Dysautonomia and small fiber neuropathy in post-COVID condition and Chronic Fatigue Syndrome.

BACKGROUND: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and post-COVID condition can present similarities such as fatigue, brain fog, autonomic and neuropathic symptoms. METHODS: The study included 87 patients with post-COVID condition, 50 patients with ME/CFS, and 50 healthy controls (HC). The hemodynamic autonomic function was evaluated using the deep breathing technique, Valsalva maneuver, and Tilt test. The presence of autonomic and sensory small fiber neuropathy (SFN) was assessed with the Sudoscan and with heat and cold evoked potentials, respectively. Finally, a complete neuropsychological evaluation was performed. The objective of this study was to analyze and compare the autonomic and neuropathic symptoms in post-COVID condition with ME/CFS, and HC, as well as, analyze the relationship of these symptoms with cognition and fatigue. RESULTS: Statistically significant differences were found between groups in heart rate using the Kruskal-Wallis test (H), with ME/CFS group presenting the highest (H = 18.3; p ≤ .001). The Postural Orthostatic Tachycardia Syndrome (POTS), and pathological values in palms on the Sudoscan were found in 31% and 34% of ME/CFS, and 13.8% and 19.5% of post-COVID patients, respectively. Concerning evoked potentials, statistically significant differences were found in response latency to heat stimuli between groups (H = 23.6; p ≤ .01). Latency was highest in ME/CFS, and lowest in HC. Regarding cognition, lower parasympathetic activation was associated with worse cognitive performance. CONCLUSIONS: Both syndromes were characterized by inappropriate tachycardia at rest, with a high percentage of patients with POTS. The prolonged latencies for heat stimuli suggested damage to unmyelinated fibers. The higher proportion of patients with pathological results for upper extremities on the Sudoscan suggested a non-length-dependent SFN.

Usefulness of thermography in the diagnosis and classification of complex regional pain syndrome.

INTRODUCTION: We propose a protocol for study of complex regional pain syndrome (CRPS) based on a battery of quantitative measures (skin thermography, electrochemical skin conductance and sensory thresholds) and apply such protocol to 5 representative cases of CRPS. PATIENTS AND METHODS: 5 CPRS cases (2 women/3 men) that met the Budapest criteria for the diagnosis of CRPS. RESULTS: All patients showed spontaneous pain and allodynia. Two cases correspond to a stage I, in both the resting basal temperature was increased in the affected limb. Three cases reflect more advanced stages with a decrease in resting temperature and a delay in the recovery of the temperature when compared to contralateral limb. DISCUSSION: These non-invasive quantitative functional tests not only improve the diagnostic accuracy of CRPS but also, they help us to stratify and understand the pathological processes of the disease.

Usefulness of thermography in the diagnosis and classification of complex regional pain syndrome / Utilidad de la termografía en el diagnóstico y clasificación del síndrome de dolor regional complejo

Introduction: We propose a protocol for study of complex regional pain syndrome (CRPS) basedon a battery of quantitative measures (skin thermography, electrochemical skin conductanceand sensory thresholds) and apply such protocol to 5 representative cases of CRPS.Patients and methods: 5 CPRS cases (2 women/3 men) that met the Budapest criteria for thediagnosis of CRPS. Results: All patients showed spontaneous pain and allodynia. Two cases correspond to a stageI, in both the resting basal temperature was increased in the affected limb. Three cases reflectmore advanced stages with a decrease in resting temperature and a delay in the recovery ofthe temperature when compared to contralateral limb.Discussion: These non-invasive quantitative functional tests not only improve the diagnosticaccuracy of CRPS but also, they help us to stratify and understand the pathological processesof the disease.(AU)

