High-Frequency Rhythmic Cortical Myoclonus in a Long-Surviving Patient With Nonketotic Hypergylcemia

Mastrangelo, Massimo; Canafoglia, Laura; Franceschetti, Silvana; Oppezzo, C; Mosca, Fabio; Menni, Francesca; Parini, Rossella; Ciano, Claudia; Scaioli, V.; Panzica, Ferruccio

doi:10.1177/0883073807308699

Cited by 3 publications

(3 citation statements)

References 8 publications

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“…Glycine is a co-agonist of NMDAR, together with glutamate (23). Its excess is thought to account for the onset of seizures in neonates with NKH, but also for their long-term neurological sequelae (20,47,48).…”

Section: Discussionmentioning

confidence: 99%

Efficacy and safety of ketamine for neonatal refractory status epilepticus: case report and systematic review

Leonardi

Nosadini

et al. 2023

Front. Pediatr.

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BackgroundEvidence-based data on treatment of neonatal status epilepticus (SE) are scarce. We aimed to collect data on the efficacy and safety of ketamine for the treatment of neonatal SE and to assess its possible role in the treatment of neonatal SE.MethodsWe described a novel case and conducted a systematic literature review on neonatal SE treated with ketamine. The search was carried out in Pubmed, Cochrane, Clinical Trial Gov, Scopus and Web of Science.ResultsSeven published cases of neonatal SE treated with ketamine were identified and analyzed together with our novel case. Seizures typically presented during the first 24 h of life (6/8). Seizures were resistant to a mean of five antiseizure medications. Ketamine, a NMDA receptor antagonist, appeared to be safe and effective in all neonates treated. Neurologic sequelae including hypotonia and spasticity were reported for 4/5 of the surviving children (5/8). 3/5 of them were seizure free at 1–17 months of life.DiscussionNeonatal brain is more susceptible to seizures due to a shift towards increased excitation because of a paradoxical excitatory effect of GABA, a greater density of NMDA receptors and higher extracellular concentrations of glutamate. Status epilepticus and neonatal encephalopathy could further enhance these mechanisms, providing a rationale for the use of ketamine in this setting.ConclusionsKetamine in the treatment of neonatal SE showed a promising efficacy and safety profile. However, further in-depth studies and clinical trials on larger populations are needed.

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Section: Discussionmentioning

confidence: 99%

Efficacy and safety of ketamine for neonatal refractory status epilepticus: case report and systematic review

Leonardi

Nosadini

et al. 2023

Front. Pediatr.

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“…The polygraphic EEG recording demonstrates the presence of myoclonias that are brief, single or repetitive, and can be very frequent or nearly continuous. Massive, usually bisynchronous, axial myoclonic jerks may start from the onset of the disease or occur later, often interspersed with erratic myoclonias [75]. Simple focal seizures are usually subtle with eye devi- Fig.…”

Section: Neonatal Epilepsy Syndromesmentioning

confidence: 99%

“…Signs of peripheral neuropathy may also occur in rare cases. Although the etiology is mostly unknown, nonketotic hyperglycinemia, pyridoxine or pyridoxal-phosphate deficiency or dependent, and congenital deficiency of the mitochondrial glutamate transporter are known to produce a sim-ilar clinical picture [75,76]. There is a high risk of familial recurrence since in most cases the disease appears to be inherited as an autosomal recessive trait.…”

Section: Neonatal Epilepsy Syndromesmentioning

confidence: 99%

EEG and the newborn

Cilio

2015

J Pediatr Neurol

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Despite the evolution of new technologies for assessing neonatal brain function, electroencephalography (EEG) remains a powerful tool for neurological diagnosis and prognosis in neonates. It is considered the gold standard for distinguishing epileptic seizures from non-epileptic paroxysmal events and for detecting subclinical seizure activity in high-risk babies. In those babies severely ill, EEG is even more efficient as predictive test than the neurological examination. The prognostic value of neonatal EEG has been long recognized in term as well as in preterm infants. Background patterns, more that patterns of ictal discharges, correlate significantly with the long-term outcome. Although most abnormalities on the neonatal EEG are nonspecific, certain patterns can be highly suggestive for diagnosis. Prognostic value can be increased by obtaining early recordings, possibly within the first 48 hr of life, prolonged recordings to include samples of different activity states, and serial EEGs at short intervals to assess rapid changes that are likely to occur in high-risk infants. It is important to distinguish neonatal seizures from neonatal-onset epilepsies and epilepsy syndromes. Both benign and malignant neonatal epilepsy syndromes exist. While benign familial neonatal seizures represents a neonatal syndrome with benign outcome, early myoclonic encephalopathy and Ohtahara syndrome are the earliest form of age-dependent severe epilepsy syndromes. Early myoclonic encephalopathy and Ohtahara syndrome are both characterized by the presence of a burst-suppression EEG pattern. This pattern is usually associated with a poor prognosis and is considered the electroencephalographic correlate of a complete disconnection within the thalamocortical systems. Migrating partial seizures in infancy is a newly recognized epilepsy syndrome whose onset can be in the neonatal period. It is characterized by multifocal intractable seizures associated with psychomotor impairment. No etiology has been found so far. Different drugs currently used in neonatal intensive care unit, especially if in the toxic range, can alter the EEG background. Those effects need to be taken in account in the interpretation of neonatal EEG.

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The network sustaining action myoclonus: a MEG-EMG study in patients with EPM1

Franceschetti¹,

Canafoglia²,

Rotondi

et al. 2016

BMC Neurol

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BackgroundTo explore the cortical network sustaining action myoclonus and to found markers of the resulting functional impairment, we evaluated the distribution of the cortico-muscular coherence (CMC) and the frequency of coherent cortical oscillations with magnetoencephalography (MEG). All patients had EPM1 (Unverricht-Lundborg) disease known to present with prominent and disabling movement-activated myoclonus.MethodsUsing autoregressive models, we evaluated CMC on MEG sensors grouped in regions of interests (ROIs) above the main cortical areas. The movement was a repeated sustained isometric extension of the right hand and right foot. We compared the data obtained in 10 EPM1 patients with those obtained in 10 age-matched controls.ResultsAs expected, CMC in beta band was significantly higher in EPM1 patients compared to controls in the ROIs exploring the sensorimotor cortex, but, it was also significantly higher in adjacent ROIs ipsilateral and contralateral to the activated limb. Moreover, the beta-CMC peak occurred at frequencies significantly slower and more stable frequencies in EPM1 patients with respect to controls. The frequency of the beta-CMC peak inversely correlated with the severity of myoclonus.Conclusionsthe high and spatially extended beta-CMC peaking in a restricted range of low-beta frequencies in EPM1 patients, suggest that action myoclonus may result not only from an enhanced local synchronization but also from a specific oscillatory activity involving an expanded neuronal pool. The significant relationship between beta-CMC peak frequency and the severity of the motor impairment can represent a useful neurophysiological marker for the patients’ evaluation and follow-up.

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High-Frequency Rhythmic Cortical Myoclonus in a Long-Surviving Patient With Nonketotic Hypergylcemia

Cited by 3 publications

References 8 publications

Efficacy and safety of ketamine for neonatal refractory status epilepticus: case report and systematic review

Efficacy and safety of ketamine for neonatal refractory status epilepticus: case report and systematic review

EEG and the newborn

The network sustaining action myoclonus: a MEG-EMG study in patients with EPM1

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