Project description:BackgroundPseudo-spontaneous nystagmus has been reported in patients with direction-changing positional nystagmus (DCPN). Recently, the concept of a "light cupula" has been introduced as a pathophysiology that can exhibit persistent geotropic DCPN. Patients with persistent DCPN could have different characteristics of nystagmus. Therefore, we investigated the pseudo-spontaneous nystagmus in patients with transient (canalolithiasis) and persistent (belong to light cupula theory) geotropic DCPN.MethodsIn this study, prospectively, 49 patients with persistent geotropic DCPN and 67 patients with transient geotropic DCPN were enrolled. We compared the incidence of pseudo-spontaneous nystagmus between persistent and transient DCPN patients and characteristics of pseudo-spontaneous nystagmus and positional nystagmus by the head roll test in these patients. A prospective study was conducted at a dizziness clinic.ResultsPatients with persistent geotropic DCPN exhibited significantly higher incidence of pseudo-spontaneous nystagmus than patients with transient geotropic DCPN. Patients with transient DCPN showed a significantly higher mean SPV value during the head roll test than patients with persistent DCPN. All patients exhibiting pseudo-spontaneous nystagmus in patients with persistent DCPN had a null plane, and all patients had nystagmus beats to the opposite side of the null plane or the lesion side.ConclusionOur results support the possibility that the mechanism between persistent and transient geotropic DCPN may be different. However, more studies are needed on the pathogenesis and mechanism of the two diseases, including the occurrence of pseudo-spontaneous nystagmus in the disease entity.
Project description:Background and purposeThis study aimed to determine the clinical features, diagnosis, and treatment of patients with persistent geotropic (pG) and persistent apogeotropic (pAG) direction-changing positional nystagmus (DCPN).MethodsThis retrospective study included 30 patients with pG-DCPN and 44 patients with pAG-DCPN. All patients underwent neurological and neurotological examinations, including an evaluation of gaze-evoked nystagmus, eye-movement tests, and assessments of limb ataxia and balance, as well as magnetic resonance imaging to exclude central causes. The characteristics of positional nystagmus were detected using the supine roll test (SRT) and bow-and-lean test (BLT). The null point (NP) at which the nystagmus disappeared was determined. All patients were treated with the barbecue maneuver, and treatment efficacy was evaluated immediately, 1 week, and 1 month after treatment.ResultsThe history of diseases associated with atherosclerosis, peripheral vestibular disorders, otological disease, and migraine differed significantly between patients with pG-DCPN and pAG-DCPN. The affected sides of persistent horizontal DCPN can be determined using the SRT and the BLT, while determining the second NP and vestibular function as well as performing an audiological evaluation can be used to assist in identifying the affected side. The efficacy rates immediately and 1 week after treatment with the barbecue maneuver were higher in patients with pAG-DCPN than in patients with pG-DCPN.ConclusionspAG-DCPN was more compatible with the characteristics of cupulolithiasis, and pG-DCPN was more likely to be associated with a light cupula rather than canalolithiasis. pAG-DCPN was more likely to be accompanied by a disease associated with atherosclerosis, while pG-DCPN was often accompanied by autoimmune-related diseases and a history of migraine. The associations between pAG-DCPN, pG-DCPN, and the above-mentioned diseases need to be clarified further. The canalith-repositioning maneuver was effective in patients with pAG-DCPN and ineffective in patients with pG-DCPN, but most cases of pG-DCPN are self-limiting.
Project description:ObjectiveTo study the long-term treatment outcome of vestibular paroxysmia (VP).Study designRetrospective study.SettingTertiary referral hospital.MethodsWe analyzed records of 29 consecutive patients who were diagnosed with VP and who were treated with VP-specific anticonvulsants for at least 3 months. Patients were followed for a minimum of 6 months. We recorded and assessed starting and target dosage of medications, time to achieve adequate therapeutic response, adverse effects, and the rates of short-term and long-term remission without medication.ResultsAll 29 patients were started on oxcarbazepine as first-line treatment, and 93.1% and 100% of patients reported good-to-excellent therapeutic response within 2 and 4 weeks, respectively. Three patients switched to other anticonvulsants at 3 months. At long-term follow-up (8-56 months), most (84.6%) oxcarbazepine-treated patients maintained good therapeutic response at doses between 300 and 600 mg/day. Eleven (37.9%) patients experienced complete remission without medication for more than 1 month, of which six (20.7%) had long-term remission off medication for more than 12 months. Nineteen (65.5%) patients had neurovascular compression (NVC) of vestibulocochlear nerve on MRI, but its presence or absence did not predict treatment response or remission.ConclusionLow-dose oxcarbazepine monotherapy for VP is effective over the long term and is generally well-tolerated. About 20% of patients with VP in our study had long-term remission off medication.
