A new approach for assessing executive functions in everyday life, among adolescents with Genetic Generalised Epilepsies.

Studies have characterised relationships between cognitive status and a variety of clinical epilepsy factors. The aim of this study was to describe a new approach for assessing executive functions in everyday life and its unique expression in adolescents with Genetic Generalised Epilepsies (GGEs) compared with typical peers. Twenty adolescents with a diagnosis of GGEs and 20 typical healthy peers, matched by age and gender, were studied. Assessment of everyday executive function was carried out using: (1) the Weekly Calendar Planning Activity (WCPA), a direct performance based and outcome measure of strategy use and cognitive performance; and (2) Behavior Rating Inventory of Executive Function (BRIEF) parental report. Adolescents with GGEs demonstrated significantly less accuracy, less efficiency and fewer strategies used, as measured by the WCPA. Parents of adolescents with GGEs rated their child's daily performance as less efficient compared with typical peers. Better ratings of executive function (low BRIEF score) were associated with greater WCPA accuracy in the entered appointments. The WCPA provides a useful evaluation of cognitive performance for adolescents with GGEs and a functionally relevant information on task efficiency, self-monitoring and effective strategy use. Direct observation of performance supplements parental ratings and has strong potential to guide intervention and measure outcomes.

Childhood-onset primary generalized epilepsy--impacts on children's preferences for participation in out-of-school activities.

The purpose of this study was to compare preferences for participation in out-of-school activities between children with childhood-onset primary generalized epilepsy and their healthy peers. Overall, participants were 56 children aged 6-11 years. The study group included 26 children with childhood-onset primary generalized epilepsy. The controls were 30 healthy children. Parents of all participants completed a demographic and health status questionnaire. All children completed the Preference Assessment of Children (PAC) that profiles the out-of-school activities the child wishes to participate in. Scores are calculated for five activity types, namely, recreational, active physical, social, skill-based, and self-improvement and for two domains of formal and informal activities. Children with generalized epilepsy showed a similar preference for participation in out-of-school activities as did their healthy peers. The study group showed a lower preference for participation in social activities but showed a higher preference for participation in self-improvement activities. In both groups, younger children (aged 6-8 years) showed a lower preference for participation in most PAC scales. Older children (aged 9-11 years) showed a higher preference for participation in social activities. Difference between genders was close to being statistically significant in the skill-based activities (F(1,21)=3.84, p=.06), where girls showed a higher preference compared with boys. Intervention policies need to be undertaken in order to encourage children with epilepsy to participate in activities together with their healthy peers, aiming to enhance the well-being of children with primary generalized epilepsy.

Obesity in children with headaches: association with headache type, frequency, and disability.

OBJECTIVE: To examine the association between obesity and the different types of primary headaches, and the relation to headache frequency and disability BACKGROUND: The association between obesity and headache has been well established in adults, but only a few studies have examined this association in children, in particular, the relationship between obesity and different types of primary headaches. METHODS: The authors retrospectively evaluated 181 children evaluated for headaches as their primary complaint between 2006 and 2007 in their Pediatric Neurology Clinic. Data regarding age, gender, headache type, frequency, and disability, along with height and weight were collected. Body mass index was calculated, and percentiles were determined for age and sex. Headache type and features were compared among normal weight, at risk for overweight, and overweight children. RESULTS: A higher prevalence (39.8%) of obesity was found in our study group compared with the general population. The diagnosis of migraine, but not of tension-type headache, was significantly associated with being at risk for overweight (odds ratio [OR] = 2.37, 95% confidence interval 1.21-4.67, P = .01) or overweight (OR = 2.29, 95% confidence interval 0.95-5.56, P = .04). A significant independent risk for overweight was present in females with migraine (OR = 4.93, 1.46-8.61, P = .006). Regardless of headache type, a high body mass index percentile was associated with increased headache frequency and disability, but not with duration of attack. CONCLUSIONS: Obesity and primary headaches in children are associated. Although obesity seems to be a risk factor for migraine more than for tension-type headache, it is associated with increased headache frequency and disability regardless of headache type.

Primary headaches, attention deficit disorder and learning disabilities in children and adolescents.

