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Cervical Dystonia Mimics: A Case Series and Review of the Literature


Srinivas Raju,

Department of Neurology, Vydehi Institute of Medical Science & Research Centre, Bangalore, IN
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Amogh Ravi,

Stepping Hill Hospital, Stockport NHS Foundation Trust, Stockport, England, GB
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LK Prashanth

Center for Parkinson’s Disease and Movement Disorders Clinic, Vikram Hospitals, Bangalore; Parkinson’s Disease and Movement Disorders Clinic, Bangalore, IN
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Background: Cervical dystonia is mostly idiopathic in nature. However, a small subset of cases are mimics, leading to diagnostic pitfalls. There is paucity of literature on pseudodystonias affecting the cervical region.

Method: We performed a retrospective review of patients attending a movement disorders clinic over a period of 7 years (2012–2018). Among them, those who were considered to have mimics of cervical dystonia based upon clinical and supportive investigations were included.

Results: Six out of 2,412 patients (0.24%) were diagnosed as cervical dystonia mimics and the causes included isolated neck extensor myopathy (2), craniovertebral junction anomalies (2), sternocleidomastoid fibrosis (1) and post traumatic sequelae (1). Among these patients, three patients had received various treatments for cervical dystonia, including botulinum toxin injections.

Discussion: Mimics of isolated cervical dystonia are rare. A high degree of suspicion and proper diligent clinical assessment assists management and prognostication.

How to Cite: Raju S, Ravi A, Prashanth L. Cervical Dystonia Mimics: A Case Series and Review of the Literature. Tremor and Other Hyperkinetic Movements. 2019;9. DOI:
  Published on 04 Dec 2019
 Accepted on 04 Nov 2019            Submitted on 25 Jun 2019


The 2013 expert consensus panel by Albanese et al. defined dystonia as “a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Dystonic movements are typically patterned, twisting, and may be tremulous. Dystonia is often initiated or worsened by voluntary action and associated with overflow muscle activation.”1 The goal of the revised definition was to exclude possible conditions that may mimic dystonias, also known as “pseudodystonias.” These pseudodystonias are thought to be caused by etiologies that are presumed to be different from the broader dystonia group.1 It is critical to diagnose these pseudodystonias, as the therapy and prognostication is significantly different from that of typical idiopathic dystonia. In this article, we review our cases of cervical pseudodystonias along with a review of the published literature.


The study involved retrospective analysis of subjects attending a movement disorders clinic over a period of 7 years (January 2012 to December 2018). Clinical records of subjects who were diagnosed to have cervical pseudodystonias based on clinical and imaging findings were included in the study. Patients who had pseudodystonia involving other body parts/segments were excluded.


Six out of the 2412 (0.24%) patients who attended the movement disorders clinic were diagnosed with cervical pseudodystonia (Table 1). These patients were evaluated by health care professionals at various levels, including general physicians and neurophysicians. The causes of cervical pseudodystonia included isolated neck extensor myopathy (n = 2), congenital sternocleidomastoid fibrosis (n = 1), Post traumatic craniocervical junction sequelae (n = 1), and developmental craniovertebral junction (CVJ) anomaly (n = 2). The following two cases illustrate the diagnostic dilemmas, leading to various therapeutic interventions.

Table 1

Clinical Features and Imaging Findings of Pseudodystonia Subjects

Video Case 1
Case 2
Case 3 Case 4 Case 5 Case 6
Video 1 Video 2 Video 3
Age at onset 45 Childhood 6 21 11 24
Current age 48 22 6 53 14 24
Gender M M F M M M
Clinical presentation Neck posturing with neck flexion Abnormal posturing of neck since childhood Abnormal posturing of neck following a trauma Posturing of neck since age of 21. Increased in the last 5–6 months with paraspinal spasm Abnormal neck movements Neck posturing and discomfort in left hand (acute onset)
Clinical findings mimicking dystonia Antecollis with facial dystonia Torticollis to the right with minimal retrocollis; left SCM is taut and string like Severe torticollis with right shoulder elevation Mild torticollis to the left present with features of antecollis Jerky neck movements (dystonic jerks vs. akathisia) Lateral shift to the right with right torticaput
Other neurological /clinical findings Neck extensor weakness (MRC grade 1/5) Thin and fibrosed left SCM None Head drop with neck extensor weakness (G3+/5) Dysmorphic features Jerky tremors of hands, bilateral hand grip weakness
MRI findings Fibrosis with fatty infiltration of deep cervical muscles Atrophy and fibrosis of the left SCM. Scoliosis the of cervical spine with convexity to the right side Subluxation of C2-C3 Deep paravertebral muscle atrophy Increased Atlanto dental distance of 100 mm with retroversion of odontoid process. Signal change and thinning of cord at C1-C2 level. Hypertrophied anterior arch of atlas, hypoplastic odontoid process Multiple anomalies in CVJ with atlanto occipital assimilation, partial fusion of C1-C2 vertebrae, hemi vertebra with fusion of right C4-C5 level along with C3-C4 spinal cord hyperintensities
Interventions done before diagnosis Anticholinergics, Benzodiazepines, Dopamine blocker, Botulinum toxin, none Two sittings of botulinum toxin injections given. Anticholinergics None None Benzodiazepines None
Final diagnosis Isolated neck extensor myopathy Muscular fibrosis Posttraumatic subluxations Isolated neck extensor myopathy Atlanto axial dislocation CVJ anomaly
Intervention after diagnosis Medical management and supportive care Option for surgical release given. Patient opted for no intervention Surgical corrections Medical management and supportive care Surgical correction Surgical referral

Abbreviations: CVJ, Craniovertebral Junction; MRC, ; SCM, Sternocleidomastoid Muscle.

