A pilot tolerability and efficacy study of levetiracetam in patients with chronic myoclonus

Frucht, Steven J.; Louis, Elan D.; Chuang, Cathy; Fahn, Stanley

doi:10.1212/wnl.57.6.1112

Cited by 107 publications

(55 citation statements)

References 8 publications

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“…Myoclonus in DLB and PD consists of a sudden brief jerk that is accompanied by a sharp transient in the contralateral sensorimotor cortex on EEG 39, 43. This type of myoclonus, called cortical myoclonus, may be caused by aberrant cortical excitability39, 43, 44 and has been successfully treated with anti‐epileptic drugs 52, 53, 54. In our study, myoclonus was associated with an earlier age of onset of cognitive impairment, raising the possibility that aberrant network excitability may contribute to cognitive dysfunction in DLB.…”

Section: Discussionmentioning

confidence: 99%

Network dysfunction in α‐synuclein transgenic mice and human Lewy body dementia

Morris

Sanchez

Verret

et al. 2015

Ann Clin Transl Neurol

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ObjectiveDementia with Lewy bodies (DLB) is associated with the accumulation of wild‐type human α‐synuclein (SYN) in neurons and with prominent slowing of brain oscillations on electroencephalography (EEG). However, it remains uncertain whether the EEG abnormalities are actually caused by SYN.MethodsTo determine whether SYN can cause neural network abnormalities, we performed EEG recordings and analyzed the expression of neuronal activity‐dependent gene products in SYN transgenic mice. We also carried out comparative analyses in humans with DLB.ResultsWe demonstrate that neuronal expression of SYN in transgenic mice causes a left shift in spectral power that closely resembles the EEG slowing observed in DLB patients. Surprisingly, SYN mice also had seizures and showed molecular hippocampal alterations indicative of aberrant network excitability, including calbindin depletion in the dentate gyrus. In postmortem brain tissues from DLB patients, we found reduced levels of calbindin mRNA in the dentate gyrus. Furthermore, nearly one quarter of DLB patients showed myoclonus, a clinical sign of aberrant network excitability that was associated with an earlier age of onset of cognitive impairments. In SYN mice, partial suppression of epileptiform activity did not alter their shift in spectral power. Furthermore, epileptiform activity in human amyloid precursor protein transgenic mice was not associated with a left shift in spectral power.InterpretationWe conclude that neuronal accumulation of SYN slows brain oscillations and, in parallel, causes aberrant network excitability that can escalate into seizure activity. The potential role of aberrant network excitability in DLB merits further investigation.

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Section: Discussionmentioning

confidence: 99%

Network dysfunction in α‐synuclein transgenic mice and human Lewy body dementia

Morris

Sanchez

Verret

et al. 2015

Ann Clin Transl Neurol

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“…Frucht and Fahn (2000) reviewed more than 100 cases of LAS and found that clonazepam, sodium valproate, and piracetam were significantly effective in approximately 50% of patients. Recently, several groups have confirmed the efficacy of levetiracetam in PHM (Frucht et al, 2001;Krauss et al, 2001;Lim and Ahmed, 2005). Clonazepam, sodium valproate, piracetam, and levetiracetam may be recommended as first-line agents to treat patients with LAS.…”

Section: Discussionmentioning

confidence: 99%

Lance-adams syndrome: a report of two cases

Yan-Xing

Liu

Jiang

et al. 2007

J. Zhejiang Univ. - Sci. B

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Chronic post-hypoxic myoclonus, also known as Lance-Adams syndrome (LAS), is a rare complication of successful cardiopulmanry resuscitation often accompanied by action myoclonus and cerebellar ataxia. It is seen in patients who have undergone a cardiorespiratory arrest, regained consciousness afterwards, and then developed myoclonus days or weeks after the event. Worldwide, 122 cases have been reported in the literature so far, including 1 case of Chinese. Here we report 2 Chinese LAS patients with detailed neuroimagings. Cranial single photon emission computed tomography (SPECT) of patient 1, a 52-year-old woman, showed a mild hypoperfusion in her left temporal lobe, whereas patient 2, a 54-year-old woman, manifested a mild bilateral decrease of glucose metabolism in the frontal lobes and a mild to moderate decrease of the N-acetyl aspartate (NAA) peak in the bilateral hippocampi by cranial [18 F]-fluorodeoxyglucose positron emission tomographic (PET) scan and cranial magnetic resonance spectroscopy (MRS), respectively. We also review the literature on the neuroimaging, pathogenesis, and treatment of LAS.

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“…18 Case reports and small open-label trials indicate that LEV reduces cortical reflex myoclonus due to various causes and is generally well tolerated. 37 Another case report found that piracetam reduced choreoathetosis. 38 Loscher and Richter studied the effects of LEV and piracetam in a mutant hamster model of paroxysmal dystonia, a form of idiopathic generalized dystonia, and found that both drugs significantly decreased the severity of dystonia.…”

Section: Discussionmentioning

confidence: 99%