Efficacy and safety profile of intravenous levetiracetam versus phenytoin in convulsive status epilepticus and acute repetitive seizures in children.

PURPOSE: Although phenytoin is one of the most commonly used antiepileptic drugs (AEDs), it has potential serious side effects and drug interactions. Levetiracetam is a relatively newer AED with favorable pharmacokinetics and could be an effective and safer option for the treatment of convulsive status epilepticus (CSE). We aimed to compare the efficacy and safety profile of intravenous levetiracetam and phenytoin as second-line treatment agents in children with CSE and acute repetitive seizures (ARS). METHOD: Two hundred seventy-seven patients aged between 1 month and 18 years who received intravenous levetiracetam or phenytoin as a second-line AED with the diagnosis of CSE or ARS were retrospectively evaluated. Drug efficacy was defined as control of seizures without the need for any additional medication after completion of the infusion and no recurrence in the following 12 h. The primary outcome was drug efficacy. The secondary outcomes included application of an additional second-line AED, induction of anesthesia, and admission to the intensive care unit (ICU), and drug-related adverse reactions. RESULTS: No differences were found between the two treatment groups with regard to patient characteristics and seizure type. The efficacy of levetiracetam was higher than that of phenytoin (77.6% vs 57.7%, P = 0.011) in children with CSE. There was no significant difference between the efficacy rates of levetiracetam and phenytoin for ARS (55.8% vs 58.8%, P = 0.791). Overall, drug efficacy was 70.9% for levetiracetam and 58.1% for phenytoin (P = 0.048). For CSE, the need for additional second-line treatment, anesthesia induction, and ICU admission was higher in the phenytoin group (P = 0.001, P = 0.038, P = 0.02, respectively). Drug-related adverse reactions were more frequent in the phenytoin group than the levetiracetam group (23.3% vs 1.4%; P < 0.001). The most common adverse reaction in the phenytoin group was hypotension. Phenytoin-related anaphylaxis was detected in one patient. No serious adverse effects related to levetiracetam were observed. CONCLUSIONS: Intravenous levetiracetam seems as effective as intravenous phenytoin in emergency treatment of children with ARS and more effective for CSE in stopping the seizure with less risk of recurrence. Levetiracetam has fewer cardiovascular side effects and has a safer profile than phenytoin. Intravenous levetiracetam is a favorable option as a first second-line AED for pediatric seizures.

Interictal epileptiform discharges on electroencephalography in children with methylenetetrahydrofolate reductase (MTHFR) polymorphisms.

OBJECTIVE: Methylenetetrahydrofolate reductase (MTHFR) is an important enzyme involved in folate metabolism. MTHFR C677T and A1298C polymorphisms are best-defined variants of MTHFR that were reported to be associated with epilepsy development. The aim of the study was to determine the incidence of interictal epileptiform discharges on electroencephalography (EEG) in asymptomatic children with C677T and A1298C polymorphisms who had no history of seizure. METHODS: Children with MTHFR C677T or A1298C polymorphisms who had normal neurological examination without a history of seizure were included in the study. Blood samples for serum folate, vitamin B12, and homocysteine levels were analyzed. Sleep and awake electroencephalograms (EEG) were recorded. RESULTS: A total of 102 children (50 girls and 52 boys) with a mean age of 59.4 ± 58.7 months were included in the study. Interictal epileptiform EEG discharges were detected in 3 children (2.9%). CONCLUSION: There was no increase in the prevalence of interictal epileptiform discharges in seizure-free and asymptomatic children with MTHFR C677T and A1298C polymorphisms.

Analysis of tissue Doppler parameters and 24-hour heart rate variations in children with newly diagnosed untreated idiopathic epilepsy in interictal period.

