Hypoparathyroidism in a Case of Transfusion Dependent Thalassemia / Journal of the ASEAN Federation of Endocrine Societies

@#Repeated blood transfusions in transfusion dependent thalassemia (TDT) leads to iron overload-related endocrine complications. Hypoparathyroidism (HPT) with severe signs of hypocalcemia is a recognized complication among these patients. A 14-year-old thalassaemic boy, on regular transfusion and on anticonvulsant therapy with a presumptive diagnosis of epilepsy for the last 1 year, was admitted with high fever and severe muscle cramps with positive Trousseau’s sign. He was diagnosed as a case of primary HPT and magnesium deficiency on the basis of low serum calcium, high phosphate, normal alkaline phosphates, very low intact parathyroid hormone (iPTH), normal serum vitamin D and very low serum magnesium level. His calcium, magnesium and phosphate level normalised following treatment with intravenous magnesium and calcium. His iPTH improved but remained at low normal. He was discharged from hospital with oral calcium, calcitriol, and magnesium supplementation. The anticonvulsant (Phenobarbitone) was successfully withdrawn gradually over the next six months without any recurrence of seizure in the subsequent 3 years of follow up. Acquired HPT (apparently from hemosiderosis) is a common cause of hypocalcemia; and magnesium depletion further complicated the situation leading to severe hypocalcemia with recurrent episodes of convulsion. Magnesium replacement improved the parathyroid hormone (PTH) value proving its role in acquired HPT. Very high phosphate level on admission and poor PTH response with respect to the low serum calcium, indicates intrinsic parathyroid pathology. Metabolic abnormalities should always be evaluated in thalassaemic subject with seizure disorder and it appears that the initial convulsive episodes were due to hypocalcemia. Muscle pain, cramps or convulsion may occur from HPT and simultaneous magnesium deficiency in transfusion dependent thalassaemic subjects. Metabolic correction is more important than anticonvulsant medication. Calcium and magnesium should both be assessed routinely in transfusion dependent thalassemic patients.

Guillain-Barré syndrome developing in a patient with Graves' disease / Journal of the ASEAN Federation of Endocrine Societies

@#<p style="text-align: justify;">Graves' disease (GD) and Guillain-Barre syndrome (GBS) are both autoimmune disorders and are triggered by interactions between genetic and environmental factors. GBS in patients who suffer from other autoimmune diseases is rarely reported, and the development of atypical GBS with cranial nerve involvement in a patient with GD has never been previously reported. Herein, we report a patient with GD and a rare form of pharyngo-cervico-brachial variety of GBS.</p>

Clinical profile of young-onset dementia: A study from Eastern India

Young-onset dementia, defined as dementia occurring under the age of 65, is an increasingly recognized cause of morbidity and disability. There are few reports of the clinical profile of young-onset dementia from India. The objective of this study was to determine the clinical profile of patients attending a specialist cognitive disorders clinic in West Bengal, an eastern state of India. Almost one-fourth (94/379, 24.5%) of all the patients with dementia were of young onset. Women constituted about one-third of these cases. There was a gradual increase in the number of cases with rising age. The most common etiologies were Alzheimer disease (33%), frontotemporal dementia (27%), and vascular dementia (20%). In contrast to other published studies of young-onset dementia, frontotemporal dementia was commoner than vascular dementia. This could be due to referral bias. A positive family history was found in close to one-fifth of the patients. Close to 10% of the patients had reversible causes of dementia. Community based study is required to confirm the findings of this study.

Is post-stroke hyperglycemia a marker of stroke severity and prognosis: A pilot study

Various physiological parameters like blood pressure, temperature, blood sugar after onset of stroke have been proposed as possible marker of stroke prognosis to study the glycaemic status after acute stroke and assess the role of glycaemic status along with other clinical parameters in influencing stroke outcome. Forty-two confirmed stroke patients attending hospital within 6 hours of onset of stroke onset were included in the study. The time lag for hospitalization, blood pressure, blood sugar, HbA1c, stroke severity according to Toronto Scale, demographic factors, stroke onset type, type of stroke, past history of stroke, diabetes, and hypertension were recorded. The outcome was whether patient survived at the end of forth week. Twenty-one percent of patients who were not known diabetic found to be hyperglycemic though their HBA1C level was normal. Eighty-nine percent of such patients died. This rate was significantly higher than patients known to be diabetic with raised sugar and HBA1c level (26% patients, 12% mortality). There was strong and significant association between stroke severity and poor outcome. Strong and significant association was also found between stroke severity and blood sugar level. Modeling of stroke outcome using decision tree analysis (QUEST) found stroke severity as most important and significant predictor especially for severe stroke cases. In mild and moderately severe stroke, high sugar level was found to be a predictor, though not statistically significant.This study suggests that stroke severity is the most important predictor of stroke outcome, with high sugar level as a marker of stroke severity.