Abdelkarim A. Al-Qudah

Neonatal Guillain-Barré syndrome

A term female infant had the clinical manifestations and accompanying electrophysiologic studies to fulfill the criteria of Guillain-Barré syndrome. At birth, she presented with generalized hypotonia, paucity of lower limb movements, and diminished muscle stretch reflexes. At 3 weeks of age, motor nerve conduction studies demonstrated evidence of demyelination and axonal involvement. Progressive clinical improvement was observed beginning at the age of 2 weeks with subsequent normalization of clinical examinations and nerve conduction studies. To our knowledge, this patient is the youngest reported with Guillain-Barré syndrome.

Etiology of intellectual impairment in Duchenne muscular dystrophy

The precise etiology of intellectual impairment in Duchenne muscular dystrophy (DMD) is unknown. Histopathologic and computed tomographic studies have revealed structural brain changes; however, to our knowledge, no cranial magnetic resonance imaging (MRI) studies have been performed on DMD patients to further delineate these structural changes. We prospectively studied 4 DMD patients by cranial MRI, DNA deletion analysis, clinical evaluation, and intelligence testing. There was no significant correlation between verbal intelligence scores and MRI findings, DNA deletion, or the clinical severity of the disease. These first MRI studies of DMD did not reveal any significant anatomic brain alteration, other than mild atrophy in 2 patients. We believe these results must be considered when investigating the etiology of intellectual impairment in DMD in future studies with larger patient samples.

Value of brain CT scan in children with febrile convulsions

Studies specifically dealing with the value of brain CT scans in different types of febrile convulsion are lacking when compared to the large amount of studies on other favorable convulsive disorders. This study is correlation study between children who presented with febrile convulsions (n = 38) and the results of their brain CT scans. Twenty four patients had risk factors for subsequent epilepsy (group A), 3 of them had abnormal CT scans in the form of mild diffuse brain atrophy, and all 3 patients were found to have prior neurodevelopmental deficits. The other 14 patients did not have such risk factors (group B) and had normal brain CT scans. Despite the small size of the study, the results are unequivocal, and it can be concluded that brain CT scan is not indicated in the management of simple or complex febrile convulsions. Brain CT may be justifiable, but may not be clinically useful in the management of febrile convulsion patients with prior neurodevelopmental deficits.

Immunoglobulins in the treatment of Guillain-Barré syndrome in early childhood.

This is a prospective study on the use of immunoglobulins in the treatment of Guillain-Barré syndrome in four children, three of whom were younger than 3 years of age. All of them were unable to walk and were still deteriorating when the treatment was started. Three patients started recovering within 24 hours, and all the patients were fully mobile within 6 weeks after receiving first dose of immunoglobulins. This report shows the efficacy of intravenous immunoglobulins in the treatment of young children presenting with acute Guillain-Barré syndrome. (J Child Neurol 1994;9:178-180).

Etiologies, outcomes, and risk factors for epilepsy in infants: A case–control study

OBJECTIVES To determine the etiologies, outcomes, and risk factors for epilepsy in infants. PATIENTS AND METHODS This retrospective study included all children who had their first afebrile seizure between 1 and 12 months of age, and who were followed in the Child Neurology Clinic at the Jordan University Hospital from January 2004 to January 2006. Medical records were reviewed to collect demographic data and the clinical data pertaining to epilepsy. An age-matched control group of healthy children in a 1:2 ratio was included to determine the risk factors for epilepsy. For statistical analysis, SPSS, Version 13, was used. RESULTS Fifty-five patients were included in the study group and 111 were in the control group. Epilepsy was classified as follows: symptomatic in 24 (43.6%) children, probably symptomatic (cryptogenic) in 28 (50.9%) children, and idiopathic in 3 (5.5%) children. The etiologies of epilepsy in the symptomatic group included hypoxic-ischemic encephalopathy (n=11), cortical malformations (n=5), neurocutaneous syndromes (n=2), metabolic disorders (n=4), leukodystrophy (n=1), and craniosynostosis (n=1). Twenty-seven patients (49%) were seizure-free at their last follow-up visit for at least the last 6 months; only six patients (10.9%) continued to have normal development at the time of their last follow-up examination. The risk factors for epilepsy included parental consanguinity (P=0.0003), a family history of global developmental delay (P=0.0002), a family history of epilepsy (P=0.010), and a positive perinatal history (P=0.011). CONCLUSION This study emphasized that afebrile convulsions in infancy are rarely benign. Furthermore, consanguinity was shown to be a major risk factor for epilepsy.

Diagnostic value of short duration outpatient video electroencephalographic monitoring

There is little published on the diagnostic value of short duration outpatient video electroencephalographic (VEEG) monitoring in children. The authors performed a prospective study on 37 patients (mean age = 10.4 years), with daily paroxysmal events who underwent short duration (mean = 3.2 hours) outpatient VEEG monitoring. Events were detected in 23 patients (62.2%), and a change in management as a result of outpatient VEEG monitoring was documented in 25 patients (67.6%). Despite the short duration of the outpatient VEEG in this study, the detection rate was comparable with the previously reported studies with longer duration monitoring. The authors found it convenient for the patient and less costly. The study demonstrated that short duration outpatient VEEG monitoring was able to differentiate between seizures and nonseizures in 11 patients (78.6%) and resulted in changing seizure classification in five patients (62.5%), and in selecting epilepsy surgery candidates in nine patients (60%). Short duration outpatient VEEG is useful as a diagnostic tool in patients with daily paroxysmal events, particularly in identifying nonepileptic events.

