Faciobrachial dystonic seizures are pathognomonic of leucine-rich glioma inactivated-1 (LGi1) antibody, non-paraneoplastic limbic encephalitis. Faciobrachial dystonic seizures usually precede limbic encephalitis by about a month. It is unknown whether, if untreated, faciobrachial dystonic seizures inevitably progress to limbic encephalitis. We present an LGi1 seropositive patient with a year’s history of faciobrachial dystonic seizures, who achieved remission spontaneously without immunotherapy or antiepileptic drug treatment, and did not develop evidence of limbic encephalitis over a three-year follow-up.
Background & Objective: Modulation of cortical excitability by low frequency repetitive transcranial
magnetic stimulation (rTMS) has demonstrated therapeutic use in epilepsy. This study aimed to evaluate
the efficacy of low-frequency rTMS on refractory epilepsy in a group of Malaysian subjects. Methods:
Nine patients with refractory epilepsy completed the study. All patients received 10 sessions of 1Hz
rTMS (1000 pulses per session) at 90% of resting motor threshold. Outcome measures included seizure
frequency, Symptom Checklist-90 (SCL-90), Beck Depression Inventory II (BDI II) and Quality of
Life in Epilepsy-31 (QOLIE-31). Responders were defined as having ≥ 50% seizure reduction. Results:
The mean age was 33.8 years (SD 11.7), with 4 male. Three patients had mesial temporal sclerosis
(MTS); 4 with focal cortical dysplasia (FCD) and two lesion-negative. Three patients achieved >50%
seizure reduction at 8 weeks post-treatment, with 2 of them had improvement in the number of IED.
All of the responders had FCD. The responders were younger (mean 24.7 vs. 38.3 years old), had
shorter duration of illness (mean 15.7 vs. 30.5 years) and had less frequent seizure frequency prior to
treatment (mean 5.5 vs. 10.8 attacks per week), as compared to the non-responders. Six patients had
improvement in BDI-II scores, two in QOLIE-31 and four in SCL-90 post treatment, irrespective of
seizure control. The mean scores in BDI-II improved significantly with treatment (p
There are 50-100 million dengue infections each year, but dengue encephalitis is relatively
uncommon. The aetiology of neuronal injury is proposed to be due to direct viral neurotropism or
host immune response-mediated inflammation causing neuronal damage. We report a case of severe
dengue encephalitis, presenting during the acute viraemic phase of the disease. This was associated
with inflammation and haemorrhage of the internal medullary lamina of both thalami which, to our
knowledge, has not yet been reported in other infections of the central nervous system.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary disease of small cerebral arteries. This case series aims to describe the mutations in NOTCH3and their phenotypes in Malaysia. We includedpatients who were genetically confirmed to have CADASIL, diagnosed at the University of Malaya Medical Centre, Malaysia. Family members who fulfilled clinical or imaging criteria, and patients from two previous published Malaysian families were also included. Six families (eleven cases) were included in this series. Genetic testing revealed NOTCH3 mutations in c.328C>T (p.Arg110Cys, R110C), c.553T>G (p.Cys185Gly, C185G), c.1630C>T (p.Arg544Cys, R544C) and c.160C>T (p.Arg54Cys, R54C). Two out of four Chinese families had R544C mutation in exon 11, with a later age of onset, absence of migraine and lack of anterior temporal pole involvement on MRI. One family with mixed Indian and Chinese ancestry had a mutation in exon 3 with R110C and another Indian family exon 4 with C185G mutation. This case series highlights the genotypic and phenotypic variability of CADASIL in a multi-ethniccountry. The finding of p.Arg544Cys mutation among the older Chinese families, similar to those reported in Jeju Island and Taiwan, suggest the need to screen the older Chinese stroke patients with typical MRI changes.