The Alice in Wonderland syndrome comprises visual and/or somaesthetic distortions, most often migrainous in origin. We present a case of Alice in Wonderland syndrome in association with a right posterior/visual cortex infarction with subsequent seizures including focal motor status (epilepsia partialis continua), the latter a hitherto unreported association. These pathophysiological changes were of right hemisphere origin, suggesting Alice in Wonderland syndrome, if present, might have localising value for seizure focus in the right hemisphere.

PURPOSE:Status epilepticus (SE) is a severe condition of unrelenting seizures requiring urgent identification and treatment. SE may be unprovoked, occurring in someone with epilepsy, or may be provoked by acute intracranial disease or metabolic derangement. Increasingly encephalitis, particularly autoimmune types, is reported to cause refractory seizures. Whilst convulsive SE is readily identified, non-convulsive SE (NCSE) can be difficult to identify clinically, and electroencephalography (EEG) is required. Therefore, it is critical to identify the key clinical features associated with NCSE on EEG to inform future use of EEG.METHODS:We conducted a multicentre, retrospective analysis of EEG requests from four general and one specialist neurology hospital in the Northwest of England (2015-2018). Cases were identified from EEG requests for patients with suspected NCSE or other indications such as encephalopathy. We compared demographic and clinical characteristics between EEG-confirmed cases of NCSE and a randomly selected sample of negative controls.RESULTS:358 EEGs were reviewed, and 8 positive cases of NCSE were identified. Epilepsy was identified as the aetiology in 2 of these cases, and autoimmune encephalitis another 2 cases (one patient with N-methyl-d-aspartate receptor antibodies and another with voltage gated potassium channel antibodies). Previous alcohol excess (p = 0.005) and subtle motor signs (p = 0.047) on examination were observed more frequently in patients with NCSE compared to controls.CONCLUSION:Physicians should have a low threshold for urgent EEG in patients with suspected or previous encephalitis, especially if autoimmunity is suspected or subtle motor signs are present.

BACKGROUND:Immunoglobulin is a blood product used in a variety of medical disorders, usually delivered intravenously (IVIg). Neurology patients, particularly those with inflammatory polyneuropathy, utilise a lot of IVIg. There is a national shortage of immunoglobulin and, thus, pressing need to ensure minimum effective dosing as well as rigorous outcome assessments to assess benefit at treatment start and subsequently, as placebo effects can be strong.METHODS:Serial audit of IVIg use at The Walton Centre against national guidelines was carried out through analysis of clinical notes of day unit patients. Review of the national immunoglobulin database and of neurology outpatient notes to benchmark our practice and provide some comparison with the wider nation was also performed.RESULTS:Serial audit led to improved adherence to guidelines, and analysis of practice identified wide variation in IVIg use.CONCLUSION:Local audit and benchmarking of practice can be used to promote quality and consistency of IVIg use across the NHS.

Chronic myelomonocytic leukaemia (CMML) is a malignant haematopoietic stem cell disorder. Autoimmune disorders including demyelinating Guillain-Barre Syndrome (GBS) have been rarely described in association, but monophasic reversible axonal polyneuropathies have not. We report a 64 year old man who presented with 8 weeks of progressive leg weakness, paresthesia of the hands and feet, and weight loss. He was diagnosed with CMML the preceding year during investigations for cutaneous vasculitis. Examination revealed symmetrical lower motor neuron weakness, distal in the arms; proximal in the legs. Sensory loss to all modalities was present. CSF analysis confirmed albumin-cytological dissociation. No infiltration was seen on contrast imaging of the neuroaxis. Nerve conduction studies showed no evidence of demyelination, but an axonal sensori-motor polyneuropathy. He deteriorated rapidly with widespread denervation and respiratory involvement. Steroids and IV immunoglobulins did not help but improvement started after 1 cycle of intermediate dose ARA-C chemotherapy followed by hydroxycarbamide. There was a transient recovery of FBC abnormalities but the CMML picture returned. After neurological rehabilitation he made a full functional recovery. Monophasic axonal polyneuropathies can be associated with CMML and can respond to chemotherapy. The exact pathophysiological immunomodulatory mechanism is unclear. Prompt recognition and treatment can result in excellent recovery.

