[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.

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.

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.

Stent angioplasty for cervical carotid artery stenosis in high-risk symptomatic NASCET-ineligible patients.

BACKGROUND AND PURPOSE: Although the North American Symptomatic Carotid Endarterectomy Trial (NASCET) has shown carotid endarterectomy (CEA) to be protective compared with medical therapy alone, its stringent eligibility criteria excluded patients with severe medical, angiographic, and neurological risk factors. We sought to determine the safety and efficacy of stent angioplasty in this high-risk subset for whom the perioperative morbidity and mortality of surgery are elevated. METHODS: Twenty-eight consecutive symptomatic NASCET-ineligible patients (10 female; median age, 72.2 years) underwent microcatheter-based carotid stent angioplasty. Half of the patients had sustained a previous stroke. Classification of surgical risk by Sundt criteria yielded no patients in grade 1, 3 patients in grade 2 (10.7%), 8 in grade 3 (28.6%), and 17 (60.7%) in grade 4. Stratification of stroke risk for medical therapy according to the European Carotid Surgery Trial (ECST) 5-point score showed 8 patients with a score of 3 (28.6%), 12 with 4 (42.8%), and 8 with 5 (28.6%). Follow-up was obtained in all patients at a median of 14 months. RESULTS: The procedure was technically successful in all cases (100%), with immediate stenosis reduction from a mean of 80.3% to 2.7%. There were no periprocedural deaths, 1 major stroke (3.6%), no minor strokes, and 3 transient ischemic attacks (10.7%). In-hospital complications included 2 nonfatal myocardial infarctions, 1 case of acute renal failure, and 1 groin hematoma requiring transfusion. There were 5 deaths during the follow-up period, all beyond 30 days after the procedure: 3 from cardiac causes, 1 from lung cancer, and 1 following unrelated surgery. The patient with major stroke died at 7.8 months during rehabilitation. No surviving patients had further strokes, and all except 1 (95.5%) remained asymptomatic. Anatomic follow-up in 20 patients showed occlusion in 2 (10%) (1 symptomatic, 1 asymptomatic) and intimal hyperplasia in 3 asymptomatic patients (15%). CONCLUSIONS: The clinical results and sustained freedom from symptoms and stroke during the short available follow-up period suggest that stent angioplasty may be useful in the treatment of symptomatic cervical carotid stenosis in high-risk patients despite a notable incidence of restenosis.

Benign paroxysmal positional vertigo.

Benign paroxysmal positional vertigo has been recognized as the most common vestibular disorder. The evolution of its pathophysiological concepts has led to current therapeutic strategies that have made it the most successfully treatable cause of vertigo.

The eye movements of syncope.

We videotaped the eye movements of syncope in 25 healthy volunteers who induced fainting by hyperventilation and Valsalva maneuver on a tilt table. In an additional three subjects, syncope was similarly induced during horizontal sinusoidal oscillation on a rotating chair while eye movements were recorded by electro-oculogram. Fourteen of 25 subjects experienced syncope on the tilt table. Six had downbeat nystagmus (DBN) at the onset that evolved into upward eye deviation, whereas seven showed isolated tonic upward deviation. In one subject the eyes remained in primary position. The gain of the vestibulo-ocular reflex (VOR) increased by 65% on average during syncope with concurrent vestibular stimulation. DBN, upward eye deviation, and increased VOR gain may all be caused by vestibular disinhibition caused by cerebellar hypoperfusion.

Continuous vertigo and spontaneous nystagmus due to canalolithiasis of the horizontal canal.

The authors present a patient with benign paroxysmal positional vertigo of the right horizontal semicircular canal who developed persistent vertigo with spontaneous horizontal nystagmus to the left and caloric hypoexcitability on the right after a head shaking maneuver. Both spontaneous nystagmus and canal paresis resolved after repeated shaking of the head. The most probable mechanism of this type of vertigo is plugging of the horizontal canal by otoconial particles with a negative endolymph pressure between plug and cupula.

The interrelations of migraine, vertigo, and migrainous vertigo.

