RESUMEN
The Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are the most common immune-mediated polyneuropathies, which can show variable clinical and electrophysiological manifestations. Rarer immune-mediated neuropathies encompass paraproteinemic neuropathies (PPN), multifocal motor neuropathy (MMN) and vasculitic neuropathies. The diagnosis usually relies on the history of symptom evolution, distribution of nerve dysfunction and particularly on characteristic features in nerve conduction studies, aided by cerebrospinal fluid (CSF) examination and nerve biopsy findings. The therapeutic toolbox encompasses corticosteroids, immunoglobulins and plasmapheresis often accompanied by long-term immunosuppression. It is important to note that immune-mediated neuropathies selectively respond to treatment and contraindications need to be considered. Despite treatment a considerable number of patients suffer from permanent neurological deficits.
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Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Inmunosupresores/toxicidad , Plasmaféresis/métodos , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/terapia , Terapia Combinada/métodos , Técnicas de Diagnóstico Neurológico , Medicina Basada en la Evidencia , Síndrome de Guillain-Barré/inmunología , Humanos , Evaluación de Síntomas/métodos , Resultado del TratamientoRESUMEN
BACKGROUND: Diabetic neuropathy is a common complication of diabetes mellitus. The length-dependent symmetrical sensorimotor type of neuropathy is the most prevalent form of diabetic neuropathy but other forms of diabetic neuropathy also need to be kept in mind. Their differential diagnosis is often more challenging but implicates specific forms of treatment other than improvement of metabolic control. AIM OF THE STUDY: This article gives an overview of the less frequent forms of diabetic neuropathy and discusses their impact, diagnostic and therapeutic implications. RESULTS: Autonomic diabetic neuropathy, diabetic small fiber neuropathy and less frequent forms of diabetic neuropathy, such as diabetic radiculoplexopathy, diabetic neuropathy of cranial nerves, therapy-induced neuropathy and alternative causes of peripheral neuropathy in patients with diabetes are described. DISCUSSION: Diagnosis of less frequent subtypes of diabetic neuropathy and differentiation towards alternative causes of peripheral neuropathy are often difficult in daily medical routine. Diagnostic clues are helpful in identifying rarer forms of diabetic neuropathy, thus enabling more specific treatment.
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Neuropatías Diabéticas/diagnóstico , Eritromelalgia/diagnóstico , Radiculopatía/diagnóstico , Diagnóstico Diferencial , HumanosRESUMEN
BACKGROUND: In the HD14 trial, 2×BEACOPPescalated+2×ABVD (2+2) has improved the primary outcome. Compared with 4×ABVD, this benefit might be compromised by more infertility in women. Therefore, we analyzed gonadal function and fertility. PATIENTS AND METHODS: Women≤45 years in ongoing remission at least 1 year after therapy were included. Hormone parameters, menopausal symptoms, measures to preserve fertility, menstrual cycle, pregnancies, and offspring were evaluated. RESULTS: Three hundred and thirty one of 579 women addressed participated (57.2%) and 263 per-protocol treated patients qualified (A=ABVD: 137, B=2+2: 126, mean time after therapy 42 and 43 months, respectively). Regular menstrual cycle after treatment (A: 87%, B: 83%) and time to recovery (≤12 months) were not different. Follicle-stimulating hormone and anti-Muellerian hormone were significantly better in arm A. However, pregnancies after therapy favored arm B (A: 15%, B: 26%, P=0.043) and motherhood rates were equivalent to the German normal population. Multivariate analysis revealed prophylactic use of gonadotropin-releasing hormone (GnRH) analogues as highly significant prognostic factor for preservation of fertility (odds ratio=12.87, P=0.001). Severe menopausal symptoms were frequent in women≥30 years (A: 21%, B: 25%). CONCLUSIONS: Hormonal levels after 2+2 indicate a reduced ovarian reserve. However, 2+2 in combination with GnRH analogues does not compromise fertility within the evaluated observation time.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Fertilidad/efectos de los fármacos , Enfermedad de Hodgkin/tratamiento farmacológico , Ovario/fisiopatología , Sobrevivientes , Adulto , Hormona Antimülleriana/sangre , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Bleomicina/efectos adversos , Bleomicina/uso terapéutico , Ciclofosfamida/efectos adversos , Ciclofosfamida/uso terapéutico , Dacarbazina/efectos adversos , Dacarbazina/uso terapéutico , Doxorrubicina/efectos adversos , Doxorrubicina/uso terapéutico , Etopósido/efectos adversos , Etopósido/uso terapéutico , Femenino , Hormona Folículo Estimulante/sangre , Hormona Liberadora de Gonadotropina/análogos & derivados , Hormona Liberadora de Gonadotropina/uso terapéutico , Humanos , Modelos Logísticos , Menopausia/efectos de los fármacos , Ciclo Menstrual/efectos de los fármacos , Persona de Mediana Edad , Análisis Multivariante , Ovario/efectos de los fármacos , Prednisona/efectos adversos , Prednisona/uso terapéutico , Embarazo , Procarbazina/efectos adversos , Procarbazina/uso terapéutico , Ensayos Clínicos Controlados Aleatorios como Asunto , Vinblastina/efectos adversos , Vinblastina/uso terapéutico , Vincristina/efectos adversos , Vincristina/uso terapéutico , Adulto JovenRESUMEN