Introducción: Proponemos un protocolo para el estudio del síndrome de dolor regionalcomplejo (SDRC) basado en una batería de medidas cuantitativas (termografía cutánea, con-ductancia electroquímica cutánea y umbrales sensoriales en la prueba sensorial cuantitativa[QST]) y aplicamos dicho protocolo a cinco casos representativos de SDRC. Pacientes y métodos: Se presentan cinco casos de SDRC (dos mujeres/tres hombres) quecumplieron con los criterios de Budapest para el diagnóstico de SDRC. Resultados: Todos los pacientes presentaron dolor espontáneo y alodinia. Dos casos correspon-den a un estadio I, en ambos, la temperatura basal de reposo se incrementó en el miembroafectado. Tres casos muestran estadios más avanzados con disminución de la temperatura dereposo y retraso en la recuperación de la temperatura, en comparación con la extremidadcontralateral, que reflejan fases más avanzadas de la enfermedad. Discusión: Estas pruebas funcionales cuantitativas no invasivas no solo mejoran la precisióndiagnóstica del SDRC sino que también nos ayudan a estratificar las diferentes fases y compren-der los procesos patológicos de la enfermedad.(AU)

Brain fog of post-COVID-19 condition and Chronic Fatigue Syndrome, same medical disorder?

BACKGROUND: Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) is characterized by persistent physical and mental fatigue. The post-COVID-19 condition patients refer physical fatigue and cognitive impairment sequelae. Given the similarity between both conditions, could it be the same pathology with a different precipitating factor? OBJECTIVE: To describe the cognitive impairment, neuropsychiatric symptoms, and general symptomatology in both groups, to find out if it is the same pathology. As well as verify if the affectation of smell is related to cognitive deterioration in patients with post-COVID-19 condition. METHODS: The sample included 42 ME/CFS and 73 post-COVID-19 condition patients. Fatigue, sleep quality, anxiety and depressive symptoms, the frequency and severity of different symptoms, olfactory function and a wide range of cognitive domains were evaluated. RESULTS: Both syndromes are characterized by excessive physical fatigue, sleep problems and myalgia. Sustained attention and processing speed were impaired in 83.3% and 52.4% of ME/CFS patients while in post-COVID-19 condition were impaired in 56.2% and 41.4% of patients, respectively. Statistically significant differences were found in sustained attention and visuospatial ability, being the ME/CFS group who presented the worst performance. Physical problems and mood issues were the main variables correlating with cognitive performance in post-COVID-19 patients, while in ME/CFS it was anxiety symptoms and physical fatigue. CONCLUSIONS: The symptomatology and cognitive patterns were similar in both groups, with greater impairment in ME/CFS. This disease is characterized by greater physical and neuropsychiatric problems compared to post-COVID-19 condition. Likewise, we also propose the relevance of prolonged hyposmia as a possible marker of cognitive deterioration in patients with post-COVID-19.

Usefulness of thermography in the diagnosis and classification of complex regional pain syndrome.

INTRODUCTION: We propose a protocol for study of complex regional pain syndrome (CRPS) based on a battery of quantitative measures (skin thermography, electrochemical skin conductance and sensory thresholds) and apply such protocol to 5 representative cases of CRPS. PATIENTS AND METHODS: 5 CPRS cases (2 women/3 men) that met the Budapest criteria for the diagnosis of CRPS. RESULTS: All patients showed spontaneous pain and allodynia. Two cases correspond to a stage I, in both the resting basal temperature was increased in the affected limb. Three cases reflect more advanced stages with a decrease in resting temperature and a delay in the recovery of the temperature when compared to contralateral limb. DISCUSSION: These non-invasive quantitative functional tests not only improve the diagnostic accuracy of CRPS but also, they help us to stratify and understand the pathological processes of the disease.

Corea/balismo secundaria a hiperglucemia no cetósica: serie de 4 casos / Chorea/ballism secondary to non-ketotic hyperglycaemia: Report of 4 cases

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[Cerebrovascular disease in a tertiary care hospital. The current situation and points to be improved]. / Patología cerebrovascular en un hospital terciario. Situación actual y puntos de mejora.

INTRODUCTION: Caring for patients suffering from a cerebrovascular diseases requires a large quantity of resources which must be optimised. The aim of this study is to analyse the management of stroke in a tertiary care hospital. PATIENTS AND METHODS: All admissions with a diagnosis of stroke were analysed retrospectively for the year 2003. Length of stay, computed tomography in the Emergency Room, origin, previous admissions during the last year, presence of vascular risk factors, stroke subtype, complications and mortality during admission and destination when discharged from hospital, were all recorded. RESULTS: 936 patients were admitted to hospital with a diagnosis of stroke. 80.22% corresponded to acute ischaemic strokes (27.14% lacunar, 18.57% transient ischaemic attacks, 10.25% cardioembolