Efficacy and safety of corpus callosotomy versus vagus nerve stimulation as long-term adjunctive therapies in children with Lennox-Gastaut syndrome: Experience of a tertiary care center.

OBJECTIVES: To compare the efficacy and safety of corpus callosotomy versus vagus nerve stimulation (VNS) as long-term adjunctive therapies in children with Lennox-Gastaut syndrome. METHODS: This retrospective study was conducted in King Fahad Medical City between 2010 and 2019. The authors identified and followed 9 patients with Lennox-Gastaut syndrome (LGS) who underwent corpus callosotomy or VNS implantation for at least 12 months; seizure frequency and major complications were monitored. Five patients with a mean age of 10.8±1.3 years had corpus callosotomy, and 4 patients with a mean age of 13.8±3.9 years were implanted with VNS stimulators. RESULTS: Reduction in seizure frequency was achieved in all 5 patients who underwent corpus callosotomy, with greater than 75% seizure reduction in more than 50% in one, and greater than 25% in 2 respectively. However, in those implanted with VNS, 2 (50%) patients achieved a reduction in seizure frequency of greater than 75% and 2 (50%) greater than 25%, respectively. No significant difference was observed between the 2 treatment groups. One patient who underwent corpus callosotomy suffered cerebrospinal fluid leakage, and swallowing difficulties in one patient who underwent VNS. CONCLUSION: Both corpus callosotomy and VNS are safe and effective as adjunctive treatments for LGS patients.

Clinical, molecular, and biochemical delineation of asparagine synthetase deficiency in Saudi cohort.

PURPOSE: Asparagine synthetase deficiency (ASNSD) is a rare neurometabolic disease. Patients may not demonstrate low asparagine levels, which highlights the advantage of molecular over biochemical testing in the initial work-up of ASNSD. We aimed to further delineate the ASNSD variant and phenotypic spectrum and determine the value of biochemical testing as a frontline investigation in ASNSD. METHODS: We retrospectively collected the clinical and molecular information on 13 families with ASNSD from the major metabolic clinics in Saudi Arabia. RESULTS: The major phenotypes included congenital microcephaly (100%), facial dysmorphism (100%), global developmental delay (100%), brain abnormalities (100%), spasticity (86%), and infantile-onset seizures (93%). Additional unreported phenotypes included umbilical hernia, osteopenia, eczema, lung hypoplasia, and hearing loss. Overall, seven homozygous variants accounted for ASNSD. The p.Tyr398Cys and p.Asn75Ile variants accounted for 54% of the cases. The clinical sensitivity and specificity of the proposed biochemical analysis of cerebrospinal fluid (CSF) for the detection of patients with ASNSD were 83% and 98%, respectively. CONCLUSION: Our study describes the largest reported ASNSD cohort with clinical, molecular, and biochemical characterization. Taking into consideration the suboptimal sensitivity of biochemical screening, the delineation of the phenotype variant spectrum is of diagnostic utility for accurate diagnosis, prognosis, counseling, and carrier screening.

Infants routine video electroencephalogram findings in a Saudi tertiary hospital.

OBJECTIVE: To report the existence and describe the non-epileptic abnormalities, interictal epileptiform discharges, and seizures in routine electroencephalograms (EEGs) of infants in a tertiary hospital out-patient neurophysiology clinics. METHODS: This is a non-interventional, retrospective descriptive study that involved the review of 172 infants` EEGs conducted from July 2018 to June 2019 in King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia. RESULTS: Total of 172 EEGs were reviewed; 152 routine EEGs for infants and 20 neonatal EEGs. Seventy-six (50%) EEGs were reported to be normal. Among the remaining EEGs, 54 were characterized by generalized slow background abnormalities (31.3 %), seven (4%) by background asymmetry, one (0.5%) by generalized low amplitude, and one (0.5 %) by an alternate trace that was dysmature for age. Interictal epileptiform discharges (IED) were present in 75 (43.6%) EEGs and they were focal in 72 (41.8%) EEGs, and generalized spike wave discharges were seen in only 3 (1.7%) EEGs with focal interictal epileptiform discharges. A diagnosis of hypsarrythmia was made from 15 (8.7%) EEGs. There were seizures in 11 (6.4%) EEGs, of which three were in neonates (15% of neonatal EEGs), and 8 in infants (4.6 % of infant EEGs). CONCLUSION: Fifty % of routine infants` EEGs had abnormalities and hypsarrythmia was the most common abnormal background associated with seizures. Ictal discharges in form of generalized electrodecremental pattern associated clinically with epileptic spasm was the most common type of seizures.

Amelioration of Levetiracetam-Induced Behavioral Side Effects by Pyridoxine. A Randomized Double Blind Controlled Study.

BACKGROUND: Levetiracetam is a relatively new-generation antiseizure drug approved for the treatment of focal and generalized seizures. Despite its favorable side effect profile and minimal drug-drug interactions, neuropsychiatric side effects are reported in up to 13% of children. A few case series have suggested that supplementation of pyridoxine may mitigate these side effects, but controlled trials are lacking. To address this issue, a randomized interventional study was carried out in a pediatric tertiary hospital to qualify and quantify the potential beneficial effect of pyridoxine in attenuating the neuropsychiatric side effects of levetiracetam in children. METHODS: A total of 105 children with epilepsy who were taking levetiracetam (as a monotherapy or an adjunct) who showed behavioral symptoms coinciding with the start of levetiracetam, were included. Patients randomly and blindly received either a therapeutic (pyridoxine group, 46 of 105, 44%) or a homeopathic dose of pyridoxine (placebo, 59 of 105, 56%). A 30-item behavioral checklist was used to qualify and quantify the behavioral side effects at baseline and at different time points following initiation of treatment. RESULTS: Both placebo and pyridoxine groups experienced a statistical reduction in behavioral scores when compared with baseline. Our study indicated that although there was a placebo effect, the improvement in neuropsychiatric symptoms was more prominent in children who received therapeutic doses of pyridoxine. CONCLUSIONS: These data provide clinicians with pertinent evidence-based information that suggests that a trial of pyridoxine in patients who experience behavioral side effects due to the use of levetiracetam may avoid unnecessary change of antiseizure medications.