1. Diagnostic Performance Of Nerve Ultrasound In CANVAS Neuronopathy, Dr Ruth Leadbetter
Introduction: Patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) have neuronopathy and pathologically small nerves on ultrasound. We evaluated the diagnostic accuracy of nerve ultrasound in CANVAS, using receiver operating characteristic (ROC) curves. Proposed diagnostic cut-off values were tested on another group of patients with neuronopathy with spinocerebellar ataxia type 2 (SCA2).
Methods: Logistic regression and ROC curves used data from 14 CANVAS patients and 42 healthy controls and diagnostic cut-off values were then tested on seven SCA2 patients with non-length dependent neuropathy.
Results: Nerve ultrasound showed excellent discrimination between patients with CANVAS and healthy controls at the upper limb sites with areas under the ROC curves between 0.97 and 0.99. Lower limb sites had moderate discrimination. Proposed CANVAS diagnostic cut-off measurements performed well when applied to the SCA2 patients.
Discussion: Nerve ultrasound is a sensitive diagnostic tool for neuronopathy in CANVAS and potentially other spinocerebellar ataxias.
2. Clinical Features Of Neuromyelitis Optica Spectrum Disorders With Myelin Oligodendrocyte Glycoprotein Or Aquaporin-4 Antibody: A Systematic Review, Dr Benson Chen
Abstract: Neuromyelitis optica (NMO) is a rare immune-mediated disorder of the central nervous system, characterised by attacks of inflammatory demyelination predominantly targeting the optic nerves and spinal cord. The spectrum of NMO disorders (NMOSD) includes isolated optic neuritis and longitudinally transverse myelitis. The pathogenesis of NMOSD relates to humoral mechanisms, with an identified disease-specific autoantibody, targeting the aquaporin-4 (AQP4) water channel. Antibodies targeting the myelin oligodendrocyte glycoprotein (MOG) have been identified in some patients with AQP4-antibody negative NMOSD. This study reviewed the features of patients with NMOSD and compared patients who were AQP4-antibody positive and those who were MOG-antibody positive. A total of 109 studies met inclusion criteria, including 38 studies describing MOG patients and 95 studies describing AQP4 patients. Data on a number of clinical and paraclinical features was collected including: patient demographics; disease presentation; findings from neuroimaging, neurophysiological, and ne uropathological studies; findings from
cerebrospinal fluid examination and optical coherence tomography; disease duration,
course and disability; and lastly treatment and treatment response. Although both MOG and
AQP-antibody positive patients present with similar clinical phenotypes, there were
differences in many of the clinical and paraclinical features. These differences reflect the fact that the underlying pathophysiology in MOG and AQP4 disease is different and therefore they should be considered separate entities.
3. The emerging picture of Wartenberg’s migrant sensory neuritis: New Zealand Experience, Dr Zoe Dyer
Background: Wartenberg’s migrant sensory neuritis is a controversial condition. Existing literature describes patients with successive involvement of multiple cutaneous nerves resulting in episodic sensory symptoms, often provoked by stretch.
Aim: To further characterise Wartenberg’s neuritis in a cohort of patients seen at Auckland Hospital.
Method: Retrospective case series. Inclusion criteria: sequential development of multiple sensory mononeuropathies over time. Patients with motor involvement or systemic disease were excluded.
Results: 12 patients were included. At the time of presentation the mean duration of symptoms was 8 months and the number of discrete sensory nerves involved ranged from 2 to 9 (mean 4.6). In six patients symptoms were notably provoked by stretch or irritation. Nerve conduction studies are available for 9 patients. Six of these demonstrate patchy sensory nerve involvement with an axonal pattern.
One patient’s condition evolved to include severe impairment of proprioception prompting treatment with immunosuppressive therapy. All other patients experienced a benign course without emergence of an underlying secondary cause or progression to generalised neuropathy. None of this group received immunosuppressive medication.
Conclusion: These data are consistent with existing descriptions of Wartenberg’s neuritis. Further investigation of this condition may be helpful in guiding diagnosis and management of patients with otherwise unexplained sensory symptoms.
