RESUMEN
Objective: We have frequently applied noninvasive positive pressure ventilation (NPPV) to treat acute respiratory failure in children with severe motor and intellectual disabilities. We investigated the features and causes of conditions requiring endotracheal intubation. We aimed to determine whether phlegm expulsion using appropriate breathing physiotherapy with NPPV could avoid the need for endotracheal intubation in such patients. Methods: Between December 2010 and November 2012, 21 children with 51 episodes of acute respiratory failure were placed on NPPV at our hospital. We investigated the ratio, background, and causes of conditions requiring endotracheal intubation. Results: Pneumonia and bronchitis caused 30 and 21 episodes of respiratory failure, respectively. Respiratory infection required endotracheal intubation in 8 of 30 episodes of pneumonia, and in none of the 21 episodes of bronchitis. Respiratory infections were caused by upper airway obstruction with large amounts of secretion (n=4), lower airway obstruction due to atelectasis (n=3) and a combination of both (n=1). The frequency of breathing physiotherapy was significantly higher for all patients who required assistance with active phlegm expulsion than in those who did not (p=0.006). More patients on endotracheal intubation also required phlegm aspiration compared with other patients (p=0.019). Conclusion: We applied NPPV to acute respiratory failure in children with severe motor and intellectual disabilities. This allowed 84% of them to avoid endotracheal intubation. Acute respiratory failure did not improve in any patient who required endotracheal intubation, but we also used NPPV with breathing physiotherapy and postural drainage. Assistance with phlegm expulsion is hampered in children with severe motor and intellectual disabilities due to conditions such as thoracic deformations, joint contracture and glossoptosis. We consider that assistance with phlegm expulsion using appropriate breathing physiotherapy with NPPV is very important for such patients.
Asunto(s)
Bronquitis/complicaciones , Discapacidad Intelectual , Trastornos del Movimiento/terapia , Ventilación no Invasiva , Neumonía/complicaciones , Adolescente , Niño , Preescolar , Humanos , Trastornos del Movimiento/complicaciones , Adulto JovenRESUMEN
OBJECTIVE: Severe muscle hypertonia in patients with the mixed type of tetraplegia may be associated with significant deterioration in the quality of life of the patients. Intermittent use of oral muscle relaxant drugs, for example, Tizanidine (Ternelin), which is a fast-acting muscle relaxant, can provide relief from the severe hypertonia in these patients, but only for short durations. METHODS: We conducted a retrospective study of the effect of continuous infusion of tizanidine via a feeding tube on the severe systemic muscle hypertonia in patients with the mixed type of tetraplegia. We mixed tizanidine with milk or other enteral nutrients and administered the mixture via a naso-duodenal tube at a constant infusion rate several hours to 5 patients with the mixed type of tetraplegia showing severe uncontrolled systemic hypertonia under intermittent treatment with oral muscle relaxant drugs. RESULTS: Significant relief from the systemic muscle hypertonia was obtained in 4 of the 5 patients with improvement of the quality of life of the patients, e. g., they could get adequate sleep. There were no serious side effects in any of the cases. CONCLUSION: We consider that continuous infusion of tizanidine via a feeding tube would be useful for the treatment of severe systemic hypertonia in patients in whom the symptom cannot be adequately controlled by intermittent use of oral muscle relaxant drugs.
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Clonidina/análogos & derivados , Relajantes Musculares Centrales/uso terapéutico , Cuadriplejía/tratamiento farmacológico , Calidad de Vida , Preescolar , Clonidina/administración & dosificación , Clonidina/uso terapéutico , Nutrición Enteral , Femenino , Humanos , Lactante , Masculino , Hipertonía Muscular/tratamiento farmacológico , Relajantes Musculares Centrales/administración & dosificación , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
PURPOSE: Both to evaluate the characteristics of food allergic children who were prescribed an adrenaline autoinjector and to assess whether it was used appropriately. METHODS: The characteristics of food allergic children who were prescribed an adrenaline autoinjector were investigated. Among these children, those who experienced severe anaphylaxis due to inadvertent ingestion were analyzed, as was whether and how the autoinjector was used. RESULTS: An adrenaline autoinjector was prescribed to 139 food allergic children, most often for egg, followed by milk and wheat allergies. Concomitant bronchial asthma, atopic dermatitis, and food allergies of other causes were present in 49 (35.3%), 68 (48.9%), and 102 cases (73.4%), respectively. The most frequent organ involved in anaphylaxis was the skin (94.2%), followed by the respiratory (78.5%), digestive (28.1%), and circulatory (24.8%) organs. A total of 24 cases experienced severe anaphylaxis after the prescription; however, the autoinjector was used in only six (25%) of those cases. The reasons given for lack of use included fear of use, unavailability of the autoinjector, prior improvement with use of an oral antihistamine and immediate visit to a hospital emergency department in eight, five, three and one case, respectively. CONCLUSION: These results suggest that the autoinjector is often not used appropriately after prescription. Therefore, children and their caregivers require more effective guidance on proper adrenaline autoinjector use.
