Elsevier

Pediatric Neurology

Volume 42, Issue 1, January 2010, Pages 1-11
Pediatric Neurology

Review Article
Childhood Periodic Syndromes

https://doi.org/10.1016/j.pediatrneurol.2009.07.001Get rights and content

This review focuses on so-called “periodic syndromes of childhood that are precursors to migraine,” as included in the second edition of the International Classification of Headache Disorders. Presentation is characterized by an episodic pattern and intervals of complete health. Benign paroxysmal torticollis is characterized by recurrent episodes of head tilt, secondary to cervical dystonia, with onset between ages 2-8 months. Benign paroxysmal vertigo presents as sudden attacks of vertigo lasting seconds to minutes, accompanied by an inability to stand without support, between ages 2-4 years. Cyclic vomiting syndrome is distinguished by its unique intensity of vomiting, affecting quality of life, whereas abdominal migraine presents as episodic abdominal pain occurring in the absence of headache. Their mean ages of onset are 5 and 7 years, respectively. Diagnostic criteria and appropriate evaluation represent the key issues. Therapeutic recommendations include reassurance, lifestyle changes, and prophylactic as well as acute antimigraine therapy.

Introduction

The outstanding feature of migraine in any of its manifestations is its paroxysmal or periodic occurrence. There is always a return to baseline, with resulting sign-free intervals. It has long been recognized that migraine is not merely a syndrome of headache, but that the headache is associated with a range of other signs, e.g., vertigo, torticollis, and visual and sensorimotor disturbances, and gastrointestinal features, e.g., anorexia, abdominal pain, nausea, and vomiting. In some patients, these signs may occur in the absence of headache, and the attacks may precede the development of migraine headaches by several years. Some of these entities were formerly known as “migraine equivalents.” Nowadays, they are called “childhood periodic syndromes.” In the most recent criteria, i.e., the International Classification of Headache Disorders, Second Edition [1], of the International Headache Society, childhood periodic syndromes appear as a ubiquitous group termed “childhood periodic syndromes that represent precursors of migraine,” under Migraine Subtype 1.3. Our review mainly focuses on the childhood periodic syndromes included in the International Classification of Headache Disorders, Second Edition.

Section snippets

Periodic Syndrome

In 1933, Wyllie and Schlesinger introduced the term “periodic disorder of childhood” to describe recurrent episodes of pyrexia, headache, vomiting, and abdominal pain in childhood, and reported that the signs persisted in adult life as migraine or bilious attacks [2]. In Australia, Cullen and MacDonald noted that periodic syndrome frequently occurred in children with a family history of migraine [3]. In Boston, Barlow [4] confirmed that periodic syndrome was a common childhood precursor of

Benign Paroxysmal Torticollis of Infancy

Benign paroxysmal torticollis is a rare paroxysmal dyskinesia characterized by recurrent stereotypic attacks of torticollis, first described by Snyder in 1969 [9]. About 50 cases have been reported since 1969, but missed diagnoses are likely [10]. There is a female preponderance (about 70%) [10]. During an attack, abnormal inclination or rotation of the head to one side occurs by itself, or is accompanied by vomiting and ataxia. Torticollis may occur on either side. Other torsional or dystonic

Benign Paroxysmal Vertigo of Childhood

Described for the first time by Basser in 1964, benign paroxysmal vertigo usually presents in young children as episodes of unexplained fright associated with balance troubles or even falls [19]. The suggested prevalence is 2-2.6% [20], [21]. The sex distribution is equal.

Onset is sudden (95%) [22], with an expression of anxiety and fear on the face of the child, who may grasp a person standing nearby or any other support, or else may sway or refuse to stand. Ataxia may remain unnoticed,

Abdominal Migraine and Cyclic Vomiting Syndrome

In the absence of pathognomonic features, cyclic vomiting syndrome and abdominal migraine are often diagnosed through a process of exclusion. Clearly, the most important evaluation of a child who presents with recurrent paroxysmal vomiting or abdominal pain includes a careful history and physical examination. Laboratory investigations, endoscopy, and imaging should be requested to answer questions raised during this initial evaluation. If a strong clinical suspicion exists without evidence of

Other Childhood Periodic Syndromes

Beyond the childhood periodic syndromes described above, other clinical entities are not yet universally accepted by neurologists, and are not included in the International Classification of Headache Disorders, Second Edition. Although these clinical entities need further validation by longitudinal data collection, and although their relationship to migraine remains uncertain, we have chosen to discuss two of them briefly in this review: cyclic vomiting syndrome plus, and recurrent limb pain.

Pathophysiology

The unusual features evident during the aura phase of a migraine attack are thought to be attributable to the transient effects of “cortical spreading depression,” wherein a region of the cerebral cortex is temporarily and reversibly disturbed and suppressed [64]. During the aura phase, a migrating wave of regional cortical excitation, followed by depolarization and oligemia, results in visual, sensory, motor, or psychic phenomena, and clinically produces a wide variety of signs. In each

Conclusion

The clinical features and prevalence of the periodic syndromes of childhood are well-documented (Table 5). Nevertheless, they are probably under-recognized. It is worth emphasizing that practitioners should be aware of these benign events to ensure the correct diagnostic approach, sparing the child and family any needless anxiety or costly and sometimes invasive diagnostic procedures. The diagnosis relies on a careful semiologic analysis, mainly based on a parental description of

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