<h3>Objective:</h3> To define the rate, timing, and clinical risk factors for development of autoimmune disease (AID) after alemtuzumab treatment multiple sclerosis (MS). <h3>Methods:</h3> We analyzed prospective serologic data from 248 patients with MS treated alemtuzumab, median follow-up 34.3 months (range 6.7–107.3). <h3>Results:</h3> Novel AID developed in 22.2%. Thyroid was most frequent (15.7%). A range hematologic, renal, dermatologic were also observed as asymptomatic novel...

Functional outcome after subarachnoid haemorrhage has traditionally been assessed using scales developed for other neurological conditions. The modified Rankin score and Glasgow Outcome Scale are most commonly used. Employment of these in is hampered by well recognized limitations. We set out to develop validate a new condition-specific tool (SAHOT). Items addressing diverse aspects the impact were collected during focus groups involving patients, next-of-kin multidisciplinary professionals...

Epilepsy prevalence is significantly higher in people with Intellectual Disability (ID) compared to epilepsy (PWE) from the general population. Increased psychological and behavioural problems, healthcare costs, morbidity, mortality treatment resistance antiepileptic drugs (AEDs) associated ID populations. Prescribing AEDs for PWE challenging influenced heavily by studies conducted Our study compares Lacosamide (LCM) response population those population; using data an UK based database...

Background A growing body of evidence suggests inequitable access to disease-modifying therapies (DMTs) for multiple sclerosis (MS) in publicly funded healthcare systems. This retrospective study examined the impact ethnicity and deprivation on DMTs. Methods All adults diagnosed with relapsing-remitting MS between 2010 2020 were included. The being offered starting any DMTs high-efficacy measured using binary, multinomial logistic Cox regression models. These analyses adjusted sex, age at...

Background A quarter of people with intellectual disability (ID) have epilepsy, compared to approximately one in a hundred across the general population. Evidence for safe and effective prescribing antiepileptic drugs (AEDs) those ID is, however, limited. Aims Study This study seeks strengthen research evidence around Eslicarbazepine Acetate (ESL), new AED, by comparing response individuals from population who do not ID. Methods single data set was created through retrospective collection...

Genetic risk for amyotrophic lateral sclerosis (ALS) is highly elevated in genetic isolates, like the island population of Malta south Europe, providing a unique opportunity to investigate genetics this disease. Here we characterize clinical phenotype and profile largest series Maltese ALS patients date identified throughout 5-year window. Cases controls underwent neuromuscular assessment analysis rare variants causative or genes following whole-genome sequencing. Potentially damaging repeat...

A 70-year-old man presented with respiratory distress and proximal muscle weakness shortly after biopsy of a left forearm mass. The showed giant cell myositis, serological investigations identified grossly elevated serum creatine kinase level, suggesting skeletal damage. Serum troponin T was also high, but I normal. antiacetylcholine receptor antibodies were positive, imaging thymoma. He recovered well following intravenous immunoglobulin corticosteroids, later underwent thymectomy. is...

Objective: Amyotrophic lateral sclerosis (ALS) is a mostly sporadic neurodegenerative disease. The role of environmental factors has been extensively investigated but associations remain controversial. Considering that substantial proportion adult life spent at work, identifying occupations and work-related exposures considered an effective way to detect increase ALS risk. This process may be further facilitated in population isolates due genetic homogeneity. Our study occupational...

Amyotrophic lateral sclerosis (ALS) is frequently caused by mutations in the SOD1 gene. Here, we report first variant Malta, an archipelago of three inhabited islands southern Europe. We describe a patient with sporadic form ALS living on island Gozo which heterozygous c.272A>C; p.(Asp91Ala) was detected. The had late onset (79 years), sensory impairments and rapid disease progression culminating respiratory failure. has not yet developed any additional family members D91A identified. None...

People with epilepsy are at increased risk of multiple co-morbidities that may influence adverse outcomes including impact on quality life and premature mortality. These factors include potentially modifiable clinical characteristics associated sudden unexpected death in (SUDEP). For services to tackle risk, the complexity target population needs be defined. While this has been comprehensively studied large, economically developed countries little knowledge these issues exist small...