Myoclonus Dystonia is associated with a mutation in the epsilon sarcoglycan gene (SGCE – DYT-11). Russell-Silver syndrome is characterised by intrauterine growth restriction, poor postnatal growth, relative macrocephaly, triangular facial appearance and fifth finger clinodactyly. It is associated with maternal uniparental disomy of chromosome 7 (mUPD 7) in 5%–10% of cases. Previous cases of myoclonus dystonia associated with Russell-Silver syndrome have been reported in patients with mUPD 7. We report a case of myoclonus dystonia and Russell-Silver syndrome in a 41 year old man with a microdeletion of chromosome 7q. The patient was born full term but was of low birth weight. He suffered from myoclonus from the age of four. Presenting to adult neurology aged 18 he was of small stature, had dysmorphic features and had multifocal myoclonus that was action-induced, stimulus-sensitive and alleviated by alcohol. Investigations for mitochondrial disorders and other causes of progressive myoclonic epilepsy were negative. At age 35 he was found to have mild torticollis and a chromosomal microarray showed a deletion of 35 genes, including SGCE. We propose that this gentleman fulfils the criteria for Russell-Silver syndrome and that this is associated with his chromosomal microdeletion, which was discovered on investigation of his movement disorder.

Medical Journal of AustraliaVolume 203, Issue 11 p. 457-457 Christmas Competition ’Twas the night before Grand Round … Richard J B Ellis MBChB(Hons), MPhil, MRCP, Corresponding Author Richard J B Ellis MBChB(Hons), MPhil, MRCP richard.ellis@thewaltoncentre.nhs.uk The Walton Centre NHS Foundation Trust, Liverpool, UKCorrespondence: richard.ellis@thewaltoncentre.nhs.ukSearch for more papers by this authorBarnaby N Hirons MBChB, MSc, MRCP, Barnaby N Hirons MBChB, MSc, MRCP Holy Spirit Northside, Brisbane, QLDSearch for more papers by this authorAlexandra E May MBChB(Hons), MRCGP, Alexandra E May MBChB(Hons), MRCGP The Ash Surgery, Liverpool, UKSearch for more papers by this authorDavid J McCreary MBChB, MRCEM, David J McCreary MBChB, MRCEM University Hospital Aintree, Liverpool, UKSearch for more papers by this authorNicholas D Peterson MBChB(Hons), MRCS, Nicholas D Peterson MBChB(Hons), MRCS Whiston Hospital, Prescot, UKSearch for more papers by this authorLeonard M Quinn MBChB(Hons), MRCS, Leonard M Quinn MBChB(Hons), MRCS Southport Hospital, Southport, UKSearch for more papers by this authorBesa Ziso MBChB, MRCP, Besa Ziso MBChB, MRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorMichael Bonello MD, MRCP, Michael Bonello MD, MRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorBrython Hywel MBBCh(Hons), MRCP, Brython Hywel MBBCh(Hons), MRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorBenedict D Michael MBChB(Hons), MRCP, PhD, Benedict D Michael MBChB(Hons), MRCP, PhD The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorSean D Brown BSc(Hons), MBChB, MRCP, Sean D Brown BSc(Hons), MBChB, MRCP Clatterbridge Hospital, Wirral, UKSearch for more papers by this authorBenjamin D Murray BSc(Hons), MBChB(Hons), FRCA, Benjamin D Murray BSc(Hons), MBChB(Hons), FRCA Liverpool Heart and Chest Hospital, Liverpool, UKSearch for more papers by this authorDuncan M Rogers MBChB, MRCGP, Duncan M Rogers MBChB, MRCGP Market Street Surgery, Newton Le Willows, UKSearch for more papers by this authorMatt Barrett BSc, Matt Barrett BSc The Old Post Office Clinic, Ingleton, UKSearch for more papers by this authorMark Doran MD, PhD, FRCP, Mark Doran MD, PhD, FRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this author Richard J B Ellis MBChB(Hons), MPhil, MRCP, Corresponding Author Richard J B Ellis MBChB(Hons), MPhil, MRCP