Case 1

A 48-year-old gentleman presented with a 2.5-year history of abnormal posturing of the neck. The symptoms started gradually, reaching a peak at around 5 months after onset. He was diagnosed with cervical dystonia and was treated with an anticholinergic, dopa agonists, and benzodiazepines without any benefit. During this course, he underwent repetitive nerve stimulation and magnetic resonance imaging (MRI) of brain, which was normal. An MRI of the cervical spine showed degenerative disc changes without any significant compression of nerve roots or cord changes. Due to persistent symptoms and impaired activities of daily living, he was referred for botulinum toxin injections. There was no significant family history or history of exposure to antipsychotics. He had anterocollis and intermittent lower facial contractions. Passive range of movements of the neck was within normal limits. Power assessment revealed severe neck extensor weakness (Grade 0–1/5 Medical Research Council (MRC)) (Video 1). There were no other associated findings.

Video 1 

Video Shows Neck Antecollis along with Lower Facial Movements. In the second part of the video, clinical examination shows difficulty in neck extension movements against gravity.

Further evaluation showed normal serum creatinine phosphokinase (CPK) (162 IU/dl). An MRI of the cervical muscles showed atrophy, fatty infiltration, and fibrosis of deep cervical muscles, especially at the level of C2, extending above and below. Trapezius and sternocleidomastoid muscles appeared normal (Figure 1A). Electromyogram of the Trapezius and Semispinalis showed very few motor unit potentials (MUP) and no recruitment. Bilateral sternocleidomastoid showed increased spontaneous activity (right > left), with normal recruitment. Clinically, he was considered to have head drop secondary to isolated neck extensor weakness (INEM), mimicking cervical dystonia.

Figure 1 

(A) T2WI Axial Section of cervical Spine Shows Atrophied Deep Paracervical Muscles with Fibrosis and Fatty Infiltration. (B) T2WI axial section of the Neck showing the atrophied left sternocleidomastoid muscle (yellow line-1) in comparison to the normal right sternocleidomastoid muscle (Yellow line-2). (C, D) T2WI Sagittal MRI (C) of CVJ area and sagittal CT section cranio-cervical junction (D) showing multiple anomalies in CVJ with atlanto occipital assimilation, partial fusion of C1-C2 vertebrae, hemi vertebra with fusion of right C4-5 level along with C3-C4 spinal cord hyperintensities.

Case 2

The 22-year-old man presented with a history of abnormal neck posture since early childhood. He was evaluated and diagnosed with cervical dystonia and treated with botulinum toxin injections. Due to limited clinical benefit, he was referred to our clinic. On evaluation, he was noted to have torticollis to the right with mild retrocollis (Video 2). In addition, he was also noted to have a thin and taut left sternocleidomastoid muscle. An MRI of the neck showed atrophy and fibrosis of the left sternocleidomastoid (Figure 1B) along with scoliosis of the cervical spine with convexity to the right side. He was diagnosed with developmental sternocleidomastoid fibrosis mimicking cervical dystonia, and a surgical release was suggested.

Video 2 

Video Shows Limitation of Rotation of Neck to the Left Along with Right Lateral Shift Torticaput to the Right. The prominence of sternocleidomastoid at its origin can be easily appreciated on the left side.

Video 3 

Video Shows Right Lateral Shift of Neck Along with Left Shoulder Elevation. In addition, limitation of rotatory movements of neck to both right and left is shown.

Discussion and literature review

Pseudodystonias are a rare group of disorders where appropriate clinical suspicion and evaluation has an impact on treatment and prognostication. Cervical pseudodystonias can be classified further into (1) vascular, (2) musculoskeletal, (3) infections, (4) mass lesions, (5) traumatic, (6) ocular causes (7) otological causes, (8) gastrointestinal causes, (9) psychogenic, and (10) others (Table 2). The aim of the discussion is to highlight potential presentations of cervical pseudodystonia.

Table 2

Cause of potential mimics of isolated idiopathic cervical dystonia Pseudodystonia

Classification Causes
Vascular causes
  1. Spinal epidural haemorrhage
  2. Cerebellar infarct/haemorrhage
  3. Lateral medullary infarct
  4. Cerebral haemorrhage
  5. Bilateral Putaminal haemorrhage
  6. Cerebral AVM
  7. Unilateral hypoplasia of Internal Carotid artery
  8. ACOM
  9. Vascular pseudo retrocollis
  1. AARS
  2. Fibrodysplasia ossificans
  3. Acute calcific tendinitis
  4. Basillar invagination
  5. Chiari 1 malformation
  6. Klippel-Fiel syndrome
  7. Syringomyelia
  8. Diastometomyelia
  9. Osteomyelitis
  10. Inter vertebral disc calcification
  11. Facetal hypertrophy
  12. Ankylosing spondylitis
  13. Fibromatosis of sternocleidomastoid
  14. Nodular fascitis of sternocleidomastoid
  15. Congenital oseous c2-c3 synostosis
  16. Cervical spondylo discitis
  17. Osteoporotic fracture
  18. Absent sternocleidomastoid muscle
  1. Septic arthritis
  2. Cat scratch disease
  3. Tuberculoma
  4. Bacterial meningitis
  5. Acute febrile torticollis
  6. Acute encephalomyelitis
  7. Paravertebral Brucellar abscess
  8. Cervical epidural abscess
  9. Pharyngeal abscess
  10. Retropharyngeal abscess
  11. Sternocleidomastoid abscess
  12. Pyomyositis of paraspinal muscles
  13. Lymphadenitis
  14. Tuberculosis of bones & joints
Mass / Space occupying lesions
  1. Tumor calcinosis of cervical spine
  2. Intra thoracic malignancy
  3. Posterior fossa tumor
  4. Arachnoid cyst
  5. Spinal cord ependymoma
  6. Cervical osteoblastoma
  7. Medullary tumor
  8. Posterior glioma
  9. Ewing’s sarcoma
  10. Osteochondroma
  11. Giant cell tumor
  12. Osteoid osteoma
  13. Cervical hemangioblastoma
  14. Fibrodysplasia ossificans progressiva
  15. Post radiation therapy of carcinoma larynx
  16. Cerebellar gangliocytoma
  17. Spinal cord astrocytoma
  18. Colloid cyst of 3rd ventricle
  19. Sternocleidomastoid tumor
  20. Cervical eosinophilic granuloma
  1. Odontoid fracture
  2. Laminar fracture
  3. Condylar fracture
  4. C1 dislocation with split atlas
  5. Fracture of c2 lamina
  6. Brachial plexus injury
  7. Post trauma foreign body
  8. Pneumomediastinum
Ocular Causes
  1. Congenital Nystagmus
  2. Nystagmus Compensation Syndrome
  3. Spasmus nutans
  4. Oculomotor apraxia
  5. Refractive error
  6. Blepharoptosis
  7. Superior oblique palsy
  8. Abducens Palsy
  9. Vertically incomitant horizontal strabismus
  10. Duane syndrome
  11. Brown’s syndrome
  12. Double elevator palsy
  13. Orbital floor fracture
  14. Endocrine ophthalmopathy
  15. Congenital fibrosis syndrome
  16. Inferior oblique muscle palsy (ocular torticollis)
Otological Causes
  1. Acute Mastoiditis
  2. Saccular dysfunction
  3. Bezold’s abscess
Gastrointestinal causes
  1. Sandifer’s syndrome
  1. Multiple sclerosis
  2. Acute disseminated encephalomyelitis
  3. Idiopathic intracranial hypertension
  4. Hypereosinophilic syndrome
  5. Widespread nevus spilus
  6. Parry-Romberg syndrome
  7. Moyamoya disease
  8. Behcet’s disease
  9. Kawasaki disease
  10. Goeminne syndrome
  11. Langerhans cell histiocytosis
  12. Allergy
  13. Iatrogenic hypoparathyroidism
  14. Congenital muscular torticollis
  15. Benign paroxysmal torticollis
  16. Grisel’s syndrome
  17. Complication of ventriculo-peritoneal shunt
  18. Familial Mediterranean fever
  19. Foreign body