BACKGROUND: Primary headaches and Learning difficulties are both common in the pediatric population. The goal of our study was to assess the prevalence of learning disabilities and attention deficit disorder in children and adolescents with migraine and tension type headaches. METHODS: Retrospective review of medical records of children and adolescents who presented with headache to the outpatient pediatric neurology clinics of Bnai-Zion Medical Center and Meyer Children's Hospital, Haifa, during the years 2009-2010. Demographics, Headache type, attention deficit disorder (ADHD), learning disabilities and academic achievements were assessed. RESULTS: 243 patients met the inclusion criteria and were assessed: 135 (55.6%) females and 108 (44.4%) males. 44% were diagnosed with migraine (35.8% of the males, 64.2% of the females, p = 0.04), 47.7% were diagnosed with tension type headache (50.4% of the males, 49.6% of the females). Among patients presenting with headache for the first time, 24% were formerly diagnosed with learning disabilities and 28% were diagnosed with attention deficit disorder (ADHD). ADHD was more prevalent among patients with tension type headache when compared with patients with migraine (36.5% vs. 19.8%, p = 0.006). Poor to average school academic performance was more prevalent among children with tension type headache, whereas good to excellent academic performance was more prevalent among those with migraine. CONCLUSIONS: Learning disabilities and ADHD are more common in children and adolescents who are referred for neurological assessment due to primary headaches than is described in the general pediatric population. There is an association between headache diagnosis and school achievements.

Mitochondrial hsp60 chaperonopathy causes an autosomal-recessive neurodegenerative disorder linked to brain hypomyelination and leukodystrophy.

Hypomyelinating leukodystrophies (HMLs) are disorders involving aberrant myelin formation. The prototype of primary HMLs is the X-linked Pelizaeus-Merzbacher disease (PMD) caused by mutations in PLP1. Recently, homozygous mutations in GJA12 encoding connexin 47 were found in patients with autosomal-recessive Pelizaeus-Merzbacher-like disease (PMLD). However, many patients of both genders with PMLD carry neither PLP1 nor GJA12 mutations. We report a consanguineous Israeli Bedouin kindred with clinical and radiological findings compatible with PMLD, in which linkage to PLP1 and GJA12 was excluded. Using homozygosity mapping and mutation analysis, we have identified a homozygous missense mutation (D29G) not previously described in HSPD1, encoding the mitochondrial heat-shock protein 60 (Hsp60) in all affected individuals. The D29G mutation completely segregates with the disease-associated phenotype. The pathogenic effect of D29G on Hsp60-chaperonin activity was verified by an in vivo E. coli complementation assay, which demonstrated compromised ability of the D29G-Hsp60 mutant protein to support E. coli survival, especially at high temperatures. The disorder, which we have termed MitCHAP-60 disease, can be distinguished from spastic paraplegia 13 (SPG13), another Hsp60-associated autosomal-dominant neurodegenerative disorder, by its autosomal-recessive inheritance pattern, as well as by its early-onset, profound cerebral involvement and lethality. Our findings suggest that Hsp60 defects can cause neurodegenerative pathologies of varying severity, not previously suspected on the basis of the SPG13 phenotype. These findings should help to clarify the important role of Hsp60 in myelinogenesis and neurodegeneration.

[Acute hemiplegia and hemianesthesia together with decreased tendon reflexes mimicking acute stroke representing a conversion disorder].

Acute hemiplegia and hemianesthesia is commonly caused by obstruction of major cortical arteries. Such a presentation secondary to a conversion reaction is very rare, especially in the pediatric age group. The authors report an adolescent presenting with acute complete left-sided hemiplegia and sensory loss together with decreased tendon reflexes mimicking an acute arterial stroke. Examination revealed Hoover's sign was present and the patient was oblivious to his stern neurological state. Movement of his paralytic limbs was observed during sleep. Cortical and spinal CT, cortical MRI, motor and somatosensory evoked potentials and a PET study were all normal. As such, the diagnosis of psychogenic hemiplegia was established, apparently within a period that the patient had experienced severe emotional stress while questioning his gender identity. After three days, the adolescent began to move the paralytic limbs along gradual resolution of sensory deficit, leading to complete clinical recovering within two months. Although extremely rare, a conversion reaction should be taken into account in children presenting with acute hemiplegia and anaesthesia, even accompanied with decreased tendon reflexes, when the patient is oblivious to his alleged grave state, and when clinical observations such as Hoover's sign remain intact, substantiated by normal extensive radiological and neurophysiological investigation. Intact motor evoked potentials serve as a key for the diagnosis of psychogenic hemiplegia and, should therefore be performed in suspected cases.

