RESUMEN
OBJECTIVE: The aim of this study was to assess how the ketogenic diet influences the blood levels of antiepileptic drugs in the first month of treatment in a pediatric population with drug-resistant epilepsy. METHODS: The plasma concentrations of antiepileptic drugs were investigated in an open study on 36 consecutive children and adolescents (20 males), aged between 6 months and 16 years (mean age 4.7 years), who were put on the ketogenic diet because of medically refractory epilepsy. The plasma levels of antiepileptic drugs were determined 30 days and immediately before the diet and on days 8, 15, 22 and 29 after the start of the diet. The daily dose of each drug was not changed during the first month of treatment, while the daily dose of benzodiazepines was reduced by up to 30% if excessive sedation or drowsiness occurred. RESULTS: While plasma concentrations of phenobarbital did not change in the first month on the ketogenic diet (mean increase of 2.3 mg/l ± 1.0), valproic acid showed a slight but not significant decrease (mean decrease of 6.7 mg/l ± 3.2), 2 weeks after the start of the diet. CONCLUSIONS: Adjustments in the daily dose of either drug before the start of the diet do not however appear to be justified.
Asunto(s)
Dieta Cetogénica , Epilepsia/dietoterapia , Epilepsia/tratamiento farmacológico , Fenobarbital/sangre , Ácido Valproico/sangre , Adolescente , Anticonvulsivantes/sangre , Anticonvulsivantes/uso terapéutico , Niño , Preescolar , Terapia Combinada , Epilepsia/sangre , Femenino , Humanos , Lactante , Masculino , Fenobarbital/uso terapéutico , Estadísticas no Paramétricas , Resultado del Tratamiento , Ácido Valproico/uso terapéuticoRESUMEN
We report a six-year clinical and electrodiagnostic follow-up of an adolescent patient with acute thallium poisoning from attempted suicide. During the acute stage the patient showed gastrointestinal disturbances, alopecia, and clinical and electrodiagnostic signs of severe polyneuropathy. Three years after poisoning, his neurological symptomatology was making progress, and electrophysiologic signs of peripheral neuropathy were mainly confined to lower limbs. Six years after intoxication, he was still complaining of weakness and sensory disturbances at the level of distal lower extremities; his neurologic and electrodiagnostic abnormalities affected mainly the feet. In this case report we underline the importance of early diagnosis and treatment to prevent neurological damage and the role of serial electromyographic and nerve conduction studies in thallium poisoning. These investigations allowed the authors to depict the electrophysiologic course of peripheral nervous system involvement over six years following poisoning.
Asunto(s)
Electrodiagnóstico/métodos , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/fisiopatología , Talio/envenenamiento , Adolescente , Humanos , Estudios Longitudinales , Masculino , Conducción Nerviosa/fisiología , Intento de SuicidioRESUMEN
A retrospective, cross-sectional study was performed on a series of HCV-related mixed cryoglobulinemia (HCV-MC) patients to assess autonomic neuropathy (AN) and its relation to peripheral neuropathy (PN). Thirty consecutive patients affected by HCV-MC underwent clinical, neurological and electrodiagnostic examinations. Autonomic nervous system (ANS) involvement was assessed by functional cardiovascular tests and sympathetic skin response (SSR) evaluation. Sural nerve biopsy was performed in 10 patients with PN. All patients received steroids, 15 also received recombinant interferon-alpha2b (RIfn-alpha2b). PN occurred in 27 patients (90.0%) and AN in 4 (13.3 %) all with signs of PN. SSR was the autonomic test more frequently altered. Biopsy disclosed axonal degeneration more evident in the 4 patients with AN. Three out of 4 patients with AN received steroids and rIFN-alpha2b and 1 steroids alone. In our study on HCV-MC, it was concluded that AN can occur also without dysautonomic symptoms, SSR appears to be one of the optional tests to use together with dysautonomic tests to identify AN and finally PN and AN do not seem to be positively influenced by addition of rIFN-alpha2b to steroid treatment.
Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/complicaciones , Crioglobulinemia/complicaciones , Nervio Sural/fisiopatología , Potenciales de Acción/fisiología , Potenciales de Acción/efectos de la radiación , Adulto , Anciano , Enfermedades del Sistema Nervioso Autónomo/tratamiento farmacológico , Estudios Transversales , Crioglobulinemia/tratamiento farmacológico , Crioglobulinemia/etiología , Crioglobulinemia/virología , Electromiografía , Femenino , Glucocorticoides/uso terapéutico , Hepatitis C/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Músculo Esquelético/fisiopatología , Conducción Nerviosa/fisiología , Prednisona/uso terapéutico , Estudios Retrospectivos , Estadísticas no Paramétricas , Nervio Sural/patologíaAsunto(s)
Arritmias Cardíacas/complicaciones , Muerte Súbita Cardíaca/prevención & control , Distrofia Muscular de Duchenne/complicaciones , Adulto , Arritmias Cardíacas/fisiopatología , Muerte Súbita Cardíaca/etiología , Electrocardiografía Ambulatoria , Humanos , Masculino , Distrofia Muscular de Duchenne/fisiopatologíaRESUMEN
Pacemaker lead extraction has been shown to be an effective and safe treatment in the case of infected per-manent pacemaker leads. However, it can lead to potentially serious complications, usually occurring during the ex-traction procedure. This report describes a case of a 74-year-old male with a persistent superior vena cava thrombo-sis related to an infected permanent pacemaker lead transvenous extraction. Clinical and surgical management are discussed.
Asunto(s)
Remoción de Dispositivos/efectos adversos , Marcapaso Artificial , Infecciones Relacionadas con Prótesis/cirugía , Síndrome de la Vena Cava Superior/etiología , Anciano , Ecocardiografía , Humanos , MasculinoRESUMEN
AIM: Paroxysmal supraventricular tachycardia (PSVT) is a very frequent type of arrhythmia. Atrioventricular nodal reciprocating tachycardia (AVNRT) and atrioventricular reciprocating tachycardia through extranodal accessory pathways (AVRT) are the most common types of paroxysmal supraventricular tachycardia. We describe our experience in diagnosing these tachycardia by electrophysiological transesophageal study (ETS). METHODS: Three hundred patients, 155 men and 145 women, (mean age, 37.2 +/- 16 years), with a history of palpitations underwent clinical evaluation and ETS. The clinical features of those with AVNRT and those with AVRT were compared. RESULTS: Of a total of 300 patients, tachycardia was diagnosed only in 234, of which 136 (58%) had AVNRT and 98 (42%) had AVRT. AVNRT patients were older than those with AVRT (P = or < 0.004); patients with AVRT had palpitations earlier (P = or < 0.0001). Dyspnea and asthenia were the most frequent symptoms in the AVNRT patients (P = or < 0,02; P = or < 0.04). There were statistically significant differences between the two patient groups in Wencke-bach time (P = or < 0.05), ventricular-atrial (V-A) interval (P = or < 0.03) and period of induced tachycardia (P = or < 0.04). CONCLUSIONS: ETS revealed important clinical and electrophysiological differences between patients with AVRT and those with AVNRT.
Asunto(s)
Taquicardia Paroxística/fisiopatología , Adulto , Electrofisiología , Esófago/fisiología , Femenino , Humanos , Masculino , Estudios Retrospectivos , Taquicardia Paroxística/diagnósticoRESUMEN
A consecutive sample of 76 chronic alcoholic patients was studied clinically, biochemically and electrophysiologically to assess clinical and/or subclinical signs of alcohol-related neuropathy as well as the most important and disputed risk factors for neuropathy such as age, parental history of alcoholism, nutritional status, alcoholic disease duration and total lifetime dose of ethanol (TLDE). The results show that alcohol-related neuropathy, especially when subclinical, seems to be frequent and mostly characterized by axonal degeneration of peripheral nerve fibres with earlier and more frequent involvement of sensory fibres and lower limbs. Moreover, positive family history of alcoholism, but above all alcoholic disease duration and TLDE, could be more important factors than malnutrition in determining neuropathy.
Asunto(s)
Alcoholismo/complicaciones , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Enfermedades del Sistema Nervioso Periférico/patología , Adolescente , Anciano , Alcoholismo/patología , Alcoholismo/fisiopatología , Estudios Transversales , Electrofisiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Conducción Nerviosa/fisiología , Estado Nutricional , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Estudios Retrospectivos , Factores de Riesgo , Nervio Sural/patología , Nervio Sural/fisiopatologíaRESUMEN
In some alcohol-related pathologies of chronic alcoholism women are more vulnerable than men. A consecutive sample of 62 chronic alcoholics was studied, 18 females and 44 males, aged between 28 and 69 years to assess the incidence and distribution of peripheral neuropathy with regard to gender. All patients underwent clinical and neurological observations, laboratory tests, and electroneurography. Total lifetime dose of ethanol (TLDE) and other risk factors for neuropathy (disease duration, age, nutritional status) were calculated and correlated to sural nerve sensory-evoked potential (SEP) amplitude. In 42 patients (67.7%), we observed the presence of clinical and/or infraclinical neuropathy, mostly axonal, in 29 males (65.9%) and 13 females (72.2%). In women, compared to men, TLDE and disease duration were significantly inversely correlated to sural nerve SEP amplitude, i.e. in women, SEP amplitude is significantly reduced in relation to TLDE and disease duration increase. These data indicate a higher sensitivity of females towards the toxic effects of ethanol, other than malnutrition, on peripheral nerve fibres.
