title: The spectrum of benign myoclonus of early infancy: Clinical and neurophysiologic features in 102 patients.
abstract: OBJECTIVE
  To redefine benign myoclonus of early infancy (BMEI) through analysis of clinical and neurophysiologic features in 102 patients with the aim to widen the spectrum of the syndrome, including a number of different clinical expressions of transient nonepileptic paroxysmal movements occurring in normal infants.
  
  
  METHODS
  We recruited patients from one center in Argentina and two in Italy, including infants with normal neurologic and psychomotor development presenting with brief paroxysmal abnormal movements. Children with motor phenomena occurring only during sleep were excluded. Patients with abnormal interictal or ictal electroencephalography (EEG) findings were also excluded. The follow-up ranged from 2-40 years.
  
  
  RESULTS
  One hundred and two infants (60 male) met the inclusion criteria. Age at onset ranged from 1-12 months, with a median age of 6.2 months. The following nonepileptic paroxysmal motor phenomena were recognized: (1) myoclonus, (2) spasms and brief tonic contractions, (3) shuddering, (4) atonia or negative myoclonus, (5) more than one type of motor phenomenon. In the majority of cases the episodes occurred only while awake and repeated several times a day. In 45 (44.1%) of the 102 cases contractions appeared in clusters.
  
  
  CONCLUSIONS
  Based on the analysis of clinical and EMG features in this large series of infants, we postulate that the spectrum of the syndrome is wider than initially suspected, and that the different transient motor manifestations and their correlation with different EMG patterns will allow recognition of this definitely benign condition comprising a variety of episodic motor phenomena in normal babies.
authors.affiliation: Department of Neurology, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina. rhcaraballo@arnet.com.ar
authors.forename: Roberto H
authors.initials: RH
authors.lastname: Caraballo
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Capovilla
authors.forename: Federico
authors.initials: F
authors.lastname: Vigevano
authors.forename: Francesca
authors.initials: F
authors.lastname: Beccaria
authors.forename: Nicola
authors.initials: N
authors.lastname: Specchio
authors.forename: Natalio
authors.initials: N
authors.lastname: Fejerman
mesh_terms: D000328:Adult; D017668:Age of Onset; D003937:Diagnosis, Differential; D004569:Electroencephalography / Q000379:methods; D004576:Electromyography / Q000379:methods*; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D018482:Muscle, Skeletal / Q000503:physiopathology*; D009460:Neurologic Examination; D055815:Young Adult