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Epinefrina/administración & dosificación , Hipersensibilidad a los Alimentos/tratamiento farmacológico , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Inyecciones Intramusculares/instrumentación , MasculinoRESUMEN
In Gaucher disease (GD), enzyme replacement therapy (ERT) results in the alleviation of hematological abnormalities and visceral infiltration as well as improvement in quality of life and life-span. However, several years may be required for skeletal manifestations, which are usually observed in type 1 and 3 GD, to respond to ERT. Infants with type 2 GD rarely present skeletal manifestations because most of these patients die within the first 2 years of life before they develop skeletal involvement. The use of ERT may prolong the lifespan of these patients and influence the natural history of the disease. The present study reports a new natural history of treated GD in which a 2-year and 7-month-old girl with type 2 GD who was receiving ERT developed valproate-induced Fanconi syndrome, pathological fractures, and pyogenic osteomyelitis. In conclusion, skeletal disease may occur in any type of GD, and Fanconi syndrome may lead to severe skeletal complications in patients with GD.
Asunto(s)
Anticonvulsivantes/efectos adversos , Síndrome de Fanconi/inducido químicamente , Fracturas Espontáneas/etiología , Enfermedad de Gaucher/fisiopatología , Osteomielitis/etiología , Ácido Valproico/efectos adversos , Anticonvulsivantes/uso terapéutico , Preescolar , Terapia de Reemplazo Enzimático , Síndrome de Fanconi/patología , Síndrome de Fanconi/fisiopatología , Femenino , Fémur/lesiones , Fémur/patología , Fracturas Espontáneas/patología , Enfermedad de Gaucher/tratamiento farmacológico , Enfermedad de Gaucher/patología , Humanos , Imagen por Resonancia Magnética , Osteomielitis/patología , Ácido Valproico/uso terapéuticoRESUMEN
OBJECTIVE: Ketogenic diets tend to cause trace mineral deficiencies. Ketonformula is a foumula for a ketogenic diet developed by Meiji Co Ltd in Japan. No reports are available on the trace mineral deficiencies associated with a use of Ketonformula. METHODS: We monitored the serum levels of selenium, zinc and copper as well as the amount of the daily intake of these minerals before and at 6 months after the initiation of the ketogenic diet with Ketonformula in six patients with intractable epilepsy associated with severe motor and intellectual disabilities. RESULT: The median serum selenium concentration decreased from 7.0 (range, 6.5-12.3) microg/dl to 6.2 (5.4-10.9) microg/dl as a result of the 6-month-treatment with Ketonformula (p < 0.05, Wilcoxon signed-rank test). The median daily selenium intake decreased from 17.8 (15.0-27.0) microg/day at the baseline to 5.5 (5.0-22.0) microg/day after 6 months on the diet (p < 0.05). The median serum zinc concentration increased slightly (from 66.0 (46.0-84.0) microg/dl to 68.0 (46.0-71.0) microg/dl), but the difference was not significant. The median daily zinc intake, however, significantly decreased from 4.2 (3.7-6.0) mg/day to 2.2 (2.0-3.0) mg/day (p < 0.05). The median serum copper concentration also showed no significant decrease (from 134.5 (119.0-168.0) microg/dl to 126.0 (86.0-183.0) microg/dl). The median daily copper intakes, however, decreased significantly from 0.80 (0.35-1.30) mg/day to 0.30 (0.26-0.40) mg/day (p < 0.05). CONCLUSIONS: The decline of the serum selenium concentrations and daily enteral intakes of selenium, zinc, and copper after 6 months on Ketonformula suggested that patients on this ketogenic formula needs close monitoring as well as supplementation of these trace minerals.
