Benign Sleep Myoclonus Research Articles

A 10-day-old baby with "lightning-like" myoclonic limb jerks during sleep: a case report

Background: Benign neonatal sleep myoclonus (BNSM) is a condition commonly mistaken for seizures. It is characterized by self-limiting "lightning-like" myoclonic limb jerks that occur primarily during sleep in the newborn phase but are not associated with epilepsy that suddenly stops when the infant is agitated. This condition is potentially resolved between the ages of 2 and 6 months. The incidence of BNSM per 1,000 births is 0.8 to 3 cases. This case report aims to explain the findings of a neonate with multiple episodes of abnormal jerks affecting the entire limb, only during sleep.Case Report: We reported a 40-day-old baby boy with multiple episodes of abnormal jerks affecting the entire limb since 10-day-old only during sleep. The jerky body movements appeared six until nine times a day with approximately an hour interval between movements. The patient did not have any neurologic deficits, pathologic reflexes and vital signs abnormalities. Electroencephalogram (EEG) revealed the normal result. We diagnosed the patient with BNSM.Conclusion: The BNSM is a self-limiting disorder. For neonatal seizures or even neonatal status epilepticus, BNSM can be misinterpreted, recognizing childhood benign sleep myoclonus is important to prevent unnecessary diagnostic studies and treatments. The prognosis was good and the patient didn't need any medication and long-term follow-up.

Non-epileptic paroxysmal events in paediatric patients: A single tertiary centre study in Egypt

PurposeThe misdiagnosis of non-epileptic seizures (NES) as epilepsy is one of the most common pitfalls in neuropsychiatric practice. This study aimed to describe the percentage and types of NES among children who were referred for a diagnosis of epilepsy in Upper Egypt. MethodsWe recruited a total of 876 patients who were referred to Sohag University Hospital, a tertiary referral centre in Upper Egypt, for the evaluation of suspected epilepsy. Relevant methods for the diagnosis of epilepsy, including medical history and examination, EEG, video-EEG, laboratory investigations, and brain imaging, were performed for all study participants. ResultsAmong the 876 patients who were referred for the diagnosis of suspected epilepsy during the period from June 2017 to October 2018, 171 patients (19.5 %) were diagnosed as having NES. In general, we found that NES in the paediatric age groups did not differ from that reported in various studies across several different populations. The most prevalent NES in our study was breath-holding spells (32.2 %), followed by syncope (17.5 %), psychogenic nonepileptic seizures (12.3 %), motor tics (9.9 %), and benign sleep myoclonus (7.6 %). Other less frequent NES included infantile masturbation (7 %), spasmus nutans (5.3 %), migraine (2.9 %), benign paroxysmal torticollis (2.9 %), night terrors (1.8 %), and shuddering attacks (0.6 %). ConclusionIdeally, neurologists should not misdiagnose NES as epilepsy, and whenever the diagnosis of NES is uncertain, an accurate diagnosis should be made using long-term video-EEG monitoring, especially in younger paediatric patients.

Benign neonatal sleep myoclonus in a child

Benign neonatal sleep myoclonus (BNSM), is a disorder generally mistaken for seizures during the newborn period. Benign neonatal sleep myoclonus is featured by myoclonic "lightninglike" jerks of the extremities that exclusively occur during sleep; it is not associated with epilepsy that occur only during sleep and cease abruptly when the child is agitated. This case reported was a 50 days-old baby boy with a history suggestive of abnormal movements for limbs noted over the preceding 7 days. Diagnosis of BNSM in infant based on history taking, clinical manifestation, and with no electroencephalographic changes. BNSM is usually not associated with any other neurologic impairment and spontaneously subsides within the first year of life. Its importance lies in the differential diagnosis with the epileptic, especially myoclonic, seizures of infancy. BNSM can be misinterpreting for neonatal seizures or even neonatal status epilepticus, the recognition of benign sleep myoclonus of infancy is imperative to elude unnecessary diagnostic studies and treatments.

