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Dr. Dr. Jitendra Prasad Yadav • Consultant Neurologist & Neuromuscular Specialist • NMC 8029
Drooping eyelids, double vision, weakness worsening with activity? You may have myasthenia gravis (MG) — an autoimmune disease affecting nerve-muscle communication. Dr. Jitendra Prasad Yadav (NMC 8029), Consultant Neurologist with 15+ years at National Trauma Center (NAMS), provides MG diagnosis, pyridostigmine management, and immunotherapy at Kathmandu Neurology Clinic & Cognitive Center, Durbar Marg, Opposite of Yak & Yeti Hotel, Kathmandu.
Nepal Medical Council Reg. No. 8029 • 15+ years National Trauma Center, NAMS • Part of Clinics Tree Healthcare Network
What is Myasthenia Gravis?
Myasthenia gravis disrupts nerve-to-muscle signaling, causing weakness that worsens with activity and improves with rest.
In myasthenia gravis, the immune system produces antibodies against components of the neuromuscular junction — the site where nerve endings communicate with muscle fibers. In most cases (80-85%), antibodies target acetylcholine receptors (AChR) on muscle membranes. When acetylcholine (the neurotransmitter released by nerves) tries to bind these receptors to trigger muscle contraction, the antibodies block, damage, or destroy the receptors, impairing signal transmission. Result: muscles receive inadequate signals and become weak, especially with sustained or repeated activity.
Key characteristic: Fluctuating weakness — worsens with activity (fatigability), improves with rest. Symptoms vary day-to-day and hour-to-hour.
Ocular MG (50% present with eye symptoms only; 15% remain ocular-only):
Generalized MG (85% eventually develop generalized symptoms):
Pattern recognition: Symptoms worsen late in day, with sustained activity, in heat; improve with rest, sleep, cool temperature.
Clinical examination:
Serologic testing:
Electrodiagnostic testing:
Edrophonium (Tensilon) test: IV injection of short-acting acetylcholinesterase inhibitor → rapid improvement in weakness if MG. Risk of bradycardia (requires atropine available). Less commonly used now due to antibody and electrodiagnostic test availability.
Imaging: Chest CT to detect thymoma (10-15% of MG patients) or thymic hyperplasia (60-70%). Thymectomy is therapeutic for thymoma (mandatory) and may benefit non-thymomatous MG.
1. Symptomatic treatment — Pyridostigmine:
Acetylcholinesterase inhibitor — prevents acetylcholine breakdown at neuromuscular junction, prolonging its action and improving muscle strength. Dose: 60 mg TID-QID (every 4-6 hours while awake), titrate based on response (max ~120 mg per dose, 600 mg/day). Onset 30-60 minutes, duration 3-4 hours. Side effects: cholinergic — abdominal cramps, diarrhea, increased salivation, muscle twitching. Provides rapid symptom relief but does NOT alter disease course or prevent progression.
2. Immunosuppression:
3. Thymectomy:
Surgical removal of thymus gland. Indications: thymoma (mandatory — risk of malignancy), non-thymomatous MG with AChR antibodies, age <65 years, generalized symptoms. Benefits: higher remission rates, reduced medication requirements, long-term disease improvement. Best outcomes when performed early in disease course. Performed by cardiothoracic surgery (sternotomy or minimally invasive approach). Available at major hospitals in Kathmandu (TUTH, private hospitals).
4. Acute exacerbations and crisis management:
Some medications worsen neuromuscular transmission and can precipitate crisis:
Always inform doctors/dentists of MG diagnosis before prescribing medications.
With treatment, most MG patients achieve good symptom control and maintain normal or near-normal quality of life. Remission (off all medications) occurs in 20-30%, more likely after thymectomy. Most patients require long-term immunosuppression. Myasthenic crisis risk decreases with optimal therapy but remains lifelong concern — patients and families should recognize warning signs (worsening weakness, breathing difficulty) and have emergency plan.
Long-term monitoring: regular neurology follow-up (every 3-6 months when stable), antibody level checks (not routine, but may guide therapy), pulmonary function testing (forced vital capacity — assess respiratory muscle strength), medication side effect monitoring (CBC, LFTs for immunosuppressants; glucose, bone density for steroids).
Seek immediate hospital evaluation for: difficulty breathing or shortness of breath at rest, inability to swallow (choking, drooling), severe sudden worsening of weakness, inability to lift head or stand. These suggest myasthenic crisis requiring ICU care, intubation, and IVIG/plasmapheresis.
Dr. Jitendra Prasad Yadav (NMC 8029), Consultant Neurologist with 15+ years at National Trauma Center (NAMS), provides:
Book MG Consultation
Kathmandu Neurology Clinic & Cognitive Center
Durbar Marg, Opposite of Yak & Yeti Hotel, Kathmandu 44600, Nepal
Phone: 974-7736624 • +977 9747978200
Sun-Sat 9AM-7PM • By Appointment • Near Narayanhiti Palace Museum • 27.7172, 85.3240
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Emergency Warning
Seek emergency care immediately for difficulty breathing, severe swallowing difficulty, or rapid worsening of weakness — these suggest myasthenic crisis requiring ICU care and mechanical ventilation.
