Publications by authors named "Helge Topka"

Introduction: In 2022 the DGN (Deutsche Gesellschaft für Neurologie) published an updated Transient Global Amnesia (TGA) guideline. TGA is characterized by a sudden onset of retrograde and anterograde amnesia for a period of one to a maximum of 24 h (with an average of 6 to 8 h). The incidence is estimated between 3 and 8 per 100,000 population/year.

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Patients with amyotrophic lateral sclerosis (ALS) can lose all muscle-based routes of communication as motor neuron degeneration progresses, and ultimately, they may be left without any means of communication. While others have evaluated communication in people with remaining muscle control, to the best of our knowledge, it is not known whether neural-based communication remains possible in a completely locked-in state. Here, we implanted two 64 microelectrode arrays in the supplementary and primary motor cortex of a patient in a completely locked-in state with ALS.

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Objectives: To evaluate daily life management and functional outcome of Idarucizumab administration in case of emergency situations in patients with Dabigatran treatment.

Design: Multicenter observational registry study.

Setting: All hospitals with full neurological departments (n = 6) in Munich, Germany INCLUDED PATIENTS: All patients treated with Idarucizumab from 01/2016 to 03/2019.

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Third nerve palsy is bilateral in only about 10% of cases, of which one in five cases is due to brainstem stroke. Bilateral oculomotor nerve palsy as an isolated clinical finding after brainstem stroke is extremely rare. We present a case of severe bilateral fascicular oculomotor nerve palsy due to distal basilar occlusion and subsequent midbrain infarction of cardioembolic origin.

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[Meningism].

Fortschr Neurol Psychiatr

May 2018

A thorough neurological examination in emergency situations requires the evaluation of meningeal signs. Even though in most settings, evaluation of meningism is technically not very demanding, the interpretation of findings may prove difficult. As opposed to a widely held belief, clinical signs of meningism are neither specific nor highly sensitive for detection of meningitis or subarachnoid hemorrhage.

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Few movement disorders seem to make a straightforward approach to diagnosis and treatment more difficult and frustrating than myoclonus, due to its plethora of causes and its variable classifications. Nevertheless, in recent years, exciting advances have been made in the elucidation of the pathophysiology and genetic basis of many disorders presenting with myoclonus. Here, we provide a review of all of the important types of myoclonus encountered in pediatric and adult neurology, with an emphasis on the recent developments that have led to a deeper understanding of this intriguing phenomenon.

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Considering the causative or contributory effects of diabetes mellitus on common neurological diseases such as polyneuropathy, stroke and dementia, modern antidiabetic drugs may be expected to reduce incidence or progression of these conditions. Nevertheless, most observed benefits have been small, except in the context of therapy for diabetes mellitus type I and new-onset polyneuropathy. Recently, semaglutide, a GLP-1 analog, has been shown to significantly reduce stroke incidence in a randomized controlled trial.

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Acute vertigo may originate from peripheral or central vestibular disorders. As central vestibular symptoms may indicate severe brainstem or cerebellar ischemia, rapid clinical differentiation is required. To this end, evaluation of spontaneous or gaze-evoked nystagm, head-impulse test as well identification of skew deviation are most helpful.

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Rarely, not stroke but peripheral weakness can result from cervical artery dissection. In these cases, a mural hematoma compressing the ipsilateral C5 and/or C6 root can be demonstrated.

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Primary dystonias are a clinically and genetically heterogeneous group of movement disorders, but only for two of them, i.e., dystonia 1 and dystonia 6, the disease causing gene has been identified.

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Superficial siderosis of the central nervous system is a very rare disease related to hemosiderin deposits in the brain, brainstem, cerebellum and spinal cord due to chronic subarachnoid hemorrhage. Chronic increased intracranial pressure develops in about one-third of affected cases. We report a patient with superficial siderosis and sudden intracranial pressure crisis.

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Predictive control of grasping forces when manipulating objects in the environment is suggested to reflect internal models that capture the causal relationship between actions and their consequences. The anatomical correlate of predictive control of grasping within the central nervous system is not completely understood. One structure which has been related to the neural representation of internal models is the cerebellum.

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Apart from the classic triad of hypokinetic gait disorder, cognitive dysfunction and urinary incontinence, the clinical spectrum of normal pressure hydrocephalus has been found to affect the upper limbs. It is unclear if the motor deficit of hand and arm movements improves with CSF evacuation. The present study was designed to quantitatively assess the effect of CSF evacuation on the hypokinesia of grasping movements in normal pressure hydrocephalus.

