Dr. Root of Barrow Neurological Institute has no relevant financial relationships to disclose.)
Dr. Robblee of Barrow Neurological Institute has no relevant financial relationships to disclose.)
Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, received honorariums from Abbie, Curelator, Ipsen Therapeutics, Lundbeck Biopharmaceuticals, Supernus Pharmaceuticals, and Theranica for consulting. He is also the principal investigator for clinical trials conducted by Amgen, ElectroCore Medical, and Teva.)
Benign paroxysmal vertigo (BPV) is one of the most common etiologies of vertigo in the pediatric population. Its diagnostic criteria have been defined by the International Classification of Headache Disorders (ICHD). Though its characteristics are well-defined, its pathophysiology remains in question. This article looks to explain the history of benign paroxysmal vertigo and its common features, diagnostic criteria, prognosis, and differential diagnosis that should be considered in pediatric patients presenting with vertigo. Although there exists a paucity of data regarding treatment of benign paroxysmal vertigo, its management will also be discussed.
The most updated version of the International Classification of Headache Disorders, ICHD 3, 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 by diagnostic criteria (Headache Classification Committee of the International Headache Society 2018). They are diagnosed in an otherwise healthy patient with a normal neurologic examination in between attacks (Gelfand 2018).
Benign paroxysmal vertigo is described as a separate entity from vestibular migraine in ICHD-3. Benign paroxysmal vertigo is predominantly described in the pediatric population, whereas vestibular migraine develops in childhood as well as during adulthood (Huang et al 2020). 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. Benign paroxysmal vertigo is considered a precursor syndrome to migraine (Headache Classification Committee of the International Headache Society 2018). The ICHD-3 “episodic syndromes that may be associated with migraine” section also contains benign paroxysmal torticollis, cyclical vomiting syndrome, and abdominal migraine. Benign paroxysmal positional vertigo is commonly confused with benign paroxysmal vertigo considering the similarity in their names. These conditions will be discussed further in the differential diagnosis section below.
• Benign paroxysmal vertigo is characterized by recurrent, brief attacks of vertigo occurring without warning and resolving spontaneously in otherwise healthy patients.
• Benign paroxysmal vertigo is one of the most common causes of vertigo in childhood, with most cases experiencing spontaneous remission.
• Patients with benign paroxysmal vertigo are likely to have a family history of migraine and have a higher risk of developing migraine in adolescence or adulthood compared to the general population.
• The diagnosis of benign paroxysmal vertigo is based on the clinical history and exclusion of other diagnoses such as posterior fossa tumors, cervical spine abnormalities, vestibular pathology, epilepsy, and metabolic disorders.
• Prevention and treatment of benign paroxysmal vertigo largely lacks sufficient evidence, though oral migraine preventive medications also frequently used.
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 headache 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 support this common background with respect to social and demographic factors, precipitating and relieving factors, and accompanying gastrointestinal, neurologic, and vasomotor features (Abu-Arafeh and Russell 1995; Russell and Abu-Arafeh 1999; Batuecas-Caletrio et al 2013).
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