Update to the Hong Kong Epilepsy Guideline: evidence-based recommendations for clinical management of women with epilepsy throughout the reproductive cycle.

Since the publication of the Hong Kong Epilepsy Guideline in 2009, there has been significant progress in antiepileptic drug development. New AEDs have emerged, and data about their uses have been published. Women require special attention in epilepsy care. Drug teratogenicity, pregnancy, breastfeeding, contraception, reproduction technology, menopause, and catamenial epilepsy are major topics. Antiepileptic drugs should be chosen individually for patients who are pregnant or may become pregnant with consideration of their teratogenicity and seizure control properties. Folate is commonly prescribed for women of childbearing age who are taking antiepileptic drugs. Spontaneous vaginal delivery and breastfeeding are not contra-indicated in most cases but need to be considered individually based on the patient's medical condition and wishes. Serum drug level monitoring of certain antiepileptic drugs during pregnancy and puerperium can guide dosage adjustment. For catamenial epilepsy, intermittent benzodiazepines such as clobazam during the susceptible phase of the menstrual cycle could be a treatment option.

Idiopathic inflammatory demyelinating disorders after acute transverse myelitis.

Acute transverse myelitis (ATM) is commonly para-infectious. Recurrent ATM occurs in connective tissue diseases (CTD), infective myelitis and idiopathic inflammatory demyelinating disorders (IIDD) including multiple sclerosis (MS) and neuromyelitis optica (NMO). Previous studies might include NMO and idiopathic recurrent transverse myelitis (IRTM) as MS. The aim was to study the outcome of patients after a first attack of idiopathic ATM. Idiopathic ATM patients over a 6-year period were retrospectively studied. Known causes of myelopathy were excluded. Among 32 patients studied, 20 (63%) had single ATM attack upon follow up for 39-93 months, three developed recurrent ATM related to CTD (two systemic lupus erythematosus and one anti-Ro antibody positive) and nine (28.1%) developed recurrent neuroinflammation compatible with IIDD. Among IIDD patients, three had NMO, two restricted variant of NMO, three IRTM and one classical MS. NMO, its variant and IRTM had mean spinal MRI abnormality of 3.7, 2.1 and 3.9 vertebral segments respectively while non-recurrent ATM had 1.6 vertebral segments. Four (80%) of the five patients with NMO or its variant had poor neurological prognosis versus only one (5%) of non-recurrent ATM patients. IRTM patients had advanced mean onset age, 62 years vs. 43 years for non-recurrent ATM patients. In IIDD patients presenting with ATM as first attack of neuroinflammation, NMO and its variant (56%) were most frequent, then IRTM (33%), with classical MS (11%) the rarest. As long-term treatments for NMO are different from MS, early recognition of NMO and its variant is important for prevention of serious neurological deficits.

An epidemiological study of motor neuron disease in Hong Kong.

Worldwide, the incidence of motor neuron disease (MND) has been increasing steadily over recent decades. We reported a follow-up epidemiology study of MND in this locality. We identified the subjects from the computer database of the government hospital system between 1 January 1997 and 31 January 2002 by searching the ICD code starting from 335.xx. Every retrieved case or their records were reviewed and validated by neurologist(s) of the responsible regional hospitals which the patients attended. One hundred and twenty cases from seven regional hospitals (serving 48.05% of the HKSAR population) were identified, validated and confirmed to be MND or related diseases. Ninety-eight new cases were diagnosed during the study period. Average age of onset was 58.76 years; SD 14.12 (28-89) years. Male to female ratio was 1.72:1. Peak age of onset was 60-64 years without sex difference. The adjusted incidence rate was 0.60/100,000/year. The adjusted point prevalence at the prevalence date (31 January 2001) was 3.04/100,000. Despite the incidence and prevalence of MND among Hong Kong Chinese, it remained low compared to worldwide figures, and our data suggested a significant rise of MND or related disease in the last decade. A territory-wide prospective epidemiological study is indicated.

Association of novel single nucleotide polymorphisms in the calcium channel alpha 1 subunit gene (Ca(v)1.1) and thyrotoxic periodic paralysis.

Thyrotoxic (hypokalemic) periodic paralysis (TPP) is a frequent complication of thyrotoxicosis among Chinese men. To determine the genetic association of TPP, we studied 97 male TPP patients, 77 Graves' disease patients without TPP, and 100 normal male subjects. Mutations of the voltage-dependent calcium channel (Ca(v)1.1), sodium channel (Na(v)1.4), and potassium channel (K(v)3.4), and association of the microsatellite markers on chromosome 1 in the region of the Na/K-ATPase subunits alpha1, alpha2, and beta1 were studied. None of the TPP patients carried the known mutations in Ca(v)1.1, Na(v)1.4, and K(v)3.4 genes. There was no association of TPP with the microsatellite markers that mapped to 1p13, 1q21-23, and 1q22-25. We detected 12 single nucleotide polymorphisms (SNPs) in Ca(v)1.1 in our population, of which three were novel. Significant differences in the SNP genotype distribution between TPP compared with Graves' disease controls and normal controls were seen at the 5' flanking region nucleotide (nt) -476 (P = 0.02), intron 2 nt 57 (P < 0.01), and intron 26 nt 67 (P < 0.001). Because these SNPs lie at or near the thyroid hormone responsive element, it is possible that they may affect the binding affinity of the thyroid hormone responsive element and modulate the stimulation of thyroid hormone on the Ca(v)1.1 gene.