Asunto(s)
Cobre/sangre , Dieta Cetogénica , Epilepsia/dietoterapia , Selenio/sangre , Zinc/sangre , Niño , Preescolar , Suplementos Dietéticos , Humanos , Lactante , Japón , Masculino , Adulto JovenRESUMEN
Febrile infection-related epilepsy syndrome (FIRES) is a severe epileptic syndrome that manifests with refractory seizures or status epilepticus in previously healthy children after banal febrile illness. The neuroimaging findings in the acute phase of FIRES are nonspecific or normal. We report the case of a 7-year-old boy with FIRES who presented with a reversible lesion in the splenium of the corpus callosum on brain magnetic resonance imaging (MRI). The patient developed clusters of clonic seizures with a deviation of the eyes after a 3-day history of fever. A reversible splenial lesion was observed on brain MRI and, therefore, the initial diagnosis was mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). However, the intractable complex partial seizures necessitated a long-term midazolam infusion, indicating that FIRES was a more likely diagnosis than MERS. All other findings of this patient met the diagnostic criteria for FIRES. With this diagnosis, a high-dose phenobarbital was administrated, and the seizures were successfully controlled. This case indicated that FIRES should be considered even in patients with a reversible splenial lesion associated with encephalitis/encephalopathy.
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Cuerpo Calloso/patología , Encefalitis/patología , Epilepsia/patología , Fiebre/patología , Niño , Diagnóstico Diferencial , Humanos , Imagen por Resonancia Magnética , Masculino , Síndrome , Resultado del TratamientoRESUMEN
In 1999, Hirose et al. reported a Japanese family with autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) associated with a neuronal nicotinic acetylcholine receptor α4 subunit mutation (S252L). We followed the siblings of this family, and found that the elder brother had Asperger's disorder without mental retardation (MR) and the younger brother had autistic disorder with profound MR. The clinical epileptic features of the siblings were very similar, and both had deficits in socialization, but their cognitive development differed markedly. It thus seems that epilepsy is the direct phenotype of the S252L mutation, whereas other various factors modulate the cognitive and social development. No patients with ADNFLE have previously been reported to have autism spectrum disorder or profound MR.
Asunto(s)
Trastorno Autístico/complicaciones , Epilepsia del Lóbulo Frontal/complicaciones , Adolescente , Anticonvulsivantes/uso terapéutico , Síndrome de Asperger/genética , Trastorno Autístico/genética , Carbamazepina/uso terapéutico , Niño , Desarrollo Infantil , Epilepsia del Lóbulo Frontal/tratamiento farmacológico , Epilepsia del Lóbulo Frontal/genética , Femenino , Humanos , Discapacidad Intelectual/etiología , Discapacidad Intelectual/psicología , Masculino , Pruebas Neuropsicológicas , Linaje , Receptores Nicotínicos/genética , Hermanos , Conducta Social , Intento de Suicidio , Escalas de WechslerRESUMEN
UNLABELLED: Grisel syndrome is a non-traumatic atlantoaxial subluxation and a rare complication of any inflammatory condition of the upper neck and otolaryngological procedures. Delayed diagnosis causes neurological impairment, ranging from radiculopathy to paralysis and death. Kawasaki disease is a very frequent and important acute febrile vasculitis of childhood that is seen worldwide, and upper neck involvement (cervical lymphadenopathy) is one of the common symptoms of Kawasaki disease. A case of Grisel syndrome that occurred as a complication of Kawasaki disease is reported. This is the first case report, in English, of Grisel syndrome as a complication of Kawasaki disease. CONCLUSION: Pediatricians should be aware of Grisel syndrome as a possible complication of Kawasaki disease.
