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Background and purpose Methods Results Conclusions Few data are available on the course of myasthenia gravis (MG) regarding disease severity and stability over time in real‐world settings. This study used the French National Health Insurance Database (SNDS) to assess markers of disease severity in patients with MG longitudinally.All patients with MG‐related claims in the SNDS between 2013 and 2020 were identified. Patients were followed for up to 8 years after the first claim. Intensive care unit (ICU) stays, treatment with intravenous immunoglobulin (IVIg) or plasma exchange (PE), and death were documented throughout the follow‐up period. Standardized mortality rates were estimated, and mortality‐related variables were identified using a Cox model.In all, 14,459 individuals constituted the full study population, including 6354 incident patients. In the incident population, 2199 (34.6%) were admitted to ICUs at least once, principally during the first year after the index date (N = 1477; 23.3%). This proportion decreased progressively to reach 3.0% in the seventh year. A total of 2817 patients received IVIg and 432 PE, again principally in the first year. In the full study population, the standardized mortality rate was 1.08 (95% confidence interval [CI] 1.03–1.13), being lower in men (0.95, 95% CI 0.89–1.02) than in women (1.15, 95% CI 1.07–1.23) and in patients aged >65 years (1.06, 95% CI 1.01–1.11) than in younger patients (1.50, 95% CI 1.24–1.76). Male gender, older age and higher comorbidity were independently associated with mortality.A subgroup of patients with MG require ICU admission and rescue therapy with IVIg or PE, indicative of poor disease control. New therapies are needed to improve disease control and reduce disease burden. [ABSTRACT FROM AUTHOR]

Background: Ictal epileptic headache (IEH) is caused by a focal epileptic seizure. The diagnosis can be challenging when the headache is isolated without any other symptoms. Case Report: A 16‐year‐old girl presented with a 5‐year history of bilateral frontotemporal headaches with severe intensity lasting for 1–3 min. Past medical, physical, and developmental histories were unremarkable. Head magnetic resonance imaging showed right hippocampal sclerosis. The diagnosis of pure IEH was confirmed by video‐electroencephalographic monitoring. The onset and cessation of frontal headache correlated with a right temporal discharge. The patient was diagnosed with right mesial temporal lobe epilepsy. Two years later, her seizures increased despite antiseizure medications. A right anterior temporal lobectomy was performed. The patient remained seizure‐free and headache‐free for 10 years. Conclusion: IEH should be considered in the differential diagnosis of brief and isolated headache, even if the headache is diffuse or contralateral to the epileptogenic focus. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Recent guidelines define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and possible CIDP. The aims of our study were to evaluate the value of diagnostic tests to support the diagnosis of CIDP in patients with possible CIDP and to identify prognostic factors of therapeutic success. Methods: We conducted an observational retrospective two‐center study between 2014 and 2019. We selected patients with a clinical presentation suggesting CIDP, but whose electrodiagnostic (EDX) test results did not meet the EFNS/PNS 2021 criteria. We analyzed epidemiologic and clinical features, axonal loss on EDX, cerebrospinal fluid (CSF), somatosensory evoked potentials (SSEPs), plexus magnetic resonance imaging (MRI), nerve biopsy, and therapeutic response. Results: We selected 75 patients, among whom 30 (40%) responded to treatment. The positivity rates of CSF analysis, MRI and SSEPs were not influenced by the clinical presentation or by the delay between symptom onset and medical assessment. A high protein level in CSF, female gender, and a relapsing–remitting course predicted the therapeutic response. Discussion: It is important to properly diagnose suspected CIDP not meeting EFNS/PNS 2021 EDX criteria by using supportive criteria. Specific epidemiological factors and a raised CSF protein level predict a response to treatment. Further prospective studies are needed to improve diagnosis and the prognostic value of diagnostic tests in CIDP. [ABSTRACT FROM AUTHOR]

Introduction: Assessment of diaphragm compound muscle action potential by noninvasive phrenic nerve stimulation at the neck is well described. However, normal values in a large cohort of healthy subjects are lacking. Our objective was to determine reference values of phrenic nerve conduction in healthy subjects.Methods: We recruited 155 healthy subjects (25-79 years old) and measured mean amplitude (PhrenAmp) and latency (PhrenLat) of motor responses according to Bolton's method.Results: The lower limit for PhrenAmp was 0.28 and 0.25 mV and the upper limit for PhrenLat was 8.41 and 8.56 ms for right and left side, respectively. PhrenLat was correlated with age. PhrenAmp, PhrenLat and area were significantly higher in men. Tolerance to phrenic nerve stimulation was excellent.Conclusions: Our study provides normative values of phrenic nerve motor responses in a large cohort of healthy subjects and identifies age and sex as factors of variation. Muscle Nerve 59:451-456, 2019. [ABSTRACT FROM AUTHOR]

