Migraine and isolated recurrent vertigo of unknown cause

Chronic recurrent attacks of vertigo, not associated with any auditory or neurological symptoms, are a common reason for referral to our neurotology clinic. Even after an extensive neurotological evaluation, some cases remain undiagnosed. We prospectively evaluated 72 consecutive patients who presented to the clinic with isolated recurrent vertigo of unknown cause. All patients underwent diagnostic evaluation to exclude identifiable causes of isolated recurrent vertigo. We compared the prevalence of migraine, according to the International Headache Society (IHS) criteria, in the isolated recurrent vertigo group, with a sex- and age-matched control group of orthopedic patients. The prevalence of migraine according to IHS criteria was higher in the isolated recurrent vertigo group (61.1%) than in the control group (10%; p < 0.01). Only 16.7% of patients had an abnormal vestibular function test. The most common abnormal finding was a unilateral vestibular weakness to caloric stimulation. Our results suggest that migraine should be considered in the differential diagnosis of isolated recurrent vertigo of unknown cause.     

Dominant recurrent ataxia and vertigo of childhood

Four families are described with an autosomal dominant illness characterized by the childhood onset of recurrent attacks of prolonged ataxia, server vertigo, and vomiting. The attacks often begin in infancy. On the average, attacks occur monthly, and last between one hour to more than a week. Variations in severity occur within families. During an attack, consciousness is unaltered, but severe vertigo makes walking impossible and vomiting is frequent and severe. An attack is marked by horizontal and vertical jerk nystagmus, accompanied by vertigo which is sometimes worsened by position; however, there is no muscular weakness. During an attack, blood gases, ammonia, and amino acid studies are normal. Between attacks patients manifest combinations of slight horizontal or vertical jerk nystagmus or mild clumsiness. Cochlear and labyrinthine studies and neurologic investigations were noncontributory. Conventional therapies for vertigo, epilepsy, and migraine were ineffective, but acetazolamide (250-500 mg/day) stopped the attacks.     

Migraine and recurrent epistaxis in children

Recurrent epistaxis is a common pediatric problem with uncertain etiology in most cases. We observed frequent complaints, or history of epistaxis in children with migraine. The aim of this study was to determine whether there is an association between epistaxis and migraine in children. A detailed questionnaire was used to conduct a study of 45 consecutive patients, ages 6-11 years, with migraine, diagnosed according to the 1997 proposed pediatric revisions to the International Headache Society criteria; the patients were evaluated in our Pediatric Neurology Clinic. Control subjects consisted of 64 children without recurrent headaches, matched as a group for age and sex, and drawn as a convenient sample from two general pediatric practices and an elementary school. Sixteen (36%) of 45 patients with migraine had epistaxis as compared with 7 (11%) of 64 control subjects (odds ratio = 4.5; 95% confidence interval 1.6-12.1; P = 0.002). Epistaxis began an average of 3 years before migraine with similar characteristics to idiopathic epistaxis in habitual nose-bleeders, such as onset in early childhood, high incidence in sleep, and family history of epistaxis. This study demonstrates a significant association between migraine and recurrent epistaxis in children. Recurrent epistaxis increased the odds of migraine more than fourfold. Moreover, these data raise the question of whether epistaxis may represent a precursor to childhood migraine. The two disorders may share a common pathogenesis, and a prospective, longitudinal study is required to define further the relationship between them.     

Recent advances in the genetics of recurrent vertigo and vestibulopathy

PURPOSE OF REVIEW: To focus on recent advances in the genetics of recurrent vertigo, with an overview on episodic ataxia, benign recurrent vertigo (mainly migraine-associated vertigo), bilateral vestibulopathy, and Ménière's disease. RECENT FINDINGS: Since the identification more than a decade ago of the genetic causes of episodic ataxia type 1 with myokymia caused by KCNA1 mutations and episodic ataxia type 2 with nystagmus caused by CACNA1A mutations, the list of episodic ataxia syndromes with distinct clinical features and genetic loci is slowly expanding, now up to episodic ataxia type 7. There is growing recognition for a correlation between benign recurrent vertigo and migraine, and acceptance for vertigo as a manifestation of migraine; efforts to identify susceptibility loci for migraine and migraine-associated vertigo are underway. A handful of families with vestibulopathy spanning several generations have been identified. Although no gene has yet been found, vestibulopathy with normal hearing variably associated with migraine is likely monogenic and heterogeneous, similar to nonsydromic deafness. There is also continuing effort to identify genetic causes of familial Ménière's disease. SUMMARY: Overlapping clinical features among different familial syndromes of recurrent vertigo and strong association with migraine suggest shared mechanisms. Collaborative efforts in patient identification and recruitment will facilitate progress in understanding disease mechanisms to improve diagnosis and treatment of recurrent vertigo.     