INTRODUCTION: Cardiac mortality has increased in patients with epilepsy. Although majority of cardiac autonomic and ventricular function abnormalities were detected in ictal and postictal period, interictal epileptogenic activity may induce the autonomic imbalance as well. In our study, we aimed to investigate the interictal, subclinical cardiac changes in terms of cardiac autonomic balance via 24-hour Holter electrocardiography (ECG) and ventricular functions by tissue Doppler echocardiography (TDI) in children with newly diagnosed untreated idiopathic epilepsy. MATERIAL AND METHODS: Thirty children with newly diagnosed untreated idiopathic epilepsy (12 males, 18 females; mean age: 125.13 ±â€¯35.2 months) (patient group) and 40 healthy, age and body mass index (BMI)-matched children (18 males, 22 females; mean age: 129.43 ±â€¯38.5 months) (control group) were enrolled. Included patients underwent 24-hour Holter electrocardiographic and tissue Doppler echocardiographic study. RESULTS: Time domain measures were found significantly lower in the patient group. Mean high frequency (HF) values were significantly lower, and mean low frequency (LF) and mean LF/HF parameters were significantly higher in the patient group. Mean isovolumetric contraction time (ICT), isovolumetric relaxation time (IRT), and myocardial performance index (MPI) values were significantly higher, and mean ejection time (ET) values were significantly lower among the patients with untreated idiopathic epilepsy. CONCLUSION: We found that patients with untreated newly diagnosed epilepsy have a significant subclinical deterioration of left ventricular functions, and they also showed changes in heart rate variability (HRV) regarding the sympathovagal imbalance in interictal period. These findings can be related with increased cardiac mortality.

Risk factors associated with epilepsy development in children with cerebral palsy.

OBJECTIVES: Epilepsy is one of the most common and important comorbidity among patients with cerebral palsy (CP). The purpose of this study was to determine the risk factors predicting the development of epilepsy considering prenatal, perinatal, and natal characteristics; associated impairments; and cranial imaging findings in our patient population with cerebral palsy at a tertiary center in Istanbul, Turkey. METHODS: This retrospective study consisted of 234 children aged between 3 and 18 years of age. Children were divided into two groups as CP patients with epilepsy (126 patients) and CP patients without epilepsy (108 patients). Demographic features and clinical and cranial magnetic resonance imaging (cMRI) findings were compared between the two groups. RESULTS: Presence of family history of epilepsy, history of neonatal seizure especially in the first 72 h of life, quadriplegic type of CP, severe degree of gross motor function and fine motor disorders, and moderate to severe mental retardation or psycho-social developmental delay were determined as risk factors for the development of epilepsy in CP patients. Also, an increased risk of epilepsy was detected in term infants and appropriate for gestational age (2500-4000 g) infants. On the other hand, presence of parental consanguinity, being born from a primiparous mother, age of mother at birth, mode of delivery, presence of multiple gestation and labor problems, history of follow-up in neonatal intensive care unit and intubation, and cMRI findings were not significant risk factors for the development of epilepsy in CP. CONCLUSION: Predicting epilepsy development by determining the risk factors in patients with CP might be useful because knowing the risk factors could provide close follow-up of these patients for epilepsy.

Increased QT and P-wave dispersion during attack-free period in pediatric patients with migraine attacks.

INTRODUCTION: Migraine is a common neurovascular disease characterised with recurrent attacks by pain-free periods. It has been suggested that both sympathetic and parasympathetic dysfunctions play a role in its pathophysiology. AIM: The aim of our study was to investigate the ECG changes during attack-free period in children with migraine, in terms of QTc interval, QTc, and P-wave dispersion to evaluate the autonomic nervous system disturbance. METHODS: Sixty children who were diagnosed with migraine were included as patient group and 50 healthy, age- and body mass index-matched children who were examined for innocent murmur were included as control group. The patients' routine ECG records were screened from the outpatient clinic files. The durations of P-wave, QT, and QTc intervals and dispersion values and heart rates (beats/minute) were compared between the patient and control groups. RESULTS: P maximum and P dispersion were significantly higher, and P minimum was significantly lower in the migraine group compared with the control group. QT-QTc maximum and QT-QTc dispersion were significantly higher and QT-QTc minimum was significantly lower in the migraine group compared with the control group. CONCLUSION: According to our findings, although migraine patients were asymptomatic and no arrhythmia was detected in the surface ECG, sympathovagal balance in the sympathetic system, which may be disrupted in favour of the sympathetic system, should continue even in the attack-free period, and we should be careful in terms of serious arrhythmias that may develop in these patients.

The adiponectin levels and asthma control in non-obese children with asthma.