4. The New Zealand Creutzfeldt-Jakob disease registry: a 20-year review, Dr Benson Chen
Introduction: Creutzfeldt-Jakob disease (CJD) is a rare, fatal transmissible spongiform encephalopathy (TSE) with an estimated incidence of between one to two cases per million population per year in New Zealand. CJD and other TSEs have been notifiable diseases in New Zealand since 1996. We analysed the findings from the New Zealand CJD registry (NZCJDR) over its first 20 years of existence.
Methods: All notifications to the New Zealand CJD registry from January 1997 until December 2016 were examined. All available clinical, radiological, biochemical and pathology results on notified cases were reviewed
Results: During the first 20 years of the NZCJDR, 119 cases of suspected CJD were notified. Of these, 93 cases (82%) fulfilled diagnostic criteria for definite or probable CJD, 6 (5%) met criteria for possible CJD and the remaining 20 were deemed not to be CJD.
Discussion: These figures suggest that the incidence of CJD in New Zealand is similar to that in other developed countries. The absence of vCJD cases is reassuring, but continued vigilance remains necessary.
5. Comparative Audit of Levetiracetam, Sodium Valproate and Lamotrigine within the Auckland Area; Details from the EpiNet Database, Dr Mary Brennan
Objectives: To perform a retrospective audit of patients in the Auckland EpiNet Database who have been prescribed levetiracetam, sodium valproate and lamotrigine at any point.
Methods: The records of all patients from Auckland in the EpiNet Database who have been prescribed levetiracetam, sodium valproate and lamotrigine at any point are being reviewed. We aim to identify who is still taking these medications and why patients have stopped their medication.
Results: Levetiracetam, sodium valproate and lamotrigine have each been prescribed to over 1,000 patients. Patients were excluded if they were from out of Auckland, were participating in randomised controlled trials, or had only started the medication in the past three months.
With regard to Levetiracetam 500 patients (56%) remain on it and 385 (44%) are no longer taking it. Of those who stopped Levetiracetam 183 patients experienced side effects, and efficacy was sub optimal in 150 patients; 46 patients (5%) were advised to stop the drug as it was considered no longer necessary. 119 patients (12%) had to stop the medication due to a mood disorder.
We are currently in the process of reviewing sodium valproate and lamotrigine, using the same methods as our levetiracetam review.
Conclusion: Of the patients in the EpiNet Database who have taken levetiracetam, 56% remain on this medication up until July 2018. Side effects were a more common reason for stopping Levetiracetam than lack of efficacy. A significant minority of patients commenced on levetiracetam developed a mood disturbance or behaviour change. We look forward to presenting comparative data of both sodium valproate and lamotrigine.
6. Assessment of the cause of acute transient vestibular syndrome, Dr Duncan Smyth
Objectives: In the majority of cases of acute transient vestibular syndrome (ATVS), the diagnosis is unknown. The aim of this study was to see whether adequate investigation can define the cause of ATVS. We present the interim results of the study.
Methods: Patients presenting with acute transient vestibular syndrome (symptoms lasting 1-24 hours) were recruited from Wellington Hospital Emergency Department. Patients with known vestibular disorders or with an obvious cause on examination, including abnormal video oculography of the horizontal semi-circular canal were excluded. Patients underwent a complete neurological and oculomotor examination, subjective visual vertical, video-oculography of horizontal and vertical canals, bithermal calorics, cervical vestibular evoked myogenic potentials and MRI brain with diffusion weighted imaging.
Results: Eleven patients were included in the study. Five patients (45%) had significant abnormalities on at least one vestibular test, although the exact vestibular disorder was not able to be defined. No patients had evidence for cerebral ischaemia.
Conclusion: Extensive vestibular investigation may establish a peripheral vestibular cause in approximately half of patients with ATVS, although the exact nature of the presumed peripheral disorder remains unknown.
1. Long term outcome of 200 patients referred to a First Seizure Clinic, Dr Eileen McManus
Aim: 1) To determine what proportion of our first seizure referrals reflected true unprovoked first seizures or epilepsy, 2) To assess the diagnostic accuracy of our First Seizure Clinic by quantify risk of subsequent seizures in our First Seizure Clinic cohort.
