Tremor in motor neuron disease may be central rather than peripheral in origin.

Adult Aged Cerebellar Diseases / complications Electromyography Humans Male Middle Aged Motor Neuron Disease / complications Tremor / etiology
cerebellum eyeblink classic conditioning motor neuron disease power spectrum tremor

Journal

European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311

Informations de publication

Date de publication:
03 2019
Historique:
received: 12 01 2018
accepted: 21 06 2018
pubmed: 29 6 2018
medline: 20 8 2019
entrez: 29 6 2018
Statut: ppublish

Résumé

Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post-mortem observations have revealed considerable variability in the phenotype. Rhythmic involuntary oscillations of the hands during action, resembling tremor, can occur in MND, but their pathophysiology has not yet been investigated. A total of 120 consecutive patients with MND were screened for tremor. Twelve patients with action tremor and no other movement disorders were found. Ten took part in the study. Tremor was recorded bilaterally using surface electromyography (EMG) and triaxial accelerometer, with and without a variable weight load. Power spectra of rectified EMG and accelerometric signal were calculated. To investigate a possible cerebellar involvement, eyeblink classic conditioning was performed in five patients. Action tremor was present in about 10% of our population. All patients showed distal postural tremor of low amplitude and constant frequency, bilateral with a small degree of asymmetry. Two also showed simple kinetic tremor. A peak at the EMG and accelerometric recordings ranging from 4 to 12 Hz was found in all patients. Loading did not change peak frequency in either the electromyographic or accelerometric power spectra. Compared with healthy volunteers, patients had a smaller number of conditioned responses during eyeblink classic conditioning. Our data suggest that patients with MND can present with action tremor of a central origin, possibly due to a cerebellar dysfunction. This evidence supports the novel idea of MND as a multisystem neurodegenerative disease and that action tremor can be part of this condition.

Sections du résumé

BACKGROUND AND PURPOSE
Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post-mortem observations have revealed considerable variability in the phenotype. Rhythmic involuntary oscillations of the hands during action, resembling tremor, can occur in MND, but their pathophysiology has not yet been investigated.
METHODS
A total of 120 consecutive patients with MND were screened for tremor. Twelve patients with action tremor and no other movement disorders were found. Ten took part in the study. Tremor was recorded bilaterally using surface electromyography (EMG) and triaxial accelerometer, with and without a variable weight load. Power spectra of rectified EMG and accelerometric signal were calculated. To investigate a possible cerebellar involvement, eyeblink classic conditioning was performed in five patients.
RESULTS
Action tremor was present in about 10% of our population. All patients showed distal postural tremor of low amplitude and constant frequency, bilateral with a small degree of asymmetry. Two also showed simple kinetic tremor. A peak at the EMG and accelerometric recordings ranging from 4 to 12 Hz was found in all patients. Loading did not change peak frequency in either the electromyographic or accelerometric power spectra. Compared with healthy volunteers, patients had a smaller number of conditioned responses during eyeblink classic conditioning.
CONCLUSIONS
Our data suggest that patients with MND can present with action tremor of a central origin, possibly due to a cerebellar dysfunction. This evidence supports the novel idea of MND as a multisystem neurodegenerative disease and that action tremor can be part of this condition.

Identifiants

DOI: 10.1111/ene.13743 PMID: 29953699
pubmed: 29953699
doi: 10.1111/ene.13743
doi:

Types de publication

Journal Article

Langues

eng

Pagination

394-e31

Informations de copyright

© 2018 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.

Auteurs

A Latorre (A)

Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, UK.
Department of Neurology and Psychiatry, Sapienza, University of Rome, Rome, Italy.
ORCID: 0000-0001-7103-5209

L Rocchi (L)

Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, UK.

M Stamelou (M)

Department of Neurology, Philipps University, Marburg, Germany.
Parkinson's Disease and Movement Disorders Department, HYGEIA Hospital, Athens, Greece.

A Batla (A)

Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, UK.

M Ciocca (M)

Department of Neurology, Fatebenefratelli Hospital, ASST Fatebenefratelli Sacco, Milan, Italy.

B Balint (B)

Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, UK.
Department of Neurology, University Hospital Heidelberg, Heidelberg, Germany.

K Sidle (K)

Department of Clinical Neuroscience, University College London Institute of Neurology, London, UK.

A Berardelli (A)

Department of Neurology and Psychiatry, Sapienza, University of Rome, Rome, Italy.
Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Neuromed, Pozzilli, Italy.
ORCID: 0000-0003-3598-3142

J C Rothwell (JC)

Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, UK.

K P Bhatia (KP)

Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, UK.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Tremor in motor neuron disease may be central...
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Sujet âgé › Tremor in motor neuron disease may be central...
questionsmedicales.fr › Maladies du système nerveux › Maladies du système nerveux central › Encéphalopathies › Maladies du cervelet › Tremor in motor neuron disease may be central...
questionsmedicales.fr › Diagnostic › Techniques et procédures diagnostiques › Myographie › Électromyographie › Tremor in motor neuron disease may be central...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Tremor in motor neuron disease may be central...
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Adulte d'âge moyen › Tremor in motor neuron disease may be central...
questionsmedicales.fr › Maladies du système nerveux › Maladies neuromusculaires › Maladies du motoneurone › Tremor in motor neuron disease may be central...
questionsmedicales.fr › États, signes et symptômes pathologiques › Signes et symptômes › Manifestations neurologiques › Dyskinésies › Tremblement › Tremor in motor neuron disease may be central...