Disequilibrium of unknown cause in older people

Many older people complain of a sense of unsteadiness when standing or walking and many are fearful of falling. The cause of this disequilibrium is often difficult to determine even after thorough clinical evaluation. To better understand the pathogenesis of this symptom in a community-dwelling population, we studied 26 patients >75 years old who complained of disequilibrium in whom no cause was evident after clinical evaluation. Each patient and each of 26 age-matched controls underwent a comprehensive evaluation that included a history and examination, a questionnaire, functional scales, a gait and balance scale, mini-mental state testing, audiometry, visual acuity, visual tracking, rotational vestibular testing, and quantitative posturography. Although none had Romberg's sign, patients tended to sway more and do poorer on semiquantitative gait and balance testing than did controls. Patients showed few differences from controls for most other measurements. However, 7 patients, designated Group V, had profoundly reduced vestibular function. In the remaining 19 patients (Group D), the average vestibular function was still significantly lower than controls. Sway velocity measured with posturography was increased in the patients compared with controls, particularly in Group V patients. Measures of vestibular function correlated with increased sway velocity both in Group D alone and among all study participants. Quantitative measurement of vestibular function should be considered in older people complaining of disequilibrium, particularly if the cause is not apparent after the initial evaluation.     

DSM-III diagnoses associated with dyspepsia of unknown cause

The frequencies of DSM-III diagnoses in 30 patients with dyspepsia of unknown origin and 20 patients with organic dyspepsia were compared. Dyspepsia of unknown origin was associated with a higher prevalence of psychiatric diagnoses (86.7% versus 25.0%), particularly anxiety disturbances (66.7%).     

Origin of isolated vertigo in rotational vertebral artery syndrome

Rotational vertebral artery syndrome (RVAS) is characterized by recurrent attacks of paroxysmal vertigo, nystagmus, and ataxia induced by head rotation. Although recent report has described the RVAS as an important but unrecognized cause of isolated vascular vertigo, the locus of injury site responsible for isolated vertigo in RVAS is still unclear. We report here two patients with RVAS who had a stereotypic clinical presentation characterized by recurrent attacks of isolated vertigo induced by head rotation. The pattern of nystagmus observed in our patients with RVAS can be best explained by the stimulation of the vestibular labyrinth bilaterally or unilaterally. In RVAS, the isolated vertigo may occur due to transient ischemia of the superior vestibular labyrinth. RAVS should be considered in the differential diagnosis of positional vertigo, especially when vertigo is developed while sitting or standing position.     

An unusual cause of recurrent chorea.

Recurrent chorea is described in a 61-year-old woman who had had chorea gravidarum when she was younger. The recurrent chorea appeared to be induced by a topical vaginal cream that contained conjugated estrogen. This case is consistent with the existence of a recurrent syndrome of hormone-induced chorea. The effect of estrogen on the basal ganglia is complex and not fully understood.     

孤立性眩晕型小脑梗死的误诊分析与早期识别

目的旨在探讨急性孤立性眩晕型小脑梗死的早期识别因子及误诊为前庭周围性眩晕的原因。方法通过回顾性分析55例表现为孤立性眩晕发作的急性小脑梗死及58例年龄基本相配前庭周围性眩晕患者的临床资料,分析漏诊和误诊的具体原因,比较分析急性小脑梗死与前庭周围性眩晕患者的临床特点与脑血管病危险因素,寻找早期识别急性小脑梗死的预测因子。结果 13例(23.64%)初诊时被误诊为前庭周围性眩晕者。36例(65.45%)没有步态检查记录,所有患者没有进行眼前庭反射检查,初诊时阳性体征(如眼球震颤、辨距不良及直线行走困难等)的发现率明显偏低。16例(29.09%)没有及时进行头部CT或MRI检查。小脑性眩晕的早期呕吐、头痛症状及眩晕间隙期步态障碍的发生率也明显高于周围性眩晕(P<0.05)。二项Logistic回归分析发现男性、Essen缺血性卒中风险评估(ESRS)>3分是预测急性小脑梗死的重要因子(P<0.05)。结论急性小脑梗死通常表现为孤立性眩晕症状,容易漏诊或误诊。病史询问不详尽、神经系统体格检查不彻底、忽视了神经影像学检查以及早期CT检查的不敏感性是容易漏诊或误诊的重要原因。ESRS>3分、早期出现呕吐或头痛、眩晕间隙期直线行走困难、前庭眼球反射正常对早期诊断小脑性急性孤立性眩晕有重要临床价值。     

Schwannoma of the vestibulocochlear nerve presenting as isolated paroxysmal vertigo

We report a patient with a schwannoma of the eighth cranial (vestibulocochlear) nerve who presented with isolated episodes of paroxysmal vertigo and positive Dix-Hallpike maneuver, and without the common features of hearing loss, disequilibrium and tinnitus. There are no previous reports of paroxysmal episodes of vertigo as the sole manifestation of schwannoma of the vestibulocochlear nerve. Hence, recurrent paroxysmal vertigo should therefore prompt the physician to rule out schwannoma of the vestibulocochlear nerve as a potential cause even in the presence of normal hearing tests.     

Reverse Shapiro's syndrome--an unusual cause of fever of unknown origin

Reverse Shapiro's syndrome was first described by Hirayama et al. in a girl with periodic hyperthermia associated with complete agenesis of the corpus callosum. Here we report another such case in a 9-month-old girl presenting with fever of unknown origin since the age of 7 months. On examination, she had mild hypotonia with delayed developmental milestones. No other neurological or physical abnormalities were noted. The cause of her prolonged fever of unknown origin was investigated and all results were negative. Her brain magnetic resonance images showed agenesis of the corpus callosum. On the basis of the previous literature, we suggest that the periodic hyperthermia of this girl was caused by dopaminergic denervation of the hypothalamic thermoregulatory center. Treatment with dopamine agonists (levodopa plus carbidopa) failed to control the hyperthermia.