Methods: We retrospectively reviewed the medical records of 200 patients referred to the first seizure clinic between May 2014 and December 2015, with review of clinical notes and telephone follow up at 28 months post diagnosis.
Results: Of the 200 patients referred to the first seizure clinic, 181 attended. At the initial assessment 22% (n=39) of these patients were diagnosed with epilepsy, with most of these patients (59%) found to have a history of previous seizures. 28% (n=50) were diagnosed with a first seizure, of which 28% were labelled as provoked seizures. 38% (n=69) of the patients received another diagnosis (syncope, NEAD, migraine or parasomnia) and 13% (n=23) were labelled as indeterminable. At 28 months follow up, 22% (n=11) of patients who received a diagnosis of first seizure subsequently received a diagnosis of epilepsy. In the remaining groups only 5 (5%) patients were diagnosed with epilepsy (of these 3 were in the indeterminable group).
Conclusions: Our study shows that approximately half of the patients referred to a First Seizure Clinic had not experienced a seizure but were given an alternative diagnosis. Secondly, our study indicates that the risk of seizure reoccurrence following a first seizure is quite low (22%). However, we believe this because a substantial proportion of the patients (22%) were diagnosed with epilepsy already at the first assessment. The high proportion of patients being diagnosed with epilepsy was mainly due to a history of previous seizures. Thirdly, patients who were given an alternative diagnosis at the first assessment had a low probability (5%) for seizure recurrence.
2. A case of atypical parkinsonism with DCTN1 mutation, Dr Eileen McManus
Abstract: A patient with parkinsonism, low mood and syncope was initially diagnosed as psychogenic parkinsonism until a family history of central hypoventilation syndrome lead to the consideration of Perry Syndrome. A mutation in the dynactin 1 (DCTN1) gene confirmed the diagnosis. Perry syndrome is an atypical parkinsonism characterised by depression, apathy, weight loss and central hypoventilation. Perry syndrome should be considered in the differential diagnosis of parkinsonism especially if there is family history.
3. Nivolumab Induced Autoimmune Encephalitis, Dr Erin Coleman
Abstract: A 67 year old male with a completely resected stage IV melanoma was entered into a clinical trial of adjuvant nivolumab, an anti-PD1 monoclonal antibody. He developed autoimmune side effects of hyperthyroidism, pneumonitis, rash and hypoadrenalism. Two months into his immunotherapy he developed fatigue and an inability to perform his work. Examination revealed confusion and a MMSE was 22/30. CSF examination showed 6 WBC but was otherwise normal. An MRI was normal. An EEG demonstrated a mild encephalopathic pattern. He was diagnosed with autoimmune encephalitis. The trial drug was stopped and he was started on IV followed by oral steroids. He remained in a period of rehabilitation for 6 months and has now returned to work and driving. Autoimmune encephalitis is a rare but serious side effect from immune checkpoint inhibitors. With increasing use of this class of medications, early recognition and prompt treatment is essential.
4. Review of First Line Anti-Epileptic Drug Prescribing in the Auckland Area as recorded in the EpiNet database, Dr Mary Brennan
Objectives: To perform a retrospective audit of first anti-epileptic drug prescribing in the Auckland area as recorded in the EpiNet database, and in particular to look at
Methods: Records were reviewed for all patients from Auckland in the first anti-epileptic drug database in EpiNet. We looked at which anti-epileptic drug each patient was commenced on. For patients started on levetiracetam, lamotrigine and sodium valproate, we reviewed patients’ dispensing records to ascertain if they were still taking the medication. If no longer taking the prescribed medication, we tried to identify why this was the case.