The postpartum dysgalactia syndrome (PDS) represents one of the most important diseases after parturition in sows. The genetic background of the disease has been investigated some time ago and heritability estimates around 0.10 have been obtained. To compute current estimates, a dataset of 1680 sampled sows and their 2001 clinically examined litters was used for variance components estimation with a threshold liability model. Affected sows were defined through clinical examination 12-48 h after parturition. Posterior mean of additive genetic variance was 0.10 and estimated heritability for PDS averaged 0.0879 with a 95% confidence interval of 0.0876 and 0.0881. The results are in agreement with those of other studies and emphasize the importance of considering the genetic predisposition for susceptibility to PDS as well as of additional factors including hygiene and management conditions.
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Trastornos de la Lactancia/veterinaria , Mastitis/veterinaria , Enfermedades de los Porcinos/genética , Animales , Femenino , Trastornos de la Lactancia/genética , Mastitis/genética , Periodo Posparto , PorcinosRESUMEN
Pain-related evoked potentials (PREPs) represent a novel method for the evaluation of peripheral and central nociceptive pathways, e.g. in the diagnosis of small fiber neuropathy (SFN) or after therapeutic interventions for headache. Compared to contact heat-evoked and laser-evoked potentials, recording of PREPs is less stressful for the subjects and technically less demanding. The clinical usefulness of PREPs has been described for SFN associated with diabetes, HIV and hepatitis C infections as well as in headache and facial pain disorders. They have also been evaluated after interventional methods, such as direct current stimulation (tDCS). The article reviews and discusses the advantages and pitfalls of this technique in the context of recent clinical studies as compared to other paradigms of peripheral electrical stimulation and delineates perspectives and possible indications.
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Potenciales Evocados Somatosensoriales/efectos de los fármacos , Potenciales Evocados Somatosensoriales/fisiología , Nociceptores/efectos de los fármacos , Nociceptores/fisiología , Dolor/tratamiento farmacológico , Dolor/fisiopatología , Adulto , Analgésicos/efectos adversos , Analgésicos/uso terapéutico , Estimulación Eléctrica , Femenino , Cefalea/tratamiento farmacológico , Cefalea/fisiopatología , Humanos , Rayos Láser , Masculino , Fibras Nerviosas Mielínicas/efectos de los fármacos , Fibras Nerviosas Mielínicas/fisiología , Dimensión del Dolor/efectos de los fármacos , Dimensión del Dolor/métodos , Umbral del Dolor/efectos de los fármacos , Umbral del Dolor/fisiología , Nervios Periféricos/efectos de los fármacos , Nervios Periféricos/fisiopatología , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Valor Predictivo de las Pruebas , Tiempo de Reacción/efectos de los fármacos , Tiempo de Reacción/fisiología , Piel/inervación , Sensación Térmica/efectos de los fármacos , Sensación Térmica/fisiología , Resultado del TratamientoRESUMEN
Although there is no clear evidence, minimally invasive hip arthroplasty seems to be associated with slightly higher complication rates compared to standard procedures. Major nerve palsy is one of the least common but most distressing complications. The key for minimizing the incidence of nerve lesions is to analyze preoperative risk factors, accurate knowledge of the anatomy and minimally invasive techniques. Once clinical signs of nerve injury are evident, the first diagnostic steps are localization of the lesion and quantification of the damage pattern. Therefore, clinical assessment of the neurological deficits should be performed as soon as possible. Apart from rare cases of isolated transient conduction blockade or complete transection, the damage pattern is mostly combined. Thus, there can be evidence for dysfunction of nerve conduction (neuropraxia) and structural nerve damage (axonotmesis or neurotmesis) simultaneously. Because the earliest signs of denervation are detectable via electromyography after 1 week, it is not possible to make any reliable prognosis within the first days after nerve injury using electrophysiological methods. This review article should serve as a guideline for prevention, diagnostics and therapy of neural lesions in minimally invasive hip arthroplasty.
