RESUMEN
BACKGROUND: It is estimated that 240,000 people suffer from multiple sclerosis in Germany. In addition to sensory, motor, vegetative, and neuropsychological functional deficits, dysphagia is a highly relevant and disabling, although not well studied symptom of MS. OBJECTIVES: The purpose of this article is to provide an overview of the scientific background, increase awareness of the symptoms of dysphagia, and to introduce diagnostic tools for its management, overall aiming at alleviating symptoms of dysphagia in persons with MS, and improving their quality of life. METHODS: A structured literature review was conducted of what is currently known on the development, manifestation, diagnosis and treatment options for MS-related dysphagia. Due to the lack of class 1 evidence, in particular for diagnosis and treatment options of dysphagia, also smaller studies or pilot projects were included and discussed in this review. RESULTS: Data from imaging methods such as Flexible Endoscopic Evaluation of Swallowing and Videofluoroscopic Swallowing Evaluation enabled the diagnosis. There was a high variablity in the reported frequency of dysphagia in published studies, largely related to differences in methodology to evaluate the swallowing (from 38 % up to 81 %). Overall, dysphagia as a symptom of multiple sclerosis was underestimated at the patient and the physician level. According to current data from the German MS register, specific treatments were only carried out in 30% of the affected patients.
Asunto(s)
Trastornos de Deglución , Esclerosis Múltiple , Deglución , Trastornos de Deglución/diagnóstico , Trastornos de Deglución/etiología , Alemania/epidemiología , Humanos , Esclerosis Múltiple/complicaciones , Calidad de VidaRESUMEN
BACKGROUND: Small fiber neuropathy (SFN) is a challenging subtype of peripheral neuropathies. Once the diagnosis has been established, there is an uncertainty how SFN may progress, whether larger fibers will become involved over time, whether quality of life may be compromised, or whether repeated diagnostic workup in patients with unknown underlying cause may increase the yield of treatable causes of SFN. METHODS: We evaluated 16 patients with documented long-time course of idiopathic SFN. RESULTS: Clinical and electrophysiological course remained stable in 75% of the patients, while 25% SFN-patients developed large fiber neuropathies. CONCLUSIONS: Our data suggest that SFN represents a benign disease course in the majority of patients without severely limiting the quality of life.
Asunto(s)
Calidad de Vida , Neuropatía de Fibras Pequeñas/patología , Adulto , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neuropatía de Fibras Pequeñas/complicacionesRESUMEN
HIV long-term nonprogressors (LTNPs) maintaining high CD4(+) T-cell counts without antiretroviral therapy (ART) are divided into elite controllers (ECs) with undetectable and viremic controllers (VCs) with low viral loads. Little is known about the long-term changes of T-cell subsets and inflammation patterns in ECs versus VCs. The aim of the study was to explore the long-term evolution of CD4(+) T-cell levels in LTNPs and to analyze cytokine profiles in ECs versus VCs. Nineteen ECs and 15 VCs were enrolled from the natural virus controller cohort (NaViC). T-cell counts were monitored over years, the mean annual change was calculated, and plasma concentrations of 25 cytokines were evaluated using a multiplex bead array. While absolute numbers of T cells did not differ between ECs and VCs over time, we observed a significant decrease of CD4(+) T-cell percentages in VCs, but not in ECs (median [interquartile range]: ECs: 37% [28-41] vs. VCs: 29% [25-34]; p = .02). ECs had lower levels of macrophage inflammatory protein-1ß (MIP-1ß, p = .003), interferon γ-induced protein-10 (IP-10, p = .03), and monokine induced by interferon-γ (MIG, p = .02). CD4(+) T-cell percentages inversely correlated with MIP 1-ß (r = -0.42, p = .017) and IP-10 (r = -0.77, p < .0001). A subtle decline of CD4(+) T-cell percentages could be observed in VCs, but not in ECs, which was associated with higher plasma levels of proinflammatory cytokines. Hence, even low levels of HIV replication might go along with a progressive decline in CD4(+) T-cell counts in LTNPs.
Asunto(s)
Citocinas/sangre , Infecciones por VIH/inmunología , Sobrevivientes de VIH a Largo Plazo , Adulto , Recuento de Linfocito CD4 , Estudios de Cohortes , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Pharmacological treatment of dyskinetic cerebral palsy (CP) is often ineffective. Data about outcome of deep brain stimulation (DBS) in these patients remains scarce. METHODS: Eight patients with dyskinetic CP and DBS of the Globus Pallidus internus were investigated. Using pre- and postoperative videos the severity of dystonia and changes thereof during standardized settings ('on') and after the stimulator had been switched off ('off') were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). Furthermore, subjective impression (SI) of the extent of postoperative change as well as gait (Leonardo Mechanograph® Gangway), speech (Frenchay Dysarthria) and swallowing performances (fiberoptic laryngoscopy) were assessed during 'on' and 'off'. RESULTS: When comparing pre- and postoperative as well as 'on' and 'off', the BFMDRS and most of the gait, speech, and swallowing parameters did not differ significantly. In contrast, patients reported significant improvement of their SI postoperatively (3.1 on a 10-point-scale). CONCLUSION: Data show that our CP-patients did not benefit from GPi-DBS when tested formally for dystonia, gait, speech and swallowing. In stark contrast, these patients reported significant subjective improvement. Taken together, and in light of current unsatisfactory medical treatment options, our data suggest that further assessment of the effects of GPi-DBS in dyskinetic CP is warranted.