title: Tremor in Charcot-Marie-Tooth disease: No evidence of cerebellar dysfunction.
abstract: OBJECTIVE
  Tremor in Charcot-Marie-Tooth disease (CMT) can be disabling. Cerebellar abnormalities are thought to underpin neuropathic tremor. Here, we aim to clarify the potential role of the cerebellum in CMT tremor.
  
  
  METHODS
  We assessed prevalence of tremor by questionnaire in 84 patients with CMT. Of those, 23 patients with CMT with and without arm tremor and healthy controls underwent a clinical assessment, classical eyeblink conditioning, electro-oculography, visuomotor adaptation test, tremor recording with surface EMG and accelerometry, and retrospective correlation with nerve conduction studies to investigate the possible mechanisms of tremor generation.
  
  
  RESULTS
  The prevalence study revealed tremor in 21% of patients and in 42% of those it caused impairment of function. Tremor recordings revealed a mild-to-moderate amplitude tremor with a weight load-invariant 7.7 Hz frequency component. Performance on classical eyeblink conditioning, visuomotor adaptation and electro-oculography were no different between tremulous and non-tremulous patients and healthy controls.
  
  
  CONCLUSIONS
  These results argue against a prominent role for an abnormal cerebellum in tremor generation in the patients studied with CMT. Rather, our results suggest an enhancement of the central neurogenic component of physiological tremor as a possible mechanism for tremor in the patients studied.
  
  
  CONCLUSIONS
  This study is the first to propose differing pathogenic mechanisms for subtypes of neuropathic tremor.
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK. Electronic address: t.saifee@ucl.ac.uk.
authors: Tabish A
authors: TA
authors: Saifee
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: Isabel
authors: I
authors: Pareés
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: Panagiotis
authors: P
authors: Kassavetis
authors: Academic Department of Neuro-otology, Faculty of Medicine, Imperial College London, London, UK.
authors: Diego
authors: D
authors: Kaski
authors: Academic Department of Neuro-otology, Faculty of Medicine, Imperial College London, London, UK.
authors: Adolfo M
authors: AM
authors: Bronstein
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: John C
authors: JC
authors: Rothwell
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: Anna
authors: A
authors: Sadnicka
authors: MRC Centre for Neuromuscular Diseases, Department of Molecular Neurosciences, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK.
authors: Michael P
authors: MP
authors: Lunn
authors: MRC Centre for Neuromuscular Diseases, Department of Molecular Neurosciences, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK.
authors: Hadi
authors: H
authors: Manji
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: James T
authors: JT
authors: Teo
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: Kailash P
authors: KP
authors: Bhatia
authors: MRC Centre for Neuromuscular Diseases, Department of Molecular Neurosciences, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK.
authors: Mary M
authors: MM
authors: Reilly
authors: Sobell Department for Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, 33 Queen Square, London WC1N 3BG, UK.
authors: Mark J
authors: MJ
authors: Edwards
mesh_terms: D000328:Adult; D000368:Aged; D001767:Blinking / Q000502:physiology; D002526:Cerebellar Diseases / Q000175:diagnosis* / Q000503:physiopathology*; D002607:Charcot-Marie-Tooth Disease / Q000175:diagnosis* / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods; D005133:Eye Movements / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D014202:Tremor / Q000175:diagnosis* / Q000503:physiopathology*
keywords: CMT; Hereditary; Motor; Neuropathy; Pathophysiology