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1.
J Neurol ; 2024 May 03.
Article in English | MEDLINE | ID: mdl-38702563

ABSTRACT

BACKGROUND: The current diagnostic workup for chronic dizziness in elderly patients often neglects neuropsychological assessment, thus missing a relevant proportion of patients, who perceive dizziness as a subjective chief complaint of a concomitant cognitive impairment. This study aimed to establish risk prediction models for cognitive impairment in chronic dizzy patients based on data sources routinely collected in a dizziness center. METHODS: One hundred patients (age: 74.7 ± 7.1 years, 41.0% women) with chronic dizziness were prospectively characterized by (1) neuro-otological testing, (2) quantitative gait assessment, (3) graduation of focal brain atrophy and white matter lesion load, and (4) cognitive screening (MoCA). A linear regression model was trained to predict patients' total MoCA score based on 16 clinical features derived from demographics, vestibular testing, gait analysis, and imaging scales. Additionally, we trained a binary logistic regression model on the same data sources to identify those patients with a cognitive impairment (i.e., MoCA < 25). RESULTS: The linear regression model explained almost half of the variance of patients' total MoCA score (R2 = 0.49; mean absolute error: 1.7). The most important risk-predictors of cognitive impairment were age (ß = - 0.75), pathological Romberg's sign (ß = - 1.05), normal caloric test results (ß = - 0.8), slower timed-up-and-go test (ß = - 0.67), frontal (ß = - 0.6) and temporal (ß = - 0.54) brain atrophy. The binary classification yielded an area under the curve of 0.84 (95% CI 0.70-0.98) in distinguishing between cognitively normal and impaired patients. CONCLUSIONS: The need for cognitive testing in patients with chronic dizziness can be efficiently approximated by available data sources from routine diagnostic workup in a dizziness center.

2.
J Neurol ; 269(10): 5239-5248, 2022 Oct.
Article in English | MEDLINE | ID: mdl-34894282

ABSTRACT

BACKGROUND: Combining a mobile application-based vestibular diary called the DizzyQuest and an iPad-based hearing test enables evaluation of the relationship between experienced neuro-otological symptoms and hearing thresholds in daily life setting. The aim was to investigate the relationship between self-reported hearing symptoms and hearing thresholds in patients with Meniere's disease (MD), using the DizzyQuest and the iPad-based hearing test simultaneously. METHODS: The DizzyQuest was administered for 3 weeks in 21 patients. Using the experience-sampling-method (ESM), it assessed hearing loss and tinnitus severity for both ears separately. Each day after the DizzyQuest, an iPad-based hearing test was used to measure hearing thresholds. A mixed model regression analysis was performed to investigate relationships between hearing thresholds and self-reported hearing loss and tinnitus severity. RESULTS: Fifteen patients were included. Overall, pure-tone averages (PTAs) were not correlated with self-reported hearing loss severity and tinnitus. Individual differences in PTA results between both ears did not significantly influence the difference in self-reported hearing loss severity between both ears. Self-reported hearing loss and tinnitus scores were significantly higher in ears that corresponded with audiometric criteria of MD (p < 0.001). Self-reported tinnitus severity significantly increased with self-reported hearing loss severity in affected (p = 0.011) and unaffected ears (p < 0.001). CONCLUSION: Combining the DizzyQuest and iPad-based hearing test, facilitated assessment of self-reported hearing loss and tinnitus severity and their relationship with hearing thresholds, in a daily life setting. This study illustrated the importance of investigating neuro-otological symptoms at an individual level, using multiple measurements. ESM strategies like the DizzyQuest should therefore be considered in neuro-otological research.


Subject(s)
Hearing Loss , Meniere Disease , Tinnitus , Audiometry, Pure-Tone , Hearing , Hearing Loss/complications , Hearing Loss/diagnosis , Humans , Meniere Disease/complications , Meniere Disease/diagnosis , Self Report
3.
J Neurol ; 267(Suppl 1): 3-14, 2020 Dec.
Article in English | MEDLINE | ID: mdl-32712867