Asunto(s)
Articulación Atlantoaxoidea , Luxaciones Articulares/diagnóstico , Luxaciones Articulares/etiología , Síndrome Mucocutáneo Linfonodular/complicaciones , Preescolar , Femenino , HumanosRESUMEN
Toothbrushing-induced seizures are rare reflex seizures triggered by the brushing of one's own teeth. We encountered an 11-year-old girl with severe mental retardation, hypotonic cerebral palsy and epilepsy who presented with toothbrushing-induced seizures. She had had spontaneous brief tonic seizures several times a day since the age of 1 year and 2 months and started presenting with the same type of seizures induced by toothbrushing from the age of 8 years. As she could not brush her teeth by herself due to her disabilities, her mother brushed her teeth daily for her. The interictal EEG showed spike-and-wave complexes in the frontal regions bilaterally. The [Tc-99m]HMPAO-SPECT at the time of the seizure induced by toothbrushing suggested that the seizures originated from the left perisylvian cortex. This is the first report of toothbrushing-induced seizures triggered by the brushing of the patient's teeth by another person ('passive toothbrushing').
Asunto(s)
Epilepsia Refleja/etiología , Cepillado Dental/efectos adversos , Corteza Cerebral/patología , Corteza Cerebral/fisiopatología , Niño , Electroencefalografía , Epilepsia Refleja/patología , Epilepsia Refleja/fisiopatología , Femenino , HumanosRESUMEN
OBJECTIVE: We investigated the efficacy, safety, and tolerability of high-dose topiramate with rapid dose titration in 12 children with symptomatic West syndrome who suffered from severe motor and intellectual disabilities. METHODS: Topiramate was introduced as add-on therapy at the daily dose of 1 mg/kg/day, followed by increments of 2 mg/kg at 3- or 4-day intervals, up to a maximum of 19 or 20 mg/kg/day. The ages at the start of topiramate therapy ranged from 5 to 22 months. Prior to the topiramate therapy, the patients had received 2 to 6 antiepileptic agents with (8 patients) or without ACTH (4 patients). RESULTS: Topiramate appeared to be effective in 8 of the 12 patients (67%); four became seizure-free;three showed greater than 90% seizure reduction; one showed greater than 50% seizure reduction. The maintenance dose was 7 to 20 mg/kg/day (mean:17.9 +/- 3.9 mg/kg/day). In 4 of these 8 patients (50%), the spasms relapsed several months after complete cessation or diminution in the frequency of the spasms following treatment with topiramate. All of the 8 topiramate-responsive patients could continue the topiramate therapy throughout this study. The duration of topiramate therapy was 7 to 42 months (median: 12.5 months). There were no severe side effects that necessitated discontinuation of topiramate, including kidney stones. CONCLUSIONS: High-dose topiramate with rapid dose titration was revealed to be effective, safe, and well-tolerated in children with symptomatic West syndrome.
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Anticonvulsivantes/uso terapéutico , Fructosa/análogos & derivados , Espasmos Infantiles/tratamiento farmacológico , Anticonvulsivantes/administración & dosificación , Anticonvulsivantes/efectos adversos , Niño , Preescolar , Relación Dosis-Respuesta a Droga , Femenino , Fructosa/administración & dosificación , Fructosa/efectos adversos , Fructosa/uso terapéutico , Humanos , Lactante , Masculino , Topiramato , Resultado del TratamientoAsunto(s)
Aberraciones Cromosómicas , Cromosomas Humanos Par 18/ultraestructura , Cejas , Trisomía/diagnóstico , Trisomía/genética , Niño , Preescolar , Facies , Femenino , Humanos , MasculinoRESUMEN
The ketogenic diet (KD) is a high-fat, low-protein, low-carbohydrate diet which is effective in the treatment of medically refractory epilepsy. Several theories have been proposed to explain the mechanism underlying the anticonvulsant efficacy of the KD, however, the precise anticonvulsant mechanism of the KD is still unknown. We speculated the mechanism underlying the effect of the KD in patients with intractable epilepsy, based on the results of the [11C] flumazenil (FMZ)-positron emission tomography (PET) study. A patient developed frontal lobe epilepsy at the age of 2 years. At the age of 4 years 11 months, she was admitted to our hospital for the initiation of a KD. At the time of admission, she had several epileptic attacks each day: frequent postural tonic seizures, hypermotor seizures, head nodding, and intermittent loss of consciousness (non-convulsive status epilepticus). MR imaging showed no abnormal signal intensity in the brain. With the KD, the seizure frequency reduced dramatically on the fifth day. Interictal [11C] FMZ-PET was performed before and 2 months after the initiation of the KD. Before the KD, the [11C] FMZ-PET images and [11C] FMZ-PET binding potential (BP) images showed extremely low accumulation of FMZ throughout the cerebral cortex. Two months after the initiation of the KD, significantly increased binding potential of [11C] FMZ was observed, implying the increased binding potential of the benzodiazepine receptors, probably due to the anticonvulsant effect of the KD. These PET findings suggested that KD may control seizures by directly or indirectly increasing the binding potential of the benzodiazepine receptors.
