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Episodes of recurrent head deviation to one side followed by irritability affect this infant, lasting for days. Migraine headaches affect mother and aunt. Is there a clue in the history?
A 6-month-old presents with episodes of recurrent head deviation to one side followed by irritability. These episodes may last for days. Both mother and aunt have migraine headaches. Examination and imaging are normal. An electroencephalogram during the event is normal.
What could explain the child’s symptoms?
Answer: Benign paroxysmal torticollis.
Benign paroxysmal torticollis (BPT) is an under-recognized torticollis of infancy and often mistaken for “congenital” torticollis. The episodes of head tilt/deviation may last from 10 minutes to 10 days, occur as early as 1 week of life, and in most cases have their onset in the first 7 months.(1,2). Resolution by 4 years of age is almost universal. Episodes rarely last for more than 2 weeks.
No gender association has been noted in most studies although some have found a slight female predominance.3 Associated symptoms may include irritability, vomiting, ataxia, pallor, and somnolence.
BPT is widely accepted as a forerunner of migraine headaches-- more specifically a manifestation of basilar migraine.4 A proposed association between BPT and a calcium channel mutation that is implicated in the etiology of other types of migraine further supports BPT’s role as a migraine precursor.5 Treatment options for the acute phase of BPT remain unsatisfactory but parental reassurance is paramount. A trial of cyproheptadine may be safe and possibly effective.6 A trial dose of 1 mg may be considered.
Differential diagnosis includes dystonic drug reactions; tumors of the posterior fossa or cervical spine; focal seizures; and local pathology, such as fibrosis or contracture of the sternocleidomastoid muscle. A thorough history may elicit the episodic and reversible nature of the symptoms and is the most important diagnostic clue. A family history of migraine also may guide the clinician. There are no biomarkers for BPT, thus in many situations it may be a diagnosis of exclusion. Other migraine “equivalents” in the first few years of life include benign paroxysmal vertigo, abdominal migraine, and cyclic vomiting.
1. Rosman NP, Douglass LM, Sharif UM, et al. The neurology of benign paroxysmal torticollis of infancy: report of 10 new cases and review of the literature. J Child Neurol. 2009;24:155-160.
2. Bhatia KP. Paroxysmal dyskinesias. Mov Disord. 2011;26:1157-1165.
3. Drigo P, Carli G, Laverda AM. Benign paroxysmal torticollis of infancy. Brain Dev. 2000;22:169-172.
4. Deonna T, Martin D. Benign paroxysmal torticollis in infancy. Arch Dis Child. 1981;56:912-919.
5. Giffin NJ, Benton S, Goadsby PJ. Benign paroxysmal torticollis of infancy: four new cases and linkage to CACN1 mutation. Dev Med Child Neurol. 2002;44:490-493.
6. Lewis DW, Gozzo Y, Avner M, et al. Primary headache disorders in children, adolescents, and young adults. In: Winner P, Lewis DW, eds. Young adult and pediatric headache management. Hamilton. Ontario, Canada: B.C. Decker, 2005:41-115.