Sarcoidosis is an idiopathic multisystem granulomatous disorder of unknown cause. Nervous system involvement (central and/or peripheral) uncommon, developing in 5%–10%. The presenting symptoms are variable, reflecting the level involvement, and frequently fluctuate progress. Diagnosing neurosarcoidosis people with previously confirmed systemic disease may be relatively straightforward, but diagnosing primary challenging. Managing primarily consensus based; corticosteroid its mainstay,...

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a subtype of stiff-person syndrome (formerly stiff-man syndrome). It rare disabling, characterised by brainstem symptoms, muscle stiffness, breathing issues autonomic dysfunction. We describe 65-year-old man who presented odynophagia together tongue neck swelling, followed multiple cranial nerve palsies culminating in bilateral vocal cord paralysis acute stridor. He subsequently developed progressive generalised hypertonia...

Alemtuzumab is a humanised monoclonal antibody approved for treatment of active relapsing remitting multiple sclerosis (RRMS). It targets CD-52 glycoprotein on the cell surface mature lymphocytes, resulting in their destruction, but thought to leave polymorphonuclear cells unaffected. A 17 year old female with aggressive RRMS completed her first alemtuzumab course. Four weeks post-infusion monitoring bloods, identified neutropenia 0.6, reaching nadir 0.4 3 days. Haematologist advised...

<h3>Background</h3> The search for treatments of progressive multiple sclerosis (PMS) necessitates meaningful markers disease progression clinical trials. <h3>Aim</h3> We investigated the responsiveness MS Muscle Strength scale (MSMSS), a recently developed physician-based instrument, to determine its value as outcome measure in PMS. <h3>Methods</h3> 54 pwPMS (SPMS=45/PPMS=9) were examined using MSMSS and again &gt;12 months. Group-level individual-level indices change calculated...

Paraneoplastic neurological syndromes are non-metastatic disorders triggered by an altered immune response to a neoplasm. Uncommon presentations increasingly reported, widening the recognised spectrum of these disorders. We present two patients with atypical manifestations that heralded malignancy. <b>Case 1</b> A 79 year old woman presented progressive asymmetrical (R&gt;L) leg weakness. Examination confirmed significant paraparesis (MRC 2/5 bilaterally) normal muscle bulk and tone,...

<h3>Background</h3> Intravenous immunoglobulin is a first line treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). ABN guidelines have highlighted the need evidence of IVIg efficacy in pwCIDP using validated instruments. <h3>Aim</h3> 1) To evaluate on CIDP collected admitted to Royal Devon and Exeter Hospital IVIg. 2) Based (1), develop an assessment tool measuring therapy pwCIDP. <h3>Methods</h3> Retrospective evaluation data from admissions RD&amp;E...

Alemtuzumab is a anti-CD52 humanised monoclonal antibody shown to be an effective treatment for relapsing multiple sclerosis (MS) in phase II studies but exhibits unique adverse event profile. In limited follow-up development of autoimmune disease has been observed 20–30% patients. Currently, the exact range and temporal evolution following remains unclear will have important implications screening safety monitoring. this study we analyse prospective clinical serological data from 225...

We report a case of paraneoplastic limbic encephalitis presenting with purely olfactory symptoms. For 7 weeks, 76-year-old man experienced distorted impressions the odour most foods and liquids hallucinatory perceptions without objective stimuli. The perceived was metallic foul, causing loss appetite weight, nausea repeated retching. No dental or ENT cause for his symptoms found. MRI brain sinuses normal. EEG showed no focal paroxysmal activity. CSF protein elevated (1.17 g) isoelectric...

Published cases of post-malaria neurological syndrome (PMNS) invariably report clinical and radiological encephalopathy. We a case brainstem spinal cord inflammation without supratentorial involvement following recovery from <i>Plasmodium falciparum</i> infection. A 48-year-old woman was diagnosed with <i>P malaria while on holiday in Sierra Leone, during which she did not take prophylaxis. She responded well to artesunate amiodaquine. Ten days later, back the UK, admitted hospital...