Acute exacerbation of myasthenia gravis with topical imiquimod use.

We report a patient with a history of well-controlled myasthenia gravis who was prescribed imiquimod for lentigo maligna. Treatment was halted after 2 weeks when the patient reported itching and irritating sensations in his throat, consistent with previous myasthenia exacerbations. The symptoms improved once imiquimod use was discontinued. We advise clinicians to be cautious when prescribing imiquimod to a patient with a history of myasthenia gravis. PMID: 28670076 [PubMed - in process]
Source: Baylor University Medical Center Proceedings - Category: Universities & Medical Training Authors: Tags: Proc (Bayl Univ Med Cent) Source Type: research

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Conclusions Stimulating the agrin-LRP4-MuSK-DOK7 AChR clustering pathway with NSC-87877, or other drugs, could represent a novel therapeutic approach for MuSK-MG and could potentially improve other NMJ disorders with reduced AChR numbers or disrupted NMJs.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Myasthenia Article Source Type: research
Myasthenia gravis (MG) with antibodies to muscle-specific kinase (MuSK) is an autoimmune disease of the neuromuscular junction with some remarkable characteristics. Although MuSK is expressed in all skeletal muscles, patients with this disease present with particularly cranial, bulbar, and respiratory muscle weakness, leading to a relatively high frequency of respiratory crises.1 Despite several available immunosuppressive or immunomodulatory treatments, patients with MuSK MG often retain residual muscle weakness and experience more often severe muscle atrophy than patients with the more frequent MG with acetylcholine rece...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Myasthenia Editorial Source Type: research
Dropped head syndrome (DHS) can be the presenting feature of a wide spectrum of neurological conditions affecting the central and peripheral nervous systems, and can be due to underlying neck extension weakness or dystonia. Among central disorders, DHS can be seen in patients with parkinsonism (Parkinson disease or multiple system atrophy) where it can be due to underlying dystonia, myopathy, or a combination of both. [1,2] Among neuromuscular disorders, DHS can be the presenting symptom of an underlying motor neuron disease, defect of neuromuscular transmission (including myasthenia gravis, congenital myasthenia and Lambe...
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction. Approximately 9% of MG patients have autoantibodies targeting the muscle specific kinase (MuSK), and are challenging therapeutically, since they often present with more severe symptoms. A useful therapy is plasmapheresis, but it is highly non-specific. Antigen-specific immunoadsorption would only remove the pathogenic autoantibodies, minimizing the possible side effects and maximizing the benefit. We used rats with human MuSK-induced experimental autoimmune MG to perform antigen-specific immunoadsorptions, and found it very effective, res...
Source: Journal of Neuroimmunology - Category: Allergy & Immunology Authors: Source Type: research
ConclusionA small subgroup of patients with generalized myasthenia gravis do not respond sufficiently to standard therapies. Refractory disease has considerable implications for both patients and health care providers and highlights an unmet need for new treatment options.
Source: Journal of Neurology - Category: Neurology Source Type: research
We describe 2 children from unrelated families who presented with hypotonia, ptosis and fatigability in early infancy with anti-AChR antibodies detected via ELISA on 2 separate occasions in the sera. Both were treated as refractory autoimmune MG due to poor clinical response to acetylcholinesterase inhibitor and immunotherapy. In view of the atypical clinical features, genetic studies of CMS were performed and both were confirmed to have novel pathogenic mutations in the COLQ gene. To the best of our knowledge, the presence of anti-AChR antibody in COLQ-related CMS has never been reported in the literature. The clinical pr...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
Immune checkpoint inhibitors (ICIs) can cause adverse reactions in the nervous system. The incidence rate is 0.1% –12% and 80% of nervous system adverse reactions occur within the first four months of application. ICIs can cause diseases of various parts of the nervous system including central nervous system diseases such as aseptic meningitis, meningeal encephalitis, necrotizing encephalitis, brainstem encep halitis, transverse myelitis, etc., and peripheral neuropathy such as cranial nerve peripheral neuropathy, multifocal nerve root neuropathy, Guillain‐Barré syndrome, spinal nerve root neuropathy, myasth...
Source: Thoracic Cancer - Category: Cancer & Oncology Authors: Tags: CLINICAL GUIDELINE Source Type: research
We thank Finsterer and associates for their interest in our study and for giving valuable comments.
Source: American Journal of Ophthalmology - Category: Opthalmology Authors: Tags: Correspondence Source Type: research
Juvenile myasthenia gravis (JMG) is an antibody mediated autoimmune disorder that manifests as progressive voluntary muscle weakness and fatigue. In medically refractory cases, thymectomy has been shown to abrogate symptoms and reduce glucocorticoid dependence. While transcervical or transsternal incisions have been the traditional approach, adult trends now favor thoracoscopic thymectomy. Little data exist to support this approach in children.
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Source Type: research
Complement-dependent disruption of motor endplate is detected in anti-acetylcholine receptor (AChR) antibody-positive myasthenia gravis (MG). We measured serum AChR α1 subunit protein levels, which may be associated with neuromuscular damage, in 55 patients with MG (47 were seropositive and 8 were negative) and in 20 controls. Serum AChR α1 subunit protein concentrations were higher in patients with anti-AChR antibody-positive MG than those in controls (P =  .04), were negatively correlated with MG activities of daily living score (P = .01), and tended to be higher in ocular MG than in generalized MG.
Source: Journal of Neuroimmunology - Category: Allergy & Immunology Authors: Tags: Short Communication Source Type: research
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