Frontotemporal dementia in Oman: cognitive behavioural profile and neuroimaging characteristics; a prospective hospital-based study.

Frontotemporal dementia is increasingly recognised as an important cause of early-onset dementia and is considered to be the second commonest neurodegenerative dementia after Alzheimer's disease. We describe the cognitive, behavioural profile and neuroimaging characteristics of 6 patients with frontal variant of Frontotemporal dementia that were evaluated at the cognitive behavioural clinic at this tertiary referral teaching hospital. All patients underwent clinical, neuropsychological, structural/functional neuroimaging, and laboratory evaluations. The male to female ratio was 1:1; mean age of onset was 54 years, and the mean duration of symptoms were 30 months. The mean scores for Addenbrooke's cognitive examination, Frontal Assessment Battery, and Mini-Mental State Examination were 70.5, 6.33 and 23.6 respectively. The mean VLOM ratio was 2.04. MRI revealed significant asymmetrical regional frontal/temporal atrophy supplemented by the evidence of circumscribed hypoperfusion in SPECT imaging. We conclude that a combination of behavioural and cognitive assessment using short bedside tests, along with structural and functional neuroimaging does facilitate early identification, and increase the diagnostic specificity of Frontotemporal dementia.

Isolated agenesis of the gallbladder.

We report a rare case of agenesis of the gallbladder, which was misdiagnosed as cholecystitis. This is the first reported case from the Middle East. Despite advances in biliary imaging, the diagnosis is usually made at surgery. Like most patients, our patient became asymptomatic after the surgery. Extensive dissection to exclude the presence of gallbladder in an ectopic site is discouraged.

Radioactive iodine in the treatment of Graves' disease.

OBJECTIVES: This study was performed to evaluate the efficacy of radioactive iodine 131I therapy of Graves' hyperthyroidism at Sultan Qaboos University Hospital, Oman and to determine the optimal dose of 131I needed to achieve the euthyroid or hypothyroid status. METHODS: The medical records of 366 patients with Graves hyperthyroidism who received a single dose of 131I at Sultan Qaboos University Hospital, Oman between 1991 and 1999 were reviewed. The diagnosis was based on clinical, biochemical grounds and 99mTc thyroid scintigraphy. The patients were followed up for a minimum period of 12 months. For the analysis, the patients were divided into 6 groups according to the 131I dose administered: Dose one (350-399), dose 2 (400-449), dose 3 (450-499), dose 4 (500-549), dose 5 (550-599) and dose 6 (> or = 600) MBq. RESULTS: Fifty-eight percent of all the patients were hypothyroid after 3 months. Three hundred and twenty two patients (88%) were treated by a single dose of 131I in 12 months (85.5% hypothyroid and 2.5% euthyroid). Forty-one patients (11.2%) required a 2nd 131I dose and only 3 patients required 3 doses of 131I. The best cure rate (93%) was observed in group dose 5 (574.0 +/- 16.4 MBq) which however, was not significantly different from other dosage levels. The female to male ratio was 2:1 and the cure rates were not gender or age related. CONCLUSION: Treatment of Graves' hyperthyroidism from a single 131I dose is our aim, rather than avoidance of hypothyroidism. Our results indicate that cure rates are higher with larger doses of 131I except in group dose 6 (special category of patients). In the future, fixed doses would be adopted in our radioactive iodine treatment practice guidelines. As the majority of our patients were hypothyroid at 3 months regular monthly follow-up is essential. Whenever appropriate, physicians are encouraged to consider early referral of Graves' hyperthyroidism patients for radioactive iodine treatment as it is cheap, effective, easy to administer and free from serious side effects.

Splenic function in Omani children with sickle cell disease: correlation with severity index, hemoglobin phenotype, iron status, and alpha-thalassemia trait.

The prevalence of functional asplenia in Omani children with sickle cell disease (SCD) has not been previously defined. In this study, the authors aim to compare the natural history of splenic dysfunction in their patients to other reports. The splenic function was studied in 72 Omani patients with sickle cell disease (50 homozygous for hemoglobin S (HbS-S), 11 double heterozygotes for HbS and beta(0)-thalassemia (HbS-beta(0)-thal), 5 HbS-beta(+)-thal, 5 patients with hemoglobin S-D disease, and 1 child with hemoglobin S oman trait) aged 4.8-16 years, using (99m)Tc-labeled tin colloid scintigraphy. The study revealed 4 groups according to their colloid uptake: group I included 20 patients (28%) with normal splenic function; group II, 6 patients (8%) with mild hyposplenism; group III, 20 (28%) with severe hyposplenism; and group IV, 26 (36%) patients with functional asplenia. Overall, more than 60% of them had preserved splenic function. Except for HbS-beta(+) patients, the developmental pattern of hyposplenism was not different among the different Hb phenotypes. Factors associated with preservation of spleen function in these patients were larger splenic size (p < .01), less clinical severity (p < .05), lower MCH (p < .01), higher HbF (p < .001), and presence of alpha-thalassemia trait (p < .05).

Are scintigraphy and ultrasonography necessary before fine-needle aspiration cytology for thyroid nodules?

OBJECTIVE: To evaluate the efficacy of scintigraphy, ultrasound and fine-needle aspiration in thyroid nodules and to establish the best diagnostic pathway in detecting thyroid cancer. METHOD: Two hundred and sixteen patients with thyroid nodules were examined using high-resolution ultrasonography, 99mTc thyroid scintigraphy and ultrasound-guided fine-needle aspiration. Of these, 113 patients subsequently underwent thyroidectomy. The remaining 103 were followed up for two years without any evidence of malignancy. RESULTS: Cytopathology classified 71% of the aspirate as benign, 3% as positive for malignancy, 21% as suspected neoplasia and 5% as unsatisfactory. Fine-needle aspiration cytology had a sensitivity of 87.5% and specificity of 80%. On ultrasound 33% of malignant nodules were hypo-echoic and on scintigraphy 16% of solitary cold nodules were malignant. Neither test could reliably diagnose thyroid cancer. CONCLUSION: Ultrasound-guided fine-needle aspiration cytology should be the first test performed in euthyroid patients with a thyroid nodule. Scintigraphy and ultrasound imaging should be reserved for follow-up studies and patients who have suppressed levels of thyroid stimulating hormone.