Abstract: A 59‐year‐old woman presented with progressive paresthesias of all of her limbs for 4 years, associated with neuropathic pain, tingling in the tongue and allodynia, consistent with small fiber neuropathy (SFN). Several systemic symptoms and signs were found on clinical examination and laboratory work‐up. Neurological investigations including neurophysiologic test and skin biopsy supported the diagnosis of SFN. Chronic exposure to N‐hexane was then disclosed and suspected to be the cause of the disease. Following the discontinuation of chronic N‐hexane exposure, the patient had a progressive improvement of all signs and symptoms, reinforcing the correlation between exposure to N‐hexane, and development of SFN. Exposure to N‐hexane may be considered as a novel reversible cause of SFN, which underlines the need to look for toxic etiologies in the diagnosis of SFN. [ABSTRACT FROM AUTHOR]

Introduction: Vincristine is an antimitotic agent used for treatment of leukemia, lymphomas, and cancers. Its main side effect is a dose-related, length-dependent (LD) axonal neuropathy.Methods: We performed electrodiagnostic (EDx) examinations in 17 children who had been treated with vincristine and who presented with the clinical picture of a peripheral neuropathy.Results: The mean dose of vincristine was 8.5 ± 4.0 mg/m(2) . Clinical motor symptoms were more frequent and more severe than sensory ones. Thirteen children presented with a motor deficit, 4 of whom could no longer walk. EDx examination showed an axonal neuropathy with a non-length-dependent (NLD) pattern in 9 children and an LD pattern in 8. A major motor predominance was encountered in 12 patients.Conclusions: The electrophysiological and clinical motor predominance described differs from the mainly sensory neuropathy reported in adults. Incomplete myelination due to young age may have resulted in greater sensitivity of some nerves to neurotoxic agents. [ABSTRACT FROM AUTHOR]

Objective: To test the influence of functional cerebral reorganization in amyotrophic lateral sclerosis (ALS) on disease progression. Methods: Nineteen predominantly right-handed ALS patients and 21 controls underwent clinical evaluation, functional Magnetic Resonance Imaging (fMRI), and diffusion tensor imaging. Patients were clinically re-evaluated 1 year later and followed until death. For fMRI, subjects executed and imagined a simple hand-motor task. Between-group comparisons were performed, and correlations were searched with motor deficit arm Medical Research Council (MRC) score, disease progression ALS Functional Rating Scale (ALSFRS), and survival time. Results: By the MRC score, the hand strength was lowered by 12% in the ALS group predominating on the right side in accordance with an abnormal fractional anisotropy (FA) limited to the left corticospinal tract (37.3% reduction vs. controls P < 0.01). Compared to controls, patients displayed overactivations in the controlateral parietal ( P < 0.004) and somatosensory ( P < 0.004) cortex and in the ipsilateral parietal ( P < 0.01) and somatosensory ( P < 0.01) cortex to right-hand movement. Movement imagination gave similar results while no difference occurred with left-hand tasks. Stepwise regression analysis corrected for multiple comparisons showed that controlateral parietal activity was inversely correlated with disease progression ( R2 = 0.43, P = 0.001) and ipsilateral somatosensory activations with the severity of the right-arm deficit ( R2 = 0.48, P = 0.001). Conclusions: Cortical Blood Oxygen Level Dependent (BOLD) signal changes occur in the brain of ALS patients during a simple hand-motor task when the motor deficit is still moderate. It is correlated with the rate of disease progression suggesting that brain functional rearrangement in ALS may have prognostic implications. Hum Brain Mapp 34:2391-2401, 2013. © 2012 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Sensory neuronopathies (SNNs) encompass paraneoplastic, infectious, dysimmune, toxic, inherited, and idiopathic disorders. Recently described diagnostic criteria allow SNN to be differentiated from other forms of sensory neuropathy, but there is no validated strategy based on routine clinical investigations for the etiological diagnosis of SNN. In a multicenter study, the clinical, biological, and electrophysiological characteristics of 148 patients with SNN were analyzed. Multiple correspondence analysis and logistic regression were used to identify patterns differentiating between forms of SNNs with different etiologies. Models were constructed using a study population of 88 patients and checked using a test population of 60 cases. Four patterns were identified. Pattern A, with an acute or subacute onset in the four limbs or arms, early pain, and frequently affecting males over 60 years of age, identified mainly paraneoplastic, toxic, and infectious SNN. Pattern B identified patients with progressive SNN and was divided into patterns C and D, the former corresponding to patients with inherited or slowly progressive idiopathic SNN with severe ataxia and electrophysiological abnormalities and the latter to patients with idiopathic, dysimmune, and sometimes paraneoplastic SNN with a more rapid course than in pattern C. The diagnostic strategy based on these patterns correctly identified 84/88 and 58/60 patients in the study and test populations, respectively. [ABSTRACT FROM AUTHOR]