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The New Type of Vertigo: Recurrent Spontaneous Vertigo with Interictal Headshaking Nystagmus

Vertigo is an abnormal sensation of motion that is characterized by a feeling of spinning around. Recurrent spontaneous vertigo is a type of vertigo that recurs and is spontaneous. Recurrent spontaneous vertigo has a variety of causes, ranging from benign conditions such as benign paroxysmal positional vertigo and Meniere's disease to severe causes such as strokes or tumors.

The New Type of Vertigo: Recurrent Spontaneous Vertigo with Interictal Headshaking Nystagmus
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However, there are also recurrent spontaneous vertigo with unknown causes and diagnosed as benign recurring vertigo which is a diagnosis of exclusion. At present, in South Korea, a new type of recurrent vertigo has been discovered that is referred to as Recurrent spontaneous vertigo with interictal headshaking nystagmus.

How to Diagnose Recurrent Spontaneous Vertigo with Interictal Headshaking Nystagmus

Recurrent spontaneous vertigo with head-shaking nystagmus is a type of recurrent vertigo with unknown etiology. Inclusion criteria for diagnosing this condition are:
  1. Changes in position or head movements do not trigger more than two spontaneous rotational vertigo attacks
  2. Not associated with migraine-type headaches and auditory symptoms during or between attacks
  3. No focal neurological symptoms during or after an attack, such as episodic ataxia, cursory stroke, or vestibular epilepsy
  4. No evidence of peripheral vestibulopathy was found on caloric, rotatory, or head-impulse examinations
  5. No asymmetrical hearing loss from pure tone audiometry
  6. No lesions on brain MRI that could explain the symptoms of recurrent vertigo
  7. There is no history of the disease that can explain the symptoms of recurrent vertigo
  8. Incompatible with the diagnosis of other vestibular disorders such as compensated vestibular neuritis, vestibular migraine, vestibular paroxysms, or Meniere's disease.
  9. Based on these inclusion criteria, the diagnosis of this disease is a diagnosis per exclusion, which can only be established when all the other etiologies of vertigo have been eliminated.
A study in South Korea from 2004 to 2015 involving 3990 patients with recurrent spontaneous vertigo found 338 cases of benign recurrent vertigo. Among these 338 cases, researchers found new vertigo subtypes accompanied by head-shaking nystagmus in about 10% of patients. This subtype is referred to as recurrent spontaneous vertigo with head-shaking nystagmus.

Causes and Risk Factors

The etiology of this type of vertigo is the same as the cause of benign recurrent vertigo, which is still not known with certainty. However, it is thought that this condition has genetic links to Meniere's disease and vestibular migraine. Epidemiological data show that a quarter of family members of patients with Meniere's disease will experience episodes of spontaneous vertigo without auditory symptoms. Furthermore, two-thirds of the families of patients with recurrent benign vertigo meet the diagnostic criteria of vestibular migraine. Recurrent benign vertigo is also usually associated with complaints of motion sickness and has a family history of vertigo complaints.

Symptoms of Recurrent Spontaneous Vertigo with Interictal Headshaking Nystagmus

  • The symptoms of recurrent spontaneous vertigo with head-shaking nystagmus are more severe than without head-shaking nystagmus.
  • The attack duration ranges from a few minutes to several days with a frequency ranging from 2-3 times per week to once a year.
  • Patients experience symptoms of nausea, vomiting, headaches that are not caused by migraines, intolerance to head movements, palpitations, chest pain, and urgency for defecation during an attack. Without any neurological and neurotological examination abnormalities in patients.
  • Nystagmus in recurrent spontaneous vertigo with head-shaking nystagmus is 2-3 times longer than nystagmus in vestibular neuritis, vestibular migraine, or Meniere's disease.
  • Nystagmus also has a higher intensity than nystagmus in vestibular neuritis and can be induced with only 2-5 seconds of head shaking in 71% of cases.


How to treat Recurrent Spontaneous Vertigo with Interictal Headshaking Nystagmus

Preventive medicines can be given to adult sufferers with frequent attacks of vertigo or severe attacks. In one-third of patients can experience partial or complete improvement with medication. Drug options that can be given include:
  • Nimodipine 2 x 5 mg,
  • Betahistine 2 x 6 mg, n
  • Flunarizine 1 x 5-10 mg,
  • Baclofen 2 x 10 mg,
  • Nortriptyline 1 x 5-20 mg,
  • Acetazolamide 2-3 X 250 mg 


Prognosis of Recurrent Spontaneous Vertigo with Interictal Headshaking Nystagmus

Although having more severe symptoms than recurrent spontaneous vertigo without head-shaking nystagmus, Recurrent spontaneous vertigo with interictal headshaking nystagmus has a good prognosis because generally, patients will experience improvement in the condition to a total resolution without complications.

Data on complications of this disease is still very minimal. There are only reports of complications from hyperacusis due to vertigo.

Conclusion
Some neurologists in South Korea have identified a new type of vertigo called recurrent spontaneous vertigo with head-shaking nystagmus. This type of vertigo has dizziness that is more severe than other benign recurrent vertigo, and nystagmus is longer than vertigo caused by vestibular conditions.

Prophylactic therapy can be given for frequent or severe vertigo. Drug options that can be provided include betahistine, baclofen, and acetazolamide. Recurrent spontaneous vertigo with head-shaking nystagmus has a good prognosis.


References
1. Hackethal V. Vertigo with An Effective Treatment but Unknown Cause? Neurologytimes.com. 2018
2. Lee SU, Choi JY, Kim HJ, Kim JS. Recurrent spontaneous vertigo with interictal headshaking nystagmus. Neurology. 2018 Jun 12;90(24):e2135-45.

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