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Artroplastia de Reemplazo de Cadera/efectos adversos , Procedimientos Quirúrgicos Mínimamente Invasivos/efectos adversos , Traumatismos de los Nervios Periféricos/etiología , Traumatismos de los Nervios Periféricos/prevención & control , Humanos , Traumatismos de los Nervios Periféricos/diagnósticoRESUMEN
Symptomatic treatment of amyotrophic lateral sclerosis (ALS) is relevant in preventing complications and improving quality of life as long as curative therapies are still out of sight. About one third of ALS patients show disabling problems associated with dysarthria, dysphagia, sialorrhea, and a pseudobulbar affective disorder already in the early stages of ALS. A multidisciplinary approach is the cornerstone of symptomatic treatment of bulbar and pseudobulbar ALS features. Except for riluzole randomized controlled trials are lacking. Here, we review the current views with regard to epidemiology, pathophysiology, diagnosis, and practical aspects of treating bulbar and pseudobulbar symptoms.
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Esclerosis Amiotrófica Lateral/terapia , Trastornos de Deglución/terapia , Disartria/terapia , Cuidados Paliativos/métodos , Grupo de Atención al Paciente , Parálisis Seudobulbar/terapia , Sialorrea/terapia , Síntomas Afectivos/diagnóstico , Síntomas Afectivos/fisiopatología , Síntomas Afectivos/terapia , Esclerosis Amiotrófica Lateral/diagnóstico , Esclerosis Amiotrófica Lateral/fisiopatología , Esclerosis Amiotrófica Lateral/psicología , Antidepresivos/uso terapéutico , Terapia Combinada , Equipos de Comunicación para Personas con Discapacidad , Trastornos de Deglución/diagnóstico , Trastornos de Deglución/fisiopatología , Disartria/diagnóstico , Disartria/fisiopatología , Antagonistas de Aminoácidos Excitadores/uso terapéutico , Humanos , Parálisis Seudobulbar/diagnóstico , Parálisis Seudobulbar/fisiopatología , Calidad de Vida/psicología , Ensayos Clínicos Controlados Aleatorios como Asunto , Riluzol/uso terapéutico , Sialorrea/diagnóstico , Sialorrea/fisiopatologíaAsunto(s)
4-Aminopiridina/uso terapéutico , Potenciales Evocados Motores , Esclerosis Múltiple/tratamiento farmacológico , Esclerosis Múltiple/fisiopatología , Bloqueadores de los Canales de Potasio/uso terapéutico , Caminata , 4-Aminopiridina/administración & dosificación , 4-Aminopiridina/farmacología , Adulto , Anciano , Esquema de Medicación , Femenino , Humanos , Pierna/inervación , Masculino , Persona de Mediana Edad , Conducción Nerviosa/efectos de los fármacos , Bloqueadores de los Canales de Potasio/administración & dosificación , Bloqueadores de los Canales de Potasio/farmacología , Valor Predictivo de las Pruebas , Estudios ProspectivosRESUMEN
INTRODUCTION: Innate immunity represents the first step of activation of the immune system and dictates the quality of adaptive immune responses. Studies have reported links between systemic inflammatory or innate immune markers and prognosis in patients with lung cancer. To our knowledge, the prospective and concomitant study of these systemic markers has never been performed. METHODS: Advanced treatment-naive non-small cell lung cancer (NSCLC) patients eligible for first-line platinum-based chemotherapy were prospectively included from December 2012 to July 2015 (N = 148). Blood samples of patients were collected before the first cycle for fresh NK cell phenotyping. Peripheral blood mononuclear cells were cryopreserved for natural cytotoxicity receptor (NCR) genotyping as