A painful stiff neck following an ear, nose, and throat surgical procedure: case report.

Grisel syndrome is a rare, nontraumatic atlantoaxial subluxation, typical of developmental ages and characterized by head flexion/rotation and painful fixation. Neurological symptoms may occur. It is secondary to head/neck infections and ear, nose, and throat surgery (adenoidectomy, tonsillectomy, and mastoidectomy). Here, we report the case of a child who presented a painful stiff neck following an adenotonsillectomy, with imaging evidencing an atlantoaxial subluxation. The child showed improvement in his condition following a conservative treatment with antibiotics, anti-inflammatory, and analgesic therapy and cervical collar. We believe it is of great significance for clinicians taking into account this peculiar condition in the differential diagnosis of a stiff neck in pediatric patients, thus avoiding misdiagnosis and delays. Indeed, its diagnosis is mainly based on a focused anamnesis associated with the detection of the typical neuroradiological findings.

Neonatal seizures and postneonatal epilepsy: a 7-y follow-up study.

BACKGROUND: Seizures are one of the most common symptoms of acute neurological disorders in newborns. This study aimed at evaluating predictors of epilepsy in newborns with neonatal seizures. METHODS: We recruited consecutively 85 neonates with repeated neonatal video-electroencephalogram (EEG)-confirmed seizures between January 1999 and December 2004. The relationship between clinical, EEG, and ultrasound (US) data in the neonatal period and the development of postneonatal epilepsy was investigated at 7 y of age. RESULTS: Fifteen patients (17.6%) developed postneonatal epilepsy. Partial or no response to anticonvulsant therapy (odds ratio (OR) 16.7, 95% confidence interval (CI): 1.8-155.8, P = 0.01; OR 47, 95% CI: 5.2-418.1, P < 0.01, respectively), severely abnormal cerebral US scan findings (OR: 5.4; 95% CI: 1.1-27.4; P < 0.04), severely abnormal EEG background activity (OR: 9.5; 95% CI: 1.6-54.2; P = 0.01), and the presence of status epilepticus (OR: 6.1; 95% CI: 1.8-20.3; P < 0.01) were found to be predictors of epilepsy. However, only the response to therapy seemed to be an independent predictor of postneonatal epilepsy. CONCLUSION: Neonatal seizures seem to be related to postneonatal epilepsy. Recurrent and prolonged neonatal seizures may act on an epileptogenic substrate, causing further damage, which is responsible for the subsequent clinical expression of epilepsy.

Ketogenic diet also benefits Dravet syndrome patients receiving stiripentol: a prospective pilot study.

We aimed to test the efficacy of ketogenic diet (KD) in patients with Dravet syndrome (DS) not satisfactorily controlled by antiepileptic drugs (AEDs). We included prospectively 15 DS patients aged >3 years with partial response to AEDs including stiripentol. All patients had a seizure diary and clinical examination with Conners and Achenbach scales before KD, at 1 month following onset and every 3 months thereafter. At 1 month, 10 patients (66%) had a decrease of seizure frequency ≥75%. Efficacy was maintained in eight responders at 3 and 6 months and in six responders at 9 months. Five patients (33%) remained on KD over 12 months, and one was seizure-free. In addition to efficacy on seizure frequency, KD was beneficial on behavior disturbances including hyperactivity. This effect was reported in all responders and in a few nonresponders. KD might have a double effect, on seizure control and on hyperactivity and behavior disturbances in patients with DS.

Care to relieve pain-stress in preterm newborns.

BACKGROUND AND AIM OF THE WORK: A variety of non-pharmacological pain-prevention and relief techniques have been studied to evaluate the pain reduction in neonates. The aim of our study was to compare the analgesic effect of sucking a pacifier with the use of eutectic mixture of local anaesthetics (EMLA) during venipuncture in preterm newborns, using physiological and behavioural parameters as indicators of pain. METHODS: We analysed the reaction to invasive procedures in 17 preterm newborns. Our patients underwent repeated vein draws without pain relief, sucking a pacifier, after the application of EMLA; we also evaluated a group of patients approached for care without pricking. For each infant we recorded the average values of the physiological parameters at rest and after pain stimuli, behavioural conditions (crying or grimaces), number and time required for blood draw. RESULTS: The maximum heart rate values, respiratory rate, and the maximum respiratory rate values presented a statistically difference only between subjects that underwent vein draws compared to subjects without pricking (p < 0.01). Moreover, the SpO2 parameter presented a significant increase in the control group compared to the others (p = 0.024). Analysis ofbehavioural parameters shows that crying seems significantly related to the duration and number ofvenipunctures (p = 0.000). CONCLUSIONS: It is clear that pain stress is more closely related to the duration and number of venipuncture than pain relief methods. Our results suggest that limiting the number and duration of vein draws could help to reduce pain stresss in preterm newborns.

Neonatal seizures: relation of ictal video-electroencephalography (EEG) findings with neurodevelopmental outcome.