Body part asymmetry in partial seizure.

Clinically differentiating between localisation related and generalised epilepsy is important because it carries significant implications for planning diagnostic management strategy. Asymmetry of body parts such as toes, popliteal crease levels, thumbs, cubital crease levels, and forehead and facial structures, are common in patients with localisation related epilepsy syndromes. We retrospectively studied 337 patients with seizure disorders. Body part asymmetry was routinely documented. Fifty-six were excluded because of non-epileptic seizures, pure psychiatric disorders, non-epileptic neurological disorders, brain tumours and strokes. The relationship between clinically detectable body asymmetry (BA) and the electro-anatomic characteristics of their epilepsy was explored. Body asymmetry was found in 88 out of 282 cases, in which 64 (73.5%) suffered from localisation related epilepsy. Among localisation related epilepsy, BA were found in 41.5% (n=64/154) of patients. In contrast, only 18.75% (n=24/128) of patients with generalised seizure disorders showed similar findings (P<0.0001). Among patients with partial onset seizures, lateralisation of BA was concordant with their seizure origin in 75.9% (n=41/54) and discordant in 24.1% (n=13/54). Investigation results of 10 partial epilepsy cases were non-lateralising at the time of study. Peak age of onset of concordant case was 0-5 years old while discordant group was 6-15 years old. We conclude that BA in patients with seizure disorder is a useful clue to diagnosis of localisation related seizure and may provide clues for lateralising seizure origin in partial onset seizures.

A prevalence study of epilepsy in Hong Kong.

OBJECTIVES: To examine epidemiological data on epilepsy for the Hong Kong west region. DESIGN. Descriptive study. SETTING: Epilepsy clinic, university teaching hospital, Hong Kong. PATIENTS AND METHODS: The epilepsy clinic of Queen Mary Hospital manages the majority of adult patients (aged 15 years or older) with chronic seizure disorders resident in the Hong Kong west area with an adult population of 475,900. All patients underwent electroencephalography examination and each subject was independently assessed by two epileptologists for diagnosis and classified according to the International League Against Epilepsy recommendations. RESULTS: Seven hundred and thirty-six patients (female, 42.9%; male, 57.1%; mean age, 40.8 years; standard deviation, 13.6 years) with epilepsy were enrolled in the study. The prevalence rate of active epilepsy in the population 15 years or older was estimated at 1.54 per 1000 on 1 January 2002. Two hundred and eighty-five (38.7%) patients had idiopathic epilepsy syndromes, 100 (13.6%) had cryptogenic epilepsy, and 285 (38.7%) had a remote symptomatic aetiology. Seizure type was partial in 408 (55.4%) patients and generalised in 285 (38.7%). Thirty-one (4.2%) patients had a positive family history. Idiopathic generalised epilepsy syndromes described as common in the literature, such as juvenile myoclonic epilepsy and childhood absence epilepsy, were infrequently seen at 0.68% and 0.95% of cases, respectively. CONCLUSIONS: This study provides baseline data for epilepsy service development and research in Hong Kong. The prevalence rate of active epilepsy in this Chinese, adult population was low compared with that reported in other developed countries. Further population-based epidemiological research is indicated to confirm the prevalence of seizure disorders in this locality.

Lateral temporal hyperperfusion in postictal psychosis assessed by 99mTc-HMPAO SPECT.

Postictal psychosis is a rare complication of epileptic seizure characterized by reversible psychotic symptoms after flurries of seizure attack. It has been attributed to a phenomenon similar to Todd's paralysis without definitive proof. We studied regional cerebral blood flow (rCBF) of six patients with postictal psychosis by (99m)Tc-HMPAO SPECT scan. Baseline rCBF was compared with the rCBF during postictal psychosis. An asymmetry index (ASI) was calculated as 200 x [(ipsilateral ROI count density - contralateral ROI count density)/(ipsilateral ROI count density + contralateral ROI count density)] %. Significant differences could be found between ASIs during postictal psychosis and interictal state SPECT scan over the lateral temporal neocortex region (P = 0.017). Although hyperperfusion abnormality in SPECT can be found in Todd's paralysis, such findings are more commonly found in cerebral hyperactivity conditions. Taking into account the clinical characteristics of postictal psychosis, namely a preceding lucid interval and crescendo-decrescendo clinical course, these may be an alternative psychopathogenic mechanism for the development of postictal psychosis.