ABSTRACT

BACKGROUND: Most questionnaires currently used for assessing symptomatology of vestibular disorders are retrospective, inducing recall bias and lowering ecological validity. An app-based diary, administered multiple times in daily life, could increase the accuracy and ecological validity of symptom measurement. The objective of this study was to introduce a new experience sampling method (ESM) based vestibular diary app (DizzyQuest), evaluate response rates, and to provide examples of DizzyQuest outcome measures which can be used in future research. METHODS: Sixty-three patients diagnosed with a vestibular disorder were included. The DizzyQuest consisted of four questionnaires. The morning- and evening-questionnaires were administered once each day, the within-day-questionnaire 10 times a day using a semi-random time schedule, and the attack questionnaire could be completed after the occurrence of a vertigo or dizziness attack. Data were collected for 4 weeks. Response rates and loss-to-follow-up were determined. Reported symptoms in the within-day-questionnaire were compared within and between patients and subgroups of patients with different vestibular disorders. RESULTS: Fifty-one patients completed the study period. Average response rates were significantly higher than the desired response rate of > 50% (p < 0.001). The attack-questionnaire was used 159 times. A variety of neuro-otological symptoms and different disease profiles were demonstrated between patients and subgroups of patients with different vestibular disorders. CONCLUSION: The DizzyQuest is able to capture vestibular symptoms within their psychosocial context in daily life, with little recall bias and high ecological validity. The DizzyQuest reached the desired response rates and showed different disease profiles between subgroups of patients with different vestibular disorders. This is the first time ESM was used to assess daily symptoms and quality of life in vestibular disorders, showing that it might be a useful tool in this population.


Subject(s)
Mobile Applications , Vestibular Diseases , Dizziness/diagnosis , Humans , Quality of Life , Retrospective Studies , Surveys and Questionnaires , Vertigo , Vestibular Diseases/diagnosis
4.
J Neurol ; 267(Suppl 1): 15-23, 2020 Dec.
Article in English | MEDLINE | ID: mdl-32654061

ABSTRACT

BACKGROUND: The DizzyQuest, an app-based vestibular diary, provides the opportunity to capture the number and nature of vertigo attacks in daily life. To accomplish this, the DizzyQuest provides different strategies: event sampling using an attack questionnaire, and time sampling using an evening questionnaire. Objective of this study was to investigate whether the number and nature of reported vertigo attacks was comparable between the two questionnaires. METHODS: Fifty-seven patients, who reported vertigo attacks, used the DizzyQuest for on average 24 days. The number and nature (including symptoms, triggers and duration) of vertigo attacks were compared between the attack and the evening questionnaire. RESULTS: The attack questionnaire was used 192 times. In contrast, at least 749 new vertigo attacks were reported in 446 evening questionnaires. A vertigo attack was not always reported in both questionnaires during the same day. Vertigo attacks that were most likely captured by both questionnaires were not always reported the same in both questionnaires regarding triggers and duration. CONCLUSION: Event sampling using an attack questionnaire has low recall bias and, therefore, reliably captures the nature of the attack, but induces a risk of under-sampling. Time sampling using an evening questionnaire suffers from recall bias, but seems more likely to capture less discrete vertigo attacks and it facilitates registration of the absence of vertigo attacks. Depending on the clinical or research question, the right strategy should be applied and participants should be clearly instructed about the definition of a vertigo attack.


Subject(s)
Vertigo , Humans , Surveys and Questionnaires , Vertigo/diagnosis , Vertigo/epidemiology
5.
Eur J Neurol ; 27(10): 2089-2098, 2020 10.
Article in English | MEDLINE | ID: mdl-32400047

