RESUMEN
Fahr's disease is a rare genetic neurodegenerative disorder described as "bilateral striopallidodentate calcinosis" (BSPDC). It is characterized by calcium deposition crossing the blood-brain barrier and calcifying different brain areas. Here, we report a case of a 26-year-old Saudi young lady, known as a case of epilepsy since childhood, a major depressive disorder with psychotic features, and hypocalcemia related to hypoparathyroidism. CT brain showed extensive coarse calcifications involving the infra and supratentorial white matter, predominantly within the basal ganglia, thalami, and dentate nuclei of cerebellar hemispheres. This report will discuss the challenging presentation, clinical symptoms, and the multidisciplinary approach to manage Fahr's syndrome symptoms. In conclusion, this case emphasizes the importance of neuroimaging and metabolic workup when investigating the seizure's etiology. The goal of treatment in Fahr's syndrome is to manage the underlying conditions.
RESUMEN
BACKGROUND: Pulmonary embolism (PE) is a common life-threatening condition with non-specific clinical presentations. The diagnosis of PE depends highly on imaging studies, which may also provide prognostic information. This study aimed to describe the clinical and imaging profiles of patients with PE, emphasizing the differences between central and peripheral PE. METHODS: After ethics review board approval, this retrospective observational study examined the non-negative results in adult patients who underwent computed tomography pulmonary angiography (CT-PA) at our hospital between May 2016 and December 2019. Demographic and clinical information and imaging findings were collected from the electronic medical records. RESULTS: The study included 85 cases that were identified after re-interpreting the 103 non-negative CT-PA scans. Six cases were excluded for incomplete data and 12 cases were false-positive. Central PE was found in 63.5% of the cases. Obesity was the most common risk factor seen in 37.6% of the cases. Furthermore, 9.4% of the patients had sickle cell disease, which tended to be associated with peripheral PE. There was no difference between the peripheral and central PE in most clinical and imaging parameters evaluated (P > 0.05). However, patients with isolated subsegmental PE were more likely to develop hemoptysis (P = 0.04). CONCLUSION: This study suggests that patients with obesity and sickle cell disease constitute an important proportion of all PE cases. Furthermore, the clinical and imaging profiles in patients with peripheral PE are similar to those in patients with central PE. Future research should focus on the clinical value of peripheral PE in patients with sickle cell disease.
RESUMEN
BACKGROUND: Pulmonary embolism (PE) is a common and life-threatening medical condition with non-specific clinical presentation. Computed tomography pulmonary angiography (CT-PA) has been the diagnostic modality of choice, but its use is not without risks. Clinical decision rules have been established for the use of diagnostic modalities for patients with suspected PE. This study aims to assess the adherence of physicians to the diagnostic algorithms and rules. METHODS: A retrospective observational study examining the utilization of CT-PA in the Emergency Department of King Fahd Hospital of Imam Abdulrahman Bin Faisal University for patients with suspected PE from May 2016 to December 2019. The electronic health records were used to collect the data, including background demographic data, clinical presentation, triage vital signs, D-dimer level (if ordered), risk factors for PE, and the CT-PA findings. The Wells score and pulmonary embolism rule-out (PERC) criteria were calculated retrospectively without knowledge of the results of D-dimer and the CT-PA. RESULTS: The study involved a total of 353 patients (125 men and 228 women) with a mean age of 46.7 ± 18.4 years. Overall, 200 patients (56.7%) were classified into the "PE unlikely" group and 153 patients (43.3%) in the "PE likely" group as per Wells criteria. Out of all the CT-PA, 119 CT-PA (33.7%) were requested without D-dimer assay (n = 114) or with normal D-dimer level (n = 5) despite being in the "PE unlikely" group. Only 49 patients had negative PERC criteria, of which three patients had PE. CONCLUSIONS: The study revealed that approximately one-third of all CT-PA requests were not adhering to the clinical decision rules with a significant underutilization of D-dimer assay in such patients. To reduce overutilization of imaging, planned interventions to promote the adherence to the current guidelines seem imperative.
