Pulse-synchronous rotational and vertical pendular eye movements in superior canal dehiscence syndrome.
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Biomedical subjects
Publications and source records attributed to M Strupp.
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We examined 103 patients with vestibular neuritis (VN) in a follow-up study (5.7 to 20.5 years, mean 9.8 years). Two patients (1.9%) had developed a second occurrence of VN 29 to 39 months after the first. VN affected the contralateral ear in both and caused less severe distressing vertigo and postural imbalance. Unlike Bell's palsy and sudden hearing loss, a relapse in the same ear did not occur.
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The authors evaluated floccular activity with fMRI during the performance of vertical smooth pursuit eye movements in four patients with downbeat nystagmus (DBN) due to cerebellar degeneration and in 16 healthy controls. Region of interest analysis revealed a significantly diminished activation of both floccular lobes during downward but not upward pursuit in DBN. These imaging data support the view that a functional deficiency of the flocculi in downward pursuit causes DBN.
The case of a patient with complete sensory and motor paraplegia for more than 1 year is presented. Leg movements were documented during sleep by video and electromyographic recording because a psychogenic cause of the symptoms was suspected. We showed the video to the patient, which illustrated that leg movements were possible. This resulted in fast and complete resolution of the neurologic symptoms. The patient has now been free of them for more than 3 years. This example suggests that the achievement of consciousness of normal motor function is a therapeutic approach for long-standing improvement of psychogenic paralysis.
The increased postural sway of patients with disorders of the vestibular system improves with vision. The suppression of pathologic nystagmus also reduces sway. Because the latter effect cannot be attributed to retinal slip as a relevant feedback for postural control, the authors investigated how eye movements rather than retinal slip affect balance. They found that slow eye movements increase sway, possibly by an efference copy, which explains why spontaneous nystagmus causes postural imbalance.
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One hundred and six patients diagnosed between 1987 and 1998 to have somatoform phobic postural vertigo were examined in a follow-up study with a self-evaluating questionnaire. The improvement rate after a mean follow-up time of 8.5 years (5 to 15.9 years) was 75% (27% of the patients reported a complete remission). While the majority of these patients experienced improvement or remission during the first year after assessment of diagnosis and a short-term psychotherapeutic approach, some patients also had considerable improvement even after two or more years. There was a negative correlation between the duration of the condition before assessment of the diagnosis and the improvement/regression rate. The improvement/regression rate was independent of gender, age, preceding vestibular or non-vestibular organic disorders, and the various medical, physical, or psychotherapeutic interventions. Transient relapses occurred in 47% of the improved patients once or repeatedly. The probability of developing a relapse remained constant throughout the entire follow-up. None of the patients required a revision of the initial diagnosis on the basis of the questionnaire.
Mitoxantrone (mitox) has been shown to be effective for secondary progressive (SP) and relapsing-remitting multiple sclerosis (MS). The aim of this open trial was to evaluate the effects of combined mitox and methylprednisolone (MP) therapy on patients with primary progressive (PP)-MS or with SP-MS. We present here the results of an interim analysis done after the study had lasted 5 years. Sixty-five patients (20 with PP-MS and 45 with SP-MS) have been included so far. The treatment involved ten cycles of combined mitox and MP. The intervals between the individual cycles were systematically prolonged from 3 months initially to 12 months, so the complete treatment took a total of 57 months. Conclusion This interim analysis indicates that mitox combined with MP beneficially reduces the progression of disability in patients with PP-MS and SP-MS. Therefore, this therapy regimen can also be considered a feasible option for PP-MS.
The effect of the potassium channel blocker 4-aminopyridine (4-AP) on spontaneous upbeat nystagmus (UBN) was investigated with the search coil technique during fixation in different gaze positions and smooth pursuit in a patient before and after ingestion of 10 mg 4-AP. UBN was reduced from 8.6 deg/s to 2.0 deg/s by 4-AP causing subjective relief from distressing oscillopsia, and impaired upward smooth pursuit was restored (gains: before medication 0.38; after medication 0.86). In the dark, UBN was slightly stronger and not affected by 4-AP. We propose that 4-AP improved the function of cerebellar pathways that mediate gaze holding and smooth pursuit by intensifying the excitability of cerebellar Purkinje cells.