OBJECTIVE: To assess the prevalence of migrainous vertigo in patients with migraine and in patients with vertigo according to explicit diagnostic criteria that are presented for discussion. METHODS: The authors prospectively evaluated 200 consecutive patients from a dizziness clinic and 200 patients from a migraine clinic for migrainous vertigo based on the following criteria: 1) recurrent vestibular symptoms (rotatory/positional vertigo, other illusory self or object motion, head motion intolerance); 2) migraine according to the criteria of the International Headache Society (IHS); 3) at least one of the following migrainous symptoms during at least two vertiginous attacks: migrainous headache, photophobia, phonophobia, visual or other auras; and 4) other causes ruled out by appropriate investigations. In addition, the authors compared the prevalence of migraine according to the IHS criteria in the dizziness clinic group with a sex- and age-matched control group of 200 orthopedic patients. RESULTS: The prevalence of migraine according to the IHS criteria was higher in the dizziness clinic group (38%) compared with the age- and sex-matched control group (24%, p < 0.01). The prevalence of migrainous vertigo was 7% in the dizziness clinic group, and 9% in the migraine clinic group. In 15 of 33 patients with migrainous vertigo, vertigo was regularly associated with migrainous headache. In 16 patients, vertigo occurred both with and without headache, and in two patients headache and vertigo never occurred together. The duration of attacks varied from minutes to days. CONCLUSION: These results substantiate the epidemiologic association between migraine and vertigo and indicate that migrainous vertigo affects a significant proportion of patients both in dizziness and headache clinics.

A modified Epley's procedure for self-treatment of benign paroxysmal positional vertigo.

We compared the efficacy of a modified Epley's procedure (MEP) and Brandt-Daroff exercises (BDE) for self-treatment of benign paroxysmal positional vertigo of the posterior semicircular canal (PC-BPPV) in 54 patients. PC-BPPV resolved within 1 week in 18 of 28 patients (64%) using the MEP and in 6 of 26 patients (23%) performing BDE (p<0.01). Type and adequate performance of the maneuver predicted treatment outcome in the multivariate analysis. The frequency of side effects was not significantly different between the two groups. The MEP is more suitable for self-treatment of PC-BPPV than conventional BDE.

Migraine and Ménière's disease: is there a link?

BACKGROUND: A possible link between Ménière's disease (MD) and migraine was originally suggested by Prosper Ménière. Subsequent studies of the prevalence of migraine in MD produced conflicting results. OBJECTIVE: To determine the lifetime prevalence of migraine in patients with MD compared to sex- and age-matched controls. METHODS: The authors studied 78 patients (40 women, 38 men; age range 29 to 81 years) with idiopathic unilateral or bilateral MD according to the criteria of the American Academy of Otolaryngology. Diagnosis of migraine with and without aura was made via telephone interviews according to the criteria of the International Headache Society. Additional information was obtained concerning the concurrence of vertigo and migrainous symptoms during Ménière attacks. The authors interviewed sex- and age-matched orthopedic patients (n = 78) as controls. RESULTS: The lifetime prevalence of migraine with and without aura was higher in the MD group (56%) compared to controls (25%; p < 0.001). Forty-five percent of the patients with MD always experienced at least one migrainous symptom (migrainous headache, photophobia, aura symptoms) with Ménière attacks. CONCLUSIONS: The lifetime prevalence of migraine is increased in patients with MD when strict diagnostic criteria for both conditions are applied. The frequent occurrence of migrainous symptoms during Ménière attacks suggests a pathophysiologic link between the two diseases. Alternatively, because migraine itself is a frequent cause of audio-vestibular symptoms, current diagnostic criteria may not differentiate between MD and migrainous vertigo.

Three hundred sixty-degree rotation of the posterior semicircular canal for treatment of benign positional vertigo: a placebo-controlled trial.

The canalithiasis hypothesis proposes that benign positional vertigo (BPV) is caused by dislodged otoconia that settle in the posterior semicircular canal (PSC). When head position is changed these particles move within the canal and induce abnormal endolymph flow. To clear the PSC from debris we developed a procedure that consists of a full circle of backward head rotation in the exact plane of the canal. Patients were seated in a three-dimensional motion device that rotated in steps of 110 degrees every 30 seconds. The first part of the study was conducted as an open trial; the second part followed a single-blinded, placebo-controlled design: Forward rotation (placebo) was applied first and backward rotation was applied 1 week later if BPV persisted. All patients were assessed with a symptom diary and, in the controlled trial, also with the Dix-Hallpike maneuver. In the open study 10 of 15 patients became asymptomatic after one treatment session. In the controlled trial all 15 patients remained symptomatic after forward rotation while 10 of 14 undergoing backward rotation were relieved from positional vertigo immediately (p = 0.004). The presence of secondary nystagmus during the procedure indicated a favorable outcome. Our findings provide evidence for the efficacy of canal-clearing procedures that validate the canalithiasis hypothesis of BPV.

