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Background: Mycosis fungoides (MF) has usually an indolent course. However, some patients develop a more aggressive disease and few prognostic parameters have been identified. Isolated cases of pustular MF (pMF) suggest an unfavourable prognosis., Objectives: We aim to describe the clinico-pathological characteristics and prognostic value of pMF., Methods: We retrospectively collected data of all cases of MF with histological pustules diagnosed from 2009 to 2020. The outcomes and clinico-pathological characteristics of pMF at diagnosis (pMFD) were compared to those of a cohort of non-pustular MF (NpMF)., Results: 33 pMF (including 22 pMFD) and 86 NpMF cases were included. The median age at diagnosis of pMF was 61 years [IQR=50-75]. The median follow-up of pMFD was 32 months [IQR=14-49]. Clinically, 33% of pMF had pustules. Large-cell transformation (LCT) occurred in 17 cases. pMFD were at a significantly more advanced-stage and more showed LCT at diagnosis than NpMF (50% vs 7%, p<0.001 and 23% vs 0%, p<0.001, respectively). In multivariate Cox analysis, the presence of histological pustule at diagnostic was associated with shorter OS in all patients (HR=13.90, CI95%[2.43-79]; p=0.003), and in early-stage patients (HR=11.09, CI95%[1.56-78.82]; p=0.02). In multivariate Fine and Gray model analysis, pMFD was associated with a higher cumulative incidence of LCT (SHR=13.90, CI95% [2.43-79]; p=0.003) in all patients. Median OS after the occurrence of histological pustules during follow-up of all pMF patients was 37 months, with a five-year OS of 25% (CI95% [0.06-0.5])., Conclusion: pMF often follows an aggressive course, with a high risk of LCT and shorter survival, even for early-stage patients. Histological pustules at diagnostic of MF might represent an independent poor prognostic factor, to be confirmed by further studies. Because pustules are not always clinically identified, histological pustules should be mentioned in pathology reports of MF and prompt discussion of a closer follow-up., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Motor neuron disorders comprise a clinically and pathologically heterogeneous group of neurologic diseases characterized by progressive degeneration of motor neurons (including both sporadic and hereditary diseases), affecting the upper motor neurons, lower motor neurons, or both. Hereditary motor neuron disorders themselves represent a vast and heterogeneous group, with numerous clinical and genetic overlaps that can be a source of error. This narrative review aims at providing an overview of the main types of inherited motor neuron disorders by recounting the stages in their historical descriptions. For practical purposes, this review of the literature sets out their various clinical characteristics and updates the list of all the genes involved in the various forms of inherited motor neuron disorders, including spinal muscular atrophy, familial amyotrophic lateral sclerosis, hereditary spastic paraplegia, distal hereditary motor neuropathies/neuronopathies, Kennedy's disease, riboflavin transporter deficiencies, VCPopathy and the neurogenic scapuloperoneal syndrome., (© 2024. Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system that can lead to severe disability from muscle weakness and sensory disturbances. Around a third of patients do not respond to currently available treatments, and many patients with a partial response have residual neurological impairment, highlighting the need for effective alternatives. Efgartigimod alfa, a human IgG1 antibody Fc fragment, has demonstrated efficacy and safety in patients with generalised myasthenia gravis. We evaluated the safety, tolerability, and efficacy of subcutaneous efgartigimod PH20 in adults with CIDP., Methods: ADHERE, a multistage, double-blind, placebo-controlled trial, enrolled participants with CIDP from 146 clinical sites from Asia-Pacific, Europe, and North America. Participants with evidence of clinically meaningful deterioration entered an open-label phase of weekly 1000 mg subcutaneous efgartigimod PH20 for no longer than 12 weeks (stage A). Those with confirmed evidence of clinical improvement (ECI; treatment responders) entered a randomised-withdrawal phase of 1000 mg subcutaneous efgartigimod PH20 weekly treatment versus placebo for a maximum of 48 weeks (stage B). Participants were randomised (1:1) through interactive response technology and stratified by their adjusted Inflammatory Neuropathy Cause and Treatment (aINCAT) score change during stage A and their most recent CIDP medication within 6 months before screening. Investigators, the clinical research organisation, and participants were masked to the treatment. The primary endpoint in stage A, evaluated in the stage A safety population, was confirmed ECI (≥1 points aINCAT decrease, ≥4 points [centile metric] Inflammatory Rasch-built Overall Disability Scale increase, or ≥8 kPa grip strength increase after four injections and two consecutive visits). The primary endpoint in stage B, evaluated in the modified intention-to-treat population, was the risk of relapse (time to first aINCAT increase of ≥1 points). ADHERE is registered with ClinicalTrials.gov (NCT04281472) and EudraCT (2019-003076-39) and is completed., Findings: Between April 15, 2020, and May 11, 2023, 629 participants were screened; 322 (114 female, 208 male) entered stage A, of whom 214 (66%, 95% CI 61·0-71·6) had confirmed ECI. In stage B, 221 participants were randomised (79 female, 142 male; 111 to subcutaneous efgartigimod PH20, 110 to placebo). Subcutaneous efgartigimod PH20 significantly reduced the risk of relapse versus placebo (hazard ratio 0·39 [95% CI 0·25-0·61]; p<0·0001). 