Abbreviations: AVM - arteriovenous malformation, ACOM - anterior communicating artery aneurysm, AARS - Atlanto Axial Rotatory Subluxation.

(1) Vascular

A number of vascular pathologies can result in true cervical dystonia, majority of them being ischemic or hemorrhagic events of the brainstem, cerebrum, and cerebellum.25 However, vascular anomalies are noted to have abnormal neck posturing due to compensatory mechanisms to aid visual defects.6 Other vascular causes like aneurysms and arteriovenous malformations (AVM) can also result in dystonia.69 An abnormal neck posture can also be seen as a compensatory mechanism to overcome visual field defects masquerading as cervical dystonia.6 Spinal epidural hemorrhage secondary to severe hemophilia can present with torticollis.10 The outcomes are variable, but spontaneous improvement is reported in almost half of the cases.210

(2) Musculoskeletal

Any anatomical/physiological variations in the cervical musculoskeletal system can present with cervical pseudodystonia. In our series, the possible cause for incorrect diagnosis of cervical dystonia in INEM was additional movements of the facial and neck muscles in an effort to lift the head, giving rise to the interpretation of neck drop as anterocollis. Clinical assessment for muscle strength was the key clinical feature in the diagnosis. Every patient should be evaluated to rule out secondary causes. INEM mimicking cervical dystonia is a rare condition and can pose a diagnostic dilemma. This condition is also known as “dropped head syndrome,” which has a wide spectrum of causes.11 Routine blood, electrophysiological, radiological, and histological investigations help in confirming isolated myopathy. Some of these causes are potentially treatable, including myasthenia, hypothyroidism, hyperparathyroidism, hypokalemia, etc. Management of INEM involves physiotherapy, orthotic collars or braces, and rarely surgical fixation.12

Congenital muscular torticollis is another common musculoskeletal cause of cervical pseudodystonia. It is commonly diagnosed during infancy and is caused due to intrauterine malposition or birth trauma. On palpation, the sternocleidomastoid muscle is taut and non-tender. Absence of tremor and sensory trick makes cervical dystonia less likely (see Case 2). There is a restriction in the range of movements of the neck. Some cases resolve spontaneously, while most of them respond well to physical therapy. Physical therapy involves passive stretching and encouraging active movements. Less than 10% of cases require surgery. Early treatment remains the key for better outcomes.1315

Various craniocervical developmental anomalies can also present with cervical pseudodystonia. Klippel–Feil syndrome is a congenital anomaly caused by the fusion of a pair or more of cervical vertebrae. It is characterized by a short neck, torticollis, limited range of movements and low posterior hairline. Symptoms become more pronounced during adolescence or young adulthood. Vertigo, tinnitus, and hearing loss are sometimes associated with this condition. It can masquerade as cervical dystonia, which becomes apparent after radiological evaluation. Treatment is mostly symptomatic. Surgery may be needed if associated with scoliosis, radiculopathy, or myelopathy. Pseudodystonic posture secondary to Klippel–feil syndrome and diastometamyelia has been reported.16 Other CVJ anomalies like Chiari malformation and basilar invagination are known to cause torticollis.1718 Syringomyelia associated with torticollis is a rare presentation. It requires a high degree of suspicion when head tilt is seen along with sensory loss. Dystonic movements of the rest of the body can also be seen occasionally. An MRI of the spine can confirm the diagnosis. Decompression of the syrinx relieves the symptoms.19