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Acute myelopathy refers to acute or subacute spinal cord dysfunction secondary to various causes. Recent studies suggest a number of distinct clinical, laboratory, MRI and outcome profiles for the various aetiologies. Nevertheless, the aetiology of acute myelopathy remains unknown in up to 60% of the patients.

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The clinical spectrum of normal pressure hydrocephalus is thought to comprise the triad of hypokinetic gait disorder, dementia and urinary incontinence. In contrast, motor abnormalities involving the upper limbs in normal pressure hydrocephalus have not yet received a great deal of attention. The present study was designed to quantitatively assess grasping movements in normal pressure hydrocephalus and to compare the performance with that in Parkinson's disease.

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Five parkinsonian subjects with chronic bilateral stimulation of the subthalamic nucleus and five sex- and age-matched healthy controls grasped, lifted, and held an instrumented object. The grip-lift task was either performed at self-determined speed or in response to an auditory cuing signal. Parkinsonian subjects performed the task with subthalamic nucleus stimulation switched ON and OFF.

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The dorso-lateral medullary syndrome (Wallenberg's syndrome) is produced by infarction of a wedge of lateral medulla posterior to the inferior olivary nucleus and is usually caused by vertebral artery occlusion. Ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion is rather rare and the anatomical structure responsible is still uncertain. Here we describe two patients presenting with ipsilateral axial lateropulsion as an initial symptom of vertebral artery occlusion.

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Objective: Earlier investigations have suggested that isolated conduction block of the facial nerve to transcranial magnetic stimulation early in the disorder represents a very sensitive and potentially specific finding in Bell's palsy differentiating the disease from other etiologies.

Methods: Stimulation of the facial nerve was performed electrically at the stylomastoid foramen and magnetically at the labyrinthine segment of the Fallopian channel within 3 days from symptom onset in 65 patients with Bell's palsy, five patients with Zoster oticus, one patient with neuroborreliosis and one patient with nuclear facial nerve palsy due to multiple sclerosis.

Results: Absence or decreased amplitudes of muscle responses to early transcranial magnetic stimulation was not specific for Bell's palsy, but also evident in all cases of Zoster oticus and in the case of neuroborreliosis.

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We investigated the differential effects of levodopa medication and STN stimulation on finger force control in Parkinson subjects grasping to lift an object and performing vertical point-to-point movements of a hand-held object. The experiments were conducted in four treatment conditions: off-drug/off-stimulation, off-drug/on-stimulation, on-drug/off-stimulation and on-drug/on-stimulation. We found that the bradykinesia in Parkinsonian subjects improved by both levodopa medication and STN stimulation.

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We investigated how patients with cerebellar degeneration control fingertip forces to resist a perturbation imposed on a handheld load. Patients and healthy sex- and age-matched control subjects held an instrumented receptacle between the index finger and thumb. A weight was dropped into the receptacle either unexpectedly from the experimenter's hand with the subject being blindfolded or expectedly from the subject's opposite hand.

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Spinocerebellar ataxia type 17 (SCA17) is a dominant progressive neurodegenerative disorder, caused by a triplet repeat expansion within the TATA-binding protein. As well as ataxia and dementia, Parkinsonism and dystonia are common in SCA17. In some pedigrees focal dystonia in the absence of ataxia has been described as a main clinical feature.

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When we repetitively lift an object, the balance between grip force normal to the object's surface and load force tangential to the object's surface is accurately programmed to match the physical object properties within a few lifts. Here, we ask if the accuracy of grip force scaling to object weight and the transfer of weight-related information from one hand to the other is impaired in cerebellar degeneration. Subjects with generalized cerebellar degenerative disorders were tested.

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Primary dystonias represent a clinically and genetically heterogeneous group of movement disorders. Mutations in the epsilon-sarcoglycan (SGCE) gene have been found recently to cause myoclonus-dystonia (MD). Considerable clinical variation of SGCE mutation carriers leads to the hypothesis that mutations in the SGCE gene might also be relevant for other subtypes of dystonias.

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We analyzed predictive and reactive grip force behavior in 15 patients with Gilles de la Tourette syndrome (GTS) and 15 sex- and age-matched healthy control subjects. Nine patients were without medication; six patients were on medication. In a first experiment, participants lifted and held instrumented objects of different weight.

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