Asunto(s)
Alcoholismo/complicaciones , Potenciales Evocados Somatosensoriales/fisiología , Enfermedades del Sistema Nervioso Periférico/epidemiología , Enfermedades del Sistema Nervioso Periférico/etiología , Nervio Sural/fisiopatología , Adulto , Anciano , Enfermedad Crónica , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Estado Nutricional , Factores de Riesgo , Sensibilidad y Especificidad , Distribución por SexoRESUMEN
Streptozotocin (STZ) is a widely used diabetogenic agent that damages pancreatic islet beta cells by activating immune mechanisms, when given in multiple low doses, and by alkylating DNA, when given at a single high dose. Actually, STZ contains a nitroso moiety. Incubation of rat islets with this compound has been found to generate nitrite; moreover, photoinduced NO production from STZ has been demonstrated. These reports have suggested that direct NO generation may be a mechanism for STZ toxicity in diabetogenesis. Several other studies have denied such a mechanism of action. This study has shown that (1) the multiple low-dose (MLDS) treatment does not stimulate NO production at the islet level; in fact, nitrite + nitrate levels and aconitase activity (also in the presence of an NO-synthase inhibitor, namely NAME) remain unmodified; RT-PCR analysis demonstrates that this treatment does not stimulate iNOS activity; (2) the high-dose (HDS) treatment does not stimulate NO production; in fact nitrite + nitrate levels remain unmodified and iNOS mRNA levels are not altered, although aconitase activity is significantly decreased. Moreover, we have confirmed that the MLDS treatment is able to decrease SOD activity by day 11 and that STZ, given in a single high dose, transiently increases superoxide dismutase (SOD) values (24 h from the administration), then dramatically lowers SOD levels. On the basis of our results, we conclude that STZ, "in vivo" is unable to generate NO, both as a MLDS or HDS treatment, thus excluding that NO exerts a role in streptozotocin-dependent diabetes mellitus.
Asunto(s)
Antibacterianos/toxicidad , Diabetes Mellitus Experimental/inducido químicamente , Diabetes Mellitus Experimental/metabolismo , Óxido Nítrico/metabolismo , Estreptozocina/toxicidad , Animales , Relación Dosis-Respuesta a Droga , Islotes Pancreáticos/metabolismo , Islotes Pancreáticos/patología , Masculino , Ratones , Ratones Endogámicos C57BL , RatasRESUMEN
Pancreases of untreated and nicotinamide (NIC)-treated pre-diabetic (10-week-old) and overtly diabetic (25-week-old) female NOD (non-obese diabetic) mice and of NON (non-obese non-diabetic) control mice were studied, with the following results. (1) Islets and ducts of overtly diabetic untreated NOD mice (25-week-old) were found to express low levels of MHC class I and II molecules, like NON controls, and high levels of adhesive molecules. (2) NIC was able to slightly affect glycaemia and insulitis, slowing down diabetes progression. Moreover it significantly decreased MHC class II expression (but not class I) in vivo by week 10, and significantly enhanced intercellular adhesion molecule-1 (ICAM-1) expression, mainly by week 25, within the pancreas, where 5-bromo-2'-deoxyuridine positive nuclei and insulin positive cells were present, demonstrating that a stimulation of endocrine cell proliferation occurs. (3) In addition, NIC partly counteracted the fall of superoxide dismutase levels, observed in untreated diabetic NOD animals. (4) In vitro studies demonstrated that NIC: (i) was able to significantly reduce nitrite accumulation and to increase NAD+NADH content significantly, and (ii) was able to increase the levels of interleukin-4, a T helper 2 lymphocyte (Th2) protective cytokine, and of interferon-alpha (IFN-alpha), which is known to be able to induce MHC class I and ICAM-1 but not MHC class II expression, as well as IFN-gamma, which is also known to be able to induce MHC class I and ICAM-1 expression. The latter, although known to be a proinflammatory Th1 cytokine, has also recently been found to exert an anti-diabetogenic role. This study therefore clearly shows that adhesive mechanisms are ongoing during the later periods of diabetes in pancreatic ducts of NOD mice, and suggests they may be involved in a persistence of the immune mechanisms of recognition, adhesion and cytolysis and/or endocrine regeneration or differentiation processes, as both NIC-increased ICAM-1 expression and 5-bromo-2'-deoxyuridine positivity imply. The effects of NIC on MHC class II (i.e. a reduction) but not class I, and, mainly, on ICAM-1 expression (i.e. an increase), together with the increase in Th2 protective cytokine levels are very interesting, and could help to explain its mechanism of action and the reasons for alternate success or failure in protecting against type 1 diabetes development.