We report a patient with juvenile myoclonic epilepsy who subsequently developed temporal lobe epilepsy, which gradually became clinically dominant. Video telemetry revealed both myoclonic seizures and temporal lobe seizures. The temporal lobe seizures were accompanied by a focal recruiting rhythm with rapid generalisation on EEG, in which the ictal EEG pattern during the secondary generalised phase was morphologically similar to the ictal pattern during myoclonic seizures. The secondary generalised seizures of the focal epilepsy responded to sodium valproate, similar to the myoclonic epilepsy. In this rare case of coexistent Juvenile Myoclonic Epilepsy and Temporal lobe epilepsy, the possibility of focal epilepsy recruiting a generalised epileptic network was proposed and discussed.
Benign epilepsy of childhood with centrotemporal spikes (BECT) was studied in Malaysian children, and was observed in Chinese, Malay and Indian children in the ratio 10:6:5. There were 12 boys and 9 girls. Fit frequency varied from almost daily to a single fit. The age of onset ranged from 2-13 years and BECT was not noted in any child over 13 years old. There was a strong circadian rhythm and fits occurred mainly in sleep. Generalised seizures were more common than partial seizures. During the 3-year study from April 1989 to April 1992, 21 children with BECT were identified from the EEG records done at the University Hospital and it was found that this genetic epilepsy which is autosomal dominant with age dependent penetrance occurs in approximately 4.8% of our epileptic children. In addition there were 3 children in whom petit mal co-existed with a BECT EEG trait.
Two rare cases of intractable epilepsy caused by Dysembryoplastic Neuroepithelial Tumours (DNET) are reported and their different management discussed. The first case required vagal nerve stimulation and radiosurgery while the later was operated with the help of neuronavigation. Both had good outcome according to Engel classification after a one year follow up.
We examined the clinical, semiological, scalp EEG, and neuropsychological features of patients with "pure" neocortical temporal lobe epilepsy (NTLE) who were successfully treated by neocortical temporal resection sparing the mesial temporal structures. This retrospective study included 17 patients with lesional NTLE who satisfied the following criteria: presence of a discrete structural lesion in the lateral temporal lobe on preoperative MRI; lateral temporal resection sparing the mesial temporal structures; follow-up for at least two years after surgery; and favourable postoperative seizure outcome (Engel Class I). The study included 10 females and seven males, and the age at surgery ranged from 15 to 48 years (mean: 30.7 years). Auras, video-recorded seizure semiology, interictal and ictal EEG, and pre- and post-operative neuropsychological data were reviewed. Twenty patients with mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis, who had a favourable postoperative seizure outcome (Engel Class I), were selected as a control group. Age at seizure onset was significantly greater in patients with NTLE than in controls. A history of febrile convulsion was significantly less frequent in NTLE patients. Epigastric ascending sensation (6% versus 40%; p=0.017), oral automatisms (29% versus 80%; p=0.003), gestural automatisms (47% versus 80%; p=0.047), and dystonic posturing (0% versus 40%; p=0.003) were significantly less frequent in NTLE than controls. Ictal unitemporal rhythmic theta activity was also significantly less frequent in NTLE than controls (35.3% versus 75%; p=0.015). Preoperative IQ score (range: 68 to 114; mean: 88.9) and preoperative memory quotient score (range: 56-122; mean: 98.1) were significantly higher in NTLE (p=0.003 and p=0.048, respectively). There were notable differences in clinical, semiological, EEG, and neuropsychological features between "pure" NTLE and MTLE. These findings may be useful to identify the epileptogenic zone.
Hearing loss has been reported with valproic acid (VPA) use. However, this is the first case of VPA-induced hearing loss that was tested and confirmed with a VPA rechallenge, supported by serial audiometry and pharmacokinetic modelling. A 39-year-old truck driver with temporal lobe epilepsy was treated with VPA at 400 mg, twice daily, and developed hearing loss after each dose, but recovered within three hours. Hearing loss fully resolved after VPA discontinuation. Audiometry performed five hours after VPA rechallenge showed significant improvement in hearing thresholds. Pharmacokinetic modelling during the VPA rechallenge showed that hearing loss occurred at a level below the therapeutic range. Brainstem auditory evoked potential at three months after VPA discontinuation showed bilateral conduction defect between the cochlear and superior olivary nucleus, supporting a pre-existing auditory deficit. VPA may cause temporary hearing threshold shift. Pre-existing auditory defect may be a risk factor for VPA-induced hearing loss. Caution should be taken while prescribing VPA to patients with pre-existing auditory deficit.
