Dropped Head Syndrome: The Importance of Neurophysiology in Distinguishing Myasthenia Gravis from Parkinson’s Disease
Abstract
1. Introduction
2. Detailed Case Description
3. Discussion
4. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Condition | Diagnosis | Clinical Signs | Notes | References |
---|---|---|---|---|
Dropped head syndrome | ||||
Myasthenia Gravis | EMG, Ach/mUSK antibodies | Dysphagia, dysphonia, weakness, fluctuating fatigue | Responds to pyridostigmine and plasmapheresis | [5,16] |
Motor Neuron Disease (ALS, PPS) * | EMG | Hyperreflexia, Babinski, weakness, fasciculations | Can have dysphonia and dysphagia | [3] |
Myopathy (PM, IBM, NM, MM, CD, FSHD, Radiation-induced, AED-induced carnitine deficiency) | Muscle biopsy | Weakness | LGMD, FSHD, IBM, other myositis, amyloid, thyroid myopathy, pay attention to epileptic patients taking valproic acid | [3,17] |
IGLON-5 Disease | IGLON-5 antibodies | Weakness, bulbar symptoms, sleep disorders | Tauopathy, rarely responds to immune therapy, severe prognosis | [18] |
Antecollis | ||||
Parkinson’s Disease | Clinical | Bradykinesia plus at least one between tremor, rigidity, postural instability | Responds to levodopa/carbidopa | [6] |
Multiple System Atrophy | Clinical | Dysautonomia, atypical Parkinsonism, cerebellar symptoms | More frequently displays antecollis than idiopathic PD | [7] |
Idiopathic Cervical Dystonia * | Clinical | Sensory trick | Responds to botulinum toxin injections in anterior neck muscle groups and anticholinergic drugs | [19] |
DPPX-Encephalopathy | DPPX antibodies | Weight loss, dysautonomia, GI symptoms | Responds to immune therapies | [20] |
Condition | Treatment | Notes |
---|---|---|
Dropped head syndrome | ||
Myasthenia Gravis | IVIG, PLEX, Steroids | May improve/resolve |
Motor Neuron Disease (ALS, PPS) | - | Consider neck brace, treat dysphagia if associated |
Myopathy (PM, IBM, NM, MM, CD, FSHD, Radiation-induced, and AED-induced carnitine deficiency) | Consider steroids in responsive forms; discontinue valproic acid in epileptic patients | Consider neck brace, physical therapy |
IGLON-5 Disease | IVIG, PLEX, Steroids | Weak response |
Antecollis | ||
Parkinson’s Disease | Discontinue dopamine-agonists or other recently added treatments; consider levodopa increase and muscle relaxants | Physical therapy, consider BoNT injections in anterior neck muscles |
Multiple System Atrophy | Same as Parkinson’s disease | - |
Idiopathic Cervical Dystonia | Consider BoNT injections in anterior neck muscles and anticholinergics | Effective, but increased risk of dysphagia |
DPPX-Encephalopathy | IVIG, PLEX, Steroids | May improve |
Treatment | Description | Mechanism of Action | Notes |
---|---|---|---|
Acetylcholinesterase inhibitors | Medications that improve communication between nerves and muscles | Inhibit breakdown of acetylcholine, increasing its availability at neuromuscular junctions | First-line treatment; includes pyridostigmine. May cause gastrointestinal side effects |
Corticosteroids | Anti-inflammatory drugs used to suppress immune response | Reduce autoantibody production and inflammation | Effective for moderate to severe MG; long-term use can cause significant side effects like osteoporosis and diabetes |
Immunosuppressants | Drugs that suppress the immune system to reduce autoantibody production | Target immune cells to decrease autoantibody levels | Include azathioprine, mycophenolate mofetil, and cyclosporine. Regular monitoring required due to risk of infection and other side effects |
Plasma exchange | Procedure aiming to remove antibodies from the blood. | Direct removal of circulating autoantibodies | Used in acute exacerbations or crisis situations. Short-term relief; risk of complications like infection or blood clots |