OBJECTIVE: To determine the relationship between adiponectin levels and asthma control in non-obese asthmatic children. METHODS: Eighty-two children with asthma who had been followed up in a single center were included. The control group included 28 children with no evidence of allergic disease. Adiponectin levels were analyzed in all children. Additionally, skin prick tests and pulmonary function tests were also performed in patients. RESULTS: Three groups were designated with respect to asthma control as; well-controlled group (n = 28), partially controlled group (n = 34) and uncontrolled group (n = 20). There was no significant difference of gender, age, height, weight, BMI and adiponectin levels between study and control groups (p > 0.05). The duration of illness, presence of atopy and sensitivities to mite, pollens, dander and cockroaches were similar between the groups (p > 0.05). Adiponectin, FEV1, FVC, and FEV1/FVC levels were significantly lower in uncontrolled group (p < 0.05). Sensitivity to Alternaria alternata was significantly higher in the uncontrolled group (p < 0.05). In logistic regression analysis, as dependent parameter, adiponectin, FEV1, FVC and FEV1/FVC levels were found to be statistically significant for uncontrolled asthma. CONCLUSION: Adiponectin levels in non-obese asthmatics were not different from controls. Lower levels of adiponectin were associated with uncontrolled asthma. Low adiponectin level can therefore be used as an indicator of uncontrolled asthma.

Neurological and Visual Outcomes in Infants and Toddlers Following Therapeutic Hypothermia for Neonatal Hypoxic-Ischemic Encephalopathy.

BACKGROUND: The majority of studies have investigated neurodevelopmental outcomes, whereas visual impairment is less explored in children with a history of neonatal (hypoxic-ischemic) encephalopathy. Our aim was to perform a detailed neurological and visual assessment and also to investigate the presence of cerebral visual impairment in infants and toddlers with neonatal encephalopathy. METHODS: Thirty participants with a history of neonatal encephalopathy, who had been hospitalized for therapeutic hypothermia, underwent a detailed neurological examination at age five to 36 months. Age-matched, 30 healthy children were also enrolled as a control group. All children in the study and control groups received neurological and a comprehensive ophthalmologic examination, including visual field and visual acuity. Presence of cerebral visual impairment was also evaluated clinically. RESULTS: Rates of cerebral palsy, severe motor impairment, cognitive impairment, epilepsy, and cerebral visual impairment were found to be 20%, 10%, 15.3%, 10%, and 20%, respectively. When compared with healthy controls, oculomotor functions, pupillary light response, refractive parameters, anterior/posterior segment examinations, ocular visual impairment rates, and last, visual acuities were found similar. However, we found a statistically significant increase in visual field defects in our study group. CONCLUSIONS: It could be better to perform a comprehensive ophthalmologic examination including visual field, visual acuity, and oculomotor functions by a pediatric ophthalmologist to accurately diagnose neurovisual deficits in infants following therapeutic hypothermia. Early identification and rehabilitation of the visual deficits might improve the neurodevelopment in these children.

Assessment of the changing trends in maternal knowledge about management of fever and antibiotic use in the last decade in Türkiye.

BACKGROUND: The wrong attitudes of parents on fever create a basis for unnecessary drug use and increased workload. The study was conducted to evaluate the knowledge and attitudes concerning fever and antibiotic use and demonstrate the changes in the last decade. METHODS: This cross-sectional study was composed of two parts, and a total of 500 participants were included. Group 1 (the new group, 50.0%) consisted of 250 participants who participated in the study between February 2020 and March 2020 and Group 2 (the old group, 50.0%) consisted of 250 participants who participated in the study between February 2010 and March 2010. All participants share the same ethnic properties and had been visiting the same center for similar reasons. A validated, structured questionnaire assessing the management of fever and antibiotic use was administered to all mothers. RESULTS: According to the fever assessment scoring, maternal knowledge of fever and its management in children significantly increased (p < 0.001). The antibiotic assessment score also increased in 2020 (p = 0.002). CONCLUSIONS: The public spotlight on the erroneous use of antibiotics and the management of febrile illnesses seems to be promising. Improving maternal/parental educational status and informational advertisements can enhance parental knowledge concerning fever and antibiotic use.

A novel de novo TET3 loss-of-function variant in a Turkish boy presenting with neurodevelopmental delay and electrical status epilepticus during slow-wave sleep.

