Status epilepticus in Auckland, New Zealand: Incidence, etiology, and outcomes.

OBJECTIVE: To determine the incidence, etiology, and outcome of status epilepticus (SE) in Auckland, New Zealand, using the latest International League Against Epilepsy (ILAE) SE semiological classification. METHODS: We prospectively identified patients presenting to the public or major private hospitals in Auckland (population = 1.61 million) between April 6, 2015 and April 5, 2016 with a seizure lasting 10 minutes or longer, with retrospective review to confirm completeness of data capture. Information was recorded in the EpiNet database. RESULTS: A total of 477 episodes of SE occurred in 367 patients. Fifty-one percent of patients were aged <15 years. SE with prominent motor symptoms comprised 81% of episodes (387/477). Eighty-four episodes (18%) were nonconvulsive SE. Four hundred fifty episodes occurred in 345 patients who were resident in Auckland. The age-adjusted incidence of 10-minute SE episodes and patients was 29.25 (95% confidence interval [CI] = 27.34-31.27) and 22.22 (95% CI = 20.57-23.99)/100 000/year, respectively. SE lasted 30 minutes or longer in 250 (56%) episodes; age-adjusted incidence was 15.95 (95% CI = 14.56-17.45) SE episodes/100 000/year and 12.92 (95% CI = 11.67-14.27) patients/100 000/year. Age-adjusted incidence (10-minute SE) was 25.54 (95% CI = 23.06-28.24) patients/100 000/year for males and 19.07 (95% CI = 16.91-21.46) patients/100 000/year for females. The age-adjusted incidence of 10-minute SE was higher in Maori (29.31 [95% CI = 23.52-37.14]/100 000/year) and Pacific Islanders (26.55 [95% CI = 22.05-31.99]/100 000/year) than in patients of European (19.13 [95% CI = 17.09-21.37]/100 000/year) or Asian/other descent (17.76 [95% CI = 14.73-21.38]/100 000/year). Seventeen of 367 patients in the study died within 30 days of the episode of SE; 30-day mortality was 4.6%. SIGNIFICANCE: In this population-based study, incidence and mortality of SE in Auckland lie in the lower range when compared to North America and Europe. For pragmatic reasons, we only included convulsive SE if episodes lasted 10 minutes or longer, although the 2015 ILAE SE classification was otherwise practical and easy to use.

EpiNet study of incidence of status epilepticus in Auckland, New Zealand: Methods and preliminary results.

The EpiNet project has been commenced to facilitate investigator-led collaborative research in epilepsy. A new Web-based data collection tool has been developed within EpiNet to record comprehensive data regarding status epilepticus and has been used for a study of status epilepticus in Auckland, New Zealand. All patients aged >4 weeks who presented to any of the five public hospitals and the major private hospital within Auckland city (population = 1.61 million) with an episode of status epilepticus between April 6, 2015 and April 5, 2016 were identified using multiple overlapping sources of information. For this study, status epilepticus was defined as any seizure exceeding 10 minutes in duration, or repeated seizures lasting >10 minutes without recovery between seizures. Patients who had either convulsive or nonconvulsive status epilepticus were included. Episodes of status epilepticus were classified according to the 2015 International League Against Epilepsy ILAE status epilepticus classification. A total of 477 episodes in 367 patients were considered as definite or probable status epilepticus; 285 episodes (62%) lasted >30 minutes, which is the duration that has previously been used for epidemiological studies of status epilepticus.

EpiNet as a way of involving more physicians and patients in epilepsy research: Validation study and accreditation process.

OBJECTIVE: EpiNet was established to encourage epilepsy research. EpiNet is used for multicenter cohort studies and investigator-led trials. Physicians must be accredited to recruit patients into trials. Here, we describe the accreditation process for the EpiNet-First trials. METHODS: Physicians with an interest in epilepsy were invited to assess 30 case scenarios to determine the following: whether patients have epilepsy; the nature of the seizures (generalized, focal); and the etiology. Information was presented in two steps for 23 cases. The EpiNet steering committee determined that 21 cases had epilepsy. The steering committee determined by consensus which responses were acceptable for each case. We chose a subset of 18 cases to accredit investigators for the EpiNet-First trials. We initially focused on 12 cases; to be accredited, investigators could not diagnose epilepsy in any case that the steering committee determined did not have epilepsy. If investigators were not accredited after assessing 12 cases, 6 further cases were considered. When assessing the 18 cases, investigators could be accredited if they diagnosed one of six nonepilepsy patients as having possible epilepsy but could make no other false-positive errors and could make only one error regarding seizure classification. RESULTS: Between December 2013 and December 2014, 189 physicians assessed the 30 cases. Agreement with the steering committee regarding the diagnosis at step 1 ranged from 47% to 100%, and improved when information regarding tests was provided at step 2. One hundred five of the 189 physicians (55%) were accredited for the EpiNet-First trials. The kappa value for diagnosis of epilepsy across all 30 cases for accredited physicians was 0.70. SIGNIFICANCE: We have established criteria for accrediting physicians using EpiNet. New investigators can be accredited by assessing 18 case scenarios. We encourage physicians with an interest in epilepsy to become EpiNet-accredited and to participate in these investigator-led clinical trials.

