BACKGROUND: Pyridostigmine is hypothesized to improve neurogenic orthostatic hypotension (nOH) symptoms without causing or exacerbating supine hypertension. The objective of this review was to evaluate the safety and efficacy of pyridostigmine for management of nOH.
METHODS: A literature search of PubMed, Embase, and CENTRAL was performed in December 2023 for prospective trials with a placebo or active comparator.
RESULTS: Four randomized and two non-randomized studies were reviewed. Three studies utilizing a single dose, crossover design found significant differences of orthostatics using adjunctive pyridostigmine. Two studies assessing longer-term endpoints demonstrated conflicting efficacy of pyridostigmine with one trial finding significant improvement in orthostatics and symptoms after three months of therapy. Use of pyridostigmine did not lead to supine hypertension with most adverse effects being cholinergic.
CONCLUSIONS: Pyridostigmine may be considered as an adjunctive medication in individuals with nOH refractory to standard treatment options as it carries a favorable safety profile with low risk for supine hypertension.

Chronic intestinal pseudo-obstruction (CIPO) may be a primary or secondary phenomenon and is often multifactorial. Treatment is largely directed at improving colonic motility. The use of cholinesterase inhibitors such as pyridostigmine has been hypothesized to increase acetylcholine in the bowel, improving symptoms and transit times.
A systematic review of the use of pyridostigmine in CIPO was conducted using scientific and commercial search engines identifying scientific studies enrolling adult human subjects, published from 2000 to 2022 in the English language.
Four studies were identified including two randomized controlled trials (RCT) and two observational studies. The studies had heterogenous inclusion criteria, dosing regimens and reported outcomes. Two studies were identified as being at high risk of bias. All studies reported improved patient outcomes with use of pyridostigmine, and low rates (4.3%) of mild cholinergic side effects. No major side effects were reported.
The use of pyridostigmine in management of CIPO is biologically plausible due to its ability to increase colonic motility, and early studies on its role are uniformly suggestive of benefit with low side-effect profile. Four clinical studies have been conducted to date, with small sample sizes, heterogeneity and high risk of bias. Further high-quality studies are required to enable assessment of pyridostigmine\'s utility as an effective management strategy in CIPO.

This review highlights the potential mechanisms of neuromuscular manifestation of COVID-19, especially myasthenia gravis (MG).
An extensive literature search was conducted by two independent investigators using PubMed/MEDLINE and Google Scholar from its inception to December 2020.
Exacerbations of clinical symptoms in patients of MG who were treated with some commonly used COVID-19 drugs has been reported, with updated recommendations of management of symptoms of neuromuscular disorders. Severe acute respiratory syndrome coronavirus 2 can induce the immune response to trigger autoimmune neurological disorders.
Further clinical studies are warranted to indicate and rather confirm if MG in the setting of COVID-19 can pre-existent subclinically or develop as a new-onset disease.

We present a case of pyridostigmine-induced coronary artery spasm in a woman with early-onset myasthenia gravis (MG) who suffered from acute chest discomfort a few days after pyridostigmine dose up-titration. Twelve-lead ECG demonstrated ST-segment elevation in inferior limb leads together with sinus arrest. Sublingual nitrate was immediately given, which rapidly relieved her symptoms concomitantly with the resolution of abnormal ECG findings. Coronary angiography showed normal coronary arteries reflecting the transient nature of the disease. A small dose of pyridostigmine was rechallenged under close monitoring in the coronary care unit and reproduced her chest discomfort. After the substitution of pyridostigmine with immunosuppressive agents and prescription of long-acting nitrate, she had no recurrence of chest discomfort, as well as well-controlled MG symptoms.

We present a case of a man with a background of myasthenia gravis who presented with a neck lump, which was diagnosed as thyrolipomatosis in continuity with a very large thymolipoma. Following removal of these lesions, the patient\'s myaesthenic symptoms improved. While thymolipomas are often seen in the context of myasthenia gravis, thyrolipomatosis is a rare entity and to our knowledge the concurrent finding of both lesions with myasthenia gravis has never been reported. We highlight the important imaging features of both entities and the clinical importance of recognising them.

OBJECTIVE: Administration of acetylcholinesterase inhibitors can bring about peripheral nerve hyperexcitability symptom in muscle-specific tyrosine kinase antibody positive myasthenia gravis, but the changes in electromyography before and after drug withdrawal have not been described in detail.
METHODS: Electromyography was performed on a case of muscle-specific tyrosine kinase antibody positive myasthenia gravis with peripheral nerve hyperexcitability correlated with the administration of pyridostigmine bromide before and after drug withdrawal, respectively.
RESULTS: Afterdischarges close after M waves appeared on the tibial nerve, common peroneal nerve, median nerve, and ulnar nerve, and these presented unique characteristics in repetitive nerve stimulation. Ten days after pyridostigmine bromide withdrawal, the second electromyography examination was carried out and showed that the afterdischarges on all nerves disappeared dramatically and the amplitude of tibial nerve F waves was elevated than before.
CONCLUSIONS: Afterdischarges can be an important indicator of muscle-specific tyrosine kinase antibody positive myasthenia gravis with peripheral nerve hyperexcitability correlated with acetylcholinesterase inhibitors.