Paroxysmal non-epileptic events in infants and toddlers: A phenomenologic analysis.

The aim of this study was to analyze in detail the clinical phenomenology of paroxysmal non-epileptic events (PNEE) in infants and toddlers. We studied all children aged ≤2 years who were diagnosed with PNEE based on video-electroencephalographic (VEEG) recordings. We analyzed the following four clinical domains of each clinical event: (i) motor manifestations (body/limb jerking, complex motor, and asymmetric limb posturing); (ii) oral/vocal (crying, vocalization, sighing); (iii) behavioral change (arrest of activity, staring); (iv) and autonomic (facial flushing, breath holding). Thirty-one of 81 (38.3%) infants and toddlers had 38 PNEE recorded during the study period (12 girls and 19 boys, mean age 10.5 months). The predominant clinical features were as follows: motor in 26/38 events, oral/verbal in 14/38 events, behavioral in 11/38 events, and autonomic in 8/38 events. Epileptic seizures and PNEE coexisted in four children (12.9%). Seventeen children (54.8%) had one or more risk factors suggestive of epilepsy. Twelve children (38.7%) had a normal neurologic examination, 10 (32.3%) had developmental delay, and eight (25.8%) had a family history of epilepsy or seizures. VEEG recorded PNEE in nearly 40% of 81 infants and toddlers referred for unclear paroxysmal events in our cohort. Non-epileptic staring spells and benign sleep myoclonus were the most common events recorded, followed by shuddering attacks and infantile masturbation. In addition, greater than one-half of the infants and toddlers had risk factors, raising a concern for epilepsy in the family and prompting the VEEG evaluation, suggesting that paroxysmal non-epileptic seizures may frequently coexist in young children with epilepsy.

Benign convulsions in newborns and infants: Occurrence, clinical course and prognosis

Background During early development severe epilepsies may appear, some with well established occurrence. Benign non-epileptic and epileptic paroxysmal syndromes with excellent prognosis occur in the same period. There are no exact data on their occurrence. Aim We have reviewed medical histories of children with benign non-epileptic or benign epileptic events: benign myoclonus of early infancy, benign neonatal sleep myoclonus, benign sleep myoclonus in infancy, benign partial epilepsy in infancy (BPEI) and benign infantile familial convulsions (BIFC) were established. The occurrence, clinical characteristics and prognosis of these syndromes were evaluated. Methods Inclusion criteria were met in 31 children. Research included retrospective analysis of clinical characteristics, laboratory values, neuroimaging and neurophysiological assessments, followed by evaluation of psychosocial development with the use of the Strengths and Difficulties Questionnaire (SDQ), fulfilled by parents. Results In our group the incidence of benign non-epileptic convulsions was 6.69 per 10 000 live births and the incidence of benign epileptic convulsions was 1.35 per 10 000. Male/female ratio in the group of children with non-epileptic events was 2.1:1. Among non-epileptic group 5 out of 23 children and among epileptic group 3 out of 8 children had minimal, mild or moderate abnormalities at neurological assessment at the time of the first clinical examination. Nonspecific changes in laboratory values were seen in 6 out of 23 in the non-epileptic and in 1 out of 8 children in the epileptic group. Neurophysiological assessments showed subtle changes in 4/23 in the non-epileptic and 6/8 in the epileptic group. Neuroimaging was not optimal in 5/23 with non-epileptic and 3/8 with epileptic events. Analysis of SDQ did not show significant deviations in psyhosocial development. Statistically significant deviation was observed only in relations with peers ( p = 0.009). Conclusions Benign neonatal and infantile convulsions are more frequent than severe epilepsies of the same age period. Results show higher proportion of males with benign non-epileptic conditions. No deviations in further development was found. Laboratory values, neuroimaging and neurophysiological assessments were normal or nonspecifically changed.

