Absolute quantification of myocardial uptake of 99mTc-DPD in patients with cardiac amyloidosis due to transthyretin deposits (ATTR).

PURPOSE: To determine the diagnostic contribution of the absolute quantification of the myocardial deposit of 99mTc-DPD in patients with cardiac amyloidosis due to transthyretin deposits (ATTR). MATERIALS AND METHODS: SPECT/CT was performed in 41 patients with positive scintigraphic results for ATTR cardiac amyloidosis. The patients were divided into two groups (Perugini grades 2 and 3) and the SUVmax at the level of the bone and both ventricles and the percentage of dose calculated in these areas were calculated. The Student's t-test was used to compare results and the area under the curve (AUC) was calculated to assess differential efficacy and establish discriminatory cut-off points between both groups of patients. RESULTS: Statistically significant differences were observed in all the study variables, with the exception of bone SUVmax. The differences with the greatest statistical power were observed in the variables SUVmaxRV and the percentage of dose in both ventricles (p < 0.001). The cut-off point obtained for the variable SUVmaxLV was 8.620 (sensitivity 87.9% and specificity 100%; AUC 0.966), while that of the variable SUVmaxRV was 6.195 (sensitivity 81.8% and specificity 100%; AUC 0.955). CONCLUSIONS: The absolute quantification of myocardial uptake of 99mTc-DPD in the SPECT/CT images of patients with suspected cardiac amyloidosis due to transthyretin deposits represents a new diagnostic tool that allows adequate classification of patients according to the Perugini visual grading scale.

Facial diplegia as unusual variant of Guillain-Barré syndrome: first case reported in Venezuela. / Diplejía facial como variante inusual del síndrome de Guillain-Barré: primer caso reportado en Venezuela.

The Guillain-Barré syndrome (GBS) is the most common cause of acute generalized paralysis. GBS is an acute inflammatory demyelinating polyradiculoneuropathy. It usually presents as a paralysis that starts in the lower limbs and then progresses symmetrically upward. The present study reports a case of bilateral facial palsy as the initial manifestation of GBS. This is a report of a case of a 37-year-old male, diabetic, that eight days after having suffered acute sinusitis, gradually presented with right hemicranial headache, dysarthria and sialorrhea. The neurological examination disclosed the absence of the bilateral frontal folds, accompanied by epiphora, bilateral lagophthalmos, bilateral Bell sign and salivary drooling through both commissures of lips. At 48 hours after hospital admission the patient showed paresis in both upper limbs. The cerebrospinal fluid analysis reported 1.1cells/mm3, fully represented by lymphocytes of normal aspect and total proteins were 196.9 mg/dL. The electromyography was consistent with acute demyelinating polyneuropathy, with a predominant motor component and a major facial involvement. With the clinical and laboratory findings, a diagnosis of GBS was established. Treatment was started with plasmapheresis and intravenous immunoglobulin, with the subsequent improvement of the clinic. The facial diplegia is part of the regional variants of GBS. Although about 60% of GBS patients present with facial weakness, it is usually preceded by weakness in the limbs. This case makes evident that GBS may present clinically as a facial diplegia.