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Background/ObjectivesTrainee medical officers (TMOs) applying for entrance into medical and surgical training programmes within Australia are initially assessed on their curriculum vitae (CV). Previous research reports that 14/47 training programmes publish publicly available standardised CV scoring criteria. Allocating points towards rural exposure (including during formative years, medical school, and postgraduate placement) is vital to ensuring the diversity of successful applicants and for endorsing fellows of the college to continue practicing in underserviced areas. The objective of this study is to determine the relative weighting or rural exposure within publicly available standardised CV scoring criteria.MethodsAll Australian and New Zealand medical and surgical specialties, outlined by the Australian Medical Association, were included, and their college, society, and faculty websites were searched for publicly available standardised CV scoring criteria.Results8/14 specialty training programmes allocate points towards rural exposure within standardised CV scoring criteria, with a mean weighting of 13.7% The ANZAN training programme allocates zero points towards rural exposure. Contrarily, the RANZCO, RANZCOG and CICM training programmes allocate the three highest weighting towards rural exposure: 28.5%, 24.6% and 14% respectively.Conclusion/DiscussionThe relative weighting of rural exposure within the ANZAN training programme is significantly less than other specialty colleges. The deliberate and strategic construction of these CV criteria may have significant implications for the future medial rural workforce in Australia. Future development of standardised CV scoring criteria should consider point allocation towards rural exposure and related activities.

BackgroundLateral medullary infarction classically results in the ‘Wallenberg syndrome’, characterised by an ipsilateral Horner’s syndrome, loss of pain and temperature in the ipsilateral face and contralateral body, vertigo, dysphagia and dysphonia. Contralateral motor weakness is uncommon but can occur with caudal extension in the ‘Babinski-Nageotte’ syndrome. Isolated, ipsilateral facial weakness is exceedingly rare. Similarly, only a few cases with contralateral facial sensory loss have been described. The presence of these two signs together in lateral medullary infarction has not been reported before.Case PresentationWe present the case of a 47-year-old gentleman presenting with an acute left Horner’s syndrome, left ocular lateropulsion and central nystagmus, in combination with left lower facial weakness sparing the frontalis and reduced pain and temperature sensation in the right face, arm and leg. MRI of the brain revealed diffusion restriction consistent with acute infarction in the left lateral medulla. There was no midbrain or pontine involvement.DiscussionIpsilateral lower facial weakness in lateral medullary infarction is postulated to occur secondary to involvement of an aberrant bundle of corticobulbar fibres that decussate in the medulla before ascending to the contralateral facial nerve nucleus in the pons. Contralateral sensory loss in the face is thought to occur secondary to involvement of the ventral trigeminothalamic tract in the medulla after its decussation.ConclusionOur case demonstrates that lower facial weakness with contralateral sensory loss can occur secondary to lateral medullary infarction. This highlights the complex organisation of the sensorimotor pathways in the brainstem.

BackgroundTrigeminal neuralgia is a pain disorder classically linked to vascular compression of the trigeminal nerve, although a number of other pathological sources of compression may cause the same clinical picture. Understanding the aetiology is an important part of the work-up and guides treatment. Meckel's cave is a cerebrospinal fluid-filled space that contains the trigeminal ganglion and through which the trigeminal nerve runs. Its absence is uncommon, and the aetiology of this is unknown. In recent years two small case series and a few case reports have suggested an association with intractable trigeminal neuralgia. Optimal management, inclusive of neurosurgical options, and prognosis, remains unknown.Case PresentationA 32 year old female was referred to Neurology clinic with symptoms of right-sided trigeminal neuralgia in the V2, V3 branches and on occasion also V1 branch of the trigeminal nerve. There was no absence of sensation on examination. An MRI brain showed an ipsilateral hypoplastic or absent right-sided Meckel's cave. She had been given amitriptyline in the community, followed by low dose carbamazepine which was up-titrated after clinic review. Subsequently she was admitted to hospital with a pain crisis. Despite maximal up-titration of carbamazepine and addition of pregabalin her symptoms were unable to be controlled, so a Neurosurgical opinion was sought.ConclusionIpsilateral absence of Meckel's cave appears to be an emerging cause of trigeminal neuralgia that is often challenging to manage with medication. We present a literature review of this rare entity, alongside the clinical developments in this case.