well as sera for NCR's ligand quantification. Data on leukocytes, neutrophils and monocyte counts and lactate dehydrogenase (LDH) levels were extracted from electronic medical records. RESULTS: Among all studied markers, monocytosis, neutrophilia, leucocytosis, high LDH and sBAG6 levels and reduced levels of NCR3 transcripts were associated with poor overall survival (OS) in univariate analysis. The levels of NCR3 transcripts was linked to age, number of metastatic sites, monocyte counts, LDH and sBAG6 levels. Neutrophilia was associated to high sBAG6 levels. NCR3 was the unique innate immune parameter that remained as an independent factor associated with both OS (P = 0.003) and progression-free survival (P = 0.009) in the multivariate analysis. CONCLUSION: This study brought evidence that these biomarkers are entangled; parameters associated with an inflammatory process were related to reduced levels of NCR3 transcripts. Finally, the level of NCR3 transcripts was independently associated with outcomes in treatment-naive patients with advanced NSCLC.
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Carcinoma de Pulmón de Células no Pequeñas/inmunología , Inmunidad Innata/inmunología , Neoplasias Pulmonares/inmunología , Receptor 3 Gatillante de la Citotoxidad Natural/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma de Pulmón de Células no Pequeñas/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/patología , Femenino , Humanos , Células Asesinas Naturales/inmunología , L-Lactato Deshidrogenasa/metabolismo , Recuento de Leucocitos , Neoplasias Pulmonares/metabolismo , Neoplasias Pulmonares/patología , Masculino , Persona de Mediana Edad , Chaperonas Moleculares/inmunología , Monocitos/inmunología , Receptor 3 Gatillante de la Citotoxidad Natural/genética , Neutrófilos/inmunología , Pronóstico , Supervivencia sin Progresión , Modelos de Riesgos Proporcionales , ARN Mensajero/metabolismo , Tasa de SupervivenciaRESUMEN
Tumor surveillance of natural killer (NK) cells is mediated by the cytotoxicity receptor natural-killer group 2 member D (NKG2D). Ligands for NKG2D are generally not expressed on healthy cells, but induced on the surface of malignant cells. To date, NKG2D ligand (NKG2D-L) induction was mainly described to depend on the activation of the DNA damage response, although the molecular mechanisms that regulate NKG2D-L expression remain largely unknown. Here, we show that the acetyltransferases CBP (CREB-binding protein) and p300 play a crucial role in the regulation of NKG2D-L on tumor cells. Loss of CBP/p300 decreased the basal cell surface expression of human ligands and reduced the upregulation of MICA/B and ULBP2 in response to histone deacetylase inhibitors or DNA damage. Furthermore, CBP/P300 deficiency abrogated the sensitivity of stressed cells to NK cell-mediated killing. CBP/p300 were also identified as major regulators of mouse NKG2D ligand RAE-1 in vitro and in vivo using the Eµ-Myc lymphoma model. Mechanistically, we observed an enhanced activation of the CBP/p300 binding transcription factor CREB (cAMP response element-binding protein) correlating to the NKG2D-L upregulation. Moreover, increased binding of CREB and CBP/p300 to NKG2D-L promoters and elevated histone acetylation were detectable. This study provides strong evidence for a major role of CBP and p300 in orchestrating NKG2D-L induction and consequently immunosurveillance of tumors in mice and humans. These findings might help to develop novel immunotherapeutic approaches against cancer.
