BACKGROUND: Regular and consistent disease assessment could provide a clearer picture of burden in generalised myasthenia gravis (gMG) and improve patient care; however, the use of assessment tools in practice lacks standardisation. This modified Delphi approach was taken to review current evidence on assessment tool use in gMG and develop expert-derived consensus recommendations for good practice.
METHODS: A European expert panel of 15 experienced gMG neurologists contributed to development of this consensus, four of whom formed a lead Sub-committee. The PICO (Population, Intervention, Control, Outcomes) framework was used to define six clinical questions on gMG assessment tools, a systematic literature review was conducted, and evidence-based statements were developed. According to a modified Delphi voting process, consensus was reached when ≥70% of the experts rated agreement with a statement as ≥8 on a scale of 1-10.
RESULTS: Eighteen expert- and evidence-based consensus statements based on six themes were developed. Key recommendations include: consistent use of the Myasthenia Gravis Activities of Daily Living score (MG-ADL) across clinical settings, followed by a simple question (e.g., Patient Acceptable Symptom State [PASS]) or scale to determine patient satisfaction in clinical practice; use of a Quantitative Myasthenia Gravis [QMG] or quality of life [QoL] assessment when the MG-ADL indicates disease worsening; and consideration of symptom state to determine the timing and frequency of recommended assessments. Expert panel consensus was reached on all 18 statements after two voting rounds.
CONCLUSIONS: This process provided evidence- and expert consensus-based recommendations for the use of objective and subjective assessment tools across gMG research and care to improve management and outcomes for patients.

Pustular psoriasis is characterised by eruptions of neutrophilic sterile pustules. The European Rare and Severe Psoriasis Expert Network consensus defines pustular psoriasis into three subtypes; generalised pustular psoriasis (GPP), palmoplantar pustulosis and acrodermatitis continua of Hallopeau (ACH). Mixed forms are categorised according to their predominant features. However, the Japanese Dermatological Association includes ACH under the diagnosis of GPP. This article aims to review the similarities and differences between ACH and GPP. Based on our review, interleukin (IL)-36RN mutations, the most frequent genetic findings in pustular psoriasis are found most commonly in GPP, followed by ACH. Genotypes of IL-36RN mutations among GPP patients and ACH patients are different between European and Asian ethnicities. IL-36 signalling pathway is the main mechanism. Metabolic diseases are common comorbidities and joint involvement can occur in 20.5%-36.4% of both conditions. Associated plaque psoriasis is more common in GPP than in ACH. Generally, ACH, even the generalised type, does not have systemic inflammation whereas GPP can occur with or without systemic inflammation. ACH can occur before, simultaneously, or after the development of GPP. However, response to treatment for GPP and ACH even in the same patients appear to be different. ACH seemed to be more recalcitrant to treatment than GPP but severe flare of GPP can lead to morbidity and mortality. Although GPP and ACH share genotypes and pathogenesis, we believe that ACH should be classified separately from GPP, and not under diagnosis of GPP. Future research is warranted to satisfactorily distinguish the two conditions.

Extrapolation of efficacy data from adults to children is accepted for focal epilepsy - the antiepileptic drug, lacosamide, has been approved for the treatment of children ≥4 years of age on this basis. Since many small-scale, open-label studies are reported in the literature before approval, a systematic review was conducted to ascertain whether results of these could be used to support extrapolation in epilepsy in the future. In the absence of randomised trials, a second analysis was conducted for reports on lacosamide use in adults with generalized epilepsies. Twenty-seven articles were included in the paediatric qualitative synthesis, and 14 in the adult. Paediatric studies were analysed separately based on seizure type: focal, generalised and mixed. In focal epilepsy, safety and seizure-related findings mirrored those observed in the adult Phase II/III trials, supporting the feasibility of data extrapolation. Few studies reported outcomes in children with epilepsies associated with generalised seizures, and those that included children with different seizure types, mostly did not provide results separately. Lacosamide treatment appeared beneficial for children and adults experiencing tonic-clonic and myoclonic seizures. Reports of seizure aggravation were inconsistent and, in many cases, could not be clearly attributed to lacosamide. Given the absence of sufficient data, evidence for the feasibility of extrapolation was not as clear-cut as it was in focal epilepsy. These results highlight the complexities of conducting trials in the generalised epilepsy setting, and the importance of studies in the real-life setting and of analysing efficacy data per generalized seizure type and syndrome.

The International League Against Epilepsy Classification of the Epilepsies, first presented in 1981, has been widely adopted across the globe. In 2017 it was revised to allow for more robust, specific, flexible and logical classification of seizures. A number of new seizure types are recognised. Classification should be timely as it plays a vital role in the diagnosis and management of patients with epilepsy. Accurate classification also underpins epilepsy research from pathophysiology to public health. Here we review the basic and extended forms of the classification. Semiology (symptoms and signs) is used as the foundation for grouping seizures under focal, generalised or of unknown onset. Focal seizures can be further classified by the presence or absence of awareness and motor signs. Generalised seizures engage bilateral networks from the onset and these can be either motor or non-motor. Seizures of unknown onset can be classified as motor, non-motor, tonic-clonic, epileptic spasms, or behaviour arrest.

Generalised granuloma annulare (GGA) is a significant cosmetic issue for patients but evidence is lacking to guide optimum treatment. We reviewed our patients with GGA treated with PUVA and narrowband UVB (NBUVB). A telephone questionnaire obtained the patients\' perspective in terms of treatment response, remission and overall satisfaction. Twenty patients, all female, were treated. Twelve patients had 15 courses of PUVA therapy (10 oral, 5 bath PUVA) and 10 had 12 courses of NB UVB (two patients had both). There was clearance or minimal residual disease (MRD) on clinical examination in eight of 12 patients after PUVA. Remission was for six months in seven patients extending to one year or more in five patients. Nine patients were contactable after PUVA therapy. They reported their satisfaction as excellent (n = 1), very good (n = 2) or good (n = 4), and two were disappointed. There was clearance or MRD in seven patients treated with NBUVB. Remission was for six months in at least three patients and greater than 1 year in at least 2 patients. Eight patients were contactable and reported satisfaction as excellent (n = 1) or good (n = 5) and two were disappointed. PUVA and NBUVB were effective in at least half of patients and they achieved satisfactory remission. Patients\' perceptions of the improvement after phototherapy were lower compared with their dermatologists\' assessment.