Vestibular migraine.

During the last decades a new vestibular syndrome has emerged that is now termed vestibular migraine (VM). The main body of evidence for VM is provided by epidemiologic data demonstrating a strong association between migraine and vestibular symptoms. Today, VM is recognized as one of the most common causes of episodic vertigo. The clinical presentation of VM is heterogeneous in terms of vestibular symptoms, duration of episodes, and association with migrainous accompaniments. Similar to migraine, there is no clinical or laboratory confirmation for VM and the diagnosis relies on the history and the exclusion of other disorders. Recently, diagnostic criteria for VM have been elaborated jointly by the International Headache Society and the Bárány Society. Clinical examination of patients with acute VM has clarified that the vast majority of patients with VM suffer from central vestibular dysfunction. Findings in the interval may yield mild signs of damage to both the central vestibular and ocular motor system and to the inner ear. These interictal clinical signs are not specific to VM but can be also observed in migraineurs without a history of vestibular symptoms. How migraine affects the vestibular system is still a matter of speculation. In the absence of high-quality therapeutic trials, treatment is targeted at the underlying migraine.

[Therapeutic apheresis for autoimmune encephalitis: a nationwide data collection]. / Therapeutische Apherese bei autoimmuner Enzephalitis: Eine bundesweite Datenerhebung.

BACKGROUND: The correlation between detection of autoantibodies and the pattern and severity of symptoms in patients with encephalitis was the crucial factor for the initiation of immune therapy. The elimination of autoantibodies using therapeutic apheresis by plasma exchange (PE) and immunoadsorption (IA) is a pathophysiologically guided therapeutic approach. The aim was to evaluate the current use of PE and for the first time also of IA for patients with autoimmune encephalitis. METHODS: A nationwide data collection was performed and the modified Rankin score (mRS) was used to evaluate the severity of neurological symptoms. RESULTS: Data of 31 treatment courses (30 patients and 1 relapse) were documented and 22 patients were positive for autoantibodies (NMDA-R, GABA, VGKC, Hu). In 23 cases PA was performed, tryptophan IA in 7 cases and in 1 patient both methods were applied. In 67 % of the treatment courses the mRS improved and the mean mRS of all patients was 3.2 before apheresis and 2.2 after apheresis (p < 0.05). All patients who were treated with IA improved clinically from a mean mRS of 3.9 before IA to 1.9 after IA (p < 0.01). CONCLUSIONS: For immune-mediated forms of encephalitis rapid elimination of autoantibodies with PA and IA seems to be an effective therapeutic option as part of a multimodal immune therapy and is already established in many clinics in Germany.

Chronic dizziness: a practical approach.

Patients with chronic dizziness pose a particular challenge to the clinician, partly because their symptoms correlate poorly with standard vestibular tests; so a 'test and think later' approach is likely to lead to diagnostic confusion rather than clarity. Rather, a meticulous clinical assessment is required. Here our approach to the chronic dizzy patient is described with an emphasis on treating the patient's symptoms.

[Vestibular migraine]. / Vestibuläre Migräne.

Vestibular migraine (VM) presents with attacks of spontaneous or positional vertigo lasting seconds to days. Headaches are often absent during acute attacks, but other symptoms of migraine, such as photophobia or auras may be present. Like migraine headaches VM triggers may include stress, sleep deprivation and hormonal changes. During acute attacks there may be central spontaneous or positional nystagmus and, less commonly, unilateral vestibular hypofunction. In the symptom-free interval vestibular testing shows mostly minor and non-specific findings. The pathogenesis of VM is uncertain but migraine mechanisms may interfere with the vestibular system at the level of the labyrinth, brainstem and cerebral cortex. Treatment includes vestibular suppressants for acute attacks and migraine prophylaxis for patients with frequent recurrences. Avoidance of triggers, stress management and biofeedback may also play a role. However, treatment efficacy has not been validated by properly controlled clinical trials. VM is not included in the 2004 International Headache Society Classification, where basilar-type migraine must have at least two posterior circulation manifestations so that isolated vertigo would not satisfy this criterion.

Screening for Menière's disease in the general population - the needle in the haystack.

CONCLUSION: Based on clinical history alone, 98.4% of the population with vestibular vertigo do not qualify for a diagnosis of Menière's disease (MD). Although frequent in dizziness clinics, MD is rare in the general population. OBJECTIVE: To narrow down the prevalence of MD in the general population. SUBJECTS AND METHODS: A representative sample adult population sample (n=4869) was screened for moderate or severe dizziness/vertigo. Subsequently, 1003 participants completed a validated neurotologic telephone interview on vestibular vertigo (VV). Prevalence of MD was determined by stepwise application of clinical criteria according to the AAO (1995): (1) at least two vertigo attacks of > or =20 min duration, (2) unilateral hearing loss, and (3) accompanying cochlear symptoms. RESULTS: Lifetime prevalence of VV was 7.4%. Of 243 participants with VV, 51 (21%) had recurrent vertigo lasting > or =20 min. Of these, nine reported unilateral hearing loss, and four had accompanying cochlear symptoms (1.6% of VV patients, population prevalence 0.12%).

