Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, receives honorariums from Allergan, Avanir Pharmaceuticals, Curelator, Depomed, Dr. Reddy's Laboratories, eNeura, INSYS Therapeutics, Lilly USA, Supernus Pharmacerticals, Thernica and Trigemina for consulting. He is also the principal investigator for a clinical trials conducted by Alder Biopharmaceuticals, Amgen, electroCore Medical, Lily USA and Teva.)
The most updated version of the International Classification of Headache Disorders (ICHD), ICHD 3-beta, has replaced “childhood periodic syndromes that are commonly precursors of migraine” with “episodic syndromes that may be associated with migraine”. This group of disorders include recurrent gastrointestinal disturbance, benign paroxysmal vertigo, and benign paroxysmal torticollis. The new term still commonly affects children but is no longer limited to this population, and they are diagnosed in an otherwise healthy patient with a normal neurologic examination in between attacks (Gelfand 2018).
Benign paroxysmal torticollis was moved from the appendix section into the main body of the document. This disorder is characterized by spontaneous, recurrent episodes of head tilting in infants and toddlers and may evolve into benign paroxysmal vertigo, which typically affects children above the age of 2 years old. Patients with the above mentioned conditions are believed to have an increased likelihood to develop migraine later in life or have them comorbidly (Teggi et al 2018). In fact, studies show a possible progression from benign paroxysmal torticollis to benign paroxysmal vertigo and from benign paroxysmal vertigo to vestibular migraine, a phenomenon termed by Brodsky and colleagues as “vestibular march” (Brodsky et al 2018b).
Cyclical vomiting syndrome and abdominal migraine were brought under a new umbrella termed “recurrent gastrointestinal disturbance”. The term expanded to also include infantile colic, alternating hemiplegia of childhood, and vestibular migraine, which are added under the appendix section (Headache Classification Subcommittee of the International Headache Society 2013; Headache Classification Subcommittee of the International Headache Society 2015; Gelfand 2015).
Motion sickness and periodic sleep disorders including sleepwalking, sleeptalking, night terrors, and bruxism are also commonly seen among this patient population (Headache Classification Subcommittee of the International Headache Society 2013).
Benign paroxysmal vertigo is described as a separate entity from vestibular migraine in ICHD-3 beta, which recognizes the former as predominant in the pediatric population, whereas the latter happening at any age. A unilateral throbbing headache may occur during attacks of benign paroxysmal vertigo but is not mandatory, whereas at least half of the episodes of vestibular migraine should be associated with a headache fulfilling migraine criteria. Essentially, the short duration of the vertigo attacks and a less prominent headache help distinguish benign paroxysmal vertigo from vestibular migraine. For clarification purposes, benign paroxysmal positional vertigo is a third separate entity that will not be described here.
In this article, the author explains the clinical manifestations, why caution must be exercised to exclude the differential diagnoses, the appropriate recommended workup, and important treatment options.
• Causes of vertigo in children as well as their epidemiology differ from that of adults.
• The diagnosis of benign paroxysmal vertigo is based on the clinical history and exclusion of a more concerning diagnosis such as posterior fossa tumors, cervical spine abnormalities, vestibular otological pathology, epilepsy, and metabolic disorders.
• Epidemiological data confirm that migraine-related syndromes are the most common cause of vertigo in children.
Historical note and terminology
Wyllie and Schlesinger introduced the term periodic disorder of childhood in 1933, describing recurrent episodes of pyrexia, headache, vomiting, and abdominal pain in children (Wyllie and Schlesinger 1933).
In 1964 Basser described benign paroxysmal vertigo of childhood as a variety of vestibular neuronitis (Basser 1964). In 1967 Fenichel reported 2 siblings who displayed the syndrome and latter progressed into classical migraine, suggesting that benign paroxysmal vertigo of childhood was instead a form of migraine peculiar to childhood (Fenichel 1967).
As knowledge of childhood cephalalgia and its phenotypic presentation advanced, it was possible to observe stronger association of periodic syndromes with adult migraine with and without aura (Winner 2013). Even though the majority of studies available rely on small case series, they have consistently shown a higher prevalence of migraine in children diagnosed with benign paroxysmal vertigo compared to the general population (Krams et al 2011; Batu et al 2015). Similarities between children with benign paroxysmal vertigo and children with migraine headaches with respect to social and demographic factors, precipitating and relieving factors, and accompanying gastrointestinal, neurologic, and vasomotor features supports this common background (Abu-Arafeh and Russell 1995; Russell and Abu-Arafeh 1999; Batuecas-Caletrio et al 2013).
The International Classification of Headache Disorders, 3rd edition describes benign paroxysmal vertigo (1.6.2) under episodic syndromes that may be associated with migraine (1.6) (Headache Classification Subcommittee of the International Headache Society 2013). The disorder is characterized by recurrent, brief attacks of vertigo occurring without warning and resolving spontaneously in otherwise healthy children, although adults may be also affected. The diagnostic criteria include: at least 5 attacks; vertigo, occurring without warning, maximal at onset and resolving spontaneously after minutes to hours without loss of consciousness; and at least 1 of the associated symptoms or signs: 1) nystagmus, 2) ataxia, 3) vomiting, 4) pallor, 5) fearfulness. The patient should have normal neurologic exam and audiometric and vestibular functions between attacks. The disorder should not be attributed to another condition (Headache Classification Subcommittee of the International Headache Society 2013).
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