Acute, painful torticollis in children with a history of fall or trauma points toward atlantoaxial rotatory subluxation (AARS).2022 It can also be due to a local inflammatory process, or connective tissue disorders. A rare case of AARS was seen associated with familial Mediterranean fever (FMF) presenting with torticollis.23 FMF is an autosomal recessive autoinflammatory condition, presenting with episodes of fever and serositis. Cervical dystonia in ankylosing spondylitis resulting in AARS and craniocervical osseous fusion (CCOF) has been reported.24 If there is no history of trauma, it is important to look for a history of recent ENT surgeries or any inflammatory conditions of the neck, as it could be Grisel’s syndrome, which is a non-traumatic atlantoaxial subluxation resulting from an ongoing local inflammatory process.2527 Grisel’s syndrome is more commonly seen in children due to laxity of ligaments along with the tenderness of the spinous process, and there may be unilateral occipital pain. A spinal cord injury should be suspected if there are associated neurological symptoms such as unsteady gait or hyperreflexia.25 Early diagnosis and management is the key. It is usually treated by manipulation under anesthesia and immobilization. For less severe cases, cervical soft collar with rest and analgesia may be sufficient. Delay in the diagnosis can lead to neurological complications and require surgery.2830

Other causes like intervertebral disc calcification, acute calcific tendinitis of longus colli, facetal hypertrophy, and nodular fasciitis of sternocleidomastoid should be considered in the differential diagnosis of musculoskeletal causes of pseudodystonia.3134

(3) Infections

Infections of the upper respiratory tract or soft tissues of the neck can cause torticollis to mimic cervical dystonia. These include cervical adenitis, lymphadenitis, retropharyngeal abscess, and sternocleidomastoid myositis.35,36 Retropharyngeal abscess is a serious condition presenting with severe pain, fever, and difficulty in breathing and swallowing along with torticollis. It requires immediate treatment with antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs), and sometimes surgical drainage of the abscess. Similarly, paravertebral brucellar abscess and sternocleidomastoid abscess are documented to present as cervical pseudodystonia.37

Other reported causes of atypical infections presenting with cervical pseudodystonias include cat scratch disease, encephalomyelitis, bacterial meningitis, neuroborreliosis, tuberculoma, tuberculosis of the bone and joints, septic arthritis, spondylodiscitis, and osteomyelitis.3846

(4) Mass lesions/space occupying lesions

Central nervous system lesions can also cause cervical pseudodystonia, along with symptoms like nausea, vomiting, headache, ataxia, visual disturbances, and cranial nerve deficits. These lesions include posterior fossa and infratentorial tumors (more commonly in the cerebellum, third ventricle, and brainstem) and spinal cord tumors like astrocytomas, medulloblastomas, and ependymomas.7,35,47,48 Cervical osteoblastoma, hemangioblastoma, osteochondroma, osteoid osteoma, fibrodysplasia ossificans progressiva, giant cell tumor, and Ewings sarcoma can all present with torticollis.7,4954 Torticollis in children requires brain and spinal cord imaging to avoid delay in diagnosis, which can be life threatening. Surgical removal of the tumor in most cases results in resolution of the symptoms.

Cervical pseudodystonia has been described in carcinoma larynx (following radiation therapy) due to fibrosis of neck muscles and in a Pancoast tumor of the lung due to possible segmental demyelination of the 11th cranial nerve.55,56

(5) Traumatic

Trauma resulting in odontoid fracture, laminar fracture, condylar fracture, osteoporotic fracture, C1 dislocation with split atlas due to various causes can all present as dystonia mimics.5761 Appropriate management will alleviate symptoms.

Other traumatic conditions presenting with a similar picture include brachial plexus injury, pneumomediastinum, foreign body, and rarely electrical injury.6265

(6) Ocular causes

Ocular causes like congenital nystagmus, nystagmus compensation (blockage) syndrome, spasmus nutans, oculomotor apraxia, refractive error, blepharoptosis, superior oblique palsy, abducens palsy, vertically incomitant horizontal strabismus, Duane syndrome, Brown’s syndrome, double elevator palsy, orbital floor fracture, endocrine ophthalmopathy, congenital fibrosis syndrome, and inferior oblique muscle palsy may present with torticollis called ocular torticollis. Abnormal head position is assumed in order to maintain binocularity and/or to optimize visual acuity. Bielschowsky head tilt test is the primary test in the ocular torticollis workup. Treatment is usually surgical and depends on the underlying cause.66

(7) Otological causes

Cervical pseudodystonias secondary to vestibular dysfunctions Contribute to otological causes. These patients have ataxia, vertigo, or nystagmus along with torticollis. Acute mastoiditis, saccular dysfunction, and Bezold’s abscess are few examples. This requires further assessment of the vestibular system and management.6769

(8) Gastrointestinal causes

In a child presenting with torticollis or side-to-side head movements associated with vomiting, regurgitation, or epigastric pain, Sandifer’s syndrome can be suspected. The symptoms can be intermittent and are associated with meals in most cases. The symptoms are probably due to the patient assuming a position to minimize the painful acid reflux. As the early symptoms resemble dystonia, evaluation is focused on neurological etiology, which is usually normal. Diagnosis is by monitoring esophageal pH and demonstrating reflux. Medical treatment for gastroesophageal reflux usually resolves the symptoms, but sometimes this condition requires surgery.70,71

(9) Psychogenic dystonias

Psychogenic dystonia, also known as functional dystonia, is a controversial diagnosis commonly associated with psychiatric comorbidities like anxiety, depression, and personality disorders. Variability of symptoms suggests a psychogenic cause. The current classification has suggested categorization of psychogenic dystonias as one of the acquired dystonias.1 Although prognosis can be poor with long-term disability, mainstay of management involves communicating the diagnosis with the patient, physiotherapy, and behavioral therapy.72,73

(10) Others

There are various other causes of cervical pseudodystonias, which are either rarely reported or documented as single association. Among these, benign paroxysmal torticollis (BPT) of infancy is a self-limiting condition, characterized by periods of unusual, sustained posture of the head and neck, during which the head tilts to one side. Episodes are often accompanied by marked autonomic features, irritability, ataxia, apathy, and drowsiness. They last several hours to a few days and often recur every few weeks. They subside within the pre-school years. It is essential to recognize this condition and to reassure parents of its benign course and not to be misdiagnosed for other disorders, such as epileptic seizures. BPT of infancy has been linked to CACNA1A mutations and are likely to be associated with familial hemiplegic migraine, episodic ataxia, and paroxysmal tonic upgaze.74,75 There is no approved medication for the disease. Some studies have demonstrated the uses of cyproheptadine and topiramate for BPT.76

Other rare neurological causes presenting as cervical pseudodystonia include idiopathic intracranial hypertension, acute demyelinating encephalomyelitis, multiple sclerosis, Guillain–Barre syndrome, hypereosinophilic syndrome, widespread nevus spilus, Parry–Romberg syndrome, moyamoya disease, atypical Kawasaki disease, Behcet’s disease, Langerhans cell histiocytosis, Goldenhar syndrome, Goeminne syndrome, iatrogenic hypoparathyroidism, and allergy.7792 These conditions have been reported in isolated case reports.