[Identical, for better and for worse].

We report on identical premature twins (monochorionic diamniotic). As fetuses, they both demonstrated sonographic and MRI evidence of cerebral bilateral ventriculomegaly. Neonatal brain US showed bilateral ventriculomegaly, similar in both twins. During follow-up, these physical and imaging similarities persisted. To the best of our knowledge, this is the first report on similar bilateral ventriculomegaly in Identical Twins (IT). A genetic origin of this finding in our IT is suggested. Should more cases of ventriculomegaly be reported in the future in several sets of IT, cerebral ventriculomegaly can then be added to the list of similar CNS features observed in identical twins.

Intractable gelastic seizures during infancy: ictal positron emission tomography (PET) demonstrating epileptiform activity within the hypothalamic hamartoma.

Gelastic seizures comprise a very rare form of epilepsy. They present with recurrent bursts of laughter voices without mirth and are most commonly associated with the evolution of a hypothalamic hamartoma. The purpose of this article is to describe the second reported ictal fluorodeoxyglucose-positron emission tomography study in a unique case of an infant with intractable gelastic seizures since the neonatal period associated with a hypothalamic hamartoma. The patient presented at 4 months old with recurrent, almost persistent, gelastic seizures consisting of laughter bouts without mirth. The seizures were noticeable at the first week of life and increased in frequency to last up to 12 hours, namely status gelasticus. These gelastic fits were accompanied with focal motor seizures, including unilateral right-eye blinking and mouth twitching. Developmental mile-stones were intact for age. Magnetic resonance imaging of the cortex demonstrated a large hypothalamic hamartoma within the third ventricle, hampering cerebrovascular fluid drainage of the lateral ventricles. An electroencephalography was nondiagnostic. Ictal fluorodeoxyglucose-positron emission tomography demonstrated a large circumscribed hypermetabolic region within the location of the hypothalamic hamartoma, representing localized intense epileptiform activity. The infant became instantly free of all seizure types given minute doses of oral benzodiazepine (clonazepam) and remains completely controlled after 12 months. Her overall development remains intact. This ictal fluorodeoxyglucose-positron emission tomography is the second reported study verifying that the main source of the epileptic activity inducing gelastic seizures originates from the hypothalamic hamartoma itself; therefore, a complementary fluorodeoxyglucose-positron emission tomography study should be considered in any patient presenting with intractable gelastic seizures, especially in those associated with hypothalamic hamartoma, in order to localize the region of epileptiform activity amenable to surgical resection if intensive drug therapy fails.

Vigabatrin, lamotrigine, topiramate and serum carnitine levels.

Clinical studies indicate a decrease in free and total carnitine in children treated with old-generation antiepileptic drugs (especially valproate). Here, we studied the effect of new-generation antiepileptic drugs on serum carnitine levels. Serum carnitine levels were measured in 91 children: 24 treated with vigabatrin, 28 treated with lamotrigine, and 21 treated with topiramate. These drugs were given as monotherapy (54 children) or polytherapy (19 children). Eighteen additional children treated with valproate served as control subjects. Reduced mean serum carnitine level was evident only in children treated with valproate, with mean free and total carnitine level of 26.9 +/- 8.6 micromol/L and 29.1 +/- 10.4 micromol/L, respectively. In contrast, the mean serum carnitine levels of children treated with vigabatrin, lamotrigine, or topiramate were similar and normal. In these children, the free carnitine levels were 38.5 +/- 7.8 micromol/L, 37.2 +/- 7.7 microg/mL, and 40.4 +/- 8.7 micromol/L, respectively, and total carnitine levels were 43.5 +/- 8.8 micromol/L, 44.4 +/- 9.2 micromol/L, and 45.5 +/- 9.8 micromol/L (+/-S.D.), respectively. Only 4 children (treated with valproate) exhibited considerably lower serum carnitine levels. None of these children had significant clinical adverse effects attributable to carnitine deficiency. In conclusion, these new-generation antiepileptic drugs probably do not cause carnitine deficiency. In contrast, valproate may induce carnitine deficiency, but most cases are asymptomatic.

Acute Q fever in children presenting with encephalitis.

We describe the cases of three children with encephalitis associated with Coxiella burnetii infection. Neurologic features ranged from status epilepticus and coma to headache and pseudotumor cerebri syndrome. Patients had good response to antibiotic treatment with doxycycline and recovered fully. Q fever should be included in the differential diagnosis of children with encephalitis, and routine serological testing should be considered, especially in endemic areas.