31 (27·9% [19·6-36·3]) participants given subcutaneous efgartigimod PH20 had a relapse versus 59 (53·6% [44·3-63·0]) given placebo. In stage A, treatment-emergent adverse events (TEAEs) occurred in 204 (63%) participants and serious TEAEs in 21 (7%). In stage B, TEAEs occurred in 71 (64%) participants on subcutaneous efgartigimod PH20 and 62 (56%) participants on placebo, and serious TEAEs in six (5%) on subcutaneous efgartigimod PH20 and six (5%) on placebo. Three deaths occurred: two in stage A (one non-related and one unlikely related to treatment) and one in stage B (placebo group)., Interpretation: ADHERE showed the efficacy of subcutaneous efgartigimod PH20 in reducing the risk of relapse versus placebo in people with CIDP who responded to treatment. Further studies are needed to provide data on the longer-term effects of efgartigimod alfa and how it compares with currently available treatment options., Funding: argenx., Competing Interests: Declaration of interests JAA reports consulting fees from Akcea Therapeutics, Alexion, Alnylam, Annexon Biosciences, argenx, CSL Behring, Grifols, Immunovant, ImmuPharma, Johnson & Johnson, and Takeda, and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Alnylam, Annexon Biosciences, argenx, CSL Behring, and Takeda. TD reports participation on a data safety monitoring board or advisory board for Dianthus Therapeutics. CE reports grants or contracts from argenx; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from argenx; support for attending meetings and travel from argenx; and stock or stock options from argenx. AT was an employee of argenx, and reports stock or stock options at the time of the study completion. JTG is an employee of argenx; reports support for attending meetings and travel from argenx; and reports stock or stock options from argenx. BVH is an employee of argenx. PU is an employee of argenx; reports patents planned, issued, or pending from argenx; reports stock or stock options from argenx; and reports other financial or non-financial interests from argenx. EH is an employee of argenx; reports patents planned, issued, or pending from argenx; and reports stock or stock options from argenx. KGG reports consulting fees from Alexion, and UCB; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Alexion, argenx, and Xeris Pharmaceuticals; and leadership or fiduciary role in other board, society, committee, or advocacy group, paid or unpaid for Myasthenia Gravis Foundation of America. FL reports grants or contracts from German Ministry of Education and Research, German Research Society DFG, HORIZON MSCA 2022 Doctoral Network, and Stiftung Pathobiochemie of the German Society for Laboratory Medicine; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Bayer, Biogen, Fresenius, Grifols, Novartis, Roche, and Teva Pharmaceuticals; support for attending meetings and travel from Bayer, Grifols, and Merck; and participation on a data safety monitoring board or advisory board for argenx, Alexion, Biogen, and Roche. MLi reports grants or contracts from Kedrion Biopharma; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from CSL Behring, Kedrion Biopharma, and Takeda; support for attending meetings and travel from CSL Behring, Kedrion Biopharma, and Takeda; and other financial or non-financial interests from argenx. MLo was an employee of argenx at the time of the study completion. LQ reports grants or contracts from argenx, CIBERER, Instituto de Salud Carlos III–Ministry of Economy and Innovation (Spain), and UCB; consulting fees from Annexon Biosciences, Alnylam, argenx, Avilar Therapeutics, CSL Behring, Dianthus Therapeutics, Janssen, LFB, Novartis, Nuvig Therapeutics, Roche, Sanofi, and Takeda; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Alnylam, argenx, CSL Behring, Novartis, Roche, and Sanofi; support for attending meetings and travel from Alnylam and Sanofi; participation on a data safety monitoring board or advisory board for argenx, CSL Behring, Sanofi, and UCB; and a leadership or fiduciary role in other board, society, committee, or advocacy group, paid or unpaid, for Inflammatory Neuropathy Consortium and Peripheral Nerve Society. NS reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Alnylam, and participation on a data safety monitoring board or advisory board for Takeda. RY reports consulting fees from Japan Tobacco and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Alnylam Japan, CSL Behring, FP Pharm, Kyowa Kirin, Ono Pharmaceutical, and Takeda Pharmaceutical. RAL reports royalties or licenses from UpToDate; consulting fees from Annexon Biosciences, argenx, CSL Behring, Dianthus Therapeutics, Grifols, Immunovant, Janssen, Nuvig Therapeutics, Sanofi, and Takeda; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from argenx, CSL Behring, Medscape, and Sanofi; participation on a data safety monitoring board or advisory board for Boehringer Ingelheim and Novartis; and leadership or fiduciary role in other board, society, committee, or advocacy group, paid or unpaid, for Peripheral Nerve Society and GBS-CIDP Foundation International. PAvD reports support for attending meetings and travel from argenx and participation on a data safety monitoring board or advisory board for argenx. All other authors declare no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved, including those for text and data mining, AI training, and similar technologies.)