In any case of presumed cervical dystonia, a diligent clinical evaluation is a major step in diagnosis and management. The examination must include a complete neurological assessment, including the power and range of movements of the neck. It is also critical not to forget the examination of the ocular and vestibular system, and the local examination of the neck for any obvious deformities. Imaging must include an X-ray of the neck for bony deformities and an MRI of the brain and neck to rule out the possibility of tumors and other spinal lesions. We have provided a simple algorithm for workup for suspected cases of cervical pseudodystonia (Figure 2).

Figure 2 

Algorithm for Mimics of Isolated Cervical Dystonia.

In patients with atypical features, a multi-disciplinary approach helps in arriving at an accurate diagnosis (pediatricians, rehabilitation experts, ENT surgeons, ophthalmologists, gastroenterologists, neurosurgeons, movement disorder specialists, and others). Dystonia mimic or pseudodystonia is an under reported entity in our movement disorder literature; this review highlights the varied causes and will help in diagnosing this chameleon-like entity and directing treating physicians toward the right intervention.


1 Citation: Raju S, Ravi A, Prashanth LK. Cervical dystonia mimics: A case series and review of the literature. Tremor Other Hyperkinet Mov. 2019; 9. doi: 10.7916/tohm.v0.707 

2 Editor: Elan D. Louis, Yale University, USA 

3 Funding: None. 

4 Financial Disclosures: None. 

5 Conflicts of Interest: The authors report no conflicts of interest. 

6 Ethics Statement: All patients that appear on video have provided written informed consent; authorization for the videotaping and publication of the videotape was provided. 


We thank Dr. Richard Walsh, Consultant Neurologist and Movement Disorders Specialist, Tallaght University Hospital, Dublin, Ireland for proof reading and editing the manuscript.


  1. Albanese, A , Bhatia, K , Bressman, SB , DeLong, MR , Fahn, S , Fung, VSC et al. Phenomenology and classification of dystonia: a consensus update. Mov Disord 2013;28(7):863–873. doi: 

  2. Ogawa, T, , Shojima, Y, , Kuroki, T and , Eguchi, H, , Hattori, N, , Miwa, H, Cervico-shoulder dystonia following lateral medullary infarction: a case report and review of the literature. J Med Case Rep 2018;12(1):34. doi: 

  3. O'Rourke, K, , O’Riordan, S and , Gallagher, J, , Hutchinson, M, Paroxysmal torticollis and blepharospasm following bilateral cerebellar infarction. J Neurol 2006;253(12):1644–1645. doi: 

  4. Shukla, AW, , Jesus, SD and , Meng, FG, , Hu, W, Focal cervical dystonia presents in the setting of acute cerebellar haemorrhage. J Neurol Sci 2017;375:307–308. doi: 

  5. Usmani, N, , Bedi, GS, , Sengun, C , Pandey, A, , Singer, C, Late onset of cervical dystonia in a 39-year-old patient following cerebellar haemorrhage. J Neurol 2011;258:149–151. doi: 

  6. Giordano, A, , Tedeschi, G , Tessitore, A, Vascular-induced compensatory pseudo-retrocollis. Neurology 2015;84(19):2005–2006. doi: 

  7. Per, H , Canpolat, M , Tumturk, A , Gumus, H , Gokoglu, A , Yikilmaz, A , et al. Different etiologies of acquired torticollis in childhood. Childs Nerv Syst 2014;30(3):431–440. doi: 

  8. Bayrakci, B, , Aysun, S , Firat, M, Arteriovenous fistula: a cause of torticollis. Pediatr Neurol 1999;20(2):146–147. doi: 

  9. Lobo-Antunes, J, , Yahr, MD , Hilal, SK, Extrapyramidal dysfunction with cerebral arteriovenous malformations. J Neurol Neurosurg Psychiatry 1974;37(3):259–268. doi: 

  10. Agha, BS, , Tehan, C , Perno, J, Torticollis: not always the usual suspects. Pediatr Emerg Care 2011;27:32–33. doi: 

  11. Martin, AR, , Reddy, R , Fehlings, MG, Dropped head syndrome: diagnosis and management. Evid Based Spine Care J 2011;2(2):41–47. doi: 

  12. Umapathi, T, , Chaudhry, V, , Cornblath, D and , Drachman, D, , Griffin, J, , Kuncl, R, Head drop and camptocormia. J Neurol Neurosurg Psychiatry 2002;73(1):1–7. doi: 

  13. Bredenkamp, J, , Hoover, L and , Berke, G, , Shaw, A, Congenital muscular torticollis: a spectrum of disease. Arch Otolaryngol Head Neck Surg 1990;116(2):212–216. doi: 

  14. Cheng, J, , Tang, S , Chen, T, Sternocleidomastoid pseudotumor and congenital muscular torticollis in infants: a prospective study of 510 cases. J Pediatr 1999;134(6):712–716. doi: 

  15. Kuo, A, , Tritasavit, S , Graham, J, Congenital muscular torticollis and positional plagiocephaly. Pediatr Rev 2014;35(2):79–87. doi: 

  16. Lopez-Vicchi, M, , Da Prat, G , Gatto, EM, Pseudodystonic posture secondary to Klippel-Feil syndrome and diastematomyelia. Tremor Other Hyperkinet Mov 2015;5:325. doi: 