Clinical Identification of the Simple Sleep-Related Movement Disorders

Simple sleep-related movement disorders must be distinguished from daytime movement disorders that persist during sleep, sleep-related epilepsy, and parasomnias, which are generally characterized by activity that appears to be simultaneously complex, goal-directed, and purposeful but is outside the conscious awareness of the patient and, therefore, inappropriate. Once it is determined that the patient has a simple sleep-related movement disorder, the part of the body affected by the movement and the age of the patient give clues as to which sleep-related movement disorder is present. In some cases, all-night polysomnography with accompanying video may be necessary to make the diagnosis. Hypnic jerks (ie, sleep starts), bruxism, rhythmic movement disorder (ie, head banging/body rocking), and nocturnal leg cramps are discussed in addition to less well-appreciated disorders such as benign sleep myoclonus of infancy, excessive fragmentary myoclonus, and hypnagogic foot tremor/alternating leg muscle activation.

Seizure-like States

Seizure-like States

Paroxysmal non-epileptic events in children: a retrospective study over a period of 10 years.

To determine the frequency, nature and clinical characteristics of paroxysmal non-epileptic events in children diagnosed by video electroencephalogram (EEG) monitoring at a tertiary referral centre. A retrospective study of children with paroxysmal non-epileptic events, aged 2 weeks to 17 years inclusive was undertaken. The study group consisted of children who had video EEG monitoring during a 10-year period (1988-99). Telemetry files, medical charts, events recorded on video and record sheets were reviewed. A total of 666 children were analysed, 269 had epileptic events recorded, 285 had non-epileptic events and 112 had no events recorded. In children with non-epileptic events, 43% were developmentally delayed, 25% had an abnormal neurological examination and 40% had epilepsy. In the study sample an epileptiform interictal EEG was common (24%). The major subgroups of non-epileptic events were: staring (34%), sleep phenomena - benign sleep myoclonus (15%), arousals (13%), motor tics (11%) and shuddering (7%). Developmental delay (57%) was common in children who presented with staring spells. A diagnosis of a specific non-epileptic event was reached in 96% of cases. Paroxysmal non-epileptic events can cause diagnostic confusion, particularly in children with developmental delay, epilepsy or an epileptiform EEG. Accurate diagnosis can be reached in the majority of cases using video EEG monitoring.

Myoclonus and epilepsies.

The possible associations of myoclonic phenomenae, progressive or non-progressive encephalopathies and epileptic features are reviewed, with special emphasis on pediatric age. This leads to recognize the following five groups of conditions: (1) Myoclonus without encephalopathy and without epilepsy; (2) Encephalopathies with non-epileptic myoclonus; (3) Progressive encephalopathies presenting myoclonus seizures of epileptic syndromes (Progressive myoclonus epilepsies); (4) Epileptic encephalopathies with myoclonic seizures; (5) Myoclonic epilepsies. Within the first group, which also includes physiologic myoclonus, a more thorough description of "Benign sleep myoclonus of newborn" and "Benign myoclonus of early infancy" is given. Characteristics of group 2 are "Kinsbourne Syndrome" and certain types of "Hyperekplexia" which pose interesting differential diagnosis with stimulus-sensitive epilepsies. In group 3, the concept of progressive encephalopathies is stressed. The fourth group refers to severe epilepsies, mainly on infancy and childhood, which lead to mental retardation irrespective of their aetiology. Group 5 comprises the true myoclonic epilepsies, differentiating syndromes recognized as idiopathic--such as "Benign myoclonic epilepsy of infancy" and "Juvenile myoclonic epilepsy"--from those which are cryptogenic and carry a more cautious prognosis--as "Cryptogenic myoclonic and myoclonoastatic epilepsies" and "Severe myoclonic epilepsy of infancy". Other epileptic syndromes not usually considered as myoclonic epilepsies, but presenting sometimes as myoclonic seizures, are finally referred.