A migraine variant in infancy

The first year of life is full of so many changes and joys. Your baby is healthy and growing. He coos and smiles when you tickle his toes or blow raspberries on his tummy. Then you notice something strange. Randomly his head tilts to one side with a slight rotation. He fusses and may vomit. His movements are jerky and his skin turns pale. Trying to control your fear, you comfort him in your arms to no avail. After several minutes his body relaxes and his head returns to a normal position. Concerned, you call your pediatrician who tells you there is nothing to worry about.

But the attacks keep happening. Some will last for hours, or even days. Between attacks, your baby appears healthy and normal. So you insist on seeing the doctor who suggests you consult a specialist. Countless tests rule out one thing after another. It’s not reflux or seizures. The doctor starts talking about “dystonia” and “torticollis.” The words are unfamiliar and frightening. He suggests some testing. So your tiny baby undergoes EEGs, ultrasounds, CT scans, various eye exams. When all tests come back normal, the doctor suggests that your child may be experiencing benign paroxysmal torticollis. He explains that this condition is not progressive and will likely go away on its own before your child starts school. He offers no treatment, either acute or preventive, and send you and baby on your way. You leave feeling confused and still afraid.

Benign paroxysmal torticollis is a rare migraine variant that is seen only in very small children. Symptoms usually appear in the first three months, starting with a tilted rotation of the head and an upward gaze. Irritability, vomiting, pallor (pale skin), malaise, and ataxia (loss of motor coordination) are common, too. Attacks can last from a few minutes to several days. At this point, there is no known treatment for the condition. Fortunately, the attacks often stop before the child enters school. Sometimes though, the affected child may develop benign paroxysmal vertigo, migraine with or without aura, or hemiplegic migraine. There is nearly always a family history of migraine.

Benign paroxysmal torticollis has been linked to mutation of the CACNA1A gene which has been implicated in the development of hemiplegic migraine. As the condition is so rare, not much is known about it. Families with a history of migraine, especially hemiplegic migraine, should be aware of the potential for this condition in babies. If symptoms appear, consultation with a pediatric headache specialist may be warranted.


ICHD-3 Diagnostic Criteria

Description: Recurrent episodes of head tilt to one side, perhaps with slight rotation, which remit spontaneously. The condition occurs in infants and small children, with onset in the first year.

Diagnostic criteria:

  1. Recurrent attacks in a young child, fulfilling criteria B and C
  2. Tilt of the head to either side, with or without slight rotation, remitting spontaneously after minutes to days
  3. At least one of the following associated symptoms or signs:

    A. pallor
    B. irritability
    C. malaise
    D. vomiting
    E. ataxia

  4. Normal neurological examination between attacks
  5. Not attributed to another disorder.

Notes:

  1. Attacks tend to recur monthly.
  2. Ataxia is more likely in older children within the affected age group.

Comments: The child’s head can be returned to the neutral position during attacks: some resistance may be encountered, but can be overcome. The differential diagnosis includes gastro-oesophageal reflux, idiopathic torsional dystonia and complex partial seizure, but particular attention must be paid to the posterior fossa and craniocervical junction where congenital or acquired lesions may produce torticollis. These observations need further validation by patient diaries, structured interviews and longitudinal data collection. Benign paroxysmal torticollis may evolve into Benign paroxysmal vertigo or Migraine with aura (particularly Migraine with brainstem aura), or cease without further symptoms.

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