ABSTRACT

BACKGROUND AND PURPOSE: Acute vestibular symptoms have a profound impact on patients' well-being. In this study, health-related quality of life (HRQoL) and functional impairment were investigated prospectively in patients with different peripheral and central vestibular disorders during the acute symptomatic stage to decipher the most relevant underlying factors. METHODS: In all, 175 patients with acute vestibular disorders were categorized as central vestibular (CV, n = 40), peripheral vestibular (PV, n = 68) and episodic vestibular disorders (EV, n = 67). All patients completed scores to quantify generic HRQoL (European Quality of Life Score Five Dimensions Five Levels, EQ-5D-5L) and disease-specific HRQoL (Dizziness Handicap Inventory, DHI). Vestibular-ocular motor signs were assessed by video-oculography, vestibular-spinal control by posturography and verticality perception by measurement of subjective visual vertical. RESULTS: Patients with PV had a poorer HRQoL compared to patients with CV and EV (EQ-5D-5L/DHI: PV, 0.53 ± 0.31/56.1 ± 19.7; CV, 0.66 ± 0.28/43.3 ± 24.0; EV, 0.75 ± 0.24/46.7 ± 21.4). After adjusting for age, gender, cardiovascular risk factors and non-vestibular brainstem/cerebellar dysfunction patients with PV persisted to have poorer generic and disease-specific HRQoL (EQ-5D-5L -0.17, DHI +11.2) than patients with CV. Horizontal spontaneous nystagmus was a highly relevant factor for subgroup differences in EQ-5D-5L and DHI, whilst vertical spontaneous nystagmus, subjective visual vertical and sway path were not. EQ-5D-5L decreased significantly with more intense horizontal subjective visual vertical in CV (rho = -0.57) and PV (rho = -0.5) but not EV (rho = -0.13). CONCLUSIONS: Patients with PV have the highest functional impairment of all patients with acute vestibular disorders. Vestibular-ocular motor disturbance in the yaw plane has more impact than vestibular-spinal or vestibular-perceptive asymmetry in the roll and pitch plane, suggesting that horizontal visual stability is the most critical for HRQoL.


Subject(s)
Quality of Life , Vestibular Diseases , Dizziness , Humans , Surveys and Questionnaires , Vertigo
7.
Nervenarzt ; 89(10): 1106-1114, 2018 Oct.
Article in German | MEDLINE | ID: mdl-30167723

ABSTRACT

Vertigo and dizziness are frequent chief complaints in clinical practice. Symptoms may originate from otological, neurological, medical and psychiatric etiologies, which poses an interdisciplinary challenge. Systematic analysis of case history and clinical examination generally allow classification into peripheral-, central- or non-vestibular disorders. The most important criteria for differentiation are the timeline, quality of symptoms, modulating factors and accompanying symptoms. As concerns the clinical examination, the following tests are relevant: head impulse test, test for spontaneous nystagmus, positional nystagmus, central ocular motor signs and the Romberg test. However, neuro-otological disorders with combined peripheral and central vestibular pathology do exist. Occlusion of the anterior inferior cerebellar artery results in ischemia of the labyrinth and cerebellum and therefore causes acute vestibular syndrome and unilateral hearing loss. Repetitive attacks of vertigo or dizziness which are accompanied by ear symptoms and headaches may be due to an overlap syndrome of Menière's disease and vestibular migraine. In this case patients often have to be treated with a dual prophylactic medication to control symptoms. In case of chronic dizziness and instability of gait a subsample of patients may suffer from CANVAS, which is a combination of bilateral vestibulopathy, a cerebellar syndrome and polyneuropathy. Chronic dizziness with signs of peripheral and central vestibular dysfunction can also originate from tumors of the cerebellopontine angle with compression of central structures. In conclusion, the diagnostic algorithm in the workup of patients with vertigo and dizziness should always include tests for peripheral and central vestibular and ocular motor function.


Subject(s)
Brain , Ear , Neurotology , Brain/pathology , Dizziness , Ear/pathology , Humans , Vertigo , Vestibular Function Tests
8.
Laryngorhinootologie ; 97(1): 14-23, 2018 01.
Article in German | MEDLINE | ID: mdl-29301160

ABSTRACT

There are currently different groups of drugs for the pharmacotherapy of vertigo, nystagmus and cerebellar disorders: antiemetics; anti-inflammatories, antimenieres, and antimigraineous medications and antidepressants, anticonvulsants, aminopyridines as well as acetyl-DL-leucine. In acute unilateral vestibulopathy, corticosteroids improve the recovery of peripheral vestibular function, but currently there is not sufficient evidence for a general recommendation. There is insufficient evidence to support the view that 16 mg t. i. d. or 48 mg t. i. d. betahistine has an effect in Menière's disease. Therefore, higher dosages are recommended. In animal studies, it was shown that betahistine increases cochlear blood flow. In vestibular paroxysmia, oxcarbazepine was effective (one randomized controlled trial (RCT)). Aminopyridines are recommended for the treatment of downbeat nystagmus (two RCTs) and episodic ataxia type 2 (EA2, one RCT). There has been no RCT on the efficacy of beta-blockers or topiramate but one RCT on flunarizine in vestibular migraine. Based on clinical experience, a treatment analogous to that for migraine without aura can be recommended. Acetyl-DL-leucine improved cerebellar ataxia (two observational studies); it also accelerated central compensation in an animal model of acute unilateral lesion, but RCTs were negative. There are ongoing RCTs on treatment of vestibular paroxysmia with carbamazepine (VESPA), acute unilateral vestibulopathy with betahistine (BETAVEST), vestibular migraine with metoprolol (PROVEMIG), benign paroxysmal positional vertigo with vitamin D (VitD@BPPV), EA2 with 4-aminopyridine versus acetazolamide (EAT-2-TREAT), and cerebellar ataxias with acetyl-DL-leucine (ALCAT).