Neonatal electroencephalographic background activity has been found to be a predictive factor of the neurodevelopmental outcome. The aim of our study was to identify if the electrical ictal findings present on the first electroencephalography (EEG) recording are related to the outcome of newborns with neonatal seizures. The study is based on the prospective evaluation of newborns consecutively admitted to the Neonatal Intensive Care Unit at the University of Parma between September 2001 and September 2004. Thirty-eight subjects were enrolled in the study on the basis of the following inclusion criteria: presence on the first EEG of at least 1 seizure, neurodevelopmental follow-up until 18 months of corrected age, and performance of several ultrasound brain scans during the neonatal period and of at least 1 cerebral MRI within the first year of life. For each seizure, the following were considered: onset topography, morphology of the epileptiform discharges, spread of the discharge, number of electrographic regions of seizure onset, number of seizures per hour, duration of the seizures, and the Ictal Fraction (= total duration of the seizures/duration of the EEG recording x hour). At the last follow-up, the unfavorable neurodevelopmental outcome seems significantly related to the moderate/severe background activity abnormalities (p = .006), to the spread of ictal discharge to the contralateral hemisphere (p = .02), and to the Ictal Fraction, when it exceeds 10 minutes (p = .036). In conclusion, the analysis of the propagation of the ictal discharge and of the Ictal Fraction might suggest significant prognostic information since the first hours of life.

Mortality risk after neonatal seizures in very preterm newborns.

We analyzed clinical and instrumental data of 403 consecutive newborns with gestational age from 24 to 32 weeks, admitted to the University-Hospital of Parma between January 2000 and December 2007, to evaluate the possible relationship between neonatal mortality and occurrence of neonatal seizures in very preterm newborns. Seventy-four subjects died during hospital stay. Seizures were present in 35 neonates, in whom the mortality rate was 37.1%. Multivariate analysis revealed that birth-weight <1000 g (odds ratio: 4.48; 95% confidence interval: 1.47-13.68; P < .01), cardiopulmonary resuscitation (odds ratio: 5.35; 95% confidence interval: 1.19-23.98; P = .02), and moderately and severely abnormal cerebral ultrasound scan findings (odds ratio: 2.48; 95% confidence interval: 1.02-6.05; P < .04; odds ratio: 9.56; 95% confidence interval: 3.45-26.51; P < .01, respectively) were related to the in-hospital mortality but not the presence of neonatal seizures. Our study suggests that neonatal seizures alone are not an independent risk factor for early death in very preterm newborns.

Motor representation of actions in children with autism.

BACKGROUND: Children with Autistic Spectrum Disorders (ASD) are frequently hampered by motor impairment, with difficulties ranging from imitation of actions to recognition of motor intentions. Such a widespread inefficiency of the motor system is likely to interfere on the ontogeny of both motor planning and understanding of the goals of actions, thus delivering its ultimate effects on the emergence of social cognition. METHODOLOGY/PRINCIPAL FINDINGS: We investigate the organization of action representation in 15 high functioning ASD (mean age: 8.11) and in two control samples of typically developing (TD) children: the first one, from a primary school, was matched for chronological age (CA), the second one, from a kindergarten, comprised children of much younger age (CY). We used nine newly designed behavioural motor tasks, aiming at exploring three domains of motor cognition: 1) imitation of actions, 2) production of pantomimes, and 3) comprehension of pantomimes. The findings reveal that ASD children fare significantly worse than the two control samples in each of the inspected components of the motor representation of actions, be it the imitation of gestures, the self-planning of pantomimes, or the (verbal) comprehension of observed pantomimes. In the latter task, owing to its cognitive complexity, ASD children come close to the younger TD children's level of performance; yet they fare significantly worse with respect to their age-mate controls. Overall, ASD children reveal a profound damage to the mechanisms that control both production and pre-cognitive "comprehension" of the motor representation of actions. CONCLUSIONS/SIGNIFICANCE: Our findings suggest that many of the social cognitive impairments manifested by ASD individuals are likely rooted in their incapacity to assemble and directly grasp the intrinsic goal-related organization of motor behaviour. Such impairment of motor cognition might be partly due to an early damage of the Mirror Neuron Mechanism (MNM).

Development of epilepsy in newborns with moderate hypoxic-ischemic encephalopathy and neonatal seizures.

BACKGROUND: Hypoxic-ischemic encephalopathy (HIE) is one of the most frequent causes of neonatal death or neurological handicaps such as cerebral palsy, mental delay, and epilepsy. Moreover, an acute consequence of HIE are neonatal seizures which can cause an additional brain damage. The neurodevelopmental outcome is known in the mild or severe cases of HIE, but in the moderate conditions the predictivity results, to date, unsatisfying. OBJECTIVE: The purpose of this prospective study was to appraise the development of post-neonatal epilepsy in a cohort of term infants with moderate HIE and neonatal seizures. METHODS: This study considered all newborns admitted to Neonatal Intensive Care Unit of the University of Parma between January 2000 and December 2002 for perinatal asphyxia, then followed by Neonatal Neurology Service. In all patients, neonatal variables such as type of delivery, birth weight, gestational age, Apgar scores, the need for resuscitation and assisted ventilation soon after birth, and arterial-blood pH were analyzed. RESULTS: Ninety-two newborns were enrolled in the study because of perinatal asphyxia. Of these, 27 subjects developed mild HIE, 25 moderate, and five severe HIE. Neonatal seizures were present in 13 subjects with moderate HIE and in all newborns with severe HIE. At the last follow-up, only three infants belonging to patients with severe HIE developed epilepsy. CONCLUSION: Moderate HIE seems not to be related to post-neonatal epilepsy either if associated or not with neonatal seizures.