Results: There were 257 patients in total on the EpiNet first anti-epileptic database. Levetiracetam was the most widely prescribed first line anti-epileptic, with 51% of patients commenced on this drug. Lamotrigine (27%) and Sodium Valproate (12%) were the next most popular first line anti-epileptics. Further review of levetiracetam, lamotrigine and sodium valproate showed that 66%, 73% and 63% respectively remained on these medications. The most common reason for stopping all three medications was side effects. Of the patients who stopped levetiracetam due to side effects, 70% (12 patients in total) did so because of a mood disorder; none of the patients who stopped lamotrigine or valproate did so for mood disorders
Conclusions: Levetiracetam is the most widely prescribed first line anti-epileptic within the Auckland area. Of the three medications reviewed, patients prescribed lamotrigine, were more likely to continue taking it. People who stop levetiracetam often do so because of a mood disorder. The numbers in this database are relatively small. Further research, with larger numbers is needed.
5. Vertebral artery aneurysms: 12 year review of outcome following endosurgical management in a single neurosurgical centre
Dr Samuel Rajadurai
Introduction: Vertebral Artery – Posterior Inferior Cerebellar Artery (VA- PICA) aneurysms are uncommon. There is no definitive treatment strategy for these aneurysms. We present our single centre experience in the management of these aneurysms both ruptured and unruptured. Vertebral artery (VA) aneurysms are mostly dissecting aneurysms and are extracranial or intracranial in location.
Aim: The objective of this study is to determine the outcome of management of these aneurysms.
Methods: This retrospective study was conducted by the neurosurgical department at Waikato Hospital, New Zealand. Inclusion criteria included all patients with VA- PICA aneurysms admitted to the unit between January 2007 and July 2018. Distal PICA aneurysms or feeding artery PICA aneurysm associated with an arteriovenous malformation (AVM) were excluded.
Various Treatment strategies were used including surgical trapping, internal coil trapping of diseased segment, proximal parent vessel occlusion and stenting with or without coiling. One patient refused treatment. A total of 29 patients were analysed using data obtained from pre and post-operative radiological imaging, clinical notes, discharge summaries and operation reports.
Results: In our study, the mean age of presentation was 54.03 years (range 37 to 77). Females were more commonly affected than males (16 vs 13). Subarachnoid haemorrhage (SAH) was seen in 62.07% of patients (18/29) and Intraventricular haemorrhage (IVH) in 48.24% of patients (14/29). The majority of VA dissection aneurysm was found in segment V4 and the remainder in segment V3. Most patients had endovascular procedures with the majority 92.30% (24/26) having no complications from the endovascular procedure itself and 7.70% (2/27) that had iatrogenic V2 segment injuries with no major sequelae. Mortalities accounted for 24.14% (7/29) in our studied population with 6 of these deaths being associated with WFNS V SAH and IVH and 1 death completely unrelated to the VA- PICA aneurysms.
Conclusion: Endovascular treatment is a safer choice than open surgery due to the minimalistic approach with less morbidity and mortality. The type of endovascular approach is tailored to the individual morphology of the aneurysm and the collateral circulation. High morbidity was related to the grade of SAH.
6. A rare case of extensive pulmonary metastases 13 years after initial resection of intracranial meningioma
Dr Samuel Rajadurai
Background: Extracranial metastases from an intracranial meningioma is a very rare entity. A current literature review reveals only few cases with extensive pulmonary involvement more than 10 years after initial intracranial meningioma resection. Only less than 0.1% of meningioma’s metastasise. Diagnosis of pulmonary meningioma is often confirmed by CT chest guided core biopsies or if feasible, surgical intervention such as video-assisted thoracic surgery (VATS) pulmonary wedge resection with intraoperative histological examination. The prognosis of extensive metastatic pulmonary meningioma however is unknown and there is no gold standard treatment option.
Case Description: The authors present a case of multiple pulmonary meningioma metastases developing 13 years after initial resection of left occipital parafalcine World Health Organisation (WHO) Grade I intracranial meningioma.
Conclusion: There are no established guidelines for the optimal management or surveillance of extensive pulmonary metastatic meningioma. Metastatic pulmonary meningioma although rare is a differential diagnosis to be considered in patients with multiple cannon ball lung lesions, especially when there is a history of recurrent or higher grade meningioma. Metastatic meningioma may occur even after a decade from initial tumour resection.
