Relationship of Lennox-Gastaut syndrome with perinatal event: A cross-sectional study.

INTRODUCTION: About one-half of children with Lennox-Gastaut syndrome (LGS) have history of birth hypoxia or other perinatal event but the knowledge about clinical, radiological profile and severity of epilepsy in these children as compared to those without a perinatal event is not known. MATERIALS AND METHODS: Thirty-one children with LGS were enrolled in this study and divided into two groups: One group with the perinatal event and other group without evidence of the perinatal event. We hypothesized that LGS with the perinatal event will have an early age of onset of LGS, more motor deficits and abnormal brain magnetic resonance imaging (MRI) and more severe epilepsy. RESULTS: There were 17 children in the perinatal event group and 14 in the other group. The mean age of onset of illness was significantly earlier in the perinatal event group (P < 0.05). More children in the perinatal event group had delayed milestones (P < 0.05), had higher seizure frequency (P < 0.05) however; there was no significant difference in number of anti-epileptic drugs consumed, motor deficits or MRI abnormalities. CONCLUSION: LGS children with the perinatal event have more severe epilepsy with early onset of disease and delayed milestones. History of perinatal insult in these children may help in predicting prognosis in LGS.

Role of P-glycoprotein in refractoriness of seizures to antiepileptic drugs in Lennox-Gastaut syndrome.

Mechanism of seizure refractoriness to antiepileptic drugs in children with Lennox-Gastaut syndrome is not known. Efflux of antiepileptic drugs due to increased expression/function of P-glycoprotein, a multidrug efflux transporter protein on the cell surface is a proposed mechanism. The authors studied the expression/function of P-glycoprotein on peripheral blood mononuclear cells of 29 children with Lennox-Gastaut syndrome, 23 children with other epilepsies, and 19 healthy children. The authors found a higher P-glycoprotein expression/function in Lennox-Gastaut syndrome, a higher percent positive cells as compared to children with other epilepsy (P < 0.001) and to healthy controls (P = 0.012), higher P-glycoprotein expression as compared to healthy controls (P = 0.003), a higher total P-glycoprotein expression (relative florescence intensity × percent positive cells) as compared to children with other epilepsies (P < 0.001) and healthy controls (P < 0.001), and a higher P-glycoprotein function as compared to children with other epilepsies (P = 0.001) and healthy controls (P = 0.002). These findings may explain seizure refractoriness to anti-epileptic drugs in Lennox-Gastaut syndome.

Cranial aspergilloma masquerading as meningioma.

Cranial aspergillosis may present as meningitis, cerebral abscess, cerebral infarcts/haemorrhages or extra-axial mass. Extra-axial cranial aspergilloma may mimic meningioma owing to mass-like characteristics and intense contrast enhancement on MRI there by delaying the diagnosis and further worsening the already bad prognosis in these patients. We present a 45-year-old gentleman who presented with signs of raised intracranial hypertension, secondary optic atrophy and a contrast-enhancing mass arising from the planum sphenoidale. Postoperatively, mass was diagnosed as aspergilloma on histopathology and culture. Despite antifungal treatment, patient could not be saved due to large artery infarcts in the immediate postoperative period. We discuss the clinical and MRI features that could help to have sufficient and early suspicion of fungal aetiology in these patients.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases.

BACKGROUND: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA) are rare primary headache syndromes characterized by spontaneous or triggered attacks of unilateral, brief, multiple, orbitofrontal pain associated with ipsilateral autonomic features. SUNCT is considered as a subset of SUNA. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or either of two is present. SUNCT/SUNA can be triggered immediately after or at the decrescendo phase of the ongoing attack without any intervening refractory period. Refractory period is usually present in trigeminal neuralgia. Absent refractory period is thought to reliably differentiate SUNCT/SUNA from trigeminal neuralgia and has been proposed for inclusion into the International Classification of Headache Disorders (ICHD) diagnostic criteria for SUNCT. CASE REPORTS: We report three patients of SUNCT syndrome with preserved intervening refractory period of variable duration observed at different times. DISCUSSION: Trigeminal neuralgias with autonomic features, SUNA and SUNCT share a common pathophysiological mechanism and actually represent a continuum. It is well known that patient with trigeminal neuralgia may transform into SUNCT/SUNA. Similarly, being a continuum, the presence or the absence of refractory period and its duration may change in a patient with SUNCT/SUNA at different time points. CONCLUSION: The presence of refractory period should not exclude the diagnosis of SUNCT in a patient with other clinical features suggestive of SUNCT.