Novel Insights from Clinical Practice Neonatology 2018;113:33–36 DOI: 10.1159/000480423 Received: April 26, 2017 Accepted after revision: June 12, 2017 Published online: September 26, 2017 Cerebellar Infarction: Unusual Manifestation with Facial Palsy, Focal Seizures, and Secondary Generalization Tobias Mühlbacher a Georg Bohner b Christoph Bührer a Christof Dame a Departments of a Neonatology and b Neuroradiology, Charité – Universitätsmedizin Berlin, Berlin, Germany Established Facts • Focal seizures are the most common symptom after neonatal cerebral infarction, while cerebellar lesions are usually clinically silent. • Subcortical seizures originating from cerebellar lesions are controversial. Novel Insights • This case indicates that cerebellar lesions after hemorrhagic infarction may exhibit epileptogenic activity, with seizure patterns recorded over the contralateral cerebral hemisphere. • Transnuchal ultrasound through the foramen magnum provides easy access for comparative imaging of both cerebellar hemispheres. Abstract Background: Cerebellar infarction is exceedingly rare in neonates, usually occurring after traumatic birth. Lifelong sequelae can result from cerebellar damage with disorders of motor function, ataxia, and also cognitive dysfunction. Objectives/Methods: We report the clinical presentation of a preterm triplet infant delivered by elective cesarean who showed peripheral facial palsy immediately after birth. Results: Tonic seizures with high-voltage discharges over the contralateral cerebral hemisphere and secondary general- © 2017 S. Karger AG, Basel E-Mail karger@karger.com www.karger.com/neo ization were successfully treated with phenobarbital. Transnuchal ultrasound through the foramen magnum and subsequent MRI examinations revealed infarction of the left cerebellar hemisphere. Conclusions: In newborn infants, acute cerebellar stroke may have direct epileptogenic potential. © 2017 S. Karger AG, Basel Case Report As the second triplet infant, a male neonate was delivered by primary cesarean at a gestational age of 34 1/7 weeks (birth weight 2,075 g) after an uneventful pregnancy. The infant showed rapid postnatal adaption (Apgar scores 8/9/10 at 1/5/10 min), but physical examination revealed a left peripheral facial palsy involving all Tobias Mühlbacher, MD Department of Neonatology, Charité – Universitätsmedizin Berlin Charité-Platz 1 DE–10117 Berlin (Germany) E-Mail Tobias.Muehlbacher @ charite.de Downloaded by: Göteborgs Universitet 130.241.16.16 - 9/27/2017 1:41:07 AM Keywords Cerebellar infarction · Facial palsy · Preterm infant · Seizures Fig. 1. a Cranial ultrasound in transversal a 3 branches. At 8 h after birth, the patient developed tonic seizures with vertical ocular deviation, stridor, and bradycardic and apneic episodes that necessitated noninvasive positive pressure ventilation. Cranial ultrasound through the anterior fontanelle and transnuchal scans through the foramen magnum showed a diffuse hyperechoic left cerebellar hemisphere lacking central arterial perfusion (Fig. 1a). MRI scans confirmed hemorrhagic infarction of the left cerebellar hemisphere shifting the adjacent structures towards the cerebellopontine angle (Fig. 1b), which was likely to be the cause of peripheral facial palsy. Amplitude-integrated encephalography indicated abnormal activities over the right parietal cortex (Fig. 2), occasionally with secondary generalization (online suppl. Fig. 1; see www.karger.com/doi/10.1159/000480423 for all online suppl. material). The seizures terminated after the administration of phenobarbital. Serial transfontanellar ultrasound examinations revealed transient dilatation of both lateral ventricles with no need for neurosurgical intervention. The facial palsy gradually resolved, with complete remission after 4 weeks. At discharge, the infant did not show neurologic disorders and was off antiepileptic medication. During the follow-up examinations at a corrected age of 3 and 6 months, the infant showed a slightly reduced motor function of the left side and trunk tonicity, but was otherwise normally developed and free of seizures. MRI scans after 7 months showed an isolated substance defect of the left cerebellar hemisphere (online suppl. Fig. 2), a normal vermis, and unsuspicious right cerebellar hemisphere, as well as supratentorial structures (online suppl. Fig. 3). Discussion Neonatal arterial ischemic stroke has a reported overall incidence of 5–43 per 100,000 neonates [1]. Hemorrhagic transformation of primary ischemic insult is often difficult to prove, two-thirds remain “idiopathic,” yet with the same risk factors as arterial stroke [2]. Both are associated with fetal risk factors such as prothrombotic 34 Neonatology 2018;113:33–36 DOI: 10.1159/000480423 b disorders, systemic illness, and inflammation, as well as maternal and obstetric factors or birth complications. Sources of presumed emboli include placental fragments or clots, thrombi in involuting fetal vessels, and thrombi or air in punctured or catheterized vessels [3]. Notably, emboli from fetal or neonatal venous circulation can enter arterial circulation by passage across the patent foramen ovale, resulting in a thromboembolic event, scarcely also symptomatic after occluding a nonterminal arterial branch [4]. Lacking collateral arterial perfusion, even small emboli in the intracranial arterial circulation can cause extended insults. The topography of infarction in arterial distribution is distinctive, mostly affecting the middle cerebral artery (>95 %) in a unilateral fashion. Focal seizures, typically occurring within the first days after birth, are the most common symptom [5]. Cerebellar infarction, however, is a rare event which has been reported in term neonates after traumatic