Scurvy Presenting As Pseudoparalysis Without Other Classical Clinical Features : A Case Report

摘要

A 28 months old boy presented in the casualty for evaluation of lower limb weakness and difficulty in walking since last 2 weeks and being referred as acute flaccid palsy suspected as Guillen Barre Syndrome for further management. The clinical presentation and imaging findings, together with the dramatic response to ascorbic acid intake, allowed us to confirm the diagnosis of infantile scurvy. Introduction Scurvy is a common nutritional disorder in developing countries due to high prevalence of malnutrition. Vitamin C performs various important functions in the body. It is required for synthesis of supporting tissues of mesenchymal ori?gin like osteoid in bone, chondroid in cartilage, dentin in teeth and collagen in capillary walls. It enhances iron absorp?tion from the gastrointestinal tract, acts as an anti-oxidant, required in the coa?gulation pathway, also in tyrosine metabolism and synthesis of corticosteroids 1 . Amla, guava and citrus fruits are rich dietary sources of vitamin C 1 . Human breast milk has five times more vitamin C than cow's milk, thus breast fed infants are less prone to develop scurvy. Delay in starting weaning foods, improper di?etary habits and recurrent diarrhea pre?dispose to scurvy 123 . Case Report A 28 months old boy presented in the casualty for evaluation of lower limb weakness and difficulty in walking since last 2 weeks and being referred as acute flaccid palsy suspected as Guillen Barre Syndrome for further management. On examination, he was irritable and thinly built. Height and weight were in the 85th percentile for his age and sex. There was no muscle weakness noticed. There was marked tenderness in the lower limbs and difficulty in walking with a wide-based, waddling gait, requiring assistance to ambulate. He had normal abdominal examination results, without organomegaly. He had no apparent joints swelling or gum hypertrophy, but there was pain with motion of his knees and hips. The remainder of his neurological examination was normal. The past history and development history was normal. His dietary history indicated that he was mainly fed with diluted feeds during infancy and very less green vegetables and citrus fruits being included in the diet. Imaging of lower limbs showed classical features of scurvy (fig.1) and hence diagnosis –pseudo paralysis of scurvy made and accordingly child was treated with 200 mg of daily ascorbic acid for one month and dietary modifications were also advised. Seventy-two hours after starting oral vitamin C supplementation, there was significant improvement in the patient's general health. He markedly improved within 2-3 weeks with follow up x ray showing signs of healing. He was able to walk in 2 weeks of treatment. Imaging of lower limbs also showed signs of healing (fig.2).The clinical presentation and imaging findings, together with the dramatic response to ascorbic acid intake, allowed us to confirm the diagnosis of infantile scurvy. Discussion Scurvy is the disease caused by deficiency of vitamin C in diet. The typical pathologic manifestations of vitamin C deficiency are noted in collagen-containing tissues and organs such as skin, cartilage, dentine, osteoid and capillary blood vessels. Pathologic changes are a function of the rate of growth of the affected tissues; hence the bone changes are often observed only in infants during periods of rapid bone growth. Defective collagen synthesis leads to defective dentine formation, hemorrhaging into the gums, and loss of teeth. Hemorrhaging is a hallmark feature of scurvy and can occur in any organ. Hair follicles are one of the common sites of cutaneous bleeding 12 . Bone involvement is typical for infantile scurvy. The bony changes occur at the junction between the end of the diaphysis and growth cartilage. Osteoblasts fail to form osteoid (bone matrix), resulting in cessation of endochondral bone formation 123 . Scurvy usually pre?sents between 6 and 24 months of age 23 . Initially there may be nonspe