Epidemiology of benign paroxysmal positional vertigo: a population based study.

OBJECTIVES: To examine the prevalence and incidence, clinical presentation, societal impact and comorbid conditions of benign paroxysmal positional vertigo (BPPV) in the general population. METHODS: Cross-sectional, nationally representative neurotological survey of the general adult population in Germany with a two stage sampling design: screening of 4869 participants from the German National Telephone Health Interview Survey 2003 (response rate 52%) for moderate or severe dizziness or vertigo, followed by validated neurotological interviews (n = 1003; response rate 87%). Diagnostic criteria for BPPV were at least five attacks of vestibular vertigo lasting <1 min without concomitant neurological symptoms and invariably provoked by typical changes in head position. In a concurrent validation study (n = 61) conducted in two specialised dizziness clinics, BPPV was detected by our telephone interview with a specificity of 92% and a sensitivity of 88% (positive predictive value 88%, negative predictive value 92%). RESULTS: BPPV accounted for 8% of individuals with moderate or severe dizziness/vertigo. The lifetime prevalence of BPPV was 2.4%, the 1 year prevalence was 1.6% and the 1 year incidence was 0.6%. The median duration of an episode was 2 weeks. In 86% of affected individuals, BPPV led to medical consultation, interruption of daily activities or sick leave. In total, only 8% of affected participants received effective treatment. On multivariate analysis, age, migraine, hypertension, hyperlipidaemia and stroke were independently associated with BPPV. CONCLUSION: BPPV is a common vestibular disorder leading to significant morbidity, psychosocial impact and medical costs.

Migrainous vertigo: prevalence and impact on quality of life.

OBJECTIVE: To investigate the epidemiology of migrainous vertigo (MV) in the general population by assessing prevalence, clinical features, comorbid conditions, quality of life, and health care utilization. METHODS: We screened a representative sample of the adult population in Germany (n = 4,869) for moderate or severe dizziness/vertigo and followed up with validated neurotologic telephone interviews (n = 1,003). Diagnostic criteria for MV were as follows: 1) recurrent vestibular vertigo; 2) migraine according to the International Headache Society; 3) migrainous symptoms during at least two vertiginous attacks (migrainous headache, photophobia, phonophobia, or aura symptoms); and 4) vertigo not attributed to another disorder. In a concurrent validation study (n = 61) the interviews had a sensitivity of 84% and a specificity of 94% for vestibular vertigo and 81% and 100% for migraine. RESULTS: The lifetime prevalence of MV was 0.98% (95% CI 0.70 to 1.37), the 12-month prevalence 0.89% (95% CI 0.62 to 1.27). Spontaneous rotational vertigo was reported by 67% of participants with MV while 24% had positional vertigo. Twenty-four percent always experienced headaches with their vertigo. Logistic regression analysis comparing participants with MV with dizziness-free migraineurs showed an independent association with coronary heart disease but not with sex, age, migrainous aura, education, stroke, hypertension, hyperlipidemia, body mass index, or depression. Age-adjusted health-related quality of life scores (SF-8 Health Survey) were consistently lower in participants with MV compared to dizziness-free controls. Two thirds of participants with MV had consulted a doctor but only 20% of these were diagnosed with MV. CONCLUSIONS: Migrainous vertigo is relatively common but underdiagnosed in the general population and has considerable personal and healthcare impact.

Short-term efficacy of Epley's manoeuvre: a double-blind randomised trial.

BACKGROUND: Benign paroxysmal positional vertigo of the posterior canal (PC-BPPV) is a common vestibular disorder and can be easily treated with Epley's manoeuvre. Thus far, the short-term efficacy of Epley's manoeuvre for treatment of PC-BPPV is unknown. OBJECTIVES: To evaluate the efficacy of Epley's manoeuvre for treatment of PC-BPPV 24 h after applying the manoeuvre. METHODS: The short-term efficacy of Epley's manoeuvre was compared with a sham procedure in 66 patients with PC-BPPV by using a double-blind randomised study design. RESULTS: 24 h after treatment, 28 of 35 (80%) patients in the Epley's manoeuvre group had neither vertigo nor nystagmus on positional testing compared with 3 of 31 (10%) patients in the sham group (p<0.001). CONCLUSION: Epley's manoeuvre is shown to resolve PC-BPPV both effectively and rapidly.