Page Information
Myasthenia gravis is a chronic autoimmune neuromuscular disease where antibodies attack acetylcholine receptors (or related proteins) at the neuromuscular junction, disrupting nerve-to-muscle signaling. This causes fluctuating muscle weakness that worsens with activity and improves with rest. Common symptoms: drooping eyelids (ptosis), double vision (diplopia), difficulty chewing/swallowing, limb weakness. Respiratory muscle weakness (myasthenic crisis) is life-threatening. MG is treatable with acetylcholinesterase inhibitors (pyridostigmine), immunosuppression (steroids, azathioprine), and in severe cases, thymectomy or plasmapheresis/IVIG.
Fluctuating weakness worsening with activity, improving with rest. Ocular MG (50% at onset): drooping eyelids (ptosis), double vision (diplopia). Generalized MG: facial weakness (difficulty smiling, chewing, facial expression), bulbar weakness (swallowing difficulty, choking, nasal speech, jaw fatigue while chewing), limb weakness (difficulty lifting arms, climbing stairs — proximal muscles most affected), neck weakness (head drop). Myasthenic crisis: severe weakness affecting respiratory muscles — shortness of breath, shallow breathing, respiratory failure requiring ventilation. Fatiguability is key — weakness worsens throughout day or with sustained activity.
Clinical exam: fatigable weakness (sustained upgaze causes ptosis, sustained arms outstretched causes dropping). Ice pack test: ice over closed eye for 2 minutes improves ptosis (suggests MG). Serologic: acetylcholine receptor (AChR) antibodies (80-85% generalized MG), MuSK antibodies (5-10%, more severe, seronegative MG), LRP4 antibodies (rare). Electrodiagnostic: repetitive nerve stimulation (RNS) shows >10% decrement in muscle response amplitude with low-frequency stimulation. Single-fiber EMG (most sensitive test, >95%, but less available). Edrophonium (Tensilon) test — short-acting acetylcholinesterase inhibitor; improvement after IV injection suggests MG (risk of bradycardia, not first-line now). Imaging: chest CT to detect thymoma (10-15% of MG patients) — thymectomy is indicated.
Autoimmune — antibodies against neuromuscular junction components. AChR antibodies (most common) block, damage, or complement-mediate destruction of receptors. MuSK antibodies (muscle-specific kinase) impair receptor clustering. Thymus abnormalities common: thymic hyperplasia (60-70%) or thymoma (10-15%). Thymectomy improves outcomes, especially in young patients with AChR-positive MG. Triggers for exacerbation: infections, surgery, certain medications (aminoglycosides, quinolones, beta-blockers, magnesium), emotional stress, pregnancy/postpartum.
Symptomatic: pyridostigmine (acetylcholinesterase inhibitor) 60mg TID-QID, titrate to effect; provides immediate symptom relief but does not alter disease course. Immunosuppression: prednisone (start low, increase gradually to avoid initial worsening), azathioprine (steroid-sparing agent, takes 6-12 months for full effect), mycophenolate mofetil (alternative steroid-sparing). Thymectomy: for thymoma (mandatory) or non-thymomatous MG <65 years with AChR antibodies (improves long-term outcomes). Acute exacerbations/crisis: plasmapheresis (plasma exchange) or IVIG — rapidly reduce circulating antibodies. Respiratory support: ICU admission, intubation/ventilation if respiratory failure. Avoid triggering medications.
Life-threatening respiratory failure due to severe MG exacerbation. Symptoms: severe weakness, difficulty breathing/swallowing, inability to protect airway, respiratory distress. Triggers: infection (most common), medication non-adherence, surgery, certain drugs. Management requires ICU admission: intubation/mechanical ventilation, plasmapheresis or IVIG, increased immunosuppression, infection treatment. Mortality has decreased with ICU care but remains significant (~5%). Patients with bulbar or respiratory involvement at baseline are at higher risk.
No cure, but MG is highly manageable. Many patients achieve remission or minimal symptoms with treatment. Goals: symptom control with minimal medication side effects, prevent crises, maintain quality of life. Thymectomy (for appropriate patients) improves long-term outcomes and may allow immunosuppression reduction. Some patients eventually taper off medications, though relapse is possible. Lifelong monitoring is required.
Kathmandu Neurology Clinic & Cognitive Center, Durbar Marg, Opposite of Yak & Yeti Hotel, Kathmandu 44600 — led by Dr. Jitendra Prasad Yadav (NMC 8029), Consultant Neurologist with 15+ years at National Trauma Center (NAMS). Services: MG diagnosis (clinical exam, antibody testing coordination, RNS/EMG), pyridostigmine prescription and titration, immunosuppression management (prednisone, azathioprine), thymoma screening (chest CT coordination), thymectomy referral (cardiothoracic surgery), acute exacerbation management and hospital coordination (plasmapheresis/IVIG), long-term monitoring (antibody levels, pulmonary function, medication side effects). Book via /book-appointment or call 974-7736624, Sun-Sat 9AM-7PM by appointment. Bring prior tests, imaging, medication list.
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