RESUMO
This clinical case report aims to highlight the unusual presentation of Sneddon syndrome with a possible association with paroxysmal hemicrania. A medical record review was performed at a tertiary hospital in Riyadh, Saudi Arabia. Data collected include clinical evaluations and laboratory and imaging results. Informed consent was obtained. Hereby, we present a 27-year-old female who presented with multiple stroke attacks, along with severe headaches involving right retro-orbital pain with an eight-year history of spotted skin lesions. Initial unenhanced computed tomography (UCT) brain in the emergency showed left insular cortex hypodensity, revealing acute ischemic insult. Subsequent magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed acute ischemic infarct in the territory of the left middle cerebral artery (MCA) involving the insula and frontoparietal lobe. Further investigations were done, including cerebrospinal fluid (CSF) analysis and autoimmune and infectious workup, which were unrevealing. Skin biopsy of the lesions showed subcutaneous fat necrosis with nonspecific scattered fibrinogen positivity and was labeled as livedo reticularis vs. livedo racemosa. A Sneddon syndrome diagnosis can be very challenging, needing a high index of suspicion to direct the diagnostic investigations. Moreover, the presence of a severe headache is an unusual phenomenon that needs further study.
RESUMO
BACKGROUND: Little is known of how accurately a first-time seizure witness can provide reliable details of a semiology. Our goal was to determine how accurately first-time seizure witnesses could identify key elements of an epileptic event that would aid the clinician in diagnosing a seizure. METHODS: A total of 172 participants over 17 years of age, with a mean (sd) of 33.12 (13.2) years and 49.4% female, composed of two groups of community dwelling volunteers, were shown two different seizure videos; one with a focal seizure that generalized (GSV), and the other with a partial seizure that did not generalize (PSV). Participants were first asked about what they thought was the event that had occurred. They then went through a history-taking scenario by an assessor using a battery of pre-determined questions about involvement of major regions: the head, eyes, mouth, upper limbs, lower limbs, or change in consciousness. Further details were then sought about direction of movement in the eyes, upper and lower limbs, the side of limb movements and the type of movements in the upper and lower limbs. Analysis was with descriptive statistics and logistic regression. RESULTS: One hundred twenty-two (71.4%) identified the events as seizure or epilepsy. The accuracy of identifying major areas of involvement ranged from 60 to 89.5%. Horizontal head movements were significantly more recognized in the PSV, while involvement of the eyes, lateralization of arm movement, type of left arm movement, leg involvement, and lateralization of leg movement were significantly more recognized in the GSV. Those shown the GSV were more likely to recognize the event as "seizure" or "epilepsy" than those shown the PSV; 78 (84.8%) vs 44 (55.7%), (OR 0.22, p < 0.0001). Younger age was also associated with correct recognition (OR 0.96, P 0.049). False positive responses ranged from 2.5 to 32.5%. CONCLUSION: First-time witnesses can identify important elements more than by chance alone, and are more likely to associate generalized semiologies with seizures or epilepsy than partial semiologies. However, clinicians still need to navigate the witness's account carefully for additional information since routine questioning could result in a misleading false positive answer.