Subject(s)
Cerebellar Diseases/drug therapy , Nystagmus, Pathologic/drug therapy , Vestibular Diseases/drug therapy , Animals , Central Nervous System Agents/therapeutic use , Humans , Randomized Controlled Trials as Topic
9.
Nervenarzt ; 88(6): 587-596, 2017 Jun.
Article in German | MEDLINE | ID: mdl-28484820

ABSTRACT

Vertigo and dizziness are among the most common chief complaints in the emergency department. Etiologies can be categorized into three subgroups: neurootological (vestibular), medical (especially cardiovascular, metabolic), and psychiatric disorders. The diagnostic approach in the emergency department is based on a systematic analysis of case history (type, time course of symptoms, modulating factors, associated symptoms), clinical examination of the vestibular, ocular motor, and cerebellar systems (head impulse test, nystagmus, skew deviation, positioning maneuver, test of gait and stance), as well as a basal monitoring (vital signs, 12-lead ECG, blood tests). For differentiation of peripheral and central etiologies in acute vestibular syndrome, the HINTS exam (head impulse test, nystagmus, test of skew) and examination of smooth pursuit and saccades should be applied. Nonselective use of neuroimaging is not indicated due to a low diagnostic yield. Cranial imaging should be done in the following constellations: (1) detection of focal neurological or central ocular motor and vestibular signs on clinical exam, (2) acute abasia with only minor ocular motor signs, (3) presence of various cardiovascular risk factors, (4) headache of unknown quality as an accompanying symptom. Besides the symptomatic therapy of vertigo and dizziness with antiemetics or analgesics, further diagnostic differentiation is urgent to guide proper treatment. Examples are the acute therapy in cerebral ischemia, the execution of positioning maneuvers in benign paroxysmal positional vertigo, the use of corticosteroids in acute unilateral vestibulopathy, as well as the readjustment of metabolic homeostasis in medical disorders.


Subject(s)
Cardiovascular Diseases/diagnosis , Dizziness/diagnosis , Emergency Medical Services/methods , Mental Disorders/diagnosis , Metabolic Diseases/diagnosis , Vertigo/diagnosis , Vestibular Diseases/diagnosis , Cardiovascular Diseases/complications , Cardiovascular Diseases/therapy , Diagnosis, Differential , Dizziness/etiology , Dizziness/therapy , Emergency Service, Hospital/organization & administration , Evidence-Based Medicine , Germany , Humans , Mental Disorders/complications , Mental Disorders/therapy , Metabolic Diseases/complications , Metabolic Diseases/therapy , Treatment Outcome , Vertigo/etiology , Vertigo/therapy , Vestibular Diseases/complications , Vestibular Diseases/therapy
10.
Fortschr Neurol Psychiatr ; 84(8): 469-79, 2016 Aug.
Article in German | MEDLINE | ID: mdl-27570904

