Family Studies and Human Leukocyte Antigen Class II Typing in Indian Probands with Seizures in Association with Single Small Enhancing Computed Tomography Lesions

Abstract

Summary:Purpose: To define the clinical features of the syndrome of seizures associated with single, small, enhancing computed tomography (CT) lesions (SSELs) in 235 Indian probands and seizure types among their family members. Human leukocyte antigen (HLA) class I1 genomic typing in randomly selected 41 probands was done to identify the role of hereditary factors in this syndrome.Methods: The seizure types among 235 probands, their clinical outcome, and seizures in their family members were studied. Family data were collected on relatives of 212 additional probands with neurologic diseases other than epilepsy. HLA class I1 antigens were studied by using polymerase chain reaction (PCR) amplified DNA and sequence-specific oligonucleotide probe (PCR-SSOP) hybridization.Results: The seizures in 86% were partial with or without generalization; 77% had fewer than five seizures before the first CT scan. Evanescent focal neurologic deficits after seizures were noted in 40%. Most patients (97%) were treated with a single antiepileptic drug (AED). Significant resolution of the CT scan lesion was noted within 6 months in 125 (53%) of 235 cases. Two thirds of patients had no seizures while taking a single AED, and an additional 18% had no seizures even after their AEDs were discontinued. Epilepsy among relatives of Indian probands having seizures in association with SSELs was more common as compared with relatives of probands with other neurologic diseases. A family history of seizures was noted in 21% probands, the ratio of affected first-to seconddegree relatives was 4.3: 1, and 60% of affected sibs had syndromic concordance with probands. There was a positive association of HLA-DRB1*13 (P, = 0.036) with this syndrome. Conc1usion.s:The syndrome of seizures in association with SSELs seems to be a benign localization-related epileptic syndrome. Our results of HLA studies point to an inherited susceptibility to an infective agent, which in most cases is of cysticercal etiology.

Seizures among families of Indian probands with different epileptic syndromes

“…In the present study all patients included in the SSELs group had a single, small, enhancing lesion that had either totally cleared or was reduced to a very small calcified dot on the repeat CT/MRI scan. Clinically these probands have a type of symptomatic localized epilepsy and were grouped separately (5.0) because this is now known to be a frequently seen benign epileptic syndrome almost exclusively confined to Indians (31, 32).…”

Neurocysticercosis in children

“…A genetic susceptibility to NCC has been suggested by a reported positive association of HLA-DRBII 13 with SSECTL(s). 69 Prevention NCC is a disease perpetuated by poor hygiene and sanitation. Lack of proper animal husbandry and meat inspection procedures in developing countries, wherein pigs are left to scavenge for food from open sewage systems, are responsible for ongoing transmission.…”

Neurocysticercosis: A disease of neglect

“…[32] From India, in patients with single, small, contrast enhancing CT lesions, a positive association of HLA-DRB1* 13, HLA-B63 and HLA-B58 and a decrease in persons with HLA-A11 has been shown. [3334] The environmental factors determining solitary cysts are young age and a patient being from the Indian subcontinent or travelers from non-endemic countries. Such patients are usually young teenagers or young adults (majority under 20 years of age) presenting with newly developed seizures.…”