Reflex epilepsy is usually induced by external stimulation, photosensitive epilepsy being the most common. Epilepsy induced by auditory stimulation is rarely studied. There are no currently published magnetoencephalographic (MEG) studies demonstrating the initiation of epileptic neuronal discharges by repeated auditory stimulations in temporal lobe epilepsy (TLE) patients. We retrospectively studied one TLE patient who underwent a MEG study to localize her epileptic focus. Auditory, somatosensory, visual and motor evoked potential studies were performed during the MEG recording. A single dipole method calculated equivalent current dipoles to localize the epileptic source. The least-squares minimization method was used to obtain the optimal solution with goodness-of-fit of greater than 80%. Periodic lateralized epileptiform discharges (PLEDs) were recorded in the temporal region when repeated auditory stimulations were done. We postulated that neuronal cortical suppression occurred during repeated stimulations which provoked epileptiform discharges (PLEDs) without any physical symptoms or aura. It was concluded that repeated stimulations could facilitate epileptiform discharges in focal area/areas in certain subjects.
Background and Objective: There is a great challenge to establish a level 4 epilepsy care offering
complete evaluation for epilepsy surgery including invasive monitoring in a resource-limited country.
This study aimed to report the setup of a level 4 comprehensive epilepsy program in Malaysia and the
outcome of epilepsy surgery over the past 4 years.
Methods: This is a retrospective study analyzing
cases with intractable epilepsy in a comprehensive epilepsy program in University Malaya Medical
Center (UMMC), Kuala Lumpur, from January 2012 to August 2016.
Results: A total of 92 cases
had comprehensive epilepsy evaluation from January 2012 till August 2016. The mean age was 35.57
years old (range 15-59) and 54 (58.7%) were male. There were 17 cases having epilepsy surgery
after stage-1 evaluation. Eleven cases had mesial temporal sclerosis and 81% achieved Engel class
I surgical outcome. Six cases had lesionectomy and 60% had Engel class I outcome. A total of 16
surgeries were performed after stage-2 evaluation, including invasive EEG monitoring in 9 cases.
Among those with surgery performed more than 12 months from the time of data collection, 5/10
(50%) achieved Engel I outcome, whereas 2 (20%) had worthwhile improvement (Engel class III)
with 75% and 90% seizure reduction.
Conclusion: Level 4 epilepsy care has an important role and is possible with joint multidisciplinary
effort in a middle-income country like Malaysia despite resource limitation.
We present our preliminary experience in neuropsychological testing in epilepsy surgery patients to demonstrate how these tests contributed to decide the laterality of epileptic focus, and to assess the effect of surgery on patient's cognitive function and quality of life. Preoperative neuropsychological tests consisting of Wechsler Adult Intelligence Scale-III (WAIS) for IQ, Wechsler Memory Scale-III (WMS) for memory and patients' quality of life (QOLIE 31) were administered to refractory epilepsy patients under evaluation for surgical treatment. These tests were repeated one year after surgery and we studied any changes in trends. A total of seven patients were recruited in this study between July 2004 and July 2006. The aetiologies of refractory epilepsy were pure mesial temporal sclerosis (MTS) in five patients, dysembryogenic neuroepithelial tumour (DNET) in one and dual lesion of cavernous angioma with ipsilateral MTS in one. The preoperative neuropsychological tests were all in concordance to MRI finding, and showed good contralateral function; five lateralises to the right and two to the left. The post-operative Engel seizure count (median 8.00, IQR 7.00-8.75), general IQ (88 vs. 79), performance IQ (94 vs. 79), verbal memory (89 vs. 71), non-verbal memory (88 vs. 75) and QOLIE (53.14 vs. 44.71) were better compared to preoperative values. The verbal IQ (84 vs. 84) was unchanged. Neuropsychological tests are useful as ancillary investigations to determine the laterality of seizure focus and integrity of function in the contralateral temporal lobe. Following successful surgical treatment, there is a trend towards improvement in memory, IQ and quality of life scores in this small group of patients.