BACKGROUND: Beck-Fahrner syndrome is caused by homozygous or heterozygous mutations in TET3 on chromosome 2p13. The general characteristics of this syndrome include behavioral abnormalities such as autistic features, attention-deficit hyperactivity disorder, learning disabilities, and epilepsy. CASE PRESENTATION: Six years old male patient was found to have a de novo TET3 loss-of-function variant by whole-exome sequencing (WES) analysis and was diagnosed with electrical status epilepticus during slow-wave sleep (ESES) based on clinical and electroencephalogram (EEG) characteristics. The patient had a neurodevelopmental delay from the age of 3 months and started experiencing generalized tonic-clonic seizures and regression at the age of 5 years. EEG findings were consistent with ESES, and WES analysis revealed a novel heterozygous nonsense NM_001366022.1:c.1594C > T (p.Arg532*) variant in TET3. Valproic acid and immunotherapy were administered for the first 6 months, and clobazam was administered orally in addition to oral valproic acid therapy for the next 6 months. Clinical improvement was noted regardless of EEG improvement for the first 6 months. EEG improvement was achieved with clobazam. No regression was observed following the discontinuation of immunotherapy. CONCLUSION: Decreased TET3 enzyme activity may be one of the new genetic etiologies of ESES.

Further clinical and genetic evidence of ASC-1 complex dysfunction in congenital neuromuscular disease.

Transcriptional coregulators modulate the efficiency of transcription factors. Bi-allelic variants in TRIP4 and ASCC1, two genes that encode members of the tetrameric coregulator ASC-1, have recently been associated with congenital bone fractures, hypotonia, and muscular dystrophy in a total of 22 unrelated families. Upon exome sequencing and data repository mining, we identified six new patients with pathogenic homozygous variants in either TRIP4 (n = 4, two novel variants) or ASCC1 (n = 2, one novel variant). The associated clinical findings confirm and extend previous descriptions. Considering all patients reported to date, we provide supporting evidence suggesting that ASCC1-related disease has a more severe phenotype compared to TRIP4-related disorder regarding higher incidence of perinatal bone fractures and shorter survival.

Cockayne syndrome type: a very rare association with hemorrhagic stroke.

BACKGROUND: Cockayne Syndrome (CS) is a rare autosomal recessive disorder that is mainly characterized by neurodevelopmental delay, cutaneous photosensitivity, and cachectic dwarfism. Genetic diagnosis is supported by the typical physical appearance and imaging findings of these patients. CASE: In our case, a 16-year-old female previously diagnosed as CS presented with right-sided hemiparesis. Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) images showed diffuse cerebral and cerebellar atrophies, volume loss of brain stem, calcification of the bilateral basal ganglia, hemorrhage on the posterior limb of the left internal capsule, thalamus, and posterior periventricular area. CONCLUSIONS: Cockayne syndrome is rarely associated with stroke; we report the clinical and neuroradiologic findings of CS presenting with a hemorrhagic stroke.

How often do neurological disorders lead to dizziness in childhood?

OBJECTIVE: The aim of the study was to determine the features and differential diagnosis of childhood dizziness and find out the prevalence of neurological diseases in children who were referred to the pediatric neurology outpatient clinic. A secondary aim was to evaluate the outcome of dizziness after 12 months. MATERIAL AND METHODS: The records of children with a complaint of dizziness that were referred to the pediatric neurology outpatient clinic were retrospectively reviewed, and detailed medical and family history, clinical characteristics, laboratory investigations including vitamin B12 levels, and neuroimaging tests were analyzed. Patients were grouped as neurological disorders and non-neurological disorders. Neurological disorders included vestibular migraine, benign paroxysmal vertigo, and epilepsy, and non-neurological disorders contained the remaining disorders. RESULTS: The study consisted of 60 children (36 females, 24 males) with a mean age of 11.7±4.1 years. The most common diagnoses were vestibular migraine (21.7%) and orthostatic hypotension (20%). We found that the incidence of neurological diseases was 40% (vestibular migraine, 21.7%; epilepsy, 10%; benign paroxysmal vertigo, 8.3%). When we compared the neurological disorders with non-neurological disorders, there was a significant difference in terms of age at onset and duration of attacks (p=0.001 for both), whereas no significant difference was detected in terms of gender, frequency of attacks, and vitamin B12 levels. We detected ongoing symptoms in 10% of the patients who were diagnosed with vestibular migraine and psychogenic dizziness. CONCLUSION: Both non-neurological and neurological diseases are common in etiology of children with dizziness.

Assessment of Nocturnal Hypoventilation by Different Methods and Definitions in Children with Neuromuscular Disease: Oxycapnography and Blood Gas Analysis.