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Antígenos de Histocompatibilidad Clase I/genética , Péptidos y Proteínas de Señalización Intercelular/genética , Linfoma/metabolismo , Subfamilia K de Receptores Similares a Lectina de Células NK/metabolismo , Proteínas Asociadas a Matriz Nuclear/genética , Proteínas de Transporte Nucleocitoplasmático/genética , Factores de Transcripción p300-CBP/metabolismo , Acetilación , Animales , Células Cultivadas , Modelos Animales de Enfermedad , Proteínas Ligadas a GPI/genética , Regulación Neoplásica de la Expresión Génica , Humanos , Linfoma/genética , Linfoma/patología , Ratones , Ratones Endogámicos C57BL , Ratones Transgénicos , Subfamilia K de Receptores Similares a Lectina de Células NK/genética , Transcripción Genética , Factores de Transcripción p300-CBP/genéticaRESUMEN
We report on two unrelated patients clinically presenting with late-onset progressive limb girdle weakness; cardiomyopathy was seen in one patient. Muscle biopsy revealed a necrotic myopathy with numerous rimmed vacuoles, ultrastructurally typical paired-helical filaments, and reduced immunohistochemical staining for alpha-dystroglycan. Quadriceps sparing hereditary inclusion body myopathy due to mutations in GNE gene, and OPMD due to PABPN1 mutations were excluded, genetically. We detected a homozygous mutation of the FKRP gene (826C>A) in both patients. Mutations of FKRP have been reported in congenital muscular dystrophies, LGMD2I, cardiomyopathy and hyperCKemia, but not in myopathies with vacuoles and paired-helical filaments. Therefore, our findings further extend the morphological variability of muscular dystrophies due to FKRP mutations.
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Distrofia Muscular de Cinturas/genética , Distrofia Muscular de Cinturas/patología , Proteínas/genética , Adulto , Citoesqueleto/patología , Humanos , Masculino , Persona de Mediana Edad , Pentosiltransferasa , Vacuolas/patologíaRESUMEN
BACKGROUND: Previous investigations in three families have shown that proximal myotonic myopathy (PROMM) is not linked to the gene loci for myotonic dystrophy (DM) or to the loci of the genes of the muscle sodium and chloride channels associated with other myotonic disorders. It is important to extend our clinical knowledge of this interesting new disorder by studying other families. PATIENTS: Thirty-five patients in 14 new families; 27 patients were examined. METHODS: Clinical examination, electromyography, muscle biopsy, DNA analysis. RESULTS: The following findings were noted: proximal without distal weakness of the legs (n = 21); myotonia on electromyograms (n = 23); intermittent clinical myotonia (n = 17); cataracts (n = 24) and a number of the cataracts were identical to the type in DM (n = 11); and peculiar muscle pain (n = 14). A few patients had cardiac arrhythmias, and others had elevations in the concentrations of serum gamma-glutamyltransferase. None of the patients had significant muscle atrophy. Muscle biopsy specimens showed mild myopathic changes. All patients had normal trinucleotide (cytosine, thymine, and guanine) repeat size of the DM gene in leukocyte DNA. Muscle DNA probes from three patients showed findings identical to those of their leukocyte DNA probes. CONCLUSIONS: Proximal myotonic myopathy is a new genetic disorder similar to, but distinct from, DM. Patients suspected of having DM but with negative DNA studies may have PROMM. The gene defect for PROMM awaits discovery. Because of the similarities between PROMM and DM, this discovery will not only shed light on the pathomechanism of PROMM, but it may also increase our understanding of DM.
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Distrofia Miotónica/complicaciones , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades Musculares/sangre , Enfermedades Musculares/complicaciones , Enfermedades Musculares/genética , Enfermedades Musculares/patología , Distrofia Miotónica/sangre , Distrofia Miotónica/genética , Distrofia Miotónica/patología , Análisis de Secuencia de ADNRESUMEN
Acute palsies of the peroneal nerve may have a variety of causes. In many patients, the cause remains undetermined. The authors report a patient with a thrombosis of a crural vein causing an acute peroneal nerve palsy. If the clinical history of patients with an acute peroneal nerve lesion is suggestive of venous thrombosis an appropriate diagnostic workup should be considered.
