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Alterations in Muscle Tone ll01:12

Alterations in Muscle Tone ll

Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
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Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
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MRI-guided Focused Ultrasound Thalamotomy for Patients with Medically-refractory Essential Tremor
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Neuropathological changes in essential tremor: 33 cases compared with 21 controls.

Elan D Louis1, Phyllis L Faust, Jean-Paul G Vonsattel

  • 1GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, USA. EDL2@columbia.edu

Brain : a Journal of Neurology
|November 21, 2007
PubMed
Summary

Neuropathological studies of essential tremor (ET) reveal heterogeneous brain changes. Most ET cases show cerebellar abnormalities, while a minority exhibit Lewy bodies in the brainstem.

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Area of Science:

  • Neuropathology
  • Neurodegenerative Disorders
  • Essential Tremor Research

Background:

  • Essential tremor (ET) is a common neurological disorder with limited neuropathological investigation.
  • Autopsy studies and case-control comparisons for ET are notably scarce.

Purpose of the Study:

  • To describe and quantify pathological changes in 33 ET and 21 control brains.
  • To correlate clinical and pathological features in essential tremor.

Main Methods:

  • Examination of autopsy tissue from the Essential Tremor Centralized Brain Repository.
  • Quantification of pathological markers including Lewy bodies, Purkinje cell counts, and Purkinje cell torpedoes.

Main Results:

  • 24.2% of ET brains had brainstem Lewy bodies; 75.8% showed cerebellar pathology without Lewy bodies.
  • ET cases without Lewy bodies exhibited reduced Purkinje cells and increased Purkinje cell torpedoes compared to controls.
  • Degeneration of the dentate nucleus, Purkinje cell heterotopias, and dendrite swellings were observed in some ET cases.

Conclusions:

  • Pathological changes in essential tremor are heterogeneous and degenerative.
  • The majority of ET cases present with cerebellar changes, while a smaller subset has brainstem Lewy bodies.
  • Further research is needed to understand clinical differences between ET cases with and without Lewy bodies.