OBJECTIVE: To investigate whether partial arterial carbon dioxide pressure (PaCO 2 ) level in arterial blood gas analysis that was used to predict nocturnal hypoventilation (NH) is concordant with nocturnal end-tidal CO 2 (PetCO 2 ) measurement obtained by a noninvasive method of oxycapnography in children with neuromuscular disease (NMD). METHODS: Twenty-one patients aged 6-18 years with a confirmed diagnosis of NMD were enrolled. Each patient underwent a nocturnal oxycapnography study using an orinasal probe and a pulse oximetry finger probe to record PetCO 2 , oxygen saturation (SpO 2 ), pulse rate, and respiratory rate. Arterial blood gas analysis was performed to record PaCO 2 levels on three occasions at night (23:00 pm, 03:00 am, 07:00 am). RESULTS: The mean overnight PaCO 2 level of the three blood gas analyses (mean PaCO 2 noct) was 41.78±4.69 mmHg. A significant change was observed between mean PaCO 2 23:00 and PaCO 2 07:00 levels (p=0.032). There was no significant difference between PaCO 2 , PetCO 2 , and SpO 2 levels in the NMD group. The interclass correlation coefficient between PaCO 2 07:00 and PetCO 2 levels was 0.791 (95% CI: 0.533-0.923); the interclass correlation coefficient between overnight mean PaCO 2 and PetCO 2 levels was 0.811 (95% CI:0.533-0.923). CONCLUSION: Our study indicates that nocturnal PetCO 2 and PaCO 2 levels were statistically comparable but the use of PaCO 2 alone is not adequate to make an early diagnosis of NH in NMD. There is a need for making more restrictive definitions for NH, and conducting studies with larger study populations to reach an agreement on the best definition of hypoventilation, and updating consensus guidelines.

Effects of tracheostomy on mothers of children with cerebral palsy.

OBJECTIVE: We aimed to investigate psychosocial changes such as burnout, caregiver burden, depression, and coping in mothers of children with cerebral palsy (CP) after tracheostomy placement. DESIGN: A total of 48 children with CP and chronic respiratory insufficiency and their mothers participated in the study. The children with tracheostomy were classified as T (+) (n = 26) and those who did not have tracheostomy were classified as T (-) (n = 22). The patients with tracheostomy through invasive mechanical ventilation were classified as TIV (+). The data about the clinical conditions of the patients and the sociodemographic characteristics of the mothers were recorded. Zarit caregiver burden, Maslach burnout, Beck depression, and Coping Orientation to Problems Experienced (COPE) questionnaires were applied to all mothers. FINDINGS: Beck depression score was 14 ± 8.24 in T (+) group and 16.09 ± 9.65 in T (-) group (p = .576). There was no significant difference between the T (+) and T (-) groups in the Maslach burnout inventory, Zarit caregiver burden scale, and their subgroups. The first two methods from the COPE scales were identical. In the T (+) group, the third coping method was found as "planning" and in the T (-) group as "active coping". Maslach burnout inventory, Zarit 1, and Zarit 2 scores were higher in patients with only tracheostomy compared to those with TIV (p = .002, p = .018, p = .021). CONCLUSION: In the study presented, the placement of tracheostomy in children with CP does not increase mother's depression tendencies and mood changes.

Acute flaccid myelitis outbreak through 2016-2018: A multicenter experience from Turkey.

AIM: We aim to describe the demographic characteristics, etiology, neurophysiology, imaging findings, treatment, prognosis, and prognostic factors of acute flaccid myelitis. METHODS: The clinical data, laboratory test and, magnetic resonance imaging (MRI) results of pediatric patients diagnosed with acute flaccid myelitis according to the Centers for Disease Control criteria between August 1, 2016, and December 31, 2018, from 13 centers in Turkey were reviewed. RESULTS: Of the 34 cases identified, 31 were confirmed (91.2%). Eighteen patients (55.9%) were boys. The median patient age was 4 years (interquartile range 2.5-6.9 years). Most of the patients were admitted in 2018 (n = 27). A preceding history of a febrile illness was reported in all patients, with a median of 4 days (interquartile range 3-7 days) before symptom onset. Thirty-one patients had T2 hyperintensity on spinal MRI, and 18 patients had cerebrospinal fluid pleocytosis. The most common infectious agents were entero/rhinoviruses (n = 5) in respiratory specimens. All patients except one received immunotherapy either alone or in combination. Among 27 patients with follow-up data 24 had persistent weakness. Involvement of four limbs together with an abnormal brain MRI at onset were associated with a poor prognosis. CONCLUSION: The number of patients with acute flaccid myelitis increased since 2012, spiking with every 2-year interval, largely in the pediatric population. The median age decreases with every outbreak. Clinicians should be aware of the clinical picture for early collection of specimens and early start of rehabilitation programs. Further studies are needed to better characterize the etiology, pathogenesis, risk factors, and treatment of this rare condition.

