Dropped Head Syndrome: Diagnosis and Management
Dropped head syndrome is characterized by severe weakness of the neck extensor muscles resulting in an inability to lift the head from a flexed position, creating a chin-on-chest deformity that significantly impacts quality of life through impaired horizontal gaze and daily functioning. 1
Etiology and Classification
Dropped head syndrome (DHS) can be categorized based on underlying causes:
- Primary/Idiopathic: Isolated neck extensor myopathy without other neurological findings
- Secondary:
- Neuromuscular disorders: Myasthenia gravis, amyotrophic lateral sclerosis
- Radiation-induced: Occurs as a delayed complication of high-dose mantle-field radiotherapy
- Movement disorders: Parkinson's disease
- Inflammatory: Myositis
- Structural: Cervical spine pathology
The radiation-induced form is particularly notable as it can manifest decades after exposure to high-dose mantle-field radiotherapy, especially in Hodgkin lymphoma survivors. It is considered irreversible and thought to result from a combination of primary muscle damage and nerve damage. 2
Clinical Presentation
Key clinical features include:
- Inability to extend the neck against gravity
- Preserved passive range of motion (distinguishing it from fixed deformities)
- Progressive chin-on-chest posture
- Significant impact on horizontal gaze
- Preserved consciousness and awareness (unlike syncope or seizure disorders)
Diagnostic Approach
Clinical assessment:
- Evaluate active neck extension against gravity
- Assess passive range of motion
- Rule out other neurological symptoms
Imaging:
- MRI cervical spine: Most sensitive for detecting soft tissue abnormalities
- Flexion-extension radiographs: To assess stability and mechanical issues
Specialized testing (based on suspected etiology):
- Electromyography and nerve conduction studies
- Muscle biopsy
- Laboratory tests for inflammatory markers
Treatment Options
Treatment should be guided by the underlying cause and severity of symptoms:
Conservative management:
- Physical therapy to improve range of motion and functional capacity
- Neck bracing for temporary support
- Pain management with nerve-stabilizing agents (pregabalin, gabapentin)
Medical treatment (for specific etiologies):
- Myasthenia gravis: Acetylcholinesterase inhibitors, immunosuppression
- Inflammatory myopathies: High-dose glucocorticoids, IVIg, plasma exchange
- Parkinson's disease: Dopaminergic therapy
Surgical intervention:
- Indicated when conservative measures fail or neurological compromise occurs
- Cervicothoracic arthrodesis has significantly higher success rates (87%) compared to cervical arthrodesis alone (29%) 1, 3
- The upper level of fusion is typically C2 in most cases (63%)
- Inclusion of thoracic vertebrae is crucial to prevent failure
Treatment Outcomes
Recent evidence strongly favors surgical intervention for definitive treatment:
- Conservative treatment shows only partial improvement in 59% of cases with total improvement in just 4.45% 4
- Surgical treatment demonstrates total improvement in nearly 100% of cases with very low failure rates 4
- All patients undergoing appropriate surgical intervention show neurological stabilization or improvement 3
Important Considerations
- Radiation-induced dropped head syndrome is generally considered irreversible 1
- Anterior surgical approaches carry higher risk of dysphagia and airway complications (75%) 3
- Regular monitoring of neurological status, horizontal gaze, and functional capacity is essential
- Early surgical intervention should be considered for progressive deformity or neurological compromise
Conclusion
Dropped head syndrome represents a significant challenge with profound impact on quality of life. While conservative measures may provide temporary relief, surgical intervention with cervicothoracic fusion offers the most definitive solution for appropriate candidates, particularly those with progressive or severe deformity.