Myasthenia gravis, a neuromuscular disorder, typically emerges later in life due to antibodies attacking muscle receptors, hindering essential chemical signals for muscle contraction. Its symptoms range from visual disturbances like drooping eyelids and double vision to muscle weakness and fatigue, often exacerbating with muscle use. Diagnosis relies on medical history, physical exams, and tests like blood tests and electromyograms to assess muscle activity.

While there's no cure for myasthenia gravis, treatments aim to manage symptoms and may include medications like anticholinesterase drugs, immunosuppressive therapies, or surgical interventions like thymectomy. Complications, notably myasthenia crisis marked by severe muscle weakness, particularly in respiratory muscles, demand prompt medical attention. Strategies to prevent crises involve medication adherence, avoiding infections, maintaining proper nutrition and activity levels, and managing stress. Early detection and proactive management are crucial for improving outcomes in individuals with myasthenia gravis.

Reference: Myasthenia Gravis. Accessed April 25, 2024. Johns Hopkins Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases/myasthenia-gravis

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder marked by chronic inflammation and progressive damage to the myelin sheath of peripheral nerves, causing muscle weakness, sensory loss, and diminished reflexes. It presents in various forms, including typical CIDP, distal acquired demyelinating symmetric neuropathy, and multifocal variants. Diagnosis involves clinical assessments, electrodiagnostic studies, and cerebrospinal fluid analysis, with imaging or nerve biopsy reserved for complex cases. Treatment focuses on immunomodulatory therapies like intravenous immunoglobulin, corticosteroids, or plasmapheresis to manage symptoms and limit nerve damage.

CIDP affects 0.67 to 10.3 per 100,000 people, more often men, typically with onset between ages 40 and 60. While treatments are effective, challenges remain with adherence, relapses, and variable patient responses. Research is advancing with emerging biologics such as neonatal Fc receptor inhibitors and complement inhibitors. Prognosis varies, with some patients achieving remission, though ongoing management is crucial to prevent relapses and disability. Continued research aims to enhance diagnostics and develop therapies tailored to individual immune responses.

Reference: Gonzalez Caldito N, Habib AA. Diagnosis of chronic inflammatory demyelinating polyneuropathy. Practical Neurology (US). 2024;23(3):19-25.

Vasculitic neuropathy (VN) involves the peripheral nervous system and can be isolated (NSVN) or part of a systemic disease (SVN). VN often presents as mononeuritis multiplex, characterized by asymmetric dysesthesias, paresthesias, and palsies. Nerve biopsy studies suggest NSVN accounts for about 28% of VN cases. VN can present in various forms, including distal sensorimotor polyneuropathy and plexopathy, and is often underdiagnosed due to its heterogeneous clinical phenotypes.

Diagnosis of VN involves a thorough medical history, physical and neurological examinations, and extensive testing to identify systemic involvement. Nerve conduction studies and EMG help reveal the neuropathy pattern and guide biopsy site selection, with nerve biopsy confirming the diagnosis. Treatment primarily involves immunosuppression, with glucocorticoid monotherapy for mild cases and combination therapy for aggressive or progressive NSVN. Early and aggressive treatment, along with physical rehabilitation, improves prognosis. Further research is needed to understand VN's natural history, treatment, and pathogenic mechanisms.

Reference: Witt A, Kodal L, Dysgaard T. Diagnosis and Treatment of Vasculitic Neuropathy. Practical Neurology. Published April 5, 2024. Accessed May 21, 2024. https://practicalneurology.com/articles/2024-apr/diagnosis-and-treatment-of-vasculitic-neuropathy?c4src=home:feed