Vertigo follows headache as the second most common key symptom--not only in neurology and ENT medicine. Careful history-taking and a physical examination are sufficient for a correct diagnostic classification of most vertigo syndromes. In most cases, the etiology is benign, the course favorable, and treatment successful. In this brief overview, the clinical presentation and treatment of the most common peripheral and central vertigo syndromes are described, and the characteristics and treatment of the--to date too rarely diagnosed--subjective vertigo are described.
Patients with episodic ataxia type 2 (EA2) can often be successfully treated with acetazolamide. The authors report three patients with EA2 (two with proven mutations in the CACNA1A gene) whose attacks were prevented with the potassium channel blocker 4-aminopyridine (4-AP; 5 mg tid). Attacks recurred after treatment was stopped; subsequent treatment alleviated the symptoms (mean follow-up time 6 months). These effects might be due to an improvement of the impaired functioning of Purkinje cells.
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A patient sought treatment for vertical oscillopsia and impaired vision during locomotion, and unsteadiness of gait. Positive fistula tests and CT of the temporal bones confirmed a diagnosis of bilateral superior canal dehiscence. An impairment of the superior canal vestibulo-ocular reflex, documented by three-dimensional search coil eye movement recordings for oblique (single) and downward pitch head motion (bilateral canal testing), is proposed to induce vertical rather than torsional-vertical oscillopsia during locomotion.
The new curriculum for medical licensure in Germany focuses on interdisciplinary and problem-based learning. In recent years, first experiences with this learning model were gathered in several German medical schools conducting courses supplementing the traditional curriculum. This article describes the course "Nervous system and behavior" at Ludwig Maximilian University in Munich. This course was established in cooperation with Harvard University in Boston, USA (The Munich-Harvard Alliance) together with three other clinical courses and has run every semester since the winter of 1999. As this course integrates neuroscience disciplines with special emphasis on neurology and psychiatry, it may serve as a role model for the implementation of these subjects in a new curriculum. This article introduces the reader to its structure and elements as well as feedback from students.
BACKGROUND: Several drugs that primarily act on gamma-aminobutyrate or muscarinic receptors have been used to treat downbeat nystagmus (DBN) syndrome despite their having only moderate success and causing several side effects that limit their effectiveness. These drugs were tested under the assumption that DBN was caused by a disinhibition of a physiologic inhibitory cerebellar input on vestibular nuclei. OBJECTIVE: To evaluate the effects of a single dose of the potassium channel blocker 3,4-diaminopyridine (3,4-DAP), which is known to increase the excitability of Purkinje cells, on DBN in a prospective, placebo-controlled, double-blind study with a crossover design. METHODS: Seventeen patients with DBN due to cerebellar atrophy (5), infarction (3), Arnold-Chiari malformation (1), or unknown etiology (8) were included in the study (1 of 18 patients had to be excluded). Mean peak slow-phase velocity (PSPV) was measured before and 30 minutes after randomized ingestion of 20 mg of 3,4-DAP or placebo orally; at least 1 week later, the treatments were switched. RESULTS: 3,4-DAP reduced mean PSPV of DBN from 7.2 +/- 4.2 degrees /s (mean +/- SD) before treatment to 3.1 +/- 2.5 degrees/s 30 minutes after ingestion of the 3,4-DAP (p < 0.001, two-way analysis of variance). Placebo had no measurable effect. In 10 of 17 subjects, the mean PSPV decreased by >50% and in 12 of 17 by >40%. In parallel, the subjects had less oscillopsia and felt more stable while standing and walking. Nine of the subjects continued to take the drug with success. Except for transient minor perioral or digital paresthesia reported by three subjects and nausea and headache reported by one, no other side effects were observed. CONCLUSIONS: In this study, the authors demonstrated that a single dose of 3,4-DAP significantly improved DBN. In view of animal studies reporting that micromolar concentrations of 4-aminopyridine increased the excitability of Purkinje cells, it is suggested that the efficacy of 3,4-DAP may be due to an increase of the physiologic inhibitory influence of the vestibulocerebellum on the vestibular nuclei.