Intravenous immunoglobulins (IVIG) | Infusion of antibodies from donated blood to modulate the immune system | Provides normal antibodies to alter immune response and neutralize autoantibodies | Used in acute exacerbations or as maintenance therapy. High cost and risk of allergic reactions |
Thymectomy | Surgical removal of the thymus | Potentially reduces the production of autoantibodies by removing a source of abnormal immune response | May lead to remission or reduced medication requirement; most effective in patients with thymoma or early-onset MG |
Monoclonal antibodies | Targeted biologic therapy that specifically inhibits components of the immune system | Block specific immune pathways, such as complement activation or B-cell function | Include rituximab and eculizumab. Used in refractory cases; expensive and require careful monitoring |
Novel/experimental therapies | |||
- Efgartigimod | Neonatal Fc receptor (FcRn) antagonist | Reduces levels of pathogenic IgG antibodies | Demonstrates efficacy in clinical trials; may cause headache, upper respiratory infections |
- Rozanolixizumab | Humanized monoclonal antibody targeting the neonatal FcRn | Lowers circulating IgG autoantibodies by blocking FcRn | Under investigation; shown promising results in reducing symptoms. Infusion-related reactions possible |
Condition | RNS Findings | Clinical Features | Diagnostic Tests | Notes |
---|---|---|---|---|
Myasthenia Gravis (MG) | Decremental response in CMAP amplitude, often U-shaped | Fluctuating muscle weakness, fatigability, dysphagia, dysphonia | Acetylcholine receptor antibodies, SFEMG | High specificity with RNS; SFEMG for inconclusive cases |
Lambert-Eaton Myasthenic Syndrome (LEMS) | Incremental response in CMAP amplitude upon high-frequency stimulation | Proximal muscle weakness, autonomic dysfunction | Voltage-gated calcium channel antibodies | CMAP amplitude increases after exercise or high-frequency stimulation |
Amyotrophic Lateral Sclerosis (ALS) | Reduced CMAP amplitude, no decremental pattern | Progressive muscle weakness, atrophy, fasciculations | EMG, clinical examination | ALS affects both upper and lower motor neurons |
Botulism | Decremental response in CMAP amplitude, improvement with rapid RNS | Acute onset of muscle weakness, cranial nerve involvement | Toxin detection in serum, stool, or food | Requires prompt diagnosis and treatment |
Congenital Myasthenic Syndromes (CMS) | Decremental response in CMAP amplitude | Early onset muscle weakness, often with ocular involvement | Genetic testing, SFEMG | Genetic forms of neuromuscular junction disorders |
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Mangiardi, M.; Magliozzi, A.; Colosimo, C.; Marsili, L. Dropped Head Syndrome: The Importance of Neurophysiology in Distinguishing Myasthenia Gravis from Parkinson’s Disease. Biomedicines 2024, 12, 1833. https://doi.org/10.3390/biomedicines12081833
Mangiardi M, Magliozzi A, Colosimo C, Marsili L. Dropped Head Syndrome: The Importance of Neurophysiology in Distinguishing Myasthenia Gravis from Parkinson’s Disease. Biomedicines. 2024; 12(8):1833. https://doi.org/10.3390/biomedicines12081833
Chicago/Turabian StyleMangiardi, Marilena, Alessandro Magliozzi, Carlo Colosimo, and Luca Marsili. 2024. "Dropped Head Syndrome: The Importance of Neurophysiology in Distinguishing Myasthenia Gravis from Parkinson’s Disease" Biomedicines 12, no. 8: 1833. https://doi.org/10.3390/biomedicines12081833
APA StyleMangiardi, M., Magliozzi, A., Colosimo, C., & Marsili, L. (2024). Dropped Head Syndrome: The Importance of Neurophysiology in Distinguishing Myasthenia Gravis from Parkinson’s Disease. Biomedicines, 12(8), 1833. https://doi.org/10.3390/biomedicines12081833