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Symptomatic neonatal seizures followed by febrile status epilepticus: The two-hit hypothesis for the subsequent development of epilepsy
No full textNeonatal seizures have been associated with the later development of postneonatal epilepsy, mainly beginning within the first year of life. Mechanisms of epileptogenesis in the immature brain still need to be fully elucidated but a two-hit hypothesis, showing that an early insult heightens later susceptibility to seizure-induced brain damage, has been demonstrated in animal models. We describe 2 cases of preterm babies sustaining recurrent neonatal seizures in the context of a severe perinatal brain damage who presented with symptomatic epilepsy only after the occurrence of an episode of febrile status epilepticus. In the context of preexisting perinatal brain damage, febrile status epilepticus acted as a second hit for developing epilepsy, confirming animal evidence
Association between neonatal Volkmann’s syndrome and perinatal ischemic stroke: Review of the literature
No full textBACKGROUND AND AIM:
Neonatal forearm compartment syndrome, also known as neonatal Volkmann's syndrome, is an uncommon condition for which the pathognomonic finding is the presence of a forearm skin lesion. Stroke in newborns occurs in about 1/1600-5000 births. Aim of the present review is to highlight the possible association of these two conditions.
METHODS:
we performed a review concerning the association of both these conditions.
RESULTS:
We found only few reports on the association of an ischemic limb injury and a perinatal stroke combined in the same newborn.
CONCLUSIONS:
In light of the high percentage of delayed diagnosis of perinatal stroke, we recommend to perform an accurate neurological evaluation and eventual neuroimaging studies in order to investigate for a cerebral vascular damage and, furthermore, to rule out thrombophilic conditions in infants with signs of a neonatal ischemic limb and/or Volkmann's syndrome
Epilepsy after neonatal seizures: Literature review
No full textIntroduction Acute neonatal seizures are the most frequent neurological complication in the neonatal intensive care units and the seizing newborns have an increased risk of long-term morbidity. However, the relationship between neonatal seizures and the development of epilepsy later in life is still unclear.
Methods We performed a literature review using the search terms "neonatal seizures AND outcome", "neonatal seizures AND epilepsy", "neonatal seizures AND post-neonatal epilepsy", including secondary sources of data such as reference lists of articles reviewed. From the studies in which data were available, the incidence of epilepsy was calculated by dividing the number of all subjects who developed epilepsy in the different studies considered with the number of all newborns enrolled to the studies less the number of patients lost at follow-up.
Results We found 44 studies published between 1954 and 2013, of which 4 were population-based studies and the remaining were hospital-based case series. The overall population evaluated was 4538 newborns and 17.9% developed post-neonatal epilepsy, with an onset within the first year of life in 68.5% of the patients. In 80.7%, epilepsy was associated with other neurological impairments.
Conclusion Estimates on epilepsy after neonatal seizures vary widely depending on selection criteria and length of the follow-up. However, it represents a common outcome of these newborns, especially in those with severe brain injury and additional neurodevelopmental disabilitie
Hereditary Hemorrhagic Telangiectasia presenting as migraine: A case report
No full textBackground: Hemorrhagic Hereditary Telangiectasia (HHT) is an autosomal dominant disease characterized by the presence of multiple arteriovenous malformations (AVMs). Migraine is described in association with HHT in adulthood, while only few paediatric cases are reported in the literature. Aim: In this paper, we describe an atypical case of HHT in a 7-year-old boy, who presented severe and recurrent episodes of migraine-like headache as the first symptom of the disease. Methods: The patient was accurately investigated both clinically (general, neurological and dermatological examinations), instrumentally (electroencephalogram, brain magnetic resonance, transcranic Eco-color-Doppler, contrast echocardiography and enhanced chest computed tomography) and genetically. Results: Familial history was positive for HHT in the paternal line. Both general and neurological examination were normal. Brain magnetic resonance imaging showed a minor old infarct in the right parietal and occipital lobes. Transcranic Eco-color-Doppler, contrast echocardiography and enhanced chest computed tomography revealed a pulmonary AVM. Cephalalgia resolved after transcatheter embolotherapy. A genetic test, identifying the mutation in endoglin gene both in the patient and in the father, confirmed the suspected diagnosis of HHT. Conclusions: Although headache is rarely reported in children as the first symptom of HHT, we warn clinicians on this possible link, as a promptly diagnosis is advisable in order to prevent potential complications
Febrile and postinfectious myoclonus: Case reports and review of the literature