Asunto(s)
Encéfalo/diagnóstico por imagen , Radioisótopos de Carbono , Dieta Cetogénica , Epilepsia del Lóbulo Frontal/diagnóstico por imagen , Epilepsia del Lóbulo Frontal/dietoterapia , Flumazenil , Tomografía de Emisión de Positrones , Radiofármacos , Receptores de GABA-A/metabolismo , Encéfalo/metabolismo , Preescolar , Epilepsia del Lóbulo Frontal/metabolismo , Femenino , Humanos , Receptores de GABA/metabolismo , Ácido gamma-Aminobutírico/metabolismoRESUMEN
Ten Japanese patients aged 1.5-17years with medication-resistant epilepsy were placed on the modified Atkins diet (MAD) for 3weeks during admission to our hospital. Dietary carbohydrate was restricted to 10g per day. We studied the efficacy of the diet regarding the seizure frequency and tolerability of the diet at the end of the 3weeks on the diet. Those who decided to continue the MAD at the time of discharge were followed up in the out-patient clinic to observe the effect of the diet on the seizure frequency. Three of the 10 patients could not continue the diet during the 3-week admission; one had rotavirus enterocolitis and the other 2 disliked the diet. Among the remaining 7 patients who could continue the diet for 3weeks, 3 achieved the seizure reduction; 2 became seizure-free and 1 showed about 75% reduction in the seizure frequency within 10days on the diet. All of these 3 patients continued the diet after the 3-week admission. The other 4 patients did not show a reduction of the seizure frequency by the end of the 3weeks on the diet. Two of them discontinued the diet on discharge. The remaining 2 still continued the diet at home and one became seizure-free 3months after the start of the diet. In total, 4 of 10 patients achieved>75% reduction in the seizure frequency, although relapse occurred in 2 of the patients, at 5months and 2years after seizure reduction, respectively. The MAD was effective and well-tolerated in children with medication-resistant epilepsy in Japan.
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Dieta Baja en Carbohidratos/efectos adversos , Epilepsia/dietoterapia , Adolescente , Pueblo Asiatico , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Convulsiones/dietoterapia , Convulsiones/prevención & controlRESUMEN
BACKGROUND: Mitochondrial DNA depletion syndromes are a group of heterogeneous autosomal recessive disorders associated with a severe reduction in mitochondrial DNA in the affected tissues. Sodium pyruvate has been reported to have a therapeutic effect in mitochondrial diseases. METHODS: We analyzed the effects of 0.5g/kg of sodium pyruvate administered through a nasogastric tube in a one-year-old patient with myopathic mitochondrial DNA depletion syndrome. To evaluate the improvement, we used the Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) and manual muscle testing. As the improvement of motor functions in this severely disabled infant could not be comprehensively detected by NPMDS, we also observed the infant's ability to perform several tasks such as pouting, winking, and number of times she could tap a toy xylophone with a stick. Blood lactate and pyruvate levels were also monitored. RESULTS: After one month's treatment, the NPMDS score in section IV, the domain for the quality of life, improved from 17 to13. The infant became capable of raising her forearm, lower leg and wrist against gravity. The maximum number of times she could repeat each task increased and the movements became brisker and stronger. No significant change of the blood lactate level or lactate-to-pyruvate ratio, both of which were mildly increased at the initiation of the therapy, was observed despite the clinical improvement. CONCLUSION: Sodium pyruvate administered at 0.5g/kg improved the muscle strength and the NPMDS score of an infant with myopathic mitochondrial DNA depletion syndrome. GENERAL SIGNIFICANCE: Sodium pyruvate may be effective for ameliorating the clinical manifestations of mitochondrial diseases. This article is part of a Special Issue entitled: Biochemistry of Mitochondria.