birth. In these cases, cerebellar hemorrhagic injury is a result of distortion leading to injury of the falx or tentorium, or a direct cerebellar contusion [6]. In very preterm infants, cerebellar hemorrhage is mostly clinically silent and only diagnosed by routine cerebral ultrasound examination. Thus, the information on incidence ranges widely between 3 and 25%. In these cases, cerebellar hemorrhage is associated with perinatal distress and, within the first 5 days after birth, with hemodynamic disorders including patent ductus arteriosus [7]. The reported case is fairly remarkable. Unilateral facial nerve palsy as the first symptom of cerebellar infarction is an extremely rare finding. The immediate observation of facial nerve palsy at birth and the subsequent Mühlbacher/Bohner/Bührer/Dame Downloaded by: Göteborgs Universitet 130.241.16.16 - 9/27/2017 1:41:07 AM scan with a high-resolution 18- to 5-MHz linear transducer and nuchal access through the foramen magnum, indicating expansive hemorrhagic infarction of the left cerebellar hemisphere. b MRI T2 scan proving stroke of the left cerebellar hemisphere with secondary hemorrhagic transformation: within the hypointense area, the underlying structure can still be identified. Medial-rostral hemorrhage is more compact with a shift of the normal tissue structures (*). Close to the cerebellopontine angle is a small area of normal cerebellar structure which can be clearly distinguished from the stroke (arrow). 12:00 13:00 14:00 EEG, μV aEEG, μV 100 100 25 25 10 10 5 5 0 0 100 25 100 25 10 10 5 5 0 0 40 40 0 0 –40 –40 40 40 0 0 –40 –40 clinical course suggest that the cerebellar insult occurred shortly prior to or during delivery. A cesarean section was electively performed without clinical signs for preeclampsia, chorioamnionitis, or other primary maternal risk factors for perinatal stroke. With regard to fetal/neonatal risk factors [3], there was no evidence of fetal thrombotic vasculopathy or vasculitis, and symptoms occurred prior to any catheterization or venous puncture for neonatal care. Moreover, coagulation screens and specific laboratory tests, including protein C and S, were unsuspicious. Extending the standard planes of cranial ultrasound by transversal scan through the foramen magnum allowed for an immediate bedside diagnosis. A high-resolution linear probe was placed in the neck, providing an excellent comparison of both cerebellar hemispheres. The subsequent MRI scan excluded a primary hemorrhage, as the underlying cerebellar structure could still be identified within the area of infarction, as well as supratentorial brain injury (online suppl. Fig. 2). While the initial ultrasound examination showed a normal ventricular size, we found secondary ventricular dilatation. On day 3 after birth, the MRI scan confirmed the increased volume of the cerebrospinal ventricular system. MR angiography showed normal perfusion of both vertebral arteries (online suppl. Fig. 4). Notably, secondary deterioration occurred after an interval with few symptoms, similar to adults with cerebellar mass infarction [8], yet not with a mere loss of consciousness. According to the classic theorem, subcortical structures have no impact on the origin of seizures. More recently, however, it has been reported that seizures can originate from hypothalamic hamartoma and tumorous cerebellar lesions, as shown in gangliogliomas using stereotactically placed depth electrodes [9–12]. Cerebellar seizures could be tracked with electro source imaging, Cerebellar Infarction Neonatology 2018;113:33–36 DOI: 10.1159/000480423 35 Downloaded by: Göteborgs Universitet 130.241.16.16 - 9/27/2017 1:41:07 AM Fig. 2. Focal seizures of the right (contralateral) cortex recorded both in raw and amplitude-integrated EEG (aEEG). which combines MR imaging and electrophysiological techniques [13]. Rarely, seizures also occur after traumatic cerebellar hemorrhage under delivery, some with concomitant cerebral affection [6]. In the case reported here, we were able to record typical focal neonatal seizure patterns over the contralateral cerebral hemisphere, although this region was normal regarding structure and volume in the initial and follow-up MRI scans, so far not indicating a crossed cerebellar-cerebro diaschisis [14]. The focal pattern strongly argues against the assumption that the abnormal ictal activity resulted from hemorrhagic contamination of the cerebrospinal fluid. Combined with the occasional secondary generalization, this observation demonstrates that a stroke-mediated cerebellar lesion exhibits epileptogenic activity in preterm neonates. The complete remission of the peripheral facial palsy and cessation of the seizures without medication at discharge support the hypothesis that the symptoms were mediated by a transient compression of the cerebellopontine angle [9]. The spreading of the seizure activity, however, provides evidence for a direct epileptogenic effect, possibly due to disinhibition by loss of inhibitory Purkinje cell efferences [10]. Such a pathomechanism may contribute to long-term sequelae of cerebellar infarction and deserves further investigation. Disclosure Statement The authors declare no conflicts of interest. References 36 stroke: the International Pediatric Stroke Study. Pediatrics 2011;128:e1402–e1410. 6 Limperopoulos C, Robertson RL, Sullivan NR, Bassan H, du Plessis AJ: Cerebellar injury in term infants: clinical characteristics, magnetic resonance imaging findings, and outcome. Pediatr Neurol 2009;41:1–8. 7 Limperopoulos C, Benson CB, Bassan H, Disalvo DN, Kinnamon DD, Moore M, Ringer SA, Volpe JJ, du Plessis AJ: Cerebellar hemorrhage in the preterm infant: ultrasonographic findings and risk factors. 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