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Neuropatías Peroneas/etiología , Trombosis de la Vena/complicaciones , Enfermedad Aguda , Anciano , Humanos , Pierna/irrigación sanguínea , Masculino , Conducción Nerviosa , Paresia/etiología , VenasRESUMEN
A 68-year-old man developed pandysautonomia with severe orthostatic dysfunction, fixed heart rate, low serum levels of norepinephrine and epinephrine, absent sympathetic skin responses, and pupillary abnormalities. CSF protein was 92 mg/dl. In spite of a good recovery following corticosteroid administration, a relapse occurred, with accompanying sensory symptoms confined to both arms. Fatal sudden cardiac arrest occurred after 4 months. Autopsy revealed numerous lymphocytic infiltrates, predominantly in autonomic and sensory ganglia and, to a lesser extent, in the nerve roots, spinal cord, and brainstem without evidence for an underlying tumor. This case provides histopathologic evidence for an inflammatory etiology of panautonomic neuropathy in some patients.
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Enfermedades del Sistema Nervioso Autónomo/diagnóstico , Encefalomielitis/diagnóstico , Radiculopatía/diagnóstico , Anciano , Autopsia , Tronco Encefálico/patología , Diagnóstico Diferencial , Encefalomielitis/patología , Epinefrina/sangre , Ganglios/patología , Humanos , Masculino , Norepinefrina/sangre , Radiculopatía/patología , Médula Espinal/patologíaRESUMEN
We report the transcranial sonography (TCS) findings of the basal ganglia in 86 patients with dystonic disorders including idiopathic dystonia (facial, cervical, upper limb, and generalized dystonia), drug-induced tardive dystonia, dopa-responsive dystonia, and kinesigenic dystonia. The TCS was focused on alterations of the lenticular, caudate, raphe nuclei, and the thalamus. Seventy-five percent of patient with idiopathic cervical and 83% of those with idiopathic upper limb dystonia had a hyperechogenic lesion of the middle segment of the lenticular nucleus on the side opposite to the clinical dystonic symptoms. The ipsilateral side was also affected in 20%. In facial dystonia, only one-third of the patients revealed lenticular nucleus lesions. The mean area of the lenticular nucleus lesion opposite to the clinically affected side was 30 mm2 in cervical dystonia, 17 mm2 in upper limb dystonia, and 7.5 mm2 in facial dystonia. These lenticular abnormalities were significantly more frequent (p < 0.001) and their areas were significantly greater (p < 0.001) compared with a control group of 50 patients afflicted with radiculopathy. There was a significant correlation of the severity of symptoms with the intensity of lenticular nucleus hyperechogenicity in patients with cervical and upper limb dystonia (p < 0.05). Increased caudate nucleus echogenicity was present in 20% of patients with cervical and upper limb dystonia, mostly contralateral to the clinically affected side and raphe abnormalities were present in 7% of all patients with idiopathic dystonia. In contrast, there were no abnormalities of the lenticular nucleus or thalamus in nonidiopathic dystonias. We conclude that idiopathic dystonia is associated with lesions in the basal ganglia, particularly the lenticular nucleus, that can be visualized by TCS. An alteration of the basal ganglia matrix may be the pathologic basis of idiopathic dystonia with secondary affliction of striatopallidothalamic pathways.
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Cuerpo Estriado/diagnóstico por imagen , Distonía/diagnóstico por imagen , Adulto , Anciano , Ganglios Basales/diagnóstico por imagen , Ganglios Basales/patología , Cuerpo Estriado/patología , Distonía/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , UltrasonografíaRESUMEN
To detect morphologic abnormalities in Parkinson's disease (PD), we examined 30 patients with PD and 30 age- and sex-matched nonparkinsonian controls by transcranial color-coded real-time sonography (TCCS). In 12 severely affected PD patients, the echogenicity of the substantia nigra was distinctly increased. In the remaining 18 PD patients and in all controls, the substantia nigra was poorly visualized or nondetectable by TCCS. The degree of hyperechogenicity of the substantia nigra closely correlated with the severity and duration of PD (p < 0.001). The increased echogenicity of the substantia nigra notably results from nigral gliosis and reflects the stage of degeneration.
