Definition
A self-limiting episodic disorder of infancy with recurrent attacks of head tilt. It is regarded as an early migraine variant.
Aetiology
- Family history of migraine is common
- CACNA1A mutations in some families, the gene of familial hemiplegic migraine
- Can evolve into benign paroxysmal vertigo of childhood or migraine
Epidemiology
- Onset in the first year, often within the first few months
- Rare, and probably underdiagnosed
Clinical Features
- Attacks of head tilt lasting hours to days, recurring at intervals of weeks
- Side of tilt can alternate between attacks
- Pallor, irritability, vomiting and ataxia during attacks
- Normal examination and development between attacks
- No sternocleidomastoid mass or fibrosis
Investigations
- Clinical diagnosis after excluding structural causes
- Cervical spine radiographs are normal
- MRI of brain and cervical spine for atypical attacks or persistent signs
Differential Diagnosis
- Congenital muscular torticollis
- Secondary torticollis
- Posterior fossa tumour
- Sandifer syndrome from gastro-oesophageal reflux
- Atlantoaxial rotatory subluxation
- Ocular torticollis
- Seizures and dystonia
Management
- Reassurance. Explanation of the benign, self-limiting course
- Symptom relief. Analgesia or antiemetics during attacks
- Referral. Paediatric neurology for atypical features or developmental delay
- No splinting or surgery. The sternocleidomastoid is normal
Prognosis
- Attacks stop in early childhood, usually by 5 years
- No neurological sequelae on long term follow up
- Later migraine or benign paroxysmal vertigo in some
References
- Drigo P, Carli G, Laverda AM. Benign paroxysmal torticollis of infancy. Brain Dev. 2000, 22(3), 169-72. PubMed
- 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), 490-3. PubMed
- Danielsson A, Anderlid BM, Stödberg T, Lagerstedt-Robinson K, Klackenberg Arrhenius E, Tedroff K. Benign paroxysmal torticollis of infancy does not lead to neurological sequelae. Dev Med Child Neurol. 2018, 60(12), 1251-5. PubMed
Reviewed by Professor Phong Tran, Head of Orthopaedic Surgery, Western Health. Last updated 19 October 2026.