7. Treatment of Vertebral-Venous Fistula with flow diverting stent – A new endovascular approach
Dr Samuel Rajadurai
Background: Vertebral-venous fistulas (VVF) are a rare vascular condition and are commonly associated with trauma, but may also be idiopathic, spontaneous or congenital. A current literature review reveals there is no level 1 evidence or established guidelines on optimal treatment for VVF. Diagnosis is often confirmed by catheter cerebral angiogram. Treatment goals are complete occlusion of the fistula, which can be achieved by both endovascular closure and/or surgical ligation treatment.
Telescopic Pipeline flow diversion stents are a common treatment modality for aneurysms and have also been described to successfully treat different types of cranial arteriovenous fistulas (AVF) including carotid-cavernous fistulas (CCF). There is however no English literature available to regarding its use in the management of vertebral-venous fistulas (VVF).
Case Description: The authors present a case of VVF, which is the first reported case to their knowledge whereby a Pipeline flow diverting stent has been used to successfully treat a VVF, whilst preserving the parent vertebral artery. The authors describe both the advantages and limitations of this approach.
Conclusion: There are no established guidelines for best treatment of VVF. This case highlights the potential and favourable clinical outcomes of flow diversion stents in the treatment of VVF. Flow diverting stent is shown to be a safe and effective method of treatment for VVF whilst preserving the parent vertebral artery although limitations need to be considered.
8. An Unusual Case Of SMART Syndrome 12 Years Post RadiotherapyDr Rakesh Biju
Abstract: A 28-year-old male presented to our emergency department with a gradual onset severe headache, right sided hemiparesis and a general tonic clonic seizure. Examination revealed a slightly disorientated and lethargic gentleman with a MRC power grade of 2/5 globally in his right lower limb and 1/5 in his right upper limb. Apart from his longstanding decreased visual acuity in the right eye, the rest of his cranial nerves examined unremarkably. A CT brain was done to rule out acute pathology and this revealed nothing of concern. Presumed to be a severe migraine accompanied by Todd’s paresis post seizure, he was commenced on analgesia and admitted under the general physicians.
At age 16, he had a primitive neuroectodermal tumor (PNET) resected from his left occipital lobe followed by targeted radiotherapy to that region. At that point, it was presumed to be terminal disease, but progression did not occur, and planned palliation terminated.
An MRI was done during his inpatient stay and a diagnosis of SMART (Stroke-like Migraine Attacks after Radiation Therapy) syndrome was made.
He was treated conservatively, and his symptoms spontaneously resolved.
SMART syndrome is a rare clinical entity which is a delayed complication of radiation therapy; in one case series, patients presented with symptoms 6 to 30 years after treatment. MRI is the gold standard for imaging and diagnosis. Most often, it is self-limiting, resolving over the course of a few weeks.
9. Risk factors, aetiology and outcome of young ischemic stroke: a Canterbury audit. Dr Sarah Wright
Abstract: Ischaemic stroke in young adults is increasingly recognized as a serious health concern and is increasing in prevalence. The aetiology of stroke in young adults differs from an older population. This study aimed to investigate the aetiology and risk factors in young ischemic stroke in Canterbury, New Zealand. We conducted a retrospective audit of young ischaemic stroke patients aged 16-50 years. Baseline demographic data, risk factors, aetiology and severity were assessed. Baseline and discharge functional neurological outcome were recorded (modified Rankin Scale, mRS). In 186 patients (62% men) with ischaemic stroke, the median age was 43 years (19-49 years) and median NIHSS on admission was 4 (range 0-26). The largest demographic group was classified as European (76%), followed by NZ Maori (10%). Cardio-embolic aetiology (31%) was the most common, and aetiology remained unknown in 27% of patients. Nine patients underwent acute stroke revascularization therapy. The neurological outcome was unfavorable in more than 32% of patients (consisting of a mRS greater than 3). In conclusion, there is a lost opportunity to prevent ischaemic strokes in young adults as modifiable vascular risk factors were present in more than 40% of patients which leads to considerable morbidity.
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