  17. Alexiou, GA and , Prodromou, N, Torticollis as an initial sign of Chiari I malformation. Pediatr Emerg Care 2009;25(3):215. doi: 

  18. Souza, PV, , Pinto, WB , Oliveira, AS, Basilar invagination in headache associated with physical exertion and recurrent torticollis. Arq Neuropsiquiatr 2014;72(11):902–903. doi: 

  19. Silva-Júnior, FP, , Santos, JG, , Sekeff-Sallem, FA , Lucato, LT, , Barbosa, ER, Cervical and axial dystonia in a patient with syringomyelia. Arq Neuropsiquiatr 2012;70(9):742–743. doi: 

  20. Hussain, K, , Abdo, MM and , AlNajjar, FJ, , Abbo, M, Not your typical torticollis: a case of atlantoaxial rotatory subluxation. BMJ Case Rep 2014;2014:bcr2013201023. doi: 

  21. Bagouri, E, , Deshmukh, S , Lakshmanan, P, Atlantoaxial rotatory subluxation as a cause of torticollis in a 5-year-old girl. BMJ Case Rep 2014;2014:bcr2013202990. doi: 

  22. Sundseth, J, , Berg-Johnsen, J, , Skaar-Holme, S , Züchner, M, , Kolstad, F, Atlantoaxial rotatory fixation-a cause of torticollis. Tidsskr Nor Legeforen 2013;133:519. doi: 

  23. Gencpinar, P, , Bozkurt, O, , Karaali, K and , Gemici, A, , Kazan, S, , Haspolat, S, A rare coincidence of torticollis in Familial Mediterranean Fever: atlanto-axial rotatory subluxation. Clin Neurol Neurosurg 2014;127:158–160. doi: 

  24. Weigel, RM, , Capelle, HH , Krauss, JK, Cervical dystonia in Bechterew disease resulting in atlantoaxial rotatory subluxation and cranio-cervical osseous fusion. Spine 2007;32(25):E781–E784. doi: 

  25. Allegrini, D, , Autelitano, A, , Nocerino, E and , Fogagnolo, P, , De Cillà, S, , Rossetti, L, Grisel’s syndrome, a rare cause of anomalous head posture in children: a case report. BMC Ophthalmol 2016;16:21. doi: 

  26. Deichmueller, C and , Welkoborsky, HJ, Grisel’s syndrome—a rare complication following “small” operations and infections in the ENT region. Eur Arch Otorhinolaryngol 2010;267:1467–1473. doi: 

  27. Elyajouri, A, , Assermouh, A, , Abilkacem, R , Agadr, A, , Mahraoui, C, Grisel’s syndrome: a rare complication following traditional uvulectomy. Pan Afr Med J 2015;20:62. doi: 

  28. Martins, J, , Almeida, S, , Nunes, P and , Prata, F, , Lobo, ML, , Marques, JG, Grisel syndrome, acute otitis media, and temporo-mandibular reactive arthritis: a rare association. Int J Pediatr Otorhinolaryngol 2015;79(8):1370–1373. doi: 

  29. Ortiz, GL, , Pratts, I , Ramos, E, Grisel’s syndrome: an unusual cause of torticollis. J Pediatr Rehabil Med 2013;6(3):175–180. doi: 

  30. Osiro, S, , Tiwari, KJ, , Matusz, P and , Gielecki, J, , Tubbs, RS, , Loukas, M, Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options. Childs Nerv Syst 2012;28:821–825. doi: 

  31. Kouamo, EI , Nour, M , Gennar, JM , Guillaume, JM , Choufani, E , Merrot, T , et al. Case study of two children with intervertebral disc calcifications. Pan Afr Med J 2016;25:34. doi: 

  32. Cuevas, Y, , Schonhaut, L, , Espinoza, A and , Schonstedt, V, , Aird, A, , Castoldi, F, Pediatric intervertebral disc calcification: a rare cause of acquired torticollis. Case report. Rev Chil Pediatr 2015;86(3):200–205. doi: 

  33. Zibis, AH, , Giannis, D, , Malizos, KN , Kitsioulis, P, , Arvanitis, DL, Acute calcific tendinitis of the longus colli muscle: case report and review of the literature. Eur Spine J 2013;22(3):S434–S438. doi: 

  34. Hemmi, S, , Murakami, T and , Shirabe, T, , Sunada, Y, A patient wsith muscular torticollis caused by nodular fasciitis in the sternocleidomastoid muscle (SCM). Rinsho Shinkeigaku 2002;42(9):864–867. PMID: .12710086 

  35. Tomczak, KK and , Rosman, NP, Torticollis. J Child Neurol 2013;28(3):365–367. doi: 

  36. Lehtinen, P, , Ruuskanen, O , Sonninen, P, An unusual life threatening cause of torticollis in a child. Arch Dis Child 2003;88(4):349. doi: 

  37. Simsek, S, , Yigitkauli, K, , Kazanci, AS , Beleu, D, , Bavbek, M, Medically treated paravertebral Brucella abscess presenting with acute torticollis: case report. Surg Neurol 2007;67(2):207–210. doi: 

  38. Rafferty, JR, , Janopaul-Naylor, E , Riese, J, Torticollis and fever in a young boy: a unique presentation of cat-scratch Disease with vertebral osteomyelitis and epidural phlegmon. Pediatr Emerg Care 2017;33(12):e164–e166. doi: 

  39. Mukherjee, S and , Sharief, N, Bacterial meningitis presenting as acute torticollis. Acta Paediatr 2004;93(7):1005–1006. doi: 

  40. van Breemen, MS, , van der Kuip, M, , Ang, CW , van Furth, AM, , Wolf, NI, Torticollis and seizures due to neuroborreliosis in a child. Ned Tijdschr Geneeskd 2012;156(51):A5157. PMID: .23249509 

