Clinical features of sleep-disordered breathing in children with neuromuscular disease / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features of sleep-disordered breathing (SDB) in children with neuromuscular disease (NMD).@*METHODS@#A retrospective analysis was performed on the medical data of 18 children who were diagnosed with NMD and underwent polysomnography (PSG) (NMD group). Eleven children without NMD who had abnormal sleeping habit and normal sleep structure on PSG were enrolled as the control group. The two groups were compared in terms of the daily and nocturnal symptoms of SDB, incidence rate of obstructive sleep apnea (OSA), pulmonary function, end-tidal partial pressure of carbon dioxide (PetCO@*RESULTS@#In the NMD group, 16 children (89%) had related daily and nocturnal symptoms of SDB, and the youngest age was 1 year at the onset of such symptoms. Compared with the control group, the NMD group had significant reductions in total sleep time and sleep efficiency (@*CONCLUSIONS@#There is a high proportion of children with SDB among the children with NMD, and SDB can be observed in the early stage of NMD, which results in the damage of sleep structure and the reduction in sleep efficiency. Respiratory events are mainly obstructive events, and oxygen reduction events are mainly observed during REM sleep.

Benign infantile convulsions with mild gastroenteritis: clinical analysis of 40 cases / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the pathogenesis, clinical characteristics and treatment of benign infantile convulsions with mild gastroenteritis (BICG).</p><p><b>METHODS</b>The clinical manifestations and laboratory findings were observed in 40 children with BICG. The antigen and antibodies of rotavirus and calicivirus in stool and cerebral spinal fluid (CSF) were tested by the golden standard method and ELISA. The neurological outcome was evaluated by a follow-up of six months or more.</p><p><b>RESULTS</b>All of the 40 children had mild gastroenteritis with or without minor dehydration. Cluster convulsions were observed in these children. There were normal findings in blood biochemistry (Na+, K+, Ca2+, Cl-, HCO3-, glucose) and cerebral CT or MRI examinations. The interictal EEG showed sprinkle central or frontal epileptiform discharges in 8 children; clear central and parietal epileptiform discharges in 1 child; and no abnormal findings were observed in the other 31 children. Positive rotavirus antigen was detected in 11 children and positive calicivirus antigen in stool samples in 4 children. Positive antibodies of rotavirus and calicivirus in CSF were not seen. Seizures recurred in 22 of 28 children who received prophylactic injections of phenobarbital(5-10 mg/kg). In a 6 months follow-up, one child developed epilepsy and the other 39 children had no seizures and neurological sequelae.</p><p><b>CONCLUSIONS</b>The digestive system manifestations are mild in children with BICG. Convulsions are always clustered in these children. The mechanism underlying convulsions is not clear. Conventional dose of phenobarbital is not effective for prevention of seizures. Most of children with BICG have a good prognosis.</p>

Follow-up of tuberous sclerosis complex complicated by epilepsy in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the treatment outcome and risk factors for intractable seizures in children with tuberous sclerosis complex(TSC)complicated by epilepsy.</p><p><b>METHODS</b>The medical data of 66 cases of TSC were retrospectively studied.</p><p><b>RESULTS</b>Of the 66 children with TSC, 47 cases were available for follow-up. The follow-up period ranged from 7 months to 9.3 years (average 4.5 + or - 2.6 years). The patients' present average age was (7.7 + or - 4.1) years (median 8 years). Among the 47 cases, 19 (40%) had infantile spasms, 24 (51%) had tonic seizures, 15 (32%) had partial seizures, and 3 (6%) had tonic-clonic seizures, and additionally, multifocal seizures, atonic seizures, atypical absence seizures and hypomotor seizures each appeared in 1 case (2%) respectively. The average number of antiepileptic drugs used was 1.9 + or - 0.86 (median 1). Among the 47 patients, 12 (26%) still had epileptic seizures and 33 (70%)were seizure-free, and 4% were dead. Three cases underwent surgery and continued to receive medication after surgery. The three patients were seizure-free in a 1.5 years follow-up. Among the 30 children over 7 years old, 17 cases (57%) were enrolled in ordinary schools, 3 cases (10%) in special schools and the other 10 cases were off-school for disabilities of intelligence and speech. The non-conditional logistic regression showed that the age of onset (RR=1.8, 95% CI 1.0- 3.2, P=0.050), administration of multiple antiepileptic drugs (RR=4.8, 95% CI 1.2-18.6, P=0.024), tonic seizures (RR=0.003, 95% CI 0.0- 0.2, P=0.04) and sex (RR=0.016, 95% CI 0.0-0.5, P=0.017) were risk factors for intractable seizures.</p><p><b>CONCLUSIONS</b>The majority (70%) of children with TSC complicated by epilepsy can be seizure-free with suitable treatment. The risk factors of poor outcome in seizure control may involve in the early onset age, tonic seizures and the administration for multiple anti-epileptic drugs.</p>

Preliminary Observation of Ketogenic Diet Therapy for Children with Intractable Epilepsy / 实用儿科临床杂志

Objective To observe the therapeutic effect of ketogenic diet therapy for children with intractable epilepsy and its safety.Methods Fifteen patients with intractable epilepsy were treated with ketogenic diet that was modified specifically for Chinese people.The compliance,seizure frequency and side effects were followed up.Results Twelve patients maintained on the treatment for 1 month.Among them,the reduction of seizure frequency in 10 patients exceed 50%.Ten patients maintained on the treatment for 3 months.Among them,the reduction of seizure frequency in 8 patients exceed 50%.Five patients maintained on the treatment for more than 6 months.The reduction of seizure frequency all exceed 50%.The reduction of seizure frequency in 4 patients exceed 90%.The seizures of 3 patients were controlled completely.Ten patients among all cases had various adverse effect,such as nausea,vomiting,diarrhea,constipation,hypoglycemia(nonsymptomatic),hyperlipemia and damage of liver function and so on,which could eliminate by anti-symptomatic treatment.Conclusions Ketogenic diet is effective and safe in Chinese children with intractable epilepsy with modified methods specifically for Chinese.The effect is unrelated with seizure types obviously.