Childhood epilepsy with occipital paroxysms: variations on the theme.

This review reports on the various presentations as well as new revealed data relating to the epileptic disorder defined as childhood epilepsy with occipital paroxysms characterized by occipital epileptic discharges recorded by electroencephalography. The initial rigidly delineated subtypes by the International League Against Epilepsy included the following: (a) Panayiotopoulos syndrome-early-onset seizure disorder presenting with prolonged infrequent, nocturnal autonomic seizures, accompanied by eye deviation and ictal vomiting. (b) Gastaut syndrome-late-onset type presenting with short diurnal frequent seizures and visual ictal manifestations along with migrainous headaches. A high percentage of children present, however, with mixed clinical phenomena making it difficult to comply with the rigidly segregated syndromes reported by several authorities including our recent report on 28 children of whom 14 showed mixed phenomena. Therefore, childhood epilepsy with occipital paroxysm should be suspected at any age presenting with nocturnal vomiting, autonomic impairment, focal motor fits with head deviation, migraine headaches, and/or visual perturbations.

Adolescence peroneal neuropathy associated with rapid marked weight reduction: case report and literature review.

PURPOSE: We report herein an uncommon presentation of peroneal nerve palsy and possible myelopathy in an adolescent associated with marked uncontrolled weight loss during a relatively short period. CASE PRESENTATION: He presented with left drop foot accompanied with sensory impairment at the foot dorsum. Neurophysiologic studies revealed a severe neuropathy of the left peroneal nerve with evidence of a conduction block at the left fibular head suggestive of entrapment neuropathy. ETIOLOGY: As for the underlying etiology of acute peroneal neuropathy (PN) and possible myelopathy associated with marked weight loss, it has been suggested that rapid loss of the subcutaneous tissue may lead to entrapment of the peroneal nerve at the fibular head leading to foot drop. Body fat percentage was found remarkably low for age at 10% (normal value for age for males: 22.3%) indicative of massive rapid weight reduction markedly depleting fat stores. CONCLUSION: When an adolescent presents with acute foot drop, PN associated with rapid uncontrolled weight reduction should be taken into account. Prompt resumption of a balanced diet including vital nutritional ingredients may avoid permanent neurological damage and probably assist in recovery from severe flaccid weakness of the lower extremity.

Typical absence epilepsy presenting prior to age of 3 years: an uncommon form of idiopathic generalized epilepsy.

PURPOSE: An attempt to allocate patients with the clinical features and electroencephalography (EEG) abnormalities of typical absence epilepsy presenting before the age of 3 years, similar to childhood and juvenile absence epilepsy (JAE) and delineate the clinical manifestations, EEG abnormalities, therapy and outcome of such an epileptic disorder by conducting a nationwide survey. RESULTS: Overall, eight infants, six males and two females, abided by the inclusion criteria of typical absence epilepsy: They were born after an unremarkable pregnancy and labor presenting at the age of 12-34 months (mean: 19.6 months) with frequent absences time-linked with an EEG demonstrating generalized occasionally irregular epileptiform discharges of 3-4 Hz spike/wave and normal background activity along with an electrographic photosensitive response in one patient. Neurological examination was intact in all infants. All eight infants were initially treated with valproic acid, of whom seven immediately responded and one had increase in frequency and duration of absences completely aborted with treatment of lamotrigine. Three relapsed after termination of therapy of whom two again presented with recurrent absences and another one with generalized tonic-clonic seizures and as such these children had virtually transformed into a later form of idiopathic generalized epilepsy (IGE) during childhood. All eight patients are seizure-free, seven still on therapy; seven children within a follow-up period of 2-7 years and the most recently diagnosed infant for 6 months. Cognitive skills were found normal in all children within the low normal range in three children with short attention and concentration spans. CONCLUSION: The data presented here delineate a very rare form of idiopathic benign generalized epilepsy presenting with typical absences before age of 3 years and a favorable outcome, similar to childhood and JAE, recognized as distinct IGE syndromes by the International League Against Epilepsy (ILAE) classification.

Childhood epilepsy with occipital paroxysms: difficulties in distinct segregation into either the early-onset or late-onset epilepsy subtypes.

