Intention Tremor | ScienceGate

Fragile-X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder that manifests with intention tremor, progressive gait ataxia, and cognitive impairment. The disease is genetically characterized by a premutation of the <i>FMR1</i>gene on the X-chromosome manifesting with a CGG triplet expansion between 55 and 200. Given the phenotypical variety of this disease, diagnosis is frequently delayed. We present and discuss a male patient whose diagnosis of FXTAS was delayed due to his concomitant alcohol abuse.

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Haploinsufficiency of POU4F1 causes an ataxia syndrome with hypotonia and intention tremor

Human Mutation ◽

10.1002/humu.24201 ◽

2021 ◽

Author(s):

Bryn D. Webb ◽

Anthony Evans ◽

Thomas P. Naidich ◽

Lynne Bird ◽

Sumit Parikh ◽

...

Keyword(s):

Intention Tremor ◽

Ataxia Syndrome

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Possible necessity for deep brain stimulation of both the ventralis intermedius and subthalamic nuclei to resolve Holmes tremor

Journal of Neurosurgery ◽

10.3171/jns.2003.99.3.0566 ◽

2003 ◽

Vol 99 (3) ◽

pp. 566-571 ◽

Cited By ~ 49

Author(s):

Pantaleo Romanelli ◽

Helen Bronté-Stewart ◽

Tracy Courtney ◽

Gary Heit

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Clinical Observation ◽

Intention Tremor ◽

Content Type ◽

Resting Tremor ◽

Holmes Tremor ◽

Tremor Analysis ◽

Deep Brain ◽

Stimulation Of

✓ Holmes tremor is characterized by resting, postural, and intention tremor. Deep brain stimulation (DBS) of both the nucleus ventralis intermedius (Vim) and the subthalamic nucleus (STN) may be required to control these three tremor components. A 79-year-old man presented with a long-standing combination of resting, postural, and intention tremor, which was associated with severe disability and was resistant to medical treatment. Neuroimaging studies failed to reveal areas of discrete brain damage. A DBS device was placed in the Vim and produced an improvement in both the intention and postural tremor, but there was residual resting tremor, as demonstrated by clinical observation and quantitative tremor analysis. Placement of an additional DBS device in the STN resolved the resting tremor. Stimulation of the Vim or STN alone failed to produce global resolution of mixed tremor, whereas combined Vim—STN stimulation produced global relief without creating noticeable side effects. Combined Vim—STN stimulation can thus be a safe and effective treatment for Holmes tremor.

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Hilbert–Huang transform based instrumental assessment of intention tremor in multiple sclerosis

Journal of Neural Engineering ◽

10.1088/1741-2560/12/4/046011 ◽

2015 ◽

Vol 12 (4) ◽

pp. 046011 ◽

Cited By ~ 5

Author(s):

Ilaria Carpinella ◽

Davide Cattaneo ◽

Maurizio Ferrarin

Keyword(s):

Multiple Sclerosis ◽

Intention Tremor ◽

Hilbert Huang Transform ◽

Instrumental Assessment

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Ventral Intermediate Thalamic Stimulation for Monoclonal Gammopathy-Associated Tremor: Case Report

Neurosurgery ◽

10.1227/neu.0b013e3182124633 ◽

2011 ◽

Vol 68 (5) ◽

pp. E1464-E1467 ◽

Cited By ~ 2

Author(s):

Donald C. Shields ◽

Alice W. Flaherty ◽

Emad N. Eskandar ◽

Ziv M. Williams

Keyword(s):

Daily Living ◽

Monoclonal Gammopathy ◽

Clinical Presentation ◽

Sensory Loss ◽

Intention Tremor ◽

Deep Brain Stimulator ◽

Thalamic Stimulation ◽

Central Nervous System Dysfunction ◽

Stimulation Parameters ◽

Deep Brain

Abstract BACKGROUND AND IMPORTANCE: Peripheral and central sensory loss are often associated with significant tremor or sensory ataxia, which can be highly refractory to medical therapy. CLINICAL PRESENTATION: We present the case of a 67-year-old man with progressive and debilitating intention tremor from monoclonal gammopathy-associated peripheral neuropathy. The patient was implanted with bilateral thalamic deep brain stimulator electrodes under microelectrode guidance. Following optimization of stimulation parameters, the patient's appendicular tremor and gait improved, as did his general activities of daily living. CONCLUSION: These initial findings suggest that deep brain stimulation may benefit not only tremor presumed to originate from central nervous system dysfunction, but also tremor originating peripherally from neuropathy-related sensory loss.

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Familial benign chorea with intention tremor: A clinical entity

The Journal of Pediatrics ◽

10.1016/s0022-3476(67)80322-6 ◽

1967 ◽

Vol 70 (5) ◽

pp. 724-729 ◽

Cited By ~ 43

Author(s):

Jonathan H. Pincus ◽

Abe Chutorian

Keyword(s):

Clinical Entity ◽

Intention Tremor

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Single-Neuron Analysis of Human Thalamus in Patients With Intention Tremor and Other Clinical Signs of Cerebellar Disease

Journal of Neurophysiology ◽

10.1152/jn.00049.2001 ◽

2002 ◽

Vol 87 (4) ◽

pp. 2084-2094 ◽

Cited By ~ 30

Author(s):

F. A. Lenz ◽

C. J. Jaeger ◽

M. S. Seike ◽

Y. C. Lin ◽

S. G. Reich

Keyword(s):

Neuronal Activity ◽

Cross Correlation ◽

Clinical Signs ◽

Emg Activity ◽

Phase Lag ◽

Cerebellar Disease ◽

Intention Tremor ◽

Cross Correlation Analysis ◽

Relay Nucleus ◽

Cerebellar Tremor

Tremor that occurs as a result of a cerebellar lesion, cerebellar tremor, is characteristically an intention tremor. Thalamic activity may be related to cerebellar tremor because transmission of some cerebellar efferent signals occurs via the thalamus and cortex to the periphery. We have now studied thalamic neuronal activity in a cerebellar relay nucleus (ventral intermediate—Vim) and a pallidal relay nucleus (ventralis oral posterior—Vop) during thalamotomy in patients with intention tremor and other clinical signs of cerebellar disease (tremor patients). The activity of single neurons and the simultaneous electromyographic (EMG) activity of the contralateral upper extremity in tremor patients performing a pointing task were analyzed by spectral cross-correlation analysis. EMG spectra during intention tremor often showed peaks of activity in the tremor-frequency range (1.9–5.8 Hz). There were significant differences in thalamic neuronal activity between tremor patients and controls. Neurons in Vim and Vop had significantly lower firing rates in tremor patients than in patients undergoing thalamic surgery for pain (pain controls). Other studies have shown that inputs to Vim from the cerebellum are transmitted through excitatory connections. Therefore the present results suggest that tremor in these tremor patients is associated with deafferentation of the thalamus from cerebellar efferent pathways. The thalamic X EMG cross-correlation functions were studied for cells located in Vim and Vop. Neuronal and EMG activity were as likely to be significantly correlated for cells in Vim as for those in Vop. Cells in Vim were more likely to have a phase lag relative to EMG than were cells in Vop. In monkeys, cells in the cerebellar relay nucleus of the thalamus, corresponding to Vim, are reported to lead movement during active oscillations at the wrist. In view of these monkey studies, the present results suggest that cells in Vim are deafferented and have a phase lag relative to tremor that is not found in normal active oscillations. The difference in phase of thalamic spike X EMG activity between Vim and Vop may contribute to tremor because lesions of pallidum or Vop are reported to relieve cerebellar tremor.

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