ABSTRACT
Acute intermittent porphyria (AIP) is an autosomal, dominant, rare metabolic disturbance that results from a defect in the activity of the heme biosynthesis. It has a heterogeneous presentation, making a prompt diagnosis challenging. We report a case of acute intermittent porphyria in a young female who underwent in vitro fertilization (IVF) and presented with recurrent abdominal pain and posterior reversible encephalopathy syndrome (PRES), progressing to acute progressive quadriparesis post-delivery.
ABSTRACT
OBJECTIVE: Sudden unexpected death in epilepsy (SUDEP) is a significant cause of mortality in people with epilepsy (PWE), with an incidence of 1 per 1000 members of the population. In Saudi Arabia, no data are available that inform local clinical practitioners about the attitudes of PWE regarding SUDEP. The aim of this study was to investigate the perspectives of Saudi PWE toward SUDEP and to assess their knowledge of SUDEP. METHODS: A cross-sectional questionnaire-based study was conducted at the neurology clinics of King Abdul-Aziz Medical City, Riyadh and Prince Sultan Military Medical City, Riyadh. RESULTS: Of the 377 patients who met the inclusion criteria, 325 completed the questionnaire. The mean age of the respondents was 32.9 ± 12.6 years. Of the study subjects, 50.5% were male. Only 41 patients (12.6%) had heard about SUDEP. Most patients (94.5%) wanted to know about SUDEP, of whom 313 (96.3%) wanted to receive this information from a neurologist. A total of 148 patients (45.5%) thought that the appropriate time to receive information about SUDEP was after the second visit, whereas only 75 (23.1%) wanted to learn about SUDEP during the first visit. However, 69 patients (21.2%) thought that the appropriate time to be informed about SUDEP was when seizure control had become more difficult. Almost half (172, 52.9%) of the patients thought that SUDEP could be prevented. SIGNIFICANCE: Our findings suggest that most Saudi PWE do not know about SUDEP and want to be counseled about their risk of SUDEP by their physicians. Therefore, education of Saudi PWE about SUDEP must be improved.