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ADN Mitocondrial/genética , Enfermedades Mitocondriales/tratamiento farmacológico , Ácido Pirúvico/uso terapéutico , Femenino , Humanos , Lactante , Enfermedades Mitocondriales/diagnóstico , Enfermedades Mitocondriales/metabolismo , SíndromeRESUMEN
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder and is the most common form of muscular dystrophy. Affected children develop muscle weakness in early childhood. Only steroids have been shown with evidence to improve muscle function in patients with DMD. We report the long-term effects of prednisolone treatment in patients with DMD, comparing the age at which 14 treated patients and 15 control patients lost their ability to walk. The prednisolone-treated patients were assigned to one of five regimens: 0.5 mg/kg/day given for the first 10 days of every month (n = 6), 0.75 mg/kg/day given for the first 10 days of every month (n = 3), 0.5 mg/kg on alternate days (n = 1), 0.75 mg/kg on alternate days (n = 1), or 5 mg/kg twice a week (n = 3). No significant difference in age of losing ambulation ability was observed between the treated group and the untreated group (mean age; 10 years and 6 months in both groups). However, 13 of the 14 patients showed an improvement in their activity of daily living other than ambulation in the treated group. The results of this study showed that the prednisolone treatment regimens used in this study did not prolong the period of ambulation.
Asunto(s)
Glucocorticoides/uso terapéutico , Distrofia Muscular de Duchenne/tratamiento farmacológico , Prednisolona/uso terapéutico , Actividades Cotidianas , Glucocorticoides/administración & dosificación , Humanos , Prednisolona/administración & dosificación , Estudios RetrospectivosRESUMEN
BACKGROUND: Epilepsy has been reported in patients with pervasive developmental disorder (PDD), with an incidence ranging from 5% to 40%; however most of the studies included patients with brain magnetic resonance imaging (MRI) abnormalities (e.g., tuberous sclerosis) and patients with epilepsy whose seizure onset was in the first year of life. METHODS: We retrospectively examined 67 patients (45 males, 22 females) with PDD and epilepsy, who did not have brain MRI abnormalities. Patients who had seizures in the first year of life were excluded. We divided the patients into two groups: group A included 34 patients with an IQ<50, and group B included 33 patients with an IQ≥50. RESULTS: The median age of epilepsy onset was higher in group A than group B (8.8 vs. 5.2 years, P<0.01). Only one patient (3%) in group A and nine patients (27%) in group B were classified with generalized epilepsy (P<0.05). At the last observation, 16 patients (47%) in group A and 25 patients (76%) in group B were seizure-free for ≥1year (not statistically significant). CONCLUSION: The relationship between PDD and epilepsy may be different between patients with lower (group A) and higher (group B) IQs in patients who do not have brain MRI abnormalities.
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Encéfalo/anomalías , Trastornos Generalizados del Desarrollo Infantil/epidemiología , Trastornos Generalizados del Desarrollo Infantil/patología , Trastornos Generalizados del Desarrollo Infantil/fisiopatología , Epilepsia/epidemiología , Epilepsia/fisiopatología , Imagen por Resonancia Magnética , Encéfalo/fisiopatología , Niño , Comorbilidad , Femenino , Humanos , Inteligencia , Masculino , Estudios RetrospectivosRESUMEN
Cloxazolam has been used mainly as an anxiolytic agent. The present study was designed to evaluate the effectiveness of cloxazolam as an add-on antiepileptic drug in patients with intractable epilepsy. A total of 32 patients with intractable epilepsy were treated with cloxazolam: 13 with generalized epilepsy, 15 with focal epilepsy, and 4 with undetermined type of epilepsy. The initial effects were evaluated at 1 month after reaching a maintenance dose (0.3-0.5 mg/kg). The long-term effects were investigated at 2 years after reaching a maintenance dose. With cloxazolam, seizure frequency was reduced by ≥ 50% in 19/32 patients (59%) during initial therapy and in 6/23 patients (26%) during long-term therapy. Two became seizure free throughout the cloxazolam therapy. During initial therapy, 8/32 patients (25%) developed 11 episodes of adverse events during the initial therapy, including 5 with drowsiness, 3 with hyperactivity, 2 with irritability, and 1 with loss of appetite. During long-term therapy, 2/23 (9%) developed drowsiness. The mean dose of cloxazolam in patients with an effective response was 0.30 ± 0.18 mg/kg for initial therapy and 0.26 ± 0.20 mg/kg for long-term therapy. Seven of the 19 effective responders developed tolerance (37%). Cloxazolam is an effective and safe antiepileptic drug for intractable epilepsy.