Project description:In 2016, the Bárány Society defined new diagnostic criteria for the neurovascular compression syndrome of the eighth nerve, called "vestibular paroxysmia" (VP), differentiating between definite (dVP) and probable (pVP) forms. The aim of this study was (1) to describe clinical symptoms and laboratory findings in a well-diagnosed large patient cohort according to those criteria, and (2) to evaluate the long-term course over years in dVP. We identified 146 patients (73 dVP, 73 pVP) from our tertiary dizziness center registry. Data of structured history-taking, clinical neurological, neuro-ophthalmological/-otological examinations as well as MRI imaging were extracted for analyses. Overall, attack frequency ranged between 5 and 30 attacks per day; spinning vertigo was the most frequent type. In two-thirds of patients, attacks occurred spontaneously; in one-quarter, they were triggered by head movements. The majority (approximately 70%) reported no accompanying symptoms; in those with symptoms, mild unilateral cochlear symptoms prevailed. One-third of patients initially showed hyperventilation-induced nystagmus without specific direction, and a deviation of the subjective visual vertical between 3° and 6°. Complete loss of peripheral vestibular function was never evident. dVP and pVP significantly differed concerning the vertigo type, e.g., spinning vertigo was more frequent in dVP. Fortunately, three-quarters of dVP patients remained attack-free during follow-up (mean 4.8 years, standardized questionnaire), more than half of them even without any medication. Patients with ongoing attacks showed significantly higher attack frequency at baseline, but reported persistent frequency reduction. Overall, the long-term prognosis of VP appears favorable, not necessarily requiring ongoing treatment.
Project description:Vestibular paroxysmia (VP) is an uncommon paroxysmal disease, characterized by vertigo, tinnitus, and postural unsteadiness. The main reason of VP is neurovascular cross compression, while few cases of VP accompanied with congenital vascular malformation were reported. Here, we describe a 22-year-old patient with VP caused by congenital anterior inferior cerebellar artery (AICA) malformation who completely recovered after taking oral medicine. This report shows that VP caused by congenital vascular malformation can occur in adults and that oral medication is effective.
Project description:The aim of this study was to investigate the clinical characteristics and underlying pathogenesis of episodic vestibular syndrome (EVS) with hyperventilation-induced downbeat nystagmus (HV-DBN).
Project description:Vestibular paroxysmia (VP) is a rare condition. The pathogenesis is linked to a neurovascular conflict (NVC) between an abnormal arterial loop and the VII/VIII cranial nerve complex in the cerebello-pontine angle. Due to its rarity, intraoperative findings are only anecdotally reported. Here we reported on a case of VP, showing the radiological images and the intraoperative surgical video of microvascular decompression (MVD). Further we discussed our findings considering the pertinent literature. We think that in case of VP the concordance between the side of tinnitus/hypoacusia and the side of NVC on magnetic resonance imaging should be always looked for before considering MVD as a therapeutic option.
Project description:RationaleVestibular paroxysmia (VP) is characterized by spontaneous, recurrent, short, paroxysmal attacks of vertigo with or without tinnitus.Patient concernsWe report a case of paroxysmal recurrent vertigo accompanying clicking tinnitus on the left side in a 61-year-old patient. He had undergone microvascular decompression to treat the left-side hemifacial spasm 6 years prior. The patient first developed vertigo attacks about 3 years after microvascular decompression, and the attacks increased in frequency over the last 4 months. Video-nystagmography revealed a background right-beating nystagmus which was reversed every 55 seconds, to left-beating nystagmus for 17 seconds.DiagnosisBrain magnetic resonance imaging and angiography demonstrated a compression of the cisternal segment of the left vestibulocochlear nerve between the tortuous right vertebral artery and the posterior wall of the left porus acusticus internus.Interventions and outcomesUnder the diagnosis of VP, 300 mg oxcarbazepine was administered daily, which relieved the symptoms dramatically.LessonThe neurovascular cross-compression of the vestibulocochlear nerve by the contralateral vertebral artery tortuosity can cause VP. Periodic paroxysms of right-beating nystagmus accompanying the left-side tinnitus during vertigo attacks in our patient can be explained by secondary central hyperactivity in both vestibular and cochlear nuclei following long-standing neurovascular cross-compression.