It is characterised by episodes of head tilt to one side for a few hours or days. The torticollis may occur without any associated symptoms or may be accompanied by pallor, vomiting, irritability or ataxia. Often there is no trigger for the events and the children are normal between spells. The spells start in first year of life and abate spontaneously usually by 2 to 3 years of age, but always by age 5.
Differential diagnoses include acute dystonias including drug-related, posterior fossa or cervical cord lesion including Chiari syndrome , cervical vertebral abnormalities like Klippel–Feil syndrome or Congenital muscular torticollis due to sternocleidomastoid fibrosis. The torticollis is persistent and not paroxysmal in case of structural lesions.
Diagnosis is by exclusion of other differentials. Ictal and interictal EEG is normal. No treatment has been shown to be effective.
It is listed as migraine variant. There is often a family history of migraine. Some older children complain of headache during a spell and many children go on to develop typical migraine after they have “outgrown” the paroxysmal torticollis.