Cervical Dystonia
The most likely diagnosis in this 54-year-old woman with progressive neck pain, persistent leftward head tilt, and head tremor that worsens with rightward head turning is cervical dystonia. 1, 2
Clinical Features Supporting Cervical Dystonia
This patient demonstrates the classic triad of cervical dystonia:
- Abnormal head posture (leftward tilt/laterocollis) that is sustained and involuntary 1, 3
- Head tremor that is position-dependent and worsens with attempted movement in the opposite direction (turning right exacerbates the tremor) 1, 4
- Associated neck pain, which occurs in approximately 80% of cervical dystonia patients 4
The progressive nature of symptoms is characteristic, as cervical dystonia typically begins with mild, intermittent movements that become constant over time. 3
Why Other Diagnoses Are Less Likely
Degenerative disc disease would not explain the directional head tilt, position-dependent tremor, or the specific pattern of symptom exacerbation with head turning. While DDD can cause neck pain, it does not produce sustained abnormal head postures or tremor. 5
Cranial nerve VI palsy affects eye movement (abduction deficit causing horizontal diplopia and esotropia), not neck positioning or head tremor. Patients with sixth nerve palsy may adopt a compensatory head turn to reduce diplopia, but this is voluntary and would not produce tremor or progressive neck pain. 6
Essential tremor produces bilateral, symmetric tremor typically affecting the hands, head (as "no-no" or "yes-yes" tremor), or voice. It would not cause sustained abnormal head posture (laterocollis) or position-dependent exacerbation with specific directional movements. 1
Cervical myopathy would present with muscle weakness, difficulty holding the head upright (head drop), and fatigue rather than involuntary sustained posturing and tremor. 1
Diagnostic Approach
The diagnosis of cervical dystonia is primarily clinical, based on recognition of characteristic symptoms and signs:
- Dystonic movements are typically spasmodic, repeated in a stereotyped pattern, and may be tonic, clonic, or tremulous 1, 3
- Range of voluntary neck movement is often reduced despite the involuntary movements 3
- Sensory tricks (geste antagonistique) may temporarily ameliorate symptoms—patients often touch their chin, back of head, or top of head to reduce dystonic posturing 1
MRI of the cervical spine should be obtained to exclude secondary causes such as structural lesions, neurodegenerative disease, or trauma, particularly given the progressive nature and associated pain. 7, 8 However, in primary (idiopathic) cervical dystonia—which represents the majority of cases—neuroimaging will be normal. 1, 2
Management
Botulinum toxin (both type A and type B) injected into affected neck muscles is the first-line treatment and most widely accepted therapy for cervical dystonia. 1, 2, 4 This approach:
- Targets pain, dystonic posturing, limited range of motion, and tremor 4
- Shows good efficacy and improved health-related quality of life in the majority of patients, even long-term 3
- Has fewer side effects compared to oral medications 4
Oral pharmacotherapy (anticholinergics, dopamine-depleting agents, muscle relaxants) can be used alone or in combination with botulinum toxin, but lacks high-quality evidence for efficacy. 2
Surgical options (selective peripheral denervation or pallidal deep brain stimulation) should be reserved for patients with inadequate response to botulinum toxin and medical management. 2, 3
Important Clinical Pearls
- Cervical dystonia can present with pain and muscle hypertrophy even before obvious abnormal movements emerge in some cases 9
- The combination of laterocollis with tremor that worsens with contralateral head turning is highly specific for cervical dystonia 1
- Approximately 20% of patients may experience spontaneous remissions, though most last less than one year 4