Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome

Journal of Neurology, Apr 2016

The leading symptoms of vestibular paroxysmia (VP) are recurrent, spontaneous, short attacks of spinning or non-spinning vertigo that generally last less than one minute and occur in a series of up to 30 or more per day. VP may manifest when arteries in the cerebellar pontine angle cause a segmental, pressure-induced dysfunction of the eighth nerve. The symptoms are usually triggered by direct pulsatile compression with ephaptic discharges, less often by conduction blocks. MR imaging reveals the neurovascular compression of the eighth nerve (3D constructive interference in steady state and 3D time-of-flight sequences) in more than 95 % of cases. A loop of the anterior inferior cerebellar artery seems to be most often involved, less so the posterior inferior cerebellar artery, the vertebral artery, or a vein. The frequent attacks of vertigo respond to carbamazepine or oxcarbazepine, even in low dosages (200–600 mg/d or 300–900 mg/d, respectively), which have been shown to also be effective in children. Alternative drugs to try are lamotrigine, phenytoin, gabapentin, topiramate or baclofen or other non-antiepileptic drugs used in trigeminal neuralgia. The results of ongoing randomized placebo-controlled treatment studies, however, are not yet available. Surgical microvascular decompression of the eighth nerve is the “ultima ratio” for medically intractable cases or in exceptional cases of non-vascular compression of the eighth nerve by a tumor or cyst. The International Barany Society for Neuro-Otology is currently working on a consensus document on the clinical criteria for establishing a diagnosis of VP as a clinical entity.

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Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome

J Neurol Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome Thomas Brandt 0 1 2 3 4 Michael Strupp 0 1 2 3 4 Marianne Dieterich 0 1 2 3 4 0 Department of Neurology, Ludwig-Maximilians University , Munich , Germany 1 Institute for Clinical Neurosciences, Ludwig-Maximilians University , Munich , Germany 2 & Thomas Brandt 3 Munich Cluster for Systems Neurology (SyNergy) , Munich , Germany 4 German Center for Vertigo and Balance Disorders, Ludwig- Maximilians University , Munich , Germany The leading symptoms of vestibular paroxysmia (VP) are recurrent, spontaneous, short attacks of spinning or non-spinning vertigo that generally last less than one minute and occur in a series of up to 30 or more per day. VP may manifest when arteries in the cerebellar pontine angle cause a segmental, pressure-induced dysfunction of the eighth nerve. The symptoms are usually triggered by direct pulsatile compression with ephaptic discharges, less often by conduction blocks. MR imaging reveals the neurovascular compression of the eighth nerve (3D constructive interference in steady state and 3D timeof-flight sequences) in more than 95 % of cases. A loop of the anterior inferior cerebellar artery seems to be most often involved, less so the posterior inferior cerebellar artery, the vertebral artery, or a vein. The frequent attacks of vertigo respond to carbamazepine or oxcarbazepine, even in low dosages (200-600 mg/d or 300-900 mg/d, respectively), which have been shown to also be effective in children. Alternative drugs to try are lamotrigine, phenytoin, gabapentin, topiramate or baclofen or other Vestibular paroxysmia; Neurovascular cross- compression; Vestibular nerve; Episodic vertigo; Carbamazepine; Review - This manuscript is part of a supplement sponsored by the German Federal Ministry of Education and Research within the funding initiative for integrated research and treatment centers. non-antiepileptic drugs used in trigeminal neuralgia. The results of ongoing randomized placebo-controlled treatment studies, however, are not yet available. Surgical microvascular decompression of the eighth nerve is the ‘‘ultima ratio’’ for medically intractable cases or in exceptional cases of non-vascular compression of the eighth nerve by a tumor or cyst. The International Barany Society for Neuro-Otology is currently working on a consensus document on the clinical criteria for establishing a diagnosis of VP as a clinical entity. Introduction The main symptoms of vestibular paroxysmia (VP) are brief attacks of spinning or non-spinning vertigo which lasts a fraction of a second to a few minutes and occurs with or without ear symptoms (tinnitus and hypo- or hyperacusis). Arteries or rarely veins in the cerebellar pontine angle are the pathophysiological cause of a segmental, pressure-induced dysfunction of the eighth nerve. As in trigeminal neuralgia, the symptoms are triggered by direct pulsatile compression and ephaptic pathological paroxysmal interaxonal transmission between neighbouring and possibly in part demyelinated axons [ 1, 2 ]. VP accounts for 3.7 % of 17,718 consecutive outpatients of the German Center for Vertigo and Balance Disorders (Fig. 1). The syndrome of neurovascular cross-compression of the eighth nerve was previously connected with so-called ‘‘disabling positional vertigo’’ [ 3 ], a very heterogeneous syndrome of vertigo with symptoms of various durations Fig. 1 Common peripheral vertigo and balance disorders of the vestibular labyrinth (right) and the vestibular nerve (left) make up about 47 % of more than 17,000 outpatients in a multidisciplinary dizziness unit (Brandt et al. 2014). Labyrinthine disorders include benign paroxysmal positional vertigo due to canalo- or cupulolithiasis, superior canal dehiscence syndrome, and Menie`re’s disease with endolymphatic hydrops. Vestibular nerve disorders include superior (from seconds to days), various characteristic features (rotatory or postural vertigo, light-headedness or gait instability without vertigo), and varying accompanying symptoms. As these vague descriptions also applied to patients with benign paroxysmal positional vertigo, Menie`re’s disease, bilateral vestibulopathy, or somatoform phobic postural vertigo, the clinical definition was subsequently made more precise and then the term ‘vestibular paroxysmia’ was introduced [ 1 ]. Whereas Møller and Jannetta [ 4 ] recommended microvascular surgical decompression with various degrees of success, we introduced the use of anticonvulsants such as carbamazepine as the medical therapy of first choice [ 1 ]. Clinical syndrome VP is suspected if brief and frequent attacks of vertigo are accompanied by the following features [ 1 ]: • Short attacks of rotatory or postural vertigo last for seconds to minutes with instability of posture and gait. • Attacks may often be triggered by particular head positions or hyperventilation, and may be influenced by changing the head posi (...truncated)


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Thomas Brandt, Michael Strupp, Marianne Dieterich. Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome, Journal of Neurology, 2016, pp. 90-96, Volume 263, Issue 1, DOI: 10.1007/s00415-015-7973-3