Myasthenia gravis (MG) is an autoimmune disease characterised by the presence of acetylcholine receptor antibodies and by blocking the transmission of the signal in the neuromuscular junction causing muscle weakness. It can be associated with several autoimmune diseases and certain drugs, between them Etanercept an anti-tumour necrosis factor (TNF) agent. A 42-year-old woman with rheumatoid arthritis (RA) refractory to methotrexate, was treated with adalimumab (ADA), a human monoclonal antibody against the TNF, in a dosage scheme of 40 mg every 14 days subcutaneously. The patient responded well to ADA therapy with sustained remission for 18 months when she developed blurred vision and eyelid ptosis of the left eye. The diagnosis of ocular MG was made. ADA has been discontinued and she started a treatment with pyridostigmine showing an excellent response and complete remission within a 2-month period. This is the first report making an association of ADA and ocular MG. Thus, rheumatologists dealing with patients treated with TNF inhibitors should be aware of the possible development of neurological adverse events, among them MG.

暂无摘要。

Pediatric chronic intestinal pseudo-obstruction is a rare disorder characterized by a severe impairment of gastrointestinal motility leading to intestinal obstruction symptoms in the absence of mechanical causes. The diagnosis is usually clinical and diagnostic work is usually aimed to rule out mechanical obstruction and to identify any underlying diseases. Treatment is challenging and requires a multidisciplinary effort. In this manuscript we describe the youngest child successfully treated with the orally administrable, longacting, reversible anti-cholinesterase drug, pyridostigmine. Like other drugs belonging to cholinesterase inhibitors, pyridostigmine enhances gut motility by increasing acetylcholine availability in the enteric nervous system and neuro-muscular junctions. Based on the direct evidence from the reported case, we reviewed the current literature on the use of pyridostigmine in severe pediatric dysmotility focusing on intestinal pseudo-obstruction. The overall data emerged from the few published studies suggest that pyridostigmine is an effective and usually well tolerated therapeutic options for patients with intestinal pseudo-obstruction. More specifically, the main results obtained by pyridostigmine included marked reduction of abdominal distension, reduced need of parenteral nutrition, and improvement of oral feeding. The present case and review on pyridostigmine pave the way for eagerly awaited future randomized controlled studies testing the efficacy of cholinesterase inhibitors in pediatric severe gut dysmotility.

BACKGROUND: The inmuno checkpoints inhibitors are new revolutionary treatment for many neoplastic diseases in advanced stadium. There are described several types of neurological complications induced by nivolumab: polyneuropathy, seizures, radiculitis and myasthenia gravis disease.
METHODS: A 65 years old man with metastatic lung adenocarcinoma who presented myasthenia gravis disease induced by avelumab therapy with good response to treatment with pyridostigmine and withdrawal of avelumab.
CONCLUSIONS: The exact mechanism by which this drug induces myasthenia gravis is still unknown and there is probably a different pathophysiological process to idiopathic myasthenia gravis. An important fact is the variability in the time of onset of myasthenia gravis after initiating treatment with inmuno checkpoints inhibitors. From the clinical point of view, most of the reported cases appeared with a generalized form of myasthenia gravis with bulbar involvement and later developed ophthalmoparesis and fluctuating palpebral ptosis. Our case as well as the review of the previous literature can be useful to alert the clinical neurologist about the possibility of the development of immune-mediated cases of this nature induced by the treatment with avelumab in clinical practice as well as to guide its clinical, prognostic and clinical characteristics.
METHODS:
BACKGROUND: Miastenia grave inducida por tratamiento con inhibidores del punto de control inmunologico: primer caso secundario a avelumab y revision de casos previamente publicados.
Introduccion. Los farmacos inhibidores del punto de control inmunologico han supuesto una revolucion en el tratamiento de varios procesos neoplasicos en estadio avanzado. Sin embargo, se han descrito numerosas complicaciones neurologicas, entre las que se encuentran polineuropatias, crisis epilepticas, radiculitis y miastenia grave. Caso clinico. Varon de 65 años con adenocarcinoma de pulmon en estadio IV en tratamiento con avelumab que desarrolla una miastenia grave ocular seropositiva, con buena respuesta a la piridostigmina y retirada de la medicacion. Conclusiones. El mecanismo exacto por el cual el avelumab induce una miastenia grave aun se desconoce, y probablemente existe un proceso fisiopatologico diferente al de la miastenia grave idiopatica. Un dato importante es la variabilidad en cuanto al tiempo de aparicion de la miastenia grave despues de iniciar el tratamiento con inhibidores del punto de control inmunologico. Desde el punto de vista clinico, la mayoria de los casos descritos comenzo con una forma de miastenia grave generalizada con afectacion bulbar que posteriormente desarrollo oftalmoparesia y ptosis palpebral fluctuante. Este caso, asi como la revision de la bibliografia, puede ser util para alertar al neurologo clinico sobre la posibilidad del desarrollo de cuadros inmunomediados de esta naturaleza inducidos por el tratamiento con avelumab en la practica clinica y para orientar sus caracteristicas clinicas, pronosticas y de tratamiento.