  41. Tey, HL, , Seet, RC , Lim, EC, Tuberculomas causing cervical dystonia. Intern Med J 2005;35(4):261–262. doi: 

  42. Warpus, JM Tuberculosis of bones and joints. Torticollis. Foot disorders. ONA J 1978;5(3):77–83. PMID: .246509 

  43. Filleron, A, , L’Kaissi, M, , Cottalorda, J, , Jeziorski, E , Rodiere, M, , Prodhomme, O, , et, al, Torticollis in children: a challenging diagnosis of C1-C2 septic arthritis. Clin Pediatr 2016;55(5):459–462. doi: 

  44. Pizzol, A, , Bramuzzo, M, , Pillon, R , Taddio, A, , Barbi, E, Torticollis as the presenting sign of cervical spondylodiscitis. Pediatr Emerg Care 2016;32(12):863–864. doi: 

  45. McKnight, P and , Friedman, J, Torticollis due to cervical epidural abscess and osteomyelitis. Neurology 1992;42(3 Pt 1):696–697. doi: 

  46. Dimaala, J, , Chaljub, G and , Oto, A, , Swischuk, L, Odontoid osteomyelitis masquerading as a C2 fracture in an 18-month-old male with torticollis: CT and MRI features. Emerg Radiol 2006;12(5):234–236. doi: 

  47. Krauss, JK, , Seeger, W, , Jankovic J Krauss, JK , Seeger, W, , Jankovic, J, Cervical dystonia associated with tumors of the posterior fossa. Mov Disord 1997;12(3):443–447. doi: 

  48. Debenedictis, CN, , Allen, JC , Kodsi, SR, Brainstem tumor presenting with tearing, photophobia, and torticollis. J AAPOS 2010;14(4):369–370. doi: 

  49. Patier de La Peña, JL, , Norman, F, , Rodríguez-Ramírez, GI , Echániz-Quintana, A, , Moreno-Cobo, MA, Cervical pain, torticollis and polyglobulia as a first manifestation of a cerebellar hemangioblastoma. Rev Clin Esp 2010;210(6):e21–e23. doi: 

  50. Akhaddar, A and , Boucetta, M, Solitary osteochondroma of the cervical spine presenting as recurrent torticollis. Pan Afr Med J 2014;17:271. doi: 

  51. Amirjamshidi, A, , Roozbeh, H, , Sharifi, G , Abdoli, A, , Abbassioun, K, Osteoid osteoma of the first 2 cervical vertebrae. Report of 4 cases. J Neurosurg Spine 2010;13(6):707–714. doi: 

  52. Moore, RE, , Dormans, JP, , Drummond, DS and , Shore, EM, , Kaplan, FS, , Auerbach, JD, Chin-on-chest deformity in patients with fibrodysplasia ossificans progressive. A case series. J Bone Joint Surg Am 2009;91(6):1497–1502. doi: 

  53. Karampalis, C, , Lenthall, R , Boszczyk, B, Solid variant of aneurysmal bone cyst on the cervical spine of a child: case report, differential diagnosis and treatment rationale. Eur Spine J 2013;22:523–531. doi: 

  54. Aydin, R, , Bilgici, MC , Dagcinar, A, A very rare cause of neck paIn: primary Ewing sarcoma of the axis. Pediatr Emerg Care 2013;29(11):1197–1200. doi: 

  55. Astudillo, L , Hollington, L , Game, X , Benyoucef, A , Boladeras, AM , Delisle, MB , et al. Cervical dystonia mimicking dropped-head syndrome after radiotherapy for laryngeal carcinoma. Clin Neurol Neurosurg 2003;106(1):41–43. doi: 

  56. Landan, I and , Cullis, PA, Torticollis following radiation therapy. Mov Disord 1987;2(4):317–319. doi: 

  57. Tsuji, S, , Inoue, S, , Tachibana, T and , Maruo, K, , Arizumi, F, , Yoshiya, S, Post-traumatic torticollis due to odontoid fracture in a patient with diffuse idiopathic skeletal hyperostosis: a case report. Medicine 2015;94(36):e1478. doi: 

  58. Balpande, DN, , Agrawal, A , Bhatlawande, VK, Torticollis as a manifestation of laminar fracture in an infant. J Pak Med Assoc 2008;58(10):591. PMID: .18998320 

  59. Chou, WC, , Huang, WC, , Shih, YH and , Lee, LS, , Wu, C, , Cheng, H, Occult occipital condyle fracture with normal neurological function and torticollis. J Clin Neurosci 2008;15(8)920–922. doi: 

  60. Ostrowski, C, , Ronan, L and , Sheridan, R, , Pearce, V, An osteoporotic fracture mimicking cervical dystonia in idiopathic Parkinson’s disease. Age Ageing 2013;42(5):658–659. doi: 

  61. Tachibana, A, , Imabayashi, H, , Yato, Y and , Nakamichi, K, , Asazuma, T, , Nemoto, K, Torticollis of a specific C1 dislocation with split atlas. Spine 2010;35(14):E672–E675. doi: 

  62. Hervey-Jumper, S, , Justice, D, , Vanaman, M , Nelson, V, , Yang, L, Torticollis associated with neonatal brachial plexus palsy. Pediatr Neurol 2011;45(5):305–310. doi: 

  63. Dekel, B, , Paret, G, , Vardi, A , Katz, M, , Barzilay, Z, Torticollis: an usual presentation of spontaneous pneumomediastinum. Pediatr Emerg Care 1996;12(5):352–353. PMID: .8897543 

  64. Walton, J, , Darr, A , George, A, An unusual case of an oesophageal foreign body presenting as torticollis. Ann R Coll Surg Engl 2016;98(3):e40–e42. doi: 

  65. Boonkongchuen, P and , Lees, A, Case of torticollis occurring following electrical injury. Mov Disord 1996;11(1):109–110. doi: 