IntroductionCreutzfeld-Jakob disease (CJD) often presents as a rapidly progressive dementia with prominent cerebellar signs and myoclonus. However, variants are recognised, including a posterior or visual presentation known as the Heidenhain variant.CaseA previously well 70-year-old man presented with a 2-month history of progressive visual and perceptual distortions. Symptoms included intense colour perception (e.g. of green grass), macropsia and micropsia (e.g. of road signs), impaired depth perception (especially when driving), and simultanagnosia (e.g. of cars and Ishihara plates). Formal neuropsychological testing was initially within normal limits. Visual acuity and formal visual field testing were normal. MRI brain performed for unrelated reasons a few weeks prior to symptom onset demonstrated increased T2/FLAIR and DWI signal restricted to the occipital and parietal cortices, mostly on the right. Repeat MRI brain at time of presentation found significant progression of these changes bilaterally. EEG showed intermittent bilateral posterior slowing but no triphasic waves or sharp wave complexes. CSF analysis revealed normal cell counts and biochemistry, along with normal protein 14–3-3 and total tau levels. However, real-time quaking-induced conversion (RT-QuIC) was positive. Sequencing of the PRNP gene did not reveal any variants to suggest familial CJD, but did demonstrate methionine/valine heterozygosity at codon 129.DiscussionThe Heidenhain variant of CJD is rare but distinctive. This case is notable for highlighting radiological changes before symptom onset, limitations of traditional CSF testing, and the usefulness of RT-QuIC. Lastly, methionine/valine heterozygosity at codon 129 contrasts with prior reported cases of the variant, which usually feature methionine homozygosity.

Background/ObjectivesThere are many different causes of hemichorea, including both structural and metabolic abnormalities. We describe a rare but reversible cause of hemichorea.MethodsA case report was written.ResultsAn 86-year-old male experienced recurrent subacute onset progressive left upper and lower limb hemichorea worsening over seven days. Prior to this, he experienced acute onset left upper and lower limb hemichorea two months ago which was attributed to diabetic striatopathy, despite lack of supportive MRI imaging (T1 MRI sequences of the basal ganglia was normal). His hemichorea was partially treatment responsive to antiglycaemic therapy and haloperidol.On examination, there was continuous irregular choreiform movements of the left arm and leg which resolved during sleep. The movements were not distractible. The rest of the neurological examination was grossly normal. His blood glucose levels were normal (7 mmol/L); however, serum magnesium was low (Mg 0.27 mmol/L). His hemichorea improved with magnesium replacement. His severe hypomagnesaemia was probably secondary to longterm proton pump inhibitor therapy for gasto-oesophageal reflux disease.Conclusions/DiscussionThis case demonstrates the importance of evaluating serum electrolytes for patients presenting with acute hemichorea.

Background/Objectives‘Pyramidal weakness’ implies that neurological examination findings can be localized to the central nervous system (CNS). Neurology trainees are taught that ‘pyramidal weakness’ infers upper limb extensor and lower limb flexor weakness. However, other weakness patterns are observed in CNS lesions and are not included in this teaching. We aim to investigate the pattern of weakness observed in CNS lesions and explore the use of the phrase ‘pyramidal weakness’ over time.MethodsWe searched Medline, PubMed, and Google Scholar up to 1/1/2022, using these terms: ‘distal weakness’, ‘upper limb flexion’, ‘lower limb extension’, pyramidal: ‘weakness’, ‘sign’, ‘tract sign’, ‘syndrome’, and sign of pyramidal: ‘lesion’, ‘disease’ and ‘involvement’. The inclusion criteria were papers relating to brain or spinal cord lesions and terms inferring their presence or the description of a motor weakness pattern.ResultsWe identified 117 studies since 1889, of which 29.9% of publications described weakness in upper limb extensors and lower limb flexors, and 26.5% reported distal weakness. We found an early reference to ‘pyramidal weakness’ in 1922; in the context of unilateral weakness in encephalitis with no description of upper limb extensor and lower limb flexor weakness. Since 1988, ‘pyramidal weakness’ has become associated with weakness in upper limb extensors and lower limb flexors. ConclusionThe phrase ‘pyramidal weakness’, used in its current format, is more frequent since the 1980s. Distal weakness and upper limb extensor and lower limb flexor weakness have been associated with CNS lesions. ‘Pyramidal weakness’ should also infer a pattern of distal weakness.