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Enfermedad de Parkinson/diagnóstico por imagen , Sustancia Negra/diagnóstico por imagen , Ultrasonografía Doppler Transcraneal , Anciano , Antiparkinsonianos/uso terapéutico , Femenino , Humanos , Masculino , Examen Neurológico , Enfermedad de Parkinson/tratamiento farmacológico , Enfermedad de Parkinson/fisiopatología , Sustancia Negra/anatomía & histología , Sustancia Negra/patologíaRESUMEN
OBJECTIVE: To investigate the usefulness of eyeball pressure testing (EP) as an indicator for impending serious bradyarrhythmias in patients with Guillain-Barré syndrome (GBS) and its relationship to motor disability. BACKGROUND: Autonomic dysfunction is a common complication in GBS and accounts for a significant number of deaths. Serious bradyarrhythmias are thought to occur only in severe cases but are difficult to predict. METHODS/DESIGN: In 13 consecutive patients with GBS aged 29 to 70 years, 156 EP (6 to 19 per patient) were done serially for up to 1 year. Bilateral moderate pressure was manually applied and sustained for 25 seconds or until abnormal bradycardia developed, defined as heart rate below 40 beats per minute. Disability was graded by a score from 0 to 5 (DS). RESULTS: Four of 13 patients (DS 2/2/3-4/5) showed abnormal sensitivity to EP at least once. In two of them, vagal overreactivity could be demonstrated repeatedly, which gradually resolved within 4 and 10 days. In one patient with a rapid progressive course requiring early cardiopulmonary resuscitation, a highly abnormal EP could be recorded until 1 day after heart arrest. Another patient (DS 3-4) with abnormal EP required cardiac pacing twice because of significant bradycardia. The only other event necessitating pacing occurred in a severely disabled patient (DS 5-4) who never showed abnormal EP. CONCLUSIONS: Vagal overreactivity could be demonstrated in approximately 30% of our patients. It was not restricted to severe motor impairment and was also present in mild-to-moderately disabled patients. In this regard, EP may be a simple and useful bedside test to indicate an increased risk of developing serious bradyarrhythmias in patients with GBS.
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Bradicardia/fisiopatología , Presión Intraocular , Polirradiculoneuropatía/fisiopatología , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Guillain-Barré syndrome (GBS), chronic idiopathic demyelinating polyradiculoneuropathy (CIDP), and multiple sclerosis (MS) are disorders with presumed immunopathogenesis. To obtain evidence for T cell activation, we determined serum concentrations of soluble interleukin-2 receptors (sIL-2 R) in 50 patients with GBS, 24 with CIDP, and 54 with MS. Both in GBS and clinically active MS sIL-2 R levels were markedly increased compared with those in patients with other neurologic diseases. Four of 24 CIDP patients had abnormally increased sIL-2 R concentrations. sIL-2 R concentrations decreased with clinical improvement in serial samples taken from GBS patients, but were not otherwise correlated with disease severity. These data establish that T cells are activated in GBS and some patients with CIDP, and corroborate earlier evidence that activated T cells are circulating in the blood of MS patients.
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Activación de Linfocitos/inmunología , Esclerosis Múltiple/inmunología , Polirradiculoneuropatía/inmunología , Receptores de Interleucina-2/sangre , Linfocitos T/inmunología , Enfermedad Crónica , Enfermedades Desmielinizantes/sangre , Enfermedades Desmielinizantes/inmunología , Ensayo de Inmunoadsorción Enzimática , Humanos , Esclerosis Múltiple/sangre , Polirradiculoneuropatía/sangreRESUMEN
Intercellular adhesion molecule ICAM-1 has a crucial role in the induction of an immune response and is instrumental in migration of T cells into inflamed tissue. We studied soluble ICAM-1 concentrations in patients with multiple sclerosis (MS), viral encephalitis, and other immunologic diseases, and compared results with those in other noninflammatory, nondemyelinating neurologic disorders as well as in healthy controls. MS patients with clinically active disease or enhancing lesions on MRI had elevated serum levels of soluble ICAM-1. Concentrations of soluble ICAM-1 were also increased in some patients with viral encephalitis. These findings raise the possibility that circulating ICAM-1 serves as a marker of acute inflammatory events in the brain and add to evidence implicating this adhesion molecule in the pathogenesis of MS.