RESUMEN
Complementary and alternative medicine (CAM) is widely used by both physicians and patients with primary headache syndromes. Despite a considerable number of articles addressing CAM in primary headache syndromes, the overall evidence for CAM is still poor. The aim of this review was to give an overview of the current evidence of the main alternative therapies used in the treatment of primary headache syndromes of childhood. MEDLINE and Cochrane Library were systematically searched for articles dealing with complementary and alternative treatment or prophylaxis of headache and migraine published within the past 20 years.
Asunto(s)
Terapias Complementarias/métodos , Trastornos de Cefalalgia/terapia , Terapia por Acupuntura/métodos , Terapia por Acupuntura/tendencias , Niño , Terapias Complementarias/tendencias , Trastornos de Cefalalgia/tratamiento farmacológico , Homeopatía/métodos , Homeopatía/tendencias , Humanos , Osteopatía/métodos , Osteopatía/tendencias , Trastornos Migrañosos/tratamiento farmacológico , Trastornos Migrañosos/terapiaRESUMEN
MPI-CDG (formally called CDG 1b), caused by phosphomannose isomerase (MPI) deficiency, leads to hypoglycaemia, protein losing enteropathy, hepatopathy, and thrombotic events, whereas neurologic development remains unaffected. Dietary supplementation of mannose can reverse clinical symptoms by entering the N-glycosylation pathway downstream of MPI. When oral intake of mannose in patients with MPI-CDG is not possible, e.g. due to surgery, mannose has to be given intravenously. We report a patient with MPI-CDG on intravenous mannose therapy that showed severe depression of consciousness and seizures without apparent cause. EEG and cranial MRI findings were compatible with metabolic coma whereas extended laboratory examinations including repeated blood glucose measurements were normal. Importantly, an intravenous bolus of glucose immediately led to clinical recovery and EEG improvement. Mannose did not interfere with glucose measurement in our assay. We suggest that in patients with MPI-CDG, intravenous mannose infusion can lead to intracellular ATP deprivation due to several mechanisms: (1) in MPI deficiency, mannose 6-P cannot be isomerised to fructose 6-P and therefore is unavailable for glycolysis; (2) animal data has shown that accumulating intracellular mannose 6-P inhibits glycolysis; and (3) elevated intracellular mannose 6-P may induce an ATP wasting cycle of dephosphorylation and rephosphorylation ("honey bee effect"). The mannose-induced metabolic inhibition may be overcome by high-dose glucose treatment. We caution that, in patients with MPI-CDG, life-threatening central nervous system disturbances may occur with intravenous mannose treatment. These may be due to intracellular energy failure. Clinical symptoms of energy deficiency should be treated early and aggressively with intravenous glucose regardless of blood glucose levels.
Asunto(s)
Trastornos Congénitos de Glicosilación/tratamiento farmacológico , Manosa-6-Fosfato Isomerasa/deficiencia , Manosa/efectos adversos , Convulsiones/inducido químicamente , Estupor/inducido químicamente , Adenosina Trifosfato/metabolismo , Biomarcadores/metabolismo , Glucemia/metabolismo , Trastornos Congénitos de Glicosilación/diagnóstico , Trastornos Congénitos de Glicosilación/enzimología , Trastornos Congénitos de Glicosilación/genética , Electroencefalografía , Metabolismo Energético , Predisposición Genética a la Enfermedad , Glucosa/administración & dosificación , Humanos , Infusiones Intravenosas , Inyecciones Intravenosas , Imagen por Resonancia Magnética , Masculino , Manosa/administración & dosificación , Manosa-6-Fosfato Isomerasa/genética , Fenotipo , Convulsiones/sangre , Convulsiones/diagnóstico , Convulsiones/tratamiento farmacológico , Estupor/sangre , Estupor/diagnóstico , Estupor/tratamiento farmacológico , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
The objective of surgical treatment of epilepsy is seizure control and improvement of quality-of-life of patients with medically intractable epilepsy. Confirmation of the diagnosis of epilepsy and its medical intractability is the essential prerequisite for epilepsy surgery. After excluding nonepileptic events such as psychogenic pseudoseizures, the clinician must establish that adequate drug trials, including verification of compliance, have been performed. A careful diagnostic evaluation is mandatory to localize the epileptogenic zone. In this review we discuss the role of different diagnostic methods with respect to patient selection and surgical outcome. Furthermore, experimental approaches are mentioned and the reasons for failures of epilepsy surgery are critically discussed.