ABSTRACT

BACKGROUND: Difficulties of walking and deficits of cognitive functions appear to be associated in the elderly. Thus, clinical assessment in geriatry and neurology should focus on: (1) diagnostic approaches covering both domains of everyday functioning; (2) therapeutic interventions that take into account possible interactions and synergies of both domains. DISCUSSION: In order to assess the capability for motor-cognitive interactions in the elderly it is recommended to investigate walking patterns during dual-tasks (e.g. walking and counting backwards, walking and naming words) and to examine clinical tests of everyday mobility tasks, such as the Timed-up-and-go-Test and spatial navigation tasks. Patients with cognitive disorders often perform inferior with a reduction of walking speed and an increase of stepping variability. Dual-task performance appears to be a reliable parameter for long-term observations of the course of the disease. Moreover, it might improve the quality of the gait examination during diagnostic or therapeutic interventions (e.g. the spinal tap test in patients with NPH). Several studies further highlight gait deficits during dual-task walking as a marker for the everyday functioning and the quality of life in elderly persons and patients with cognitive disorders.Therapeutic approaches in this context comprise complex motor-cognitive interventions, such as Thai Chi and Dalcroze rhythmic exercises. These interventions appear to act synergistically in motor and cognitive domains. First evidence for the efficacy for improving executive functions and reducing the fall risk of patients with cognitive impairments is given, thought randomized, controlled trials are rare.


Subject(s)
Alzheimer Disease/diagnosis , Alzheimer Disease/psychology , Cognitive Dysfunction/diagnosis , Cognitive Dysfunction/psychology , Walking , Aged , Alzheimer Disease/therapy , Attention , Cognitive Dysfunction/therapy , Comorbidity , Gait , Humans , Hydrocephalus, Normal Pressure/diagnosis , Hydrocephalus, Normal Pressure/psychology , Neurologic Examination , Neuropsychological Tests , Risk Factors , Spinal Puncture
11.
J Neurol ; 263(9): 1819-27, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27334906

ABSTRACT

Postural instability is a frequent symptom of patients with idiopathic normal pressure hydrocephalus (iNPH), and might be due to the misperception of body verticality. The objective of this study was to assess the usefulness of the subjective body vertical (SBV) as a potential tool for diagnosing iNPH. Twenty patients with iNPH underwent tests of SBV in the pitch and roll planes before and after cerebrospinal fluid (CSF) drainage. Ten patients with other central gait disorders served as controls and also underwent tests for SBV. Before CSF drainage, patients with iNPH showed an impaired verticality perception in the pitch plane with a significant backward deviation of the SBV as compared to the control group (iNPH: mean ± SD -3.7 ± 3.6°; control group: -0.8 ± 2.2°; t value = -2.30, p t-test = 0.03). After CSF drainage, the SBV of the iNPH patients normalized for the pitch plane (-0.9 ± 1.9°). There was a correlation between the backward deviation of the SBV and the ventricular enlargement of the frontal horns (Evan's index; r = -0.52; p Pearson = 0.02). An even stronger correlation was found with the enlargement of the third ventricle (Thalamus index; r = -0.64; p Pearson = 0.002). The new and clinically relevant finding of this study is that verticality perception of patients with iNPH is primarily impaired the pitch plane, and it improves after CSF drainage. This disturbance in pitch might be due to a bilateral central vestibular dysfunction of the thalamus. Determination of the SBV in pitch promises to increase diagnostic accuracy in the cases of suspected iNPH.


Subject(s)
Hydrocephalus, Normal Pressure/diagnosis , Neurologic Examination/methods , Perception , Postural Balance , Proprioception , Accidental Falls , Aged , Biomechanical Phenomena , Cerebrospinal Fluid Shunts , Drainage , Female , Gait Disorders, Neurologic/diagnosis , Humans , Hydrocephalus, Normal Pressure/diagnostic imaging , Hydrocephalus, Normal Pressure/therapy , Male , Neuropsychological Tests , Severity of Illness Index , Third Ventricle/diagnostic imaging
12.
Nervenarzt ; 86(12): 1573-84; quiz 1585-6, 2015 Dec.
Article in German | MEDLINE | ID: mdl-26643594

ABSTRACT

Depending on the temporal course, three forms of vertigo syndrome can be differentiated: 1) vertigo attacks, e.g. benign paroxysmal positional vertigo (BPPV), Menière's disease and vestibular migraine, 2) acute spontaneous vertigo lasting for days, e.g. acute unilateral vestibulopathy, brainstem or cerebellar infarction and 3) symptoms lasting for months or years, e.g. bilateral vestibulopathy and functional vertigo. The specific therapy of the various syndromes is based on three principles: 1) physical treatment with liberatory maneuvers for BPPV and balance training for vestibular deficits, 2) pharmacotherapy, e.g. for acute unilateral vestibulopathy (corticosteroids) and Menière's disease (transtympanic administration of gentamicin or steroids and high-dose betahistine therapy); placebo-controlled pharmacotherapy studies are currently being carried out for acute unilateral vestibulopathy, vestibular paroxysmia, prophylaxis of BPPV, vestibular migraine, episodic ataxia type 2 and cerebellar ataxia; 3) psychotherapy for functional dizziness.


