Antimyoclonic Effect of Levetiracetam in 13 Patients with Unverricht–Lundborg Disease: Clinical Observations

Magaudda, Adriana; Gélisse, Philippe; Genton, Pierre

doi:10.1111/j.0013-9580.2004.56902.x

Cited by 98 publications

(52 citation statements)

References 12 publications

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“…The diagnosis of EPM1 was hypothesised on the basis of its typical electroclinical presentation and confirmed by the finding of a dodecamer expansion of the cstb gene (Virtaneva et al 1997). The enrolled patients presented with mildly to moderately severe myoclonus (a score of two or three on a simplified rating scale, Magaudda et al 2004) and were receiving similar anti-epileptic treatment: valproate (10), levetiracetam (3), topiramate (2), benzodiazepine (1), bromazepam (1), clonazepam (1), lamotrigine (1), phenobarbital (1), and piracetam (1).…”

Section: Subjectsmentioning

confidence: 96%

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

et al. 2014

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Multimodal human brain mapping has been proposed as an integrated approach capable of improving the recognition of the cortical correlates of specific neurological functions. We used simultaneous EEG-fMRI (functional magnetic resonance imaging) and EEG-TDfNIRS (time domain functional near-infrared spectroscopy) recordings to compare different hemodynamic methods with changes in EEG in ten patients with progressive myoclonic epilepsy and 12 healthy controls. We evaluated O 2 Hb, HHb and Blood oxygen level-dependent (BOLD) changes and event-related desynchronization/synchronization (ERD/ERS) in the a and b bands of all of the subjects while they performed a simple motor task. The general linear model was used to obtain comparable fMRI and TDfNIRS activation maps. We also analyzed cortical thickness in order to evaluate any structural changes. In the patients, the TD-NIRS and fMRI data significantly correlated and showed a significant lessening of the increase in O 2 Hb and the decrease in BOLD. The post-movement b rebound was minimal or absent in patients. Cortical thickness was moderately reduced in the motor area of the patients and correlated with the reduction in the hemodynamic signals. The fMRI and TD-NIRS results were consistent, significantly correlated and showed smaller hemodynamic changes in the patients. This finding may be partially attributable to mild cortical thickening. However, cortical hyperexcitability, which is known to generate myoclonic jerks and probably accounts for the lack of EEG b-ERS, did not reflect any increased energy requirement. We hypothesize that this is due to a loss of inhibitory neuronal components that typically fire at high frequencies.

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Section: Subjectsmentioning

confidence: 96%

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

et al. 2014

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“…The diagnosis of ULD was hypothesized on the basis of the typical electroclinical presentation and confirmed by the finding of dodecamer expansion at the cstb gene (Virtaneva et al 1997). Myoclonus severity was scored using a simplified rating scale ranging from 0 (no myoclonus) to 5 (severe myoclonus) (Magaudda et al 2004). A neuroradiologist visually assessed all structural MRI images in order to exclude macroscopic anatomical abnormalities.…”

Section: Participantsmentioning

confidence: 99%

Abnormal ERD/ERS but Unaffected BOLD Response in Patients with Unverricht–Lundborg Disease During Index Extension: A Simultaneous EEG-fMRI Study

et al. 2010

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Electrophysiological studies indicate that Unverricht-Lundborg's disease (ULD), the most common form of progressive myoclonus epilepsy in Europe, is characterized by the involvement of multiple cortical regions in degenerative changes that lead to enhanced excitation and deficient inhibition. We searched for the haemodynamic correlates of these effects using functional MRI (fMRI) of self-paced index extensions, a well-accepted task highlighting significant differences. EEG and fMRI were simultaneously acquired in 11 ULD patients and 16 controls, performing the index extensions individually (event-related task) as well as repetitively (block task). ERD/ERS analysis was performed for the EEG data in the alpha and beta bands. fMRI time-series were analyzed using the traditional general linear model, as well as with an assumption-free approach, and by means of cross-region correlations representing functional connectivity. In line with the existing literature, ULD patients had enhanced desynchronization in the alpha band and reduced post-movement synchronization in the beta band. By contrast, fMRI did not reveal any difference between the two groups; there were no activation intensity, latency or extent effects, no significant engagement of additional regions, and no changes to functional connectivity. We conclude that, so long as the patients are executing a task which does not induce obvious action myoclonus, the hypothesized abnormalities in pyramidal neuron and interneuron dynamics are relatively subtle, embodied in processes which are not metabolically-demanding and take place at a time-scale invisible to fMRI.

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“…Routine hematologic/biochemical investigations and neuropsychological testing were performed in all cases. Severity of myoclonus was scored according to a simplified myoclonus rating scale, as described (5). Electrophysiologic study included EEG, somatosensory evoked potentials (SEPs), and Long-Loop Reflex I (LLRI).…”

mentioning

confidence: 99%

Late‐onset and Slow‐progressing Lafora Disease in Four Siblings with EPM2B Mutation

et al. 2005

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Summary:We report a family with four brothers affected by Lafora disease (LD). Mean age at onset was 19.5 years (range, 17-21). In all cases, the initial obvious symptoms were diffuse myoclonus and occasional generalized tonic-clonic seizures (GTCSs), followed by cognitive difficulties. Severity of myoclonus, seizure diaries, and neurologic and neuropsychological status were finally evaluated in March 2005. The duration of follow-up was >10 years for three subjects. Daily living activities and social interaction were preserved in all cases and, overall, the progression of the disease was slow. Genetic study revealed the homozygous mutation D146N in the EPM2B gene. We suggest that this mutation may be associated with a less severe LD phenotype.

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Antimyoclonic Effect of Levetiracetam in 13 Patients with Unverricht–Lundborg Disease: Clinical Observations

Cited by 98 publications

References 12 publications

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

Hemodynamic and EEG Time-Courses During Unilateral Hand Movement in Patients with Cortical Myoclonus. An EEG-fMRI and EEG-TD-fNIRS Study

Abnormal ERD/ERS but Unaffected BOLD Response in Patients with Unverricht–Lundborg Disease During Index Extension: A Simultaneous EEG-fMRI Study

Late‐onset and Slow‐progressing Lafora Disease in Four Siblings with EPM2B Mutation

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