Recurrent Upper Motor Neuron Facial Weakness of Possible Epileptic Etiology: A Case Report

Abstract

We report on an otherwise healthy infant who presented with clusters of alternating central facial nerve paresis. At 11 months of age, the patient showed intermittent facial asymmetry compatible with right-sided upper motor neuron facial paresis and accompanying incomplete upper motor neuron hypoglossal paresis. Laboratory work-up and imaging studies did not reveal signs of infection, infarction or structural lesions, and after one week, symptoms spontaneously resolved. Similar episodes affecting alternate sides were noted at ages of 17, 27 and 49 months lasting for 4–14 days. At 49 months, EEG showed right temporo-occipital benign sharp waves with activation during drowsiness and sleep. A diagnosis of benign focal epilepsy with negative motor phenomena was made. She is now 60 months old and no further episodes have occurred without antiepileptic treatment. Ictal orofacial phenomena are the clinical hallmark of benign focal epilepsy with centro-temporal sharp waves (BECTS). As in our patient, negative epileptic motor phenomena frequently lead to a broad diagnostic work-up. In infants presenting with episodic central facial nerve paresis, the possibility of negative epileptic motor phenomena should be considered.

Key words

facial paresis - epilepsy - benign epilepsy with centrotemporal sharp waves - infants - EEG

References

• 1 Abe T, Kobayashi M, Araki K. et al . Infantile convulsions with mild gastroenteritis.  Brain Dev. 2000;  22 301-306
• 2 Capovilla G, Vigevano F. Benign idiopathic partial epilepsies in infancy.  J Child Neurol. 2001;  16 874-881
• 3 de Saint-Martin A, Petiau C, Massa R. et al . Idiopathic rolandic epilepsy with “interictal” facial myoclonia and oromotor deficit: a longitudinal EEG and PET study.  Epilepsia. 1999;  40 614-620
• 4 Deonna TW, Roulet E, Fontan D. et al . Speech and oromotor deficits of epileptic origin in benign partial epilepsy of childhood with rolandic spikes (BPERS). Relationship to the acquired aphasia-epilepsy syndrome.  Neuropediatrics. 1993;  24 83-87
• 5 Gobbi G, Boni A, Filippini M. The spectrum of idiopathic Rolandic epilepsy syndromes and idiopathic occipital epilepsies: from the benign to the disabling.  Epilepsia. 2006;  47 (S 02) 62-66
• 6 Muellbacher W, Boroojerdi B, Ziemann U. et al . Analogous corticocortical inhibition and facilitation in ipsilateral and contralateral human motor cortex representations of the tongue.  J Clin Neurophysiol. 2001;  18 550-558
• 7 Narchi H. Benign afebrile cluster convulsions with gastroenteritis: an observational study.  BMC Pediatr. 2004;  4 2
• 8 Panayiotopoulos CP. Benign childhood partial epilepsies: benign childhood seizure susceptibility syndromes.  J Neurol Neurosurg Psychiatry. 1993;  56 2-5
• 9 Panayiotopoulos CP, Michael M, Sanders S. et al . Benign childhood focal epilepsies: assessment of established and newly recognized syndromes.  Brain. 2008;  131 2264-2286
• 10 Sweney MT, Silver K, Gerard-Blanluet M. et al . Alternating hemiplegia of childhood: early characteristics and evolution of a neurodevelopmental syndrome.  Pediatrics. 2009;  123 e534-e541
• 11 Tenney JR, Schapiro MB. Child neurology: alternating hemiplegia of childhood.  Neurology. 2010;  74 e57-e59

Correspondence