Autonomic dysfunction is a major feature of cerebellar ataxia, neuropathy, vestibular areflexia 'CANVAS' syndrome.

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a recently recognized neurodegenerative ganglionopathy. Prompted by the presence of symptomatic postural hypotension in two patients with CANVAS, we hypothesized that autonomic dysfunction may be an associated feature of the syndrome. We assessed symptoms of autonomic dysfunction and performed autonomic nervous system testing among 26 patients from New Zealand. After excluding three patients with diabetes mellitus, 83% had evidence of autonomic dysfunction; all patients had at least one autonomic symptom and 91% had more than two symptoms. We also found a higher rate of downbeat nystagmus (65%) than previously described in CANVAS. We confirmed that sensory findings on nerve conduction tests were consistent with a sensory ganglionopathy and describe two patients with loss of trigeminal sensation consistent with previous pathological descriptions of trigeminal sensory ganglionopathy. Our results suggest that autonomic dysfunction is a major feature of CANVAS. This has implications for the management of patients with CANVAS as the autonomic symptoms may be amenable to treatment. The findings also provide an important differential diagnosis from multiple system atrophy for patients who present with ataxia and autonomic failure.

Using the Internet to recruit patients for epilepsy trials: results of a New Zealand pilot study.

PURPOSE: We created a database that could be accessed via the Internet by any neurologist or pediatric neurologist in New Zealand. The database was designed to facilitate recruitment of patients for investigator-driven drug trials. METHODS: We established an epilepsy database, and invited neurologists and pediatric neurologists throughout New Zealand to register patients via the Internet when they were uncertain of the optimal management. Details regarding seizure type and frequency, epilepsy syndrome, etiology, drug history, and investigations were collected. We produced an algorithm to select patients who had failed to respond to a single antiepileptic drug (AED). These patients were randomized immediately via the Internet to receive a different drug. Participants were not reimbursed. RESULTS: The pilot study recruited patients from mid-June to December 2007. Sixteen neurologists participated; neurologists were based in eight different cities. One hundred thirty-seven patients were registered, of whom 113 were considered suitable for drug trials. Thirty-five patients who had used a single antiepileptic drug AED were enrolled, and 14 of these were successfully randomized online to a different drug. Follow-up information was entered via the Internet for all 108 patients who were seen again during the following year. DISCUSSION: We have demonstrated that patients can be recruited for trials and randomized from routine clinics via the Internet. Trials could compare AEDs or look at other aspects of epilepsy management. An international pilot study is planned. Neurologists are invited to enroll patients with epilepsy, who would be suitable for randomized controlled trials, into a Web-based register.

Temporal lobe resection for refractory temporal lobe epilepsy at Auckland Hospital.

AIMS: To analyse the long-term outcome of patients who underwent temporal lobe resection for intractable temporal lobe epilepsy at Auckland Hospital. METHODS: We performed a retrospective analysis of 176 patients who underwent temporal lobe resection at Auckland Hospital, New Zealand between 1987 and 2007. We had at least 1 year of follow-up on 174 patients. RESULTS: Overall 98/174 (56%) individuals were seizure-free at 1 year (Engel Class 1) with a marked improvement in quality of life. A further 61/174 (35%) had rare seizures or had significant improvement in seizure frequency (Engel Classes II or III). At last follow up (mean 4.3 years) 95/174 (55%) were seizure-free (Engel Class 1). Hippocampal sclerosis was the pathological finding in 129 patients. Surgical complications included 2 (1.1%) deaths, while 6 (3.4%) patients had symptomatic visual field defects, and 8 (4.5%) had other permanent neurological problems. A further 18 (10.3%) patients had temporary complications including infection, pulmonary embolus, and aseptic meningitis. New psychological symptoms occurred during the first year after surgery in 52% of 114 patients for whom we had detailed psychiatric assessments. CONCLUSION: Temporal lobe resection is effective in controlling medically intractable seizures, but there are potentially serious complications that need to be considered when counselling patients for such a procedure.

Bringing epilepsy out of the shadows in New Zealand.

Epilepsy is a common neurological disorder. Most patients with epilepsy have seizures that are relatively easily controlled, but a significant minority of patients have seizures that are resistant to standard anti-epileptic drugs. The New Zealand chapter of the International League against epilepsy (NZLAE) has recently been formed to improve the care of patients with epilepsy in New Zealand. NZLAE intends to work with the lay group, Epilepsy New Zealand to help raise the profile of epilepsy within the general community, and to improve access to some of the newer drugs that are not currently available here. NZLAE is also keen to promote research into epilepsy in New Zealand, and members have recently started recruiting patients for several research projects.