The Commission on Classification and Terminology of the International League Against Epilepsy Childhood rigidly segregated epilepsy with occipital paroxysms into 2 separate syndromes with different predominant seizure types: early-onset seizure susceptibility type consisting of prolonged infrequent, nocturnal autonomic seizures and accompanied by eye deviation and ictal vomiting and late onset with short diurnal frequent seizures and visual ictal manifestations along with throbbing headaches. Epileptic clinical manifestations and electroencephalographic data were analyzed in 28 patients with suspected occipital lobe epilepsy in an attempt to segregate them into either the early or late forms according to the International League Against Epilepsy classification. Electroencephalography in 25 children demonstrated occipital epileptiform paroxysms compatible with the suspected epileptic syndrome. Only 14 (50%) children complied with the rigid criteria of either early-onset or late-onset presentations. The other 14 (50%) children presented with mixed diverse epileptic phenomena such as short-lived seizures in infancy or prolonged seizures during childhood, not complying with either rigid syndrome (ie, short-lived epileptic blindness at an early age or vomiting during later childhood). Despite present attempts to rigidly segregate childhood epilepsy with occipital paroxysms into 2 distinct epileptic syndromes, a high percentage of children still present with various mixed clinical phenomena. Therefore, clinicians should be aware of possible unique and unusual presentations of occipital lobe epilepsy at various ages.

Congenital myopathies in Israeli families.

The clinical features of 37 patients from 32 Israeli families with congenital myopathies evaluated between 1983 and 2004 are described: 13 children were diagnosed with congenital fiber type disproportion, 10 had myotubular myopathy, 7 had nemaline myopathy, 5 had central core disease, 1 had actin myopathy, and 1 had multi-minicore disease. There were 7 families (22%) that had parental consanguinity, and 4 families (12%) had more than 1 patient with congenital myopathy. Of the patients, 31 (84%) presented with clinical symptoms before 4 months of age, and 6 children (16%) presented after 1 year of age. Thirteen children (35%) had a severe phenotype with chronic ventilatory dependence or mortality before the age of 11 years. Facial weakness was associated with a severe phenotype. There was a high rate of a severe clinical phenotype in patients with myotubular myopathy (60%) and in patients with nemaline myopathy (57%), whereas in patients with congenital fiber type disproportion and in patients with central core disease, the proportion of a severe phenotype was lower (23% and 0%, respectively).

Effect of high-dose methyl-prednisolone on brainstem encephalopathy and basal ganglia impairment complicating cat scratch disease.

Cat scratch disease (CSD) is a zoonotic illness caused by the Gram negative bacillus Bartonella henselae characterized by a small skin lesion at the site of a bite, lick or scratch by a cat, commonly followed by regional lymphadenopathy 1 or 2 weeks later. We report herein on severe neurological complications of CSD combining brainstem encephalopathy and basal ganglia impairment. This 12-year-old female acutely presented to a local hospital with profound coma and a prolonged tonic posturing of extremities. On the neurological examination she was deeply comatose with pin-point pupils and lack of vestibulo-ocular responses, suggestive of brainstem encephalopathy, along with marked rigid hypertonicity suggestive also of basal ganglia impairment. Initially suspecting Herpes simplex encephalitis or acute disseminated encephalomyelitis she was promptly started with high-dose methyl-prednisolone and acyclovir. Her parents apparently reported that she was scratched by a kitten some 4 weeks prior to her present admission and as such, suspecting CSD, she was begun with doxycycline and rifampicin. Her serology had proven positive for IgM antibodies to Bartonella henselae establishing the diagnosis. She regained consciousness after 4 days and the signs of brainstem and extra-pyramidal impairment also gradually abated and disappeared after 10 days. A follow-up exam after a month disclosed mild extra-pyramidal abnormalities which disappeared after 3 months. Although extremely rare, CSD should be also considered in a patient presenting with a severe encephalopathy and associated basal ganglia impairment. The prompt administration of high-dose methyl-prednisolone upon admission may have contributed to the favorable outcome in our patient and therefore should be advocated in any patient presenting with profound encephalopathy regardless the underlying etiology recovered later.

Childhood-onset idiopathic intracranial hypertension: relation of sex and obesity.