Subject(s)
Anti-Inflammatory Agents/administration & dosage , Physical Therapy Modalities , Psychotherapy/methods , Vertigo/diagnosis , Vertigo/therapy , Combined Modality Therapy/methods , Evidence-Based Medicine , Humans , Treatment Outcome
13.
Nervenarzt ; 86(10): 1277-90, 2015 Oct.
Article in German | MEDLINE | ID: mdl-26440631

ABSTRACT

The key to diagnosing vertigo and balance disorders is systematic analysis of case history with clinical examination of the vestibular, oculomotor, and cerebral systems in particular. Important criteria for differentiating between the various vertigo syndromes are 1) the time course of symptoms, 2) the type of symptoms, 3) modulating factors, and 4) associated symptoms. For clinical examination of the vestibular system, six important tests are available: assessment of spontaneous nystagmus, head impulse test, dynamic visual acuity, subjective visual verticality, positioning manoeuvre, and the Romberg test/gait analysis with eyes open and closed. On the basis of five clinical signs (vertical divergence, central fixation nystagmus, gaze-evoked nystagmus, saccades, normal head impulse test), the clinical examination is able to differentiate between acute central and peripheral vestibular syndromes with a sensitivity and specificity of over 90%. The most relevant laboratory examinations are caloric irrigation and the video head-impulse test for canal function and the vestibular evoked myogenic potentials for otolith function. Finally, treatment is based upon four therapeutic principles: physiotherapy, pharmacotherapy, psychotherapy, and in rare cases, surgery.


Subject(s)
Medical History Taking/methods , Vertigo/diagnosis , Vertigo/therapy , Vestibular Diseases/diagnosis , Vestibular Diseases/therapy , Vestibular Function Tests/methods , Clinical Decision-Making/methods , Diagnosis, Differential , Diagnostic Techniques, Neurological , Humans , Vestibular Diseases/complications
14.
Fortschr Neurol Psychiatr ; 83(9): 490-8, 2015 Sep.
Article in German | MEDLINE | ID: mdl-26421856

ABSTRACT

There are currently different groups of drugs for the pharmacotherapy of vertigo, nystagmus and cerebellar disorders: antiemetics; anti-inflammatories, antimenieres, and antimigraineous medications and antidepressants, anticonvulsants, aminopyridines as well as acetyl-DL-leucine. In acute unilateral vestibulopathy, corticosteroids improve the recovery of peripheral vestibular function, but currently there is not sufficient evidence for a general recommendation. There is insufficient evidence to support the view that 16 mg t. i. d. or 48 mg t. i. d. betahistine has an effect in Menière's disease. Therefore, higher dosages are recommended. In animal studies, it was shown that betahistine increases cochlear blood flow. In vestibular paroxysmia, oxcarbazepine was effective (one randomized controlled trial (RCT)). Aminopyridines are recommended for the treatment of downbeat nystagmus (two RCTs) and episodic ataxia type 2 (EA2, one RCT). There has been no RCT on the efficacy of beta-blockers or topiramate but one RCT on flunarizine in vestibular migraine. Based on clinical experience, a treatment analogous to that for migraine without aura can be recommended. Acetyl-DL-leucine improved cerebellar ataxia (two observational studies); it also accelerated central compensation in an animal model of acute unilateral lesion, but RCTs were negative. There are ongoing RCTs on treatment of vestibular paroxysmia with carbamazepine (VESPA), acute unilateral vestibulopathy with betahistine (BETAVEST), vestibular migraine with metoprolol (PROVEMIG), benign paroxysmal positional vertigo with vitamin D (VitD@BPPV), EA2 with 4-aminopyridine versus acetazolamide (EAT-2-TREAT), and cerebellar ataxias with acetyl-DL-leucine (ALCAT).


