Disinhibition of somatosensory and motor cortex in mitochondriopathy without myoclonus

Objective: To test electrophysiologically, if patients with mitochondriopathy but without evidence of myocloni have subclinical signs of disinhibition in motor and somatosensory cortices. Methods: Two patients were studied and compared with age-matched control groups. Results: In both patients, gian...

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Bibliographic Details
Published in:Clinical neurophysiology 2001-05, Vol.112 (5), p.917-922
Main Authors: Liepert, J, Haueisen, J, Hegemann, S, Weiller, C
Format: Article
Language:English
Subjects:
600 Hz
Adult
Biological and medical sciences
Disinhibition
Disorders of higher nervous function. Focal brain diseases. Central vestibular syndrome and deafness. Brain stem syndromes
Electric Stimulation
Evoked Potentials, Somatosensory - physiology
Female
Giant somatosensory evoked potentials
Humans
Intracortical facilitation
Intracortical inhibition
Magnetics
Magnetoencephalography
Median Nerve - physiopathology
Medical sciences
Middle Aged
Mitochondrial Myopathies - genetics
Mitochondrial Myopathies - physiopathology
Mitochondriopathy
Motor Cortex - physiology
Motor Cortex - physiopathology
Motor Neurons - physiology
Myoclonus
Nervous system (semeiology, syndromes)
Neurology
Reference Values
Sensory Thresholds
Somatosensory Cortex - physiology
Somatosensory Cortex - physiopathology
Transcranial magnetic stimulation
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Description
Summary:Objective: To test electrophysiologically, if patients with mitochondriopathy but without evidence of myocloni have subclinical signs of disinhibition in motor and somatosensory cortices. Methods: Two patients were studied and compared with age-matched control groups. Results: In both patients, giant somatosensory evoked potentials after median nerve stimulation and a reduced intracortical inhibition tested by transcranial magnetic stimulation in a paired pulse paradigm indicated a dysfunction of inhibitory circuits in the motor as well as the somatosensory cortex. In addition, the somatosensory evoked 600 Hz activity recorded by magnetoencephalography was abolished. Conclusions: Patients with mitochondriopathy may suffer from a subclinical disturbance of inhibition in the sensorimotor cortex. The loss of 600 Hz activity indicates that these high-frequency oscillations could reflect the activity of inhibitory neurons in the somatosensory cortex.
ISSN:1388-2457
1872-8952
DOI:10.1016/S1388-2457(01)00521-1