Effects of valproic acid and levetiracetam monotherapy on carotid intima-media and epicardial adipose tissue thickness in non-obese children with epilepsy.

OBJECTIVE: The aim of the study was to investigate the risk of subclinical atherosclerosis independent from obesity and high blood lipid levels in pediatric patients with idiopathic epilepsy receiving valproic acid or levetiracetam monotherapy by evaluating carotid intima-media thickness (CIMT) and Epicardial adipose tissue thickness (EATT). METHODS: A total of 75 patients (38 males, 37 females; mean age 127.2 ±â€¯37.9 months) with epilepsy receiving either valproic acid or levetiracetam monotherapy for more than 12 months (Epilepsy Group) and 75 sex, age, body mass index (BMI) matched healthy children (40 males, 35 females; mean age 133.8 ±â€¯38.7 months) (Control Group) were included in the study. The mean duration of therapy was 27.6 ±â€¯10.5 months. Serum lipid levels (total cholesterol, triglycerides, low density lipoprotein, high density lipoprotein) and CIMT-EATT of the patients and controls were assessed. Also, epilepsy group were divided according to antiepileptic drugs (valproic acid group and levetiracetam group). RESULTS: The CIMT was determined as 0.6 ±â€¯0.08 mm in epilepsy group and 0.49 ±â€¯0.15 mm in control group (p < 0.001). The EATT was measured as 5.96 ±â€¯0.8 mm in epilepsy group and 3.7 ±â€¯0.5 mm in control group (p < 0.001). Of epileptic patients, 45 were using valproic acid monotherapy and 30 were on levetiracetam monotherapy. There was no significant difference in terms of CIMT between valproic acid and levetiracetam groups (0.61 ±â€¯0.09 mm vs. 0.57 ±â€¯0.07 mm; p = 0.07). EATT measurements were significantly higher in valproic acid group compared to levetiracetam group (6.14 ±â€¯0.8 mm vs. 5.7 ±â€¯0.7 mm; p = 0.02). CIMT and EATT values were not associated with the dosage and duration of each antiepileptic drug. CONCLUSION: Non-obese children with epilepsy receiving valproic acid or levetiracetam monotherapy might have an increased risk for developing subclinical atherosclerosis despite normal lipid levels. The effect of valproic acid was more evident especially on EATT.

Prognostic Factors Associated With Recovery in Children With Bell's Palsy.

OBJECTIVES: The aim of the study was to determine the prognosis of children with Bell's palsy and analyze the prognostic factors affecting early recovery. METHODS: The records of children with a diagnosis of Bell's palsy were retrospectively analyzed. Demographic and clinical features including age, gender, House-Brackmann Facial Nerve Grading System House-Brackmann Grading Scale (HBGS) grade at admission and follow-up, and the dosage and onset of steroid treatment were reviewed. Laboratory findings such as red blood cell distribution width and neutrophil-to-lymphocyte ratio were noted. The patients who were recovered within the first month (early recovery) were compared with the patients who were recovered after first month (late recovery) in terms of demographic, clinical characteristics, laboratory findings and treatment modalities in order to determine the risk factors affecting early recovery. RESULTS: A total of 102 children (65 girls and 37 boys) with a mean age of 10.37 ± 4.2 years were included in the study. The complete recovery was detected in 101 children (%99) with Bell's palsy. Statistically significant difference was found in terms of dosage and time of onset of steroid treatment (P = .04, P = .035, respectively) and House-Brackmann Facial Nerve Grading System grade on the 10th day (P = .001) between the early and late recovery groups. CONCLUSION: The prognosis of Bell's palsy in children was very good. The prognostic factors affecting the early recovery were being House-Brackmann Facial Nerve Grading System grade 2 or 3 on the 10th day and receiving steroid treatment in the first 24 hours. Neutrophil-to-lymphocyte ratio and red blood cell distribution width were not found to be predictive factors for early recovery.

Pregabalin Treatment of a Patient With Complex Regional Pain Syndrome.

