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Published on: March 1, 2015
Recurrent upper motor neuron facial weakness of possible epileptic etiology: a case report
1Department of Paediatrics, Hannover Medical School, Hannover, Germany. hartmann.hans@mh-hannover.de
Insights
Episodic facial and hypoglossal nerve weakness in infants may signal benign focal epilepsy. Early diagnosis of this rare condition, characterized by negative epileptic motor phenomena, is crucial for appropriate management.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurophysiology
Background:
- Benign focal epilepsy with centro-temporal sharp waves (BECTS) is a common childhood epilepsy syndrome.
- Ictal orofacial phenomena are characteristic but can be challenging to diagnose, especially in infants.
- Negative epileptic motor phenomena, such as facial nerve paresis, can mimic other neurological conditions.
Observation:
- An infant presented with recurrent, alternating episodes of central facial nerve paresis and hypoglossal nerve paresis.
- Symptoms resolved spontaneously, and initial investigations ruled out infection, infarction, or structural lesions.
- Electroencephalogram (EEG) revealed benign sharp waves in the right temporo-occipital region during sleep.
Findings:
- The patient was diagnosed with benign focal epilepsy presenting with negative motor phenomena.
- Antiepileptic treatment led to the cessation of further episodes.
- The case highlights the atypical presentation of BECTS in infants.
Implications:
- Episodic facial nerve paresis in infants warrants consideration of epileptic etiologies, specifically BECTS.
- Recognizing negative epileptic motor phenomena can prevent extensive and unnecessary diagnostic work-ups.
- This case underscores the importance of EEG in diagnosing subtle epileptic manifestations in pediatric neurology.
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.
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