  66. Rubin, SE and , Wagner, RS, Ocular torticollis. Surv Ophthalmol 1986;30(6):366–376. doi: 

  67. Bredenkamp, JK and , Maceri, DR, Inflammatory torticollis in children. Arch Otolaryngol Head Neck Surg 1990;116(3):310–313. doi: 

  68. Hallberg, A, , Standring, RT , Ahsan, S, Congenital torticollis and saccular dysfunction. JAMA Otolaryngol Head Neck Surg 2013;139(6):639–642. doi: 

  69. McMullan, B Bezold’s abscess: a serious complication of otitis media. J Paediatr Child Health 2009;45:616–618. doi: 

  70. Lehwald, N, , Krausch, M, , Franke, C and , Assmann, B, , Adam, R, , Knoefel, W, Sandifer syndrome – a multidisciplinary diagnostic and therapeutic challenge. Eur J Pediatr Surg 2007;17(3):203–206. doi: 

  71. Wasserman, JK, , Jimenez-Rivera, C , Doja, A, Refractory head movements secondary to Sandifer syndrome treated with enteral feeding. Mov Disord 2010;25(11):1754–1755. doi: 

  72. Schmerler, DA and , Espay, AJ, Functional dystonia. Handb Clin Neurol 2016;139:235–245. doi: 

  73. Hallett, M Functional (psychogenic) movement disorders – clinical presentations. Parkinsonism Relat Disord 2016;22(1):S149–S152. doi: 

  74. Giffin, NJ, , Benton, S , Goadsby, PJ, Benign paroxysmal torticollis of infancy: four new cases and linkage to CACNA1A mutation. Dev Med Child Neurol 2002;44(7):490–493. doi: 

  75. Shin, M, , Douglass, LM and , Milunsky, JM, , Rosman, NPJ, The genetics of benign paroxysmal torticollis of infancy: is there an association with mutations in the CACNA1A gene? Child Neurol 2016;31(8):1057–1061. doi: 

  76. Hadjipanayis, A, , Efstathiou, E , Neubauer, D, Benign paroxysmal torticollis of infancy: an underdiagnosed condition. J Paediatr Child Health 2015;51(7):674–678. doi: 

  77. Saifudheen, K Idiopathic intracranial hypertension presenting as stiff neck and torticollis. Neurol India 2010;58(6):982. doi: 

  78. Har-Gil, M, , Evrani, M , Watemberg, N, Torticollis as the only manifestation of acute disseminated encephalomyelitis. J Child Neurol 2010;25(11):1415–1418. doi: 

  79. Rüegg, SJ, , Bühlmann, M, , Renaud, S and , Steck, AJ, , Kappos, L, , Fuhr, P, Cervical dystonia as first manifestation of multiple sclerosis. J Neurol 2004;251(11):1408–1410. doi: 

  80. Berger, B, , Rijntjes, M and , Stehlin, L, , Stich, O, Corticosteroid-responsive torticollis as a presenting symptom of multiple sclerosis. J Neurol Sci 2014;340(1–2):239–240. doi: 

  81. Planas, AM, , Occhiuzzo, FP, , Oferill, JC , Marfa, MP, , Cubells, CL, Torticollis and spinal hyperextension. Unusual presentation of Guillain-Barré syndrome. An Pediatr 2008;69(4):394–395. doi: 

  82. Das, JK, , Gupta, K and , Deshmukh, S, , Shrivastava, R, A rare case of hypereosinophilic syndrome presenting with unilateral proptosis and torticollis. Indian J Ophthalmol 2018;66(10):1508–1511. doi: 

  83. Marti, N, , Jorda, E, , Martinez, E , Gamez, L, , Ramon, MD, Widespread nevus spilus associated with torsion dystonia: a case report. Pediatr Dermatol 2010;27(6):654–656. doi: 

  84. Panda, AK, , Gopinath, G , Singh, S, Parry-Romberg syndrome with hemimasticatory spasm in pregnancy; A dystonia mimic. J Neurosci Rural Pract 2014;5(2):184–186. doi: 

  85. Yasutomo, K, , Hashimoto, T and , Miyazaki, M, , Kuroda, Y, Recurrent Torticollis As a presentation of Moyamoya disease. J Child Neurol 1993;8(2):187–188. doi: 

  86. Runel-Belliard, C, , Lasserre, S and , Quinet, B, , Grimprel, E, Febrile torticollis: an atypical presentation of Kawasaki disease. Arch Pédiatr 2009;16(2):115–117. doi: 

  87. Pellecchia, MT, , Cuomo, T, , Striano, S , Filla, A, , Barone, P, Paroxysmal dystonia in Behçet’s disease. Mov Disord 1999;14(1):177–179. doi:<177::aid-mds1037>;2-n9918371 

  88. Kostaridou, S, , Anastasopoulos, J, , Veliotis, C and , Nikas, J, , Stefanaki, K, , Tzortzatou-Stathopoulou, F, Recurrent torticollis secondary to Langerhans cell histiocytosis: a case report. Acta Orthop Belg 2005;71(1):102–106. PMID: .15792216 

  89. Al Kaissi, A, , Ben Chehida, F, , Ganger, R , Klaushofer, K, , Grill, F, Distinctive spine abnormalities in patients with Goldenhar syndrome: tomographic assessment. Eur Spine J 2015;24(3):594–599. doi: 

  90. Goeminne, L A New probably X-linked inherited syndrome: congenital muscular torticollis, multiple keloids cryptorchidism and renal dysplasia. Acta Genet Med Gemellol 1968;17(3):439–467. doi: 

  91. Mancini, F, , Zangaglia, R, , Cristina, S, , Tassorelli, C , Uggetti, C, , Nappi, G, , Pacchetti, C, Secondary cervical dystonia in iatrogenic hypoparathyroidism associated with extensive brain calcifications. Funct Neurol 2006;21(3):165–166. PMID: .17049136 

  92. Randolph, TG Allergy as a cause of Acute Torticollis. Am Pract Dig Treat 1950;1(10):1062–1067. PMID: .14771412 

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