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Benzodiazepinas/uso terapéutico , Epilepsia/tratamiento farmacológico , Adolescente , Adulto , Anticonvulsivantes/efectos adversos , Anticonvulsivantes/uso terapéutico , Distribución de Chi-Cuadrado , Niño , Preescolar , Tolerancia a Medicamentos , Femenino , Humanos , Lactante , Masculino , Tiempo , Resultado del TratamientoRESUMEN
The authors describe the use of a modified Atkins diet for the treatment of 2 children with nonconvulsive status epilepticus. Patient 1 was a 4-year-and-11-month-old girl diagnosed with frontal lobe epilepsy. Since the age of 3 years and 10 months, she had daily nonconvulsive status epilepticus resistant to antiepileptic agents. Patient 2 was a 5-year-and-5-month-old girl with subcortical band heterotopia. She had nonconvulsive status epilepticus daily since the age of 5 years. They were treated with the modified Atkins diet, in which carbohydrate intake was restricted to 10 g/d without restriction on protein, caloric, or fluid intake. The nonconvulsive status epilepticus disappeared 5 and 10 days after the initiation of the diet treatment, respectively. They have been on the diet treatment and free from nonconvulsive status epilepticus for 19 and 4 months, respectively. The modified Atkins diet appears to be very effective for the treatment of nonconvulsive status epilepticus.
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Dieta Baja en Carbohidratos , Epilepsia/dietoterapia , Estado Epiléptico/dietoterapia , Carbohidratos/deficiencia , Corteza Cerebral/metabolismo , Corteza Cerebral/fisiopatología , Preescolar , Carbohidratos de la Dieta/efectos adversos , Carbohidratos de la Dieta/metabolismo , Electroencefalografía , Epilepsia/fisiopatología , Potenciales Evocados/fisiología , Femenino , Alimentos Formulados , Humanos , Cuerpos Cetónicos/sangre , Recuperación de la Función/fisiología , Estado Epiléptico/fisiopatología , Resultado del TratamientoRESUMEN
The prevalences of cerebral palsy (CP) among 6-year-old children in Shiga Prefecture born during 1977-2000 were compared in eight successive 3-year periods (period I-VIII) with reference to birth weight and gestational age. During the 24-year surveillance period, among 349,514 children born in Shiga Prefecture, 569 children had CP at the age of 6 years, thus the prevalence was 1.63 per 1000 live births. The prevalence increased significantly in period V compared to previous periods. There were no significant differences among period V-VIII. The proportion of CP with low birth weight and lower gestational age increased progressively, and two-thirds of CP in the period VIII had a LBW and/or preterm gestation.
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Peso al Nacer , Parálisis Cerebral/epidemiología , Edad Gestacional , Niño , Femenino , Humanos , Recién Nacido , Japón/epidemiología , Masculino , PrevalenciaRESUMEN
Etiologies and risk factors of 569 patients with cerebral palsy (CP) in Shiga Prefecture born during 1977-2000 were investigated. The patients with CP born at preterm and at term were separately analyzed. The main etiologies or risk factors for CP in preterm children were periventricular leukomalacia (PVL, 58%), multiple births (25%) and mechanical ventilation (45%). The prevalence rates of these increased in the latter half of the study period. The main etiologies or risk factors for CP in term children were brain dysplasia (17%), fetal brain vascular disorders (15%), hypoxic ischemic encephalopathy (14%), PVL (8%) and intrauterine growth retardation (19%). The prevalence rates among etiologies did not change during the study period.