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We report a case of 15-yr-old child that was presented with headache, polyuria, polydipsia, recent ocular motor and abducens nerve palsies and rapid visual loss. He had a long history of progressive symmetric muscular weakness predominant in the lower limb girdle. Water deprivation revealed central diabetes insipidus. Hormonal explorations demonstrated preserved pituitary function with mild hyperprolactinemia at 21.5 ng/ml (N: 2.6 to 13.1 ng/ml). Magnetic resonance imaging showed an extensive isosignal T1 and hyposignal T2 enhanced lesion infiltrating the pituitary gland, optic-chiasmal hypothalamic region, cavernous sinus, cerebrum tent and sphenoid and temporal meningeal spaces. The serum level of angiotensin converting enzyme and cerebrospinal fluid analysis were normal. No other systemic localisation was identified. Muscle biopsy objectified dystrophic changes. Genetic study identified a delT 521 mutation characteristic of Limb-girdle muscular dystrophy type 2C. Corticotherapy rapidly ameliorated the neurological symptoms. This patient was diagnosed as having neurosarcoidosis. Neurosarcoidosis is rarely reported in childhood. We discuss the problems related to diagnosis in such a situation below.

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BACKGROUND: Insulin treated diabetic patients often do not adjust their insulin doses. We developed a method to provide a quantitative and qualitative assessment of this behavior. METHODS: Fourteen patients provided logbook pages of their self-monitoring of blood glucose (SMBG) data and insulin doses. We compared the actual decisions of patients in real-life to what they would decide on the same SMBG, as an a posteriori exercise. We also compared these decisions and those proposed by 6 diabetologists on the same sets of data to the recommendations made by HumaLink, an automated insulin dosage system. RESULTS: 1) Patients in real-life modified their insulin doses least often. However, given a chance to make these decisions a posteriori, they modified their insulin doses more often. HumaLink proposed changes even more often, and diabetologists were the most aggressive in changing insulin doses. 2) The decisions proposed by the patients in real-life or a posteriori and by the diabetologists were compared to the recommendations made by HumaLink, using a decisions analysis grid (DAG). For these three groups, full disagreement with HumaLink (patient or physician increases while HumaLink decreases and the opposite) was observed for less than 5% of the cases. 3) By comparison to HumaLink, patient decisions seemed guided by the desire to avoid hypoglycemia. By contrast, decisions by diabetologists seemed often to be guided by the desire to avoid hyperglycemia. CONCLUSION: These methods provide an objective evaluation of insulin dose adjustments by patients with diabetes and may be useful to assess the effectiveness of educational programs.

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INTRODUCTION: Langerhans cell histiocytosis is a rare entity. Involvement of the pituitary region is frequently delayed and rarely studied. CASES: We report some clinical and paraclinical particularities of the disease in four women. Diabetes insipidus was the initial symptom in all cases, accompanied by panhypopituitarism at initial presentation in one woman. Hypopituitarism was diagnosed from 6 months to 4 years after the diabetes insipidus in the other cases, and systemic lesions appeared 3 to 6 years thereafter. Magnetic resonance imaging of the hypothalamic-pituitary axis found tumors in two patients and infundibular thickening in the other two. One patient also had a partially empty sella. DISCUSSION: In adults, pituitary involvement seems to occur predominantly in women, and panhypopituitarism is possible at initial presentation. The clinical polymorphism of Langerhans cell histiocytosis makes this disease difficult and often slow to diagnose.

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Radioodine-131 has an important place in the management of well-differentiated thyroid cancer. Patient preparation for radioiodine-131 administration must be rigorous and is based on the stimulation of endogenous TSH production, which requires a hypothyroid state after withdrawal of suppressive T4-therapy. The introduction of recombinant human TSH would simplify the protocol of preparation and improve the quality of life of patients. The diagnosis place of radioiodine-131 knew significant changes following the introduction of the serum thyroglobulin measurement. This tumour marker has a central role in the strategy of follow-up and tends to be the principal element of indication for a diagnosis exploration with radioidine-131. The systematic ablation of thyroid remnants remains controversial particularly in patients with good prognosis factors; the efficacy of low activities is also still debatable. The optimal follow-up strategy and the indication of remnant ablation must take in account the prognosis factors of survival and recurrence. Radioiodine-131 therapy permits frequently the cure of distant metastases, particularly in infraradiological pulmonary forms. This fact outlines the importance of an early detection of tumour recurrence based on the conjunction of radioiodine-131 and thyroglobulin. Side effects of radioiodine-131 therapy are generally limited if the precautionary measures are well applied; leukaemia constitutes the main risk but this complication is very uncommon and occurs after a high cumulative activity.

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