Autonomic Seizures in an Infant: Unusual Cutaneous and Cardiac Manifestations

Abstract

SUMMARY An eight‐month‐old infant developed autonomic seizures, manifested by skin reaction (harlequin‐like syndrome) and paroxysmal bradycardia. Interictal EEG showed multifocal spikes. 24‐hour EEG/ECG monitoring disclosed episodes of cerebral decremental response associated with cardiac nodal rhythm. Head CT and other laboratory tests were normal. Despite excellent seizure control with carbamazepine, the child has moderate psychomotor retardation. RÉSUMÉ Crises sympathiques chez un nourrisson: manifestations cutanées et cardiaques inhabituelles Un nourrisson de huit mois a présenté des crises sympathiques, traduites par une réaction cutanée (syndrome d'Arlequin) et une bradycardie paroxystique. L'EEG intercritique montra des pointes multifocales. Un enregistrement EEG/ECG sur 24 heures révéla des réponses cérébrales de ralentissement associées à un rythme cardiaque nodal. Le scanner céphalique et les autres examens de laboratoire étaient normaux. En dépit d'un excellent contrôle des crises par carbamazepine, l'enfant a un retard psychomoteur modéré. ZUSAMMENFASSUNG Autonome Anfälle bei einem Säugling: ungewöhnliche Haut‐ und Herzmanifestationen Ein acht Monate alter Säugling entwickelte autonome Anfälle, manifestiert durch Hautreaktionen (Harlequin Syndrom) und paroxysmale Bradicardien. Das interiktal abgeleitete EEG zeigte multifokale Spikes. Die 24 Stunden Überwachung von EEG und EKG zeigte Episoden verminderter cerebraler Aktivität bei gleichzeitigem AV‐Block. Die Computertomogramme des Kopfes und die Laboruntersuchungen waren normal. Trotz sehr guter medikamentöser Einstellung hatte das Kind eine mäz̀ige psychomotorische Retardierung. RESUMEN Crisis autonómicas en un lactante: manifestaciones cardiacas y cutáneas inusuales Un lactante de ocho meses de edad sufrió crisis autonómicas que se manifestaban por reacciones cutáneas (sindrome de tipo Arlequin) y bradicardia paroxística. El EEG intercrítico mostraba puntas multifocales. Un EEG/ECG monitorizado durante 24 horas puso de manifesto episodios de decreción de respuesta cerebral en asociación con un ritmo cardiaco nodal. La TAC cerebral y otras exploraciones fueron normales. A pesar de un excelente control de las convulsiones con carbamacepina el niño tenía un retraso psicomotor moderado.

Child Neurology: An infant with episodic facial flushing

“…1 Other diagnoses should be ruled out including autonomic seizures and autonomic cephalgia. 7,8 Of note, our patient's episodes were too long to be considered Abbreviation: NA = information unavailable or not specified.…”

The Harlequin phenomenon

“…Regardless of age, even in the most extreme episodes, the lips and the tongue are consistently spared. [1][2][3][4][5][6][7]9,[12][13][14][15][16][17][18][19][20]…”

“…[1][2][3][4][5][6][7][8][9] The few reports on the HF outside the neonatal period are characterized by a variable duration that ranged from minutes, when associated with a congenital Horner syndrome to 1 h in the sequence of an anaesthesia; 3 h, in a 2-year-old child after surgery for a cystic hygroma; 4 days, presenting in an almost continuous way, preceding surgery for cyanotic cardiopathy with post-operative resolution; definitive, in a 3-month-old baby submitted to surgery for a retinoblastoma of the sympathetic cervical chain located at the level of the carotid bifurcation, which presented with Horner syndrome and was complicated by iatrogenic sympathetic cut-off; and also definitive, in a 17-year-old girl, in which the HF was a sequel of a mediastinal neurinoma. 6,[12][13][14][15][16][17][18][19][20]22,24,26 Physiopathology and eliciting factors…”

“…The exact etiopathogenic mechanism of the HF is unknown but all evidence suggests the presence of a vasomotor instability caused by sympathetic disautonomy which is a consequence of hypothalamic peripheral vascular tonus control immaturity in the newborn. [1][2][3][4][5][6][12][13][14] In other ages, it may be secondary to a lesion, in the hypothalamus, brainstem, pre-ganglionic, ganglionic or post-ganglionic cervical sympathetic nervous system, or in the second and third spinal cord thoracic segments. The nature of the lesion can be traumatic, tumoural or vascular in its origin 10,[15][16][17][18][19][20][21][22][23][24][25][26]28 (Table 1).…”

“…[1][2][3][4][5][6][7][8][9] Strangely, the existing reports on this phenomenon are remarkably more common beyond the neonatal period, whether in infants, other paediatric ages or even in adults. 6,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] In this review, we will focus on the discussion of various aspects of the Harlequin phenomenon (HF), namely its incidence, aetiology, evolving pathophysiology concepts, clinical aspects and finally the historical origin of the name.…”