BACKGROUND: Complex regional pain syndrome (CRPS) is a painful and disabling neurovascular condition. There is no consensus on the etiopathogenesis or the treatment. We present a patient with CRPS type 1 accompanied by a psychiatric disorder to discuss the relationship between CRPS and psychiatric disease and to emphasize the response of this case to treatment with pregabalin. PATIENT DESCRIPTION: A 15-year-old girl presented with swelling, severe pain, edema, hyperesthesia, allodynia, and sweating changes in the left arm and was diagnosed as CRPS type 1. The presence of disturbed family relations was revealed on psychiatric examination, and a diagnosis of major depression was made. Her symptoms did not respond to selective serotonin reuptake inhibitors and noradrenergic and specific serotonergic antidepressives, gabapentin, or stellate ganglion blockage, but the patient's pain resolved with pregabalin. Symptom-oriented measures and psychiatric support enabled ongoing treatment. A social services evaluation led to her being placed in the care of social services to protect her from the chaotic and traumatic family life. CONCLUSION: Detailed psycological and psychiatric evaluation is recommended in individuals with CRPS because psychiatric support and improvement of associated psychosocial concerns in addition to pregabalin seems to facilitate treatments in some patients.

The frequency of late-onset Pompe disease in pediatric patients with limb-girdle muscle weakness and nonspecific hyperCKemia: A multicenter study.

The aim of this multicenter study was to screen for late-onset Pompe disease in high-risk children with limb-girdle muscle weakness and nonspecific hyperCKemia using the dried blood spot (DBS) test. Seventy-two children from four pediatric neurology departments in Turkey were enrolled in the study: 37 with limb-girdle muscle weakness and 35 with nonspecific hyperCKemia. Acid α-glucosidase (GAA) activity was measured on DBS by tandem mass spectrometry. Six patients tested positively for Pompe disease. In three patients, one with the limb-girdle muscle weakness and two with nonspecific hyperCKemia, this was confirmed by genetic analysis. The overall frequency of late-onset Pompe disease in the study population was 4.2%. The c.1784C>T mutation found in one patient is a new mutation whereas the c.1655T>C mutation detected in the other two patients is not novel. In conclusion, Pompe disease should be suspected in patients with limb-girdle muscle weakness and nonspecific hyperCKemia. The DBS test is a safe and reliable method of diagnosis but must be confirmed by genetic analysis. In patients with a positive DBS test and negative genetic analysis, tissue assay of GAA should be considered.

Epilepsy classification and additional definitions in occipital lobe epilepsy.

AIM: To evaluate epileptic children with occipital lobe epilepsy (OLE) in the light of the characteristics of Panayiotopoulos syndrome and late-onset occipital lobe epilepsy of Gastaut (OLE-G). METHODS: Patients were categorized into six groups: primary OLE with autonomic symptoms (Panayiotopoulos syndrome), primary OLE with visual symptoms (OLE-G), secondary OLE with autonomic symptoms (P-type sOLE), secondary OLE with visual symptoms (G-type sOLE), and non-categorized primary OLE and non-categorized secondary OLE according to characteristic ictal symptoms of both Panayiotopoulos syndrome and OLE-G, as well as aetiology (primary or secondary). Patients were compared with regards to seizure symptoms, aetiology, cranial imaging, EEG, treatment and outcome. RESULTS: Of 108 patients with OLE (6.4±3.9 years of age), 60 patients constituted primary groups (32 with Panayiotopoulos syndrome, 11 with OLE-G, and 17 with non-categorized primary OLE); the other 48 patients constituted secondary groups (eight with P-type sOLE, three with G-type sOLE, and 37 with non-categorized sOLE). Epileptiform activity was restricted to the occipital area in half of the patients. Generalized epileptiform activity was observed in three patients, including a patient with Panayiotopoulos syndrome (PS). Only one patient had refractory epilepsy in the primary groups while such patients made up 29% in the secondary groups. CONCLUSION: In OLE, typical autonomic or visual ictal symptoms of Panayiotopoulos syndrome and OLE-G do not necessarily indicate primary (i.e. genetic or idiopathic) aetiology. Moreover, primary OLE may not present with these symptoms. Since there are many patients with OLE who do not exhibit the characteristics of Panayiotopoulos syndrome or OLE-G, additional definitions and terminology appear to be necessary to differentiate between such patients in both clinical practice and studies.