richard.ellis@thewaltoncentre.nhs.uk The Walton Centre NHS Foundation Trust, Liverpool, UKCorrespondence: richard.ellis@thewaltoncentre.nhs.ukSearch for more papers by this authorBarnaby N Hirons MBChB, MSc, MRCP, Barnaby N Hirons MBChB, MSc, MRCP Holy Spirit Northside, Brisbane, QLDSearch for more papers by this authorAlexandra E May MBChB(Hons), MRCGP, Alexandra E May MBChB(Hons), MRCGP The Ash Surgery, Liverpool, UKSearch for more papers by this authorDavid J McCreary MBChB, MRCEM, David J McCreary MBChB, MRCEM University Hospital Aintree, Liverpool, UKSearch for more papers by this authorNicholas D Peterson MBChB(Hons), MRCS, Nicholas D Peterson MBChB(Hons), MRCS Whiston Hospital, Prescot, UKSearch for more papers by this authorLeonard M Quinn MBChB(Hons), MRCS, Leonard M Quinn MBChB(Hons), MRCS Southport Hospital, Southport, UKSearch for more papers by this authorBesa Ziso MBChB, MRCP, Besa Ziso MBChB, MRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorMichael Bonello MD, MRCP, Michael Bonello MD, MRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorBrython Hywel MBBCh(Hons), MRCP, Brython Hywel MBBCh(Hons), MRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorBenedict D Michael MBChB(Hons), MRCP, PhD, Benedict D Michael MBChB(Hons), MRCP, PhD The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this authorSean D Brown BSc(Hons), MBChB, MRCP, Sean D Brown BSc(Hons), MBChB, MRCP Clatterbridge Hospital, Wirral, UKSearch for more papers by this authorBenjamin D Murray BSc(Hons), MBChB(Hons), FRCA, Benjamin D Murray BSc(Hons), MBChB(Hons), FRCA Liverpool Heart and Chest Hospital, Liverpool, UKSearch for more papers by this authorDuncan M Rogers MBChB, MRCGP, Duncan M Rogers MBChB, MRCGP Market Street Surgery, Newton Le Willows, UKSearch for more papers by this authorMatt Barrett BSc, Matt Barrett BSc The Old Post Office Clinic, Ingleton, UKSearch for more papers by this authorMark Doran MD, PhD, FRCP, Mark Doran MD, PhD, FRCP The Walton Centre NHS Foundation Trust, Liverpool, UKSearch for more papers by this author First published: 14 December 2015 https://doi.org/10.5694/mja15.00803Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease 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A 67-year-old woman presented with a seven-month history of progressive psychiatric disturbance and sub-acute cognitive decline mimicking Creuztfeldt-Jakob Disease.She presented initially to Psychiatry with worsening anxiety, depression, lassitude, impaired concentration and insomnia. She was given a diagnosis of agitated depression.She subsequently experienced episodes of disorientation, indecision and inability to complete routine, learned tasks as well as visual hallucinations. Initial investigations for causes of subacute dementia were unremarkable including a non-diagnostic MRI. However, five months into her presentation, there was further rapid deterioration with fluctuating consciousness and deteriorating mobility. She became bedbound, incontinent and cortically blind. She had right hemiparesis, hemineglect, extrapyramidal features, and extensor plantars.A 4D computerised tomography angiogram demonstrated a dural arteriovenous (AV) fistula with retrograde filling of superior sagittal sinus and cortical venous congestion. She underwent emergency Onyx embolisation. Over a few weeks, there was gradual improvement in her anxiety, cognition and mobility. She had a mild residual right hemiparesis.Intracranial dural AV fistulae can present with a spectrum of neurological symptoms, including cognitive decline. A lowhigh index of suspicion combined with close liaison between Neuroradiology, Neurology and Neurosurgery ensures prompt diagnosis and maximises the potential for cognitive recovery.