Epidemiology of vestibular vertigo: a neurotologic survey of the general population.

OBJECTIVE: The purpose of this study was to determine the prevalence and incidence of vestibular vertigo in the general population and to describe its clinical characteristics and associated factors. METHODS: The neurotologic survey had a two-stage general population sampling design: nationwide modified random digit dialing sampling for participation in the German National Telephone Health Interview Survey 2003 (response rate 52%) with screening of a random sample of 4,869 participants for moderate or severe dizziness or vertigo, followed by detailed neurotologic interviews developed through piloting and validation (n = 1,003, response rate 87%). Diagnostic criteria for vestibular vertigo were rotational vertigo, positional vertigo, or recurrent dizziness with nausea and oscillopsia or imbalance. Vestibular vertigo was detected by our interview with a specificity of 94% and a sensitivity of 84[corrected]% in a concurrent validation study using neurotology clinic diagnoses as an accepted standard (n = 61). RESULTS: The lifetime prevalence of vestibular vertigo was 7.4[corrected]%, the 1-year prevalence was 4.9[corrected]%, and the incidence was 1.4[corrected]%. In 80% of affected individuals, vertigo resulted in a medical consultation, interruption of daily activities, or sick leave. Female sex, age, lower educational level, and various comorbid conditions, including tinnitus, depression, and several cardiovascular diseases and risk factors, were associated with vestibular vertigo in the past year in univariate analysis. In multivariable analysis, only female sex, self-reported depression, tinnitus, hypertension, and dyslipidemia had an independent effect on vestibular vertigo. CONCLUSIONS: Vestibular vertigo is common in the general population, affecting [corrected] 5% of adults in 1 year. The frequency and health care impact of vestibular symptoms at the population level have been underestimated.

Disclosing to parents newborn carrier status identified by routine blood spot screening.

BACKGROUND: Newborn blood spot screening programmes are designed to detect serious conditions affecting individuals, where early treatment can improve health. It is suggested that screening can improve the experience of diagnosis for parents. For example, without newborn screening, when a child with cystic fibrosis becomes symptomatic a period of uncertainty can arise prior to diagnosis. These potential advantages of screening need to be weighed against potential disadvantages of screening at individual and population levels. Some newborn screening programmes inadvertently identify newborn infants who, although not affected by the condition, carry a gene for it and can pass on that gene to their children; these are 'genetic carriers'. Knowledge of newborn carrier status can lead to: testing of parents and family members, and concern about possible affected future siblings should both parents be identified as carriers; the possibility of such testing revealing the putative father is not the biological father; concern about the child's future reproductive choices; and unjustified anxiety about the health of the carrier newborn. There is an urgent need to develop clear guidance as to how to respond, with advances in technology fuelling the expansion of newborn blood spot screening and raised expectations of informed consent and disclosing test results. Depending on the condition for which screening is offered, options include: employing tests that do not identify carrier status, if available; identifying acceptable ways of disclosing carrier status; or identifying acceptable ways of not disclosing carrier status. These options are illustrated by screening programmes for sickle cell disorders and cystic fibrosis. Currently, there are no screening tests available for sickle cell disorders that do not identify carrier status. For cystic fibrosis, the policy choice is between an extended period of testing, and a screening result that is available sooner for most newborns, but inadvertently identifies carrier babies. OBJECTIVES: The aim of this review was to assess the impact of disclosing to parents newborn carrier status inadvertently identified by routine newborn blood spot screening. SEARCH STRATEGY: We searched for reports addressing disclosing newborn carrier status to parents following newborn screening for sickle cell disorders and cystic fibrosis in: commercially available electronic databases (October 2002), specialist registers, online journals, online abstracts and conference abstracts. We also scanned the reference lists of included papers. SELECTION CRITERIA: Studies addressing the impact of disclosing carrier status using a soundly controlled trial or randomised controlled trial. DATA COLLECTION AND ANALYSIS: Two researchers independently scanned titles and abstracts for relevance using the pre-specified inclusion criteria. Full reports of selected citations were then located and screened again for relevance by two researchers independently. At each stage, results were compared and discrepancies resolved by discussion. MAIN RESULTS: We found no controlled trials about disclosing carrier status. REVIEWERS' CONCLUSIONS: There is a need to develop and evaluate the effects of interventions to support the disclosure of carrier status to parents following newborn screening.

Benign paroxysmal positional vertigo predominantly affects the right labyrinth.