ObjectiveExplore the effects of baseline characteristics on long-term safety/efficacy of avalglucosidase alfa (AVA) in subgroups of patients with late-onset Pompe disease enrolled in COMET (Phase 3; NCT02782741).MethodsCOMET randomised treatment-naïve patients to AVA (n=51) or alglucosidase alfa (ALG; n=49). After a 49-week primary analysis period, all patients from the AVA-arm continued and 44 patients in the ALG-arm switched to AVA (extended treatment period); total duration 145 Weeks. Subgroups and analyses included: 1) the impact of baseline age (≥18–<45y, ≥45y) on the change in 6-minute walk test (6MWT) distance and upright forced vital capacity (FVC) % predicted, 2) the impact of baseline 6MWT (<403.5, ≥403.5m) on the change in FVC % predicted, and 3) the impact of baseline FVC % predicted (<55, ≥55%) on the change in 6MWT distance. Mean estimates (95% CI) were calculated from linear mixed effects models stratified by treatment group.ResultsOverall, the change from baseline to Week 145 was stable or improved for all subgroups analysed for the different outcomes. From baseline to Week 145: younger patients in the AVA-arm had a significant improvement in 6MWT distance (9.8m [4.4,15.1]; p=0.0004) and AVA-arm patients with baseline FVC ≥55% had a significant improvement in 6MWT distance (6.8m [2.4,11.2]; p=0.0026). Changes over time remained stable in all other subgroups with nonsignificant p values.ConclusionsThese data indicate that the positive changes seen during treatment with AVA are not driven by any subgroup and demonstrate that AVA is effective in patients with varying baseline characteristics. Funded: Sanofi.

BackgroundProgressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare but debilitating disease within the stiff-person syndrome (SPS) spectrum and is characterised by muscle rigidity, spasms, myoclonus, dysautonomia and brainstem dysfunction. The exact pathogenetic mechanism is unclear, although there is an association with the presence of glycine receptor antibodies in serum and cerebrospinal fluid, and some cases are paraneoplastic.ResultsHere we report a complex case of paraneoplastic PERM associated with an otherwise subclinical monoclonal B-cell lymphocytosis (MBL) of the non-chronic lymphocytic leukaemia (CLL) phenotype, which may be in turn likely secondary to long-term methotrexate use (i.e. methotrexate-associated lymphoproliferative disorder, MTX-LPD) or underlying autoimmune disease.ConclusionsPERM often has a paraneoplastic aetiology and likely exerts antitumour response against malignancies, thus potentially rendering the primary malignancies indolent or atypical in presentation. This is, to our knowledge, the first reported association between PERM and MBL, or between PERM and MTX-LPD.

Background/ObjectivesFunctional neurological disorder (FND) is a common, disabling, and usually chronic neurological condition. After a long period of neglect, research interest in the area has grown rapidly in recent years. People with FND (pwFND) often express interest in enrolling in any research studies on the condition, and recruitment is frequently a rate limiting factor in studies.The traditional method of recruiting for studies: advertising and relying on prospective participants to make contact, is imperfect. It puts an onus on patients to monitor multiple sources for notification of new studies, meaning that people who might have enrolled in a study may not find out about it, or enrol later than they otherwise might have. Research recruitment databases have been shown to reduce this problem in other conditions.MethodsWe established a research recruitment database, FND Research Connect (www.fnd-research.org) where pwFND can prospectively register to be contacted about studies for which they meet inclusion criteria, and to have the information they provide for screening purposes used in an anonymised form for epidemiological research.ResultsOn a ‘soft’ initial release to Australia only, 284 pwFND enrolled for the database in the first month. One study is already being prepared for publication using data from the database. Worldwide release is planned for early March 2024.Conclusion/DiscussionFND Research Connect appears to provide a meaningful increase in the ease with which pwFND can find studies, and FND researchers can recruit for studies.

BackgroundDot sign- punctate foci of diffusion restriction on MRI DWI (diffusion weighted imaging) has been established as a radiological finding in Transient Global Amnesia (TGA).1 This sign can also be seen in non TGA presentations, and we outline a status epilepticus presentation below.CaseA 66 year old scientist went to sleep at 8pm and woke up his wife at midnight with his generalised tonic clonic seizure like activity lasting minutes. His GCS (Glasgow coma scale) was 7 and he was intubated and ventilated in the regional Hospital Emergency department before being flown to a tertiary hospital Intensive care unit, for further review, and neurological investigations.Patient had no known history of seizures and his past medical history included ischaemic heart disease, undergoing coronary bypass graft in 2020, Hypertension and Type 2 Diabetes Mellitus.He spontaneously recovered and was extubated and back to baseline GCS 15 on the neurological inpatient ward.His MRI Brain demonstrated classic \"dot sign\" in the left hippocampal region.EEG demonstrated diffuse bilateral slowing.Conclusion\"Dot sign\" is an interesting finding, not always related to TGA, and has been shown to be associated with status epilepticus.2 References Park JH, Oh CG, Kim SH, Lee SH, Jang JW. Hippocampal lesions of diffusion weighted magnetic resonance image in patients with headache without symptoms of transient global amnesia. Dement Neurocogn Disord. 2017 Sep;16(3):87–90. Meletti S, Monti G, Mirandola L, Vaudano AE, Giovannini G. Neuroimaging of status epilepticus. Epilepsia. 2018;59(S2):113–119.