Can Meige syndrome be associated with dropped neck (cervical dystonia) in a patient with a history of facial myokymia and potential neurological symptoms?

Can Meige Syndrome Be Associated With Dropped Neck?

Yes, Meige syndrome can extend to involve cervical musculature, including neck dystonia, though this represents an evolution of the cranial dystonia rather than classic "dropped head syndrome."

Understanding Meige Syndrome and Cervical Involvement

Meige syndrome is a segmental cranial dystonia characterized by blepharospasm and oromandibular dystonia that frequently extends to include cervical muscle groups 1, 2, 3, 4. The syndrome can evolve beyond its core craniofacial manifestations to involve adjacent cervical musculature 2, 3.

Clinical Presentation of Cervical Involvement

• Cervical dystonia is a recognized component of Meige syndrome, occurring as the disorder progresses from isolated blepharospasm to involve orofacial and then cervical musculature 2, 4
• The cervical involvement typically manifests as dystonic spasms of neck muscles rather than pure weakness 3, 4
• Spasms of neck muscles are generally mild when they accompany the cranial dystonia 3

Important Distinction: Cervical Dystonia vs. Dropped Head Syndrome

Cervical Dystonia in Meige Syndrome

• Characterized by painful dystonic spasms of cervical muscles with abnormal posturing 5
• Results from the same pathophysiologic process affecting basal ganglia or mesencephalic/diencephalic regions 3
• Presents with active muscle contraction and spasm rather than weakness 4

True Dropped Head Syndrome

• Dropped head syndrome is a distinct entity caused by weakness of neck extensor muscles resulting in inability to extend the neck 5
• This condition is most commonly seen as a rare delayed complication of high-dose mantle-field radiotherapy for conditions like Hodgkin lymphoma, manifesting decades after treatment 5
• The pathophysiology involves primary muscle damage and nerve damage, not dystonia 5
• This condition is irreversible and debilitating, managed only with supportive care 5

Clinical Assessment Algorithm

When evaluating a patient with Meige syndrome and neck symptoms:

1. Determine the nature of neck involvement:

   • Is there active dystonic spasming and abnormal posturing? → Cervical dystonia (part of Meige syndrome) 5
   • Is there pure weakness with inability to hold head up against gravity? → Consider alternative diagnosis 5

2. Assess for radiation history:

   • History of high-dose neck/chest radiation? → Consider dropped head syndrome as separate entity 5
   • No radiation history? → Cervical involvement likely part of dystonic syndrome 2, 4

3. Evaluate associated features:

   • Presence of blepharospasm and oromandibular dystonia confirms Meige syndrome 1, 3, 4
   • Look for upper motor neuron signs, which can occasionally coexist 2

Management Approach for Cervical Involvement in Meige Syndrome

First-Line Treatment

• Botulinum toxin type A injections into affected cervical muscles for pain management and spasm control 5, 6
• Referral to rehabilitation specialist for comprehensive neuromusculoskeletal management 5, 6

Adjunctive Medical Management

• Nerve-stabilizing agents including pregabalin, gabapentin, or duloxetine for pain and spasm control 5, 6
• GABA antagonists, dopamine antagonists, and anticholinergics for short-term management 2

Advanced Treatment Options

• Bilateral globus pallidus internus (GPi) deep brain stimulation for refractory cases, which has shown 75-84% improvement in dystonia symptoms including cervical involvement at long-term follow-up 1, 4
• GPi-DBS produces sustained improvement in both movement and disability scores 1, 4

Critical Pitfalls to Avoid

• Do not confuse cervical dystonia with dropped head syndrome - they have different pathophysiology, prognosis, and treatment approaches 5
• Avoid splinting in dystonia as it may increase attention to affected area, promote compensatory movements, cause muscle deconditioning, and increase pain 6
• Do not use serial casting for dystonia as it has been associated with worsening symptoms and complex regional pain syndrome 6