CONGENITAL MUSCULAR DYSTROPHY IN JORDANIAN CHILDREN

This is a consecutive study on 28 patients who have been diagnosed as having congenital muscular dystrophy at Jordan University Hospital in the period from January 1990 to February 1997. Of 75 patients diagnosed as having muscle disease, 55 (73.3%) had muscular dystrophy. Of 55 muscular dystrophy patients, 28 (50.9%) had congenital muscular dystrophy, 11 (20%) had Duchenne muscular dystrophy, 9 (16.4%) had Becker muscular dystrophy, 4 (7.3%) had myotonic dystrophy, 2 (3.6%) had limb-girdle dystrophy, and 1 (1.8%) patient had facioscapulohumeral dystrophy. Age of onset of symptoms of congenital muscular dystrophy (hypotonia and weakness) was documented antenatally or in the first few months in the majority (92.9%) of patients. Parental consanguinity was documented in 21 (75%) of congenital muscular dystrophy cases, and family history of possible similar cases in 15 (53.6%). Congenital muscular dystrophy patients with normal cognitive milestones (n = 16; 57.1%) were slightly more common than patients with cognitive delay. In contrast to previous reports, congenital muscular dystrophy is probably more common in communities with high rates of parental consanguinity than other dystrophies. Our study adds significant support to the most recent literature on this finding. (J Child Neurol 1998;13:383-386).

Screening for congenital hypothyroidism in cognitively delayed children

During the period January 1994 to December 1997, a total of 560 cognitively delayed children were prospectively screened for congenital hypothyroidism in the paediatric neurology clinic at Jordan University Hospital. Seventeen (3%) children were diagnosed to have congenital hypothyroidism. Mean age at diagnosis was 15 months (range 1.8-72 months). Main presenting symptoms (other than cognitive delay) were seizures, lethargy, poor feeding, constipation and prolonged neonatal jaundice. Thirteen (76.5%) of the 17 extrathyroid-associated anomalies involved the central nervous system (CNS). This finding reflects the study population chosen for the present study. All 17 patients had some degree of cognitive improvement ranging from slight improvement to achieving normal milestones after taking L-thyroxine. Congenital hypothyroidism is common in cognitively delayed children. The presence of major abnormality of the CNS should not be regarded as the only possible cause of cognitive delay and thyroid function should be assessed in such children.

Patterns of antiepileptic drugs use in epileptic pediatric patients in Jordan.

Objectives: To determine prescribing patterns of antiepileptic drugs (AEDs) in pediatric patients with confirmed diagnosis of epilepsy, and to provide knowledge of general practice of physicians. Methods: The study was a multi-center cross-sectional observational study, in specialized clinics for management of epilepsy in north, central and south Jordan. This study was conducted from January 2014 to July 2014. These were 3 from university tertiary care hospitals and 4 from governmental tertiary care hospitals. Results: A total of 694 pediatric patients were included. Monotherapy AED use had the highest frequency 465 (67.0%), followed by dual therapy 162 (23.3%). The frequency of monotherapy in university hospitals was lower than governmental hospitals (p<0.05); however, Polytherapy was more frequent in younger children. Two old AEDs were most frequently prescribed as a monotherapy; Valproic acid 235 (50.5%) and carbamazepine 155 (33.3%). The most common combination in dual therapy was valproic acid with carbamazepine 28 (17.3%). The second most common combinations were carbamazepine with levetiracetam 21 (13.0%) or valproic acid with levetiracetam 20 (12.3%). Conclusion: Older AED remain first line drugs for use in both monotherapy and combination therapy for epileptic disorders. Polytherapy is associated with younger kids and being treated in a university hospital.

Clinical Patterns of Neuronal Migrational Disorders and Parental Consanguinity

The role of inheritance in neuronal migrational disorders is under intense investigation. Studies on neuronal migrational disorders (NMDs) from developing countries that have a high rate of parental consanguinity are lacking. The present study included 29 children (aged 15 days-12 years, mean age 1.4 years) who were diagnosed to have NMDs, from a non-selected population with seizures and non-selected population of cognitive developmental delay, in the period January 1994 to April 1997. Seventeen (58.6 per cent) patients had lissencephaly, four (13.8 per cent) patients had pachygyria, three (10.3 per cent) patients had neuronal heterotopia, four (13.8 per cent) patients had schizencephaly, one patient (3.4 per cent) had hemimegalencephaly, and 14 (48.2 per cent) patients with NMDs had other associated conditions. Lissencephalic patients had a high rate of parental consanguinity (88.2 per cent) and family history of possible similar cases (76.4 per cent). In conclusion, lissencephaly is probably the commonest neuronal migrational disorder in communities with a high rate of parental consanguinity, adding significant support to the literature on the genetic aetiology of lissencephaly.