The purpose of the present study was to perform a meta-analysis of all children with idiopathic intracranial hypertension reported since 1997 combined with our experience in order to investigate sex distribution and frequency of obesity among young children up 11 years of age vs adolescents at age 12-17 years. Overall, 244 children diagnosed with idiopathic intracranial hypertension were found suitable for the proposed meta-analysis: 132 (54%) were younger than 11 years of age; 72 (55%) were male and 60 (44%) were female. In contrast, of 112 older children (age 12-17 years), 79 (70%) were female. The association between age and obesity could be analyzed in 147 patients: only 19 (26%) out of 74 younger children up to age 11 years were reportedly obese, whereas 47 (64%) out of 73 older children were found obese. Differences in age at presentation, sex, and obesity were statistically significant (P < 0.01). Thus, a wide-scale meta-analysis of childhood-onset idiopathic intracranial hypertension revealed that the female/male ratio in children younger than age 11 years seems to be fairly equal, with a relatively low rate of obesity, contrasting to a majority of females in the group of adolescents at high risk to become obese.

Pediatric-onset gelastic seizures: clinical data and outcome.

Gelastic seizures are an extremely rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. The objective was to survey all Israeli children found to develop recurrent gelastic seizures and report presenting symptoms, electroencephalographic and radiologic data, and response to either antiepileptic drugs or surgery. Ten children who developed gelastic seizures at the age of 1 week to 6.5 years (mean, 25 months) at a frequency from 3 bouts per week to >10 prolonged bouts per day were followed for a period of 1.3-12 years (mean, 6 years). Seven cases were defined as symptomatic: cortical magnetic resonance imaging revealed a hypothalamic hamartoma in four patients and cortical abnormalities in three others. Seizure control was achieved in four patients, including a neonate with status gelasticus and hypothalamic hamartoma, and partial control in one more. Five children remained resistant to polytherapy, including three with hypothalamic hamartoma even after two of them underwent hemartoma excision. Thus, children with gelastic seizures may respond relatively well to drug therapy. Four of the 10 patients became seizure free with drug therapy; in three intractable symptomatic cases, surgery was tried but failed in two of the three.

Tumor necrosis factor blockade in the management of children with orphan diseases.

Tumor necrosis factor (TNF) blockade has been used successfully to treat a number of rheumatic disorders that have a substantial burden of illness. In children, the TNF antagonists are used mainly for the treatment of juvenile idiopathic arthritis (JIA). There are, however, a variety of rare systemic inflammatory diseases, in which TNF blockade appears promising. Preliminary data in adults suggest that several forms of vasculitis appear to be responsive to TNF antagonists-Behcet's disease, polyarteritis nodosa, Wegener granulomatosis, among others. Some of them respond better to infliximab, a chimeric monoclonal anti-TNF antibody, than to etanercept, a recombinant p75 TNF receptor. We describe our limited experience with infliximab in the treatment of three children with rare vasculitic conditions.

Current therapeutic options in severe Guillain-Barré syndrome.

The classical form of Guillain-Barré syndrome (GBS) refers to an acute monophasic demyelinating motor and sensory polyneuropathy characterized by symmetric ascending flaccid weakness, along with sensory impairment and, less commonly, autonomic perturbations. Pure motor axonal forms, axonal motor, and sensory forms, as well as pure autonomic forms, have also been identified. A complex immune-mediated process leads to segmental demyelination accompanied with axonal involvement in protracted cases. Establishing strategies of immunomodulation may therefore halt and even reverse the harmful autoimmune insult to peripheral nerves. The present article reviews the current immunomodulatory options in severe GBS. A recent Cochrane meta-analysis of 6 randomized studies showed no significant improvement using corticosteroids, including either oral or intravenous methylprednisolone. Combined methylprednisolone and immunoglobulins shortened the time lapse to regain independent walking. Plasmapheresis (PE) was the first effectively proven method of immunomodulation, followed by intravenous immunoglobulins (IVIG). Both methods are comparable in their beneficial effect and were used either separately or in combination, but PE was more frequently associated with severe adverse effects requiring cessation of therapy, including a bleeding diathesis. In addition, PE is feasible only in major referral centers requiring the appropriate equipment and trained personnel. In addition, younger children may be at risk for bleeding after insertion of wide catheters. Therefore, in cases of severe GBS, IVIG is recommended as the first-line drug using a total empiric dose of 2 g/kg administered over 2 consecutive days, especially in children proven highly effective with negligible adverse effects. In protracted cases, the addition of intravenous corticosteroids to IVIG should be considered, which may shorten the duration to regain independent walking. If such therapy fails, PE should be applied using centrifugal blood separators with 5% albumin as the substitute solution.