results Indian women were significantly shorter and lighter compared with the American women and had lower plasma concentration of vitamins B12, folate and B6, essential amino acids and glutathione, but higher concentration of total homocysteine. The homocysteine response to methionine load was higher in Indian women. The plasma concentrations of glycine and serine increased in the Indian women after methionine (in juice) load. A significant negative correlation between plasma B6 and homocysteine (r= −0.70), and plasma folate and glycine and serine levels were observed in the Indian group (P<0.05) but not in the American group. Conclusion Multi-nutrient insufficiency in the Indian women caused unique changes in markers of whole body protein and one carbon metabolism. These data would be useful in developing nutrient intervention strategies.

SYNOPSIS A patient with early onset myotonic dystrophy, with associated neuropathy and epilepsy, is presented. It is postulated that his disorder was inherited through a recessive, pleomorphic gene. His differential diagnosis is discussed and the literature reviewed. The clinical variability of myotonic dystrophy is stressed and the diagnostic difficulties encountered in the younger age group.The occurrence of myotonic dystrophy in children as compared with adults is relatively uncommon. There have been reports of its incidence between the ages 6 and 14 years by Maas and Patterson (1943). However, it was Vanier (1960), Dodge et al. (1965), and Pruzanski (1966) who put the subject into proper perspective, recognizing the atypical nature of the disease in children. They described, besides the classical type, the dysembryoplasic and dysphagic types (Dodge et al., 1965;Pruzanski, 1966), and divided their patients into three groups according to the clinical manifestation. Watters and Williams (1967) added a fourth group.The features considered to be characteristic in adults, such as ptosis, cataracts, baldness, endocrine abnormalities, and percussion myotonia, are seldom seen in the younger age group (Watters and Williams, 1967). Found instead are congenital anomalies such as high arched palate and skeletal abnormalities like talipes equinovarus and kyphoscoliosis (Aberfield et al., 1965;Pearson et al., 1967).Recently there have been reports of evidence of neuropathy associated with myotonic dystrophy (McComas et al., 1971;Caccia et al., 1972;Kalyanaraman et al., 1973).The purpose of this paper is to stress the pleomorphism of myotonic dystrophy in the There was no family history of similar difficulties; both his parents were in good health as was one younger sister.He was a pleasant adolescent of normal intelligence. The face was long and thin; the head scaphocephalic; the neck thin and swan-like, with extreme wasting of the sternocleidomastoid muscles ( Fig. 1) and marked weakness of the flexor muscles of the neck so that the head could not be lifted against gravity; the palate was high-arched and the teeth malaligned. The cardiac, respiratory, and gastro-

We report two patients in whom the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) developed during the administration of psychotherapeutic drugs. In one, the syndrome occurred after administration of a phenothiazine drug and in the other, a butyrophenone. Both the patients were diagnostically studied for evidence of other disorders, either neurologic or systemic, which could cause this syndrome with negative results. They responded to fluid and free water restriction with remarkable recovery and no sequelae. It is stressed that psychotherapeutic drug administration must be considered as one of the iatrogenic causes of SIADH.

SYNOPSIS Clinicopathological correlations are presented in a case of facial myokymia with facial palsy. The causative lesions were considered to be metastatic tumours to the pons and it was concluded that both the facial palsy and the myokymia were due to interruption of supranuclear pathways impinging on the facial nucleus.