Nuclear magnetic resonance and gamma camera tumor imaging using gadolinium-labeled monoclonal antibodies.

Chelate-derivatized monoclonal antibody labeled with paramagnetic gadolinium-3+ ion has been evaluated as a tumor-specific contrast-enhancing agent in nuclear magnetic resonance imaging in the Rauscher murine erythroleukemia system. With 10(-7) M concentrations of Gd3+ delivered to the tumor target, a small but reproducible difference in proton relaxation times (T1S) was observed in excised tumors. Nuclear magnetic resonance imaging of animals, however, failed to show significant contrast enhancement of the tumor; by comparison, gamma camera images with 153Gd-labeled specific antibody did permit clear tumor visualization without subtraction. The potential use of monoclonal antibodies in tumor imaging appears to be far greater in gamma camera and positron imaging than in nuclear magnetic resonance imaging.

Horizontal linear vestibulo-ocular reflex testing in patients with peripheral vestibular disorders.

UNLABELLED: Horizontal eye movements in response to lateral head translation [linear vestibulo-ocular reflex (LVOR)] in normal subjects and in patients with bilateral vestibular failure (n = 14), unilateral vestibular nerve section (n = 9), and benign positional vertigo (n = 14), were studied. LVORs were elicited in darkness by step acceleration (0.24 g) of the whole body along the interaural axis. RESULTS AND CONCLUSIONS: (1) In patients with bilateral vestibular failure, LVORs were either absent or abnormal with asymmetries, diminished velocities, and prolonged latencies. Measurements of dynamic visual acuity during linear self-motion showed decreased performance in patients at 1.0 and 1.5 Hz, which correlated with absent or delayed LVORs. These findings demonstrate the functional role of LVORs for dynamic visual acuity. (2) Early after vestibular nerve section, LVORs were diminished or absent with head acceleration toward the operated ear and normal in the opposite direction. After 6-10 weeks, responses were symmetrical again. Thus, a single utricle appears to be polarized with respect to the LVOR early after unilateral vestibular loss generating mostly contraversive responses. (3) Patients with benign positional vertigo showed mostly normal LVORs, which can be explained by minor utricular damage or central compensation of a chronic unilateral deficit.

Natural history and outcome of psychogenic seizures: a clinical study in 50 patients.

The natural history and the outcome of psychogenic seizures was studied in 50 patients by retrospective analysis and follow-up after a mean of 2 years. Concomitant epilepsy was definite in only 8% and possible in 14%, while 50% took anticonvulsants. Overall, 66% of patients showed heterogeneous psychiatric abnormalities, most commonly a depressive syndrome (24%), whereas hysterical personality features were rare (8%). Forty-two percent of patients were unemployed. Follow-up of 41 patients (82%) showed that 34% had become seizure free. Outcome was poor in those with a long history of psychogenic seizures and pathological psychiatric findings. In contrast, almost all patients with recent onset of psychogenic seizures and normal psychological status had become seizure free. At follow-up, 56% of patients were found to be in a poor or very poor state, which resulted from a combination of physical, psychic and social problems in most cases.

How to identify psychogenic disorders of stance and gait. A video study in 37 patients.

Thirty-seven patients with psychogenic disorders of stance and gait were clinically evaluated, recorded on video, and analysed with regard to clinical phenomenology. Characteristic, suggestive and unspecific features were identified. Six characteristic features proved most valuable for diagnosis of psychogenesis, as they occurred alone or in combination in 97% of patients: (1) momentary fluctuations of stance and gait, often in response to suggestion; (2) excessive slowness or hesitation of locomotion incompatible with neurological disease; (3) "psychogenic" Romberg test with a build-up of sway amplitudes after a silent latency or with improvement by distraction; (4) uneconomic postures with wastage of muscular energy; (5) the "walking on ice" gait pattern, which is characterized by small cautious steps with fixed ankle joints; (6) sudden buckling of the knees, usually without falls. Seventy-three percent of patients had additional suggestive features. Classification into characteristic subtypes was not found useful because predominant features varied from patient to patient and occurred in various combinations. Factitious impairment of stance and gait was studied in 13 healthy drama students. Simulated gait dysfunction appeared less conspicuous and more difficult to diagnose than the clinical psychogenic disorders.