Benign paroxysmal positional vertigo (BPPV) occurs when there are freely moving particles in a semicircular canal and the head is turned in the plane of the affected canal. The aim of the present study was to clarify whether BPPV manifests equally in both labyrinths or whether there is a preponderance for one side. We conducted a PubMed literature search of BPPV case series which specified the affected side and a retrospective chart review of 80 consecutive patients with BPPV of the posterior canal who had presented at our dizziness clinic. Eighteen studies with a total of 3426 patients were identified. In our own series the right side was affected in 54 of 80 patients (right/left ratio 2.08). Altogether, in 3506 patients the right labyrinth was involved 1.41 times more often than the left (95% CI 1.37 to 1.45). We think that the reason for the predominant involvement of the right ear in BPPV is the habit-of most patients-of sleeping on the right side.

[Benign paroxysmal positional vertigo]. / Benigner paroxysmaler Lagerungsschwindel.

Benign paroxysmal positional vertigo is the most common vestibular disorder, accounting for about 20% of referrals in specialized dizziness clinics. Nowadays, canalolithiasis of the posterior semicircular canal has been widely accepted as the biological basis for typical benign paroxysmal positional vertigo as it is compatible with all clinical features of the disorder. Better understanding of its pathophysiological concepts has led to specific therapeutic strategies, which aim to clear the affected semicircular canal from mobile particles. After a single maneuver both Epley's and Semont's procedures lead to complete recovery in about 60% of patients and in nearly 100% when performed repeatedly. These positioning maneuvers have made benign paroxysmal positional vertigo the most successfully treatable cause of vertigo.

[Migrainous vertigo]. / Migräneschwindel.

Migrainous vertigo is one of the commonest cause of episodic vertigo and is increasingly recognized among neurootologists and migraine specialists. The clinical presentation is heterogeneous with spontaneous and positional vertigo lasting seconds to days and inconsistent temporal relationship to headache and other migrainous features. Findings during the acute episode suggest that several pathophysiological mechanisms can be involved. There are no evidence-based guidelines for therapy available. Presently, migrainous vertigo is not included in the classification of the International Headache Society (IHS). We propose that the diagnosis of definite migrainous vertigo can be made in a patient with migraine according to the IHS with at least two attacks of vestibular vertigo accompanied by migrainous symptoms when other causes are excluded.

Self-treatment of benign paroxysmal positional vertigo: Semont maneuver vs Epley procedure.

The authors compared the efficacy of a self-applied modified Semont maneuver (MSM) with self-treatment with a modified Epley procedure (MEP) in 70 patients with posterior canal benign paroxysmal positional vertigo. The response rate after 1 week, defined as absence of positional vertigo and torsional/upbeating nystagmus on positional testing, was 95% in the MEP group (n = 37) vs 58% in the MSM group (n = 33; p < 0.001). Treatment failure was related to incorrect performance of the maneuver in the MSM group, whereas treatment-related side effects did not differ significantly between the groups.

Trimethoprim-sulfamethoxazole exacerbates posthypoxic action myoclonus in a patient with suspicion of Pneumocystis jiroveci infection.

We describe a 58-year-old patient with relapsing high-grade non-Hodgkin's lymphoma who exhibited exacerbation of posthypoxic action myoclonus during high-dose intravenous trimethoprim-sulfamethoxazole (TMP-SMX) treatment for highly suspicious Pneumocystis jiroveci pneumonia (PCP). Three months previously the patient had experienced a hypoxic insult caused by respiratory arrest due to an anaphylactic reaction to antibiotic therapy. He had developed posthypoxic action myoclonus (Lance-Adams syndrome), which was well controlled by oral treatment with piracetam. However, after TMP-SMX therapy (115 mg/kg daily) was started for suspicion of newly developed PCP, posthypoxic action myoclonus worsened dramatically resulting in complete disability. Anti-myoclonic therapy with increased doses of piracetam and valproic acid did not significantly improve his clinical condition. Only when TMPSMX doses were reduced (38 mg/kg daily) on day 12 did action myoclonus cease within 2 to 3 days. We suggest that TMP-SMX can exacerbate posthypoxic action myoclonus.

Vertigo and dizziness related to migraine: a diagnostic challenge.

Vertigo and dizziness can be related to migraine in various ways: causally, statistically or, quite frequently, just by chance. Migrainous vertigo (MV) is a vestibular syndrome caused by migraine and presents with attacks of spontaneous or positional vertigo lasting seconds to days and migrainous symptoms during the attack. MV is the most common cause of spontaneous recurrent vertigo and is presently not included in the International Headache Society classification of migraine. Benign paroxysmal positional vertigo (BPPV) and Ménière's disease (MD) are statistically related to migraine, but the possible pathogenetic links have not been established. Moreover, migraineurs suffer from motion sickness more often than controls. Persistent cerebellar symptoms may develop in the course of familial hemiplegic migraine. Dizziness may also be due to orthostatic hypotension, anxiety disorders or major depression which all have an increased prevalence in patients with migraine.