Subject(s)
Cerebellar Diseases/drug therapy , Nystagmus, Pathologic/drug therapy , Vestibular Diseases/drug therapy , Animals , Humans
16.
Parkinsonism Relat Disord ; 21(8): 905-10, 2015 Aug.
Article in English | MEDLINE | ID: mdl-26071126

ABSTRACT

OBJECTIVE: Primary orthostatic tremor (OT) is a rare neurological disease of unknown pathophysiology characterized by a high-frequency tremor mainly of the legs when standing. The aim of this study was to examine its long-term course by subjective estimation and objective recording by serial posturography and to obtain further standardized epidemiological and clinical data on patients with OT. METHODS: A clinical cohort of 37 patients with the diagnosis of primary OT was screened for this longitudinal follow-up study. Eighteen patients consented to participate. During study visit all patients underwent a standardized neurological examination and completed subjective scales and scores. Posturographic recordings at follow-up were compared to prior clinical posturographic measurements in 15 cases. RESULTS: In our cohort the mean duration of symptoms was 14.1 ± 6.8 years. Subjectively, 78% of patients reported progression of the disease. Posturographic data (5.4 ± 4.0 years) revealed a significant increase of the total sway path (standing on firm ground with eyes open) from 2.4 ± 1.3 to 3.4 ± 1.4 m/min (p = 0.022) and of the total root mean square values from 9.8 ± 4.3 to 12.4 ± 4.8 mm (p = 0.028). None of these observations are explained by aging of the patients. Mean frequency of the tremor did not change over time (14.7 ± 1.9 Hz vs. 14.9 ± 2.0 Hz at follow-up). Clinically, most patients had signs of cerebellar dysfunction and a substantial portion also showed proprioceptive deficits in the long-term course. CONCLUSIONS: This long-term follow-up study indicates, that primary OT is a progressive disorder. Furthermore, the clinical observation of cerebellar dysfunction in most OT patients in the long-term course might indicate an important role of the cerebellum in its pathophysiology.


Subject(s)
Cerebellum/physiopathology , Disease Progression , Dizziness/physiopathology , Postural Balance/physiology , Proprioception/physiology , Tremor/physiopathology , Aged , Electromyography , Female , Humans , Longitudinal Studies , Male , Middle Aged
18.
Neurology ; 77(2): 101-9, 2011 Jul 12.
Article in English | MEDLINE | ID: mdl-21613601

ABSTRACT

OBJECTIVE: To determine how postural imbalance and falls are related to regional cerebral glucose metabolism (PET) and functional activation of the cerebral postural network (fMRI) in patients with progressive supranuclear palsy (PSP). METHODS: Sixteen patients with PSP, who had self-monitored their frequency of falls, underwent a standardized clinical assessment, posturographic measurement of balance during modified sensory input, and a resting [¹8F]FDG-PET. In addition, patients performed an fMRI paradigm using mental imagery of standing. Results were compared to healthy controls (n = 16). RESULTS: The frequency of falls/month in patients (range 1-40) correlated with total PSP rating score (r = 0.90). Total sway path in PSP significantly correlated with frequency of falls, especially during modulated sensory input (eyes open: r = 0.62, eyes closed: r = 0.67, eyes open/head extended: r = 0.84, eyes open/foam-padded platform: r = 0.87). Higher sway path values and frequency of falls were associated with decreased regional glucose metabolism (rCGM) in the thalamus (sway path: r = -0.80, falls: r = -0.64) and increased rCGM in the precentral gyrus (sway path: r = 0.79, falls: r = 0.64). Mental imagery of standing during fMRI revealed a reduced activation of the mesencephalic brainstem tegmentum and the thalamus in patients with postural imbalance and falls. CONCLUSIONS: The new and clinically relevant finding of this study is that imbalance and falls in PSP are closely associated with thalamic dysfunction. Deficits in thalamic postural control get most evident when balance is assessed during modified sensory input. The results are consistent with the hypothesis that reduced thalamic activation via the ascending brainstem projections may cause postural imbalance in PSP.