Background: The tongue is an essential organ for the development of certain crucial functions such as swallowing and speech. The examination of the tongue can be very useful in neurology, as the various types of lingual alterations can lead to certain specific diagnoses, the tongue being a kind of 'mirror' of some neurological function., Review Summary: To discuss the elements of clinical examination of the tongue in relation to neurological disorders. After reviewing the different superficial lesions of the tongue, we deal with various movement disorders of the tongue (fasciculations/myokimia, orolingual tremor, choreic movements of the tongue, dystonia of the tongue, lingual myoclonus, and psychogenic movements), disorders of taste and lingual sensitivity and lingual pain., Conclusions: Examination of the tongue should not be limited to studying its motility and trophicity. It is equally important to check the sensory function and understand how to interpret abnormal movements involving the tongue. This study also aimed to demonstrate the importance of nonmotor tongue function in neurological practice., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: The tongue is an essential organ for the development of certain crucial functions, such as swallowing and language. The examination of the tongue can be very useful in neurology, as the various types of lingual alterations can lead to certain specific diagnoses, the tongue being a kind of "mirror" of some neurological function., Review Summary: In this study, we reviewed the literature on anatomy, physiology, and the various aspects of the examination of the tongue., Conclusions: Examination of the tongue should be an integral part of the clinical examination of the cranial nerves. This study aimed to demonstrate the importance of tongue motor and non-motor functions in neurological practice., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Noninvasive ventilation (NIV) improves survival and quality of life in amyotrophic lateral sclerosis (ALS) patients. NIV initiation is mostly conducted at hospital, but a recurrent lack of hospital beds led to the necessity of exploring an at-home initiation process. Here, we report data from our NIV initiation cohort of ALS patients. Could our at-home NIV initiation process with telemonitoring in ALS patients be an efficient solution for adherence and nocturnal hypoxaemia correction?, Methods: We performed a retrospective analysis of data collected from 265 ALS patients treated at the Bordeaux ALS Centre for whom NIV initiation was carried out between September 2017 and June 2021, with two modalities: at-home initiation or in-hospital initiation. The primary outcome was adherence to NIV at 30 days. The secondary outcome was at-home NIV initiation process efficiency of nocturnal hypoxaemia correction., Results: At 30 days, NIV adherence (mean >4 h·day -1 ) was 66% of the total population, 70% of the at-home NIV initiation subgroup and 52% of the in-hospital NIV initiation subgroup. Nocturnal hypoxaemia correction was observed in 79% of adherent patients in the at-home NIV initiation subgroup. Mean delay of NIV prescription and at-home NIV initiation was 8.7 days (+/-6.5) versus 29.5 days in hospital., Conclusion: Our study shows that our at-home NIV initiation process in ALS patients is a good option to provide rapid access to NIV with good adherence and efficiency. Further literature on the benefits of at-home NIV initiation is welcomed, especially to evaluate long-term efficiency and global cost analysis., Competing Interests: Conflict of interest: T. Réginault reports support for the present manuscript from the Bordeaux University Foundation; and travel support from Vivisol and personal fees from Zéphyr Paramed, outside the submitted work. B. Bouteleux reports personal fees from Zéphyr Paramed outside the submitted work. P. Wibart reports support for the present manuscript from the Bordeaux University Foundation, and personal fees from Zéphyr Paramed outside the submitted work. L. Grassion received grants or contracts from AADAIRC, outside the submitted work; payment of honoraria for lectures, presentations, speakers' bureaus, manuscript writing or educational events from SOS Oxygene, ASTEN Santé, and ALMS, outside the submitted work; support for attending meetings and/or travel from SOS Oxygen, VIVISOL and ASTEN Santé, outside the submitted work; and participation on a data safety monitoring board or advisory board for VIVISOL, outside the submitted work. All other authors have nothing to disclose., (Copyright ©The authors 2023.)