No full textAbstract Purpose Myoclonus associated with fever or following an infectious event (parainfectious myoclonic jerks) is a transient event with an abrupt onset. It is not common and often not recognized. In these reports, its clinical features and its favorable outcome are described. Patients and Methods We identified three pediatric patients with parainfectious myoclonic jerks who were treated at Children's Neuropsychiatry Unit at the University-Hospital of Parma (Italy). Results The myoclonic jerks presented an abrupt onset and were characterized by brief involuntary muscle contractions temporally related to febrile episodes or following infectious illnesses. The outcome was positive as they resolve spontaneously with cessation of febrile/infective events. Conclusion Parainfectious myoclonic jerks are temporary benign phenomena that could occur in association with the fever or following an infectious event. It is quite important to make a correct diagnosis as early as possible to avoid unnecessary hospitalizations or investigations
Neonatal status epilepticus: Differences between preterm and term newborns
No full textBackground Despite the many studies on neonatal seizures, neonatal status epilepticus (NSE) remains a controversial entity, with no general consensus about its definition. We report the characteristics of newborns with NSE in order to assess whether they showed homogeneous features or displayed clinical and/or instrumental differences depending on gestational age (GA). Preterm and term neonates were compared and risk factors for adverse outcome evaluated. Methods From 154 newborns with video-EEG confirmed neonatal seizures admitted to the NICU of Parma University Hospital between January 1999 and December 2012, we collected a cohort of 47 newborns (19 preterm, 28 full-term) with NSE. NSE was defined as continuous seizure activity for at least 30 min or recurrent seizures lasting a total of 30 min without definite return to the baseline neurologic condition between seizures. Outcome was assessed at least at one year. We applied the χ2 test to compare nominal data, and multivariate logistic regression analysis to determine independent risk factors for adverse outcome. Results Only Apgar scores and neurologic examination (p ≤.02) were different between the groups. None of the preterm newborns had a favourable outcome compared to 25% of the full-term ones (p =.032). Moreover, 52.6% of preterm neonates died compared to 17.8% of the full-term newborns (p =.01; OR = 5.11). The only variable related to outcome was Apgar score at 5 min (p =.02). Conclusion Newborns with NSE represented a quite homogeneous group regardless of the GA. Outcome was unfavourable in most of the subjects; however adverse outcome and death were more represented in preterm newborns
What is new: Talk about status epilepticus in the neonatal period
No full textNowadays, no general consensus was achieved regarding neonatal status epilepticus and its definition. Indeed, different criteria (mainly based on seizure duration) were used. Whereas a recent proposal has been developed to define status epilepticus in older ages, it seems that the peculiar characteristics of neonatal seizures and of the immature brain make difficult to find a tailored definition for this period of life. Achieving a consensus on this entity would mean to make the first step toward a targeted therapeutic strategy of intervention
The role of electroencephalogram in neonatal seizure detection
No full textThe role of EEG in neonatal seizure detection is well-established, being the multichannel video-EEG the gold standard. However, in the clinical practice often amplitude integrated EEG (aEEG) is used, in order to overcome the difficulties related to EEG use. Areas covered: An overview regarding neonatal seizures, current tools used to detect these (multichannel EEG versus aEEG) with respective strenghts and limitations, and some tools that can implement the use of multichannel EEG in the NICU. Expert commentary: Multichannel video-EEG is still a gold standard for seizure detection. Indeed, this tool allows to avoid both underestimation of neonatal seizure incidence and overtreatment with anticonvulsant drugs. Furthermore, it has to be acknowledged that multichannel video-EEG monitoring is not limited to the only seizure detection, providing also the information needed for a more accurate assessment of the background activity and some specific waves/pattern and features indicative of the brain development
Phenobarbital for neonatal seizures:response rate and predictors of refractoriness
Full text linkBackground : Phenobarbital is the first-line choice for neonatal seizures treatment, despite a response rate of approximately 45%. Failure to respond to acute anticonvulsants is associated with poor neurodevelopmental outcome, but knowledge on predictors of refractoriness is limited. Objective : To quantify response rate to phenobarbital and to establish variables predictive of its lack of efficacy. Methods : We retrospectively evaluated newborns with electrographically confirmed neonatal seizures admitted between January 1999 and December 2012 to the neonatal intensive care unit of Parma University Hospital (Italy), excluding neonates with status epilepticus. Response was categorized as complete (cessation of clinical and electrographic seizures after phenobarbital administration), partial (reduction but not cessation of electrographic seizures with the first bolus, response to the second bolus), or absent (no response after the second bolus). Multivariate analysis was used to identify independent predictors of refractoriness. Results : Out of 91 newborns receiving phenobarbital, 57 (62.6%) responded completely, 15 (16.5%) partially, and 19 (20.9%) did not respond. Seizure type (p = 0.02), background electroencephalogram (EEG; p ≤ 0.005), and neurologic examination (p ≤ 0.005) correlated with response to phenobarbital. However, EEG (p ≤ 0.02) and seizure type (p ≤ 0.001) were the only independent predictors. Conclusion : Our results suggest a prominent role of neurophysiological variables (background EEG and electrographic-only seizure type) in predicting the absence of response to phenobarbital in high-risk newborns
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