Subject(s)
Accidental Falls , Postural Balance/physiology , Supranuclear Palsy, Progressive/pathology , Supranuclear Palsy, Progressive/physiopathology , Thalamus/physiopathology , Aged , Brain Mapping , Disability Evaluation , Eye , Female , Fluorodeoxyglucose F18 , Humans , Image Processing, Computer-Assisted , Magnetic Resonance Imaging/methods , Male , Middle Aged , Oxygen/blood , Positron-Emission Tomography/methods , Rest , Statistics as Topic , Supranuclear Palsy, Progressive/diagnostic imaging , Thalamus/blood supply , Thalamus/diagnostic imaging
19.
Nervenarzt ; 81(12): 1450-5, 2010 Dec.
Article in German | MEDLINE | ID: mdl-21088822

ABSTRACT

In humans, central control of gait and spatial orientation can be visualized by functional neuroimaging techniques. However, in many cases data acquisition has to be uncoupled from the actual movement in space. Optic and PET methods allow the measurement of brain activity during real overground walking. Virtual reality, mental imagination, and the reduction to basic movement components (foot movement) are used in functional MRI to image different aspects of the locomotor network. Results show that - in humans as in cats - the locomotor signal is transmitted from the frontal cortex via basal ganglia and locomotor regions in the brain stem and cerebellum to the spinal pattern generators. The hippocampal formation (on the right side) and its interaction with frontal and parietal cortex are essential for spatial navigation. Functional imaging of gait in patients is still in its infancy, but already broadens the knowledge on the pathophysiology of gait disturbances in degenerative brain disease.


Subject(s)
Gait Disorders, Neurologic/physiopathology , Gait , Locomotion , Magnetic Resonance Imaging/methods , Neurodegenerative Diseases/physiopathology , Proprioception , Animals , Brain Mapping/methods , Cats , Gait Disorders, Neurologic/etiology , Humans , Neurodegenerative Diseases/complications
20.
Nervenarzt ; 80(8): 875-86, 2009 Aug.
Article in German | MEDLINE | ID: mdl-19662450

ABSTRACT

The German BMBF (German Ministry of Education and Research) has decided to establish an Integrated Center for Research and Treatment of Vertigo, Balance and Ocular Motor Disorders (IFB(LMU)) in Munich in 2010. After funding over a 10-year period, the long-term continuation of the IFB(LMU) by the medical faculty and the hospital is envisioned. Vertigo is one of the most common complaints in medicine. Despite its high prevalence patients with vertigo generally receive either inappropriate or inadequate treatment. This deplorable situation is internationally well known and its causes are multiple: insufficient interdisciplinary cooperation, no standardized diagnostics and therapy, the failure to translate findings of basic science into clinical applications and the scarcity of clinical multicenter studies. The IFB(LMU) will constitute a suitable tool with which these structural, clinical, and scientific deficits can be overcome. It will also make possible the establishment of an international interdisciplinary referral center. Munich has become the site of a unique concentration of leading experts on vertigo, balance and ocular motor disorders, both in the clinical and basic sciences. Academic structures have paved the way for the creation of an interdisciplinary horizontal network that also allows structured, vertical academic career paths via the Bachelor's and Master's degree programs in neuroscience, a Graduate School of Systemic Neurosciences, and the Munich Center for Neuroscience "Brain and Mind". The IFB(LMU) has the following objectives with regard to structure and content: to create an independent patient-oriented clinical research center under the auspices of the Medical Faculty but with autonomic administration and budget; to overcome existing clinical and academic barriers separating the traditional specializations, to establish a standardized interdisciplinary longitudinal and transversal network at one site for the management of patients. This should professionalize both the management and the international recruitment of patients (integrated care, telemedicine); to organize the study infrastructure for prospective multicenter clinical studies as well as to free clinical scientists from administrative tasks; to promote translational research with a focus on the innovative topics of molecular functional and structural imaging, experimental and clinical pharmacotherapy, clinical research of vertigo and balance disorders, mathematical modelling, interaction between biological and technical systems (robotics) and research on functionality and the quality of life; to offer new attractive educational paths and career images for medical doctors, students of the natural sciences and engineers in clinical research in order to overcome traditional hierarchical structures. This should promote the principles of efficiency and self-reliance; to supplement the existing excellence with up to eight groups of young scientists and up to eight professorships (tenure track). This should also be seen as an incentive that will attract the best young scientists; to incorporate IFB(LMU) competence into the existing medical and biological graduate schools. The IFB(LMU) is a unique reference center worldwide.


Subject(s)
Biomedical Research/organization & administration , Delivery of Health Care, Integrated/organization & administration , Government Programs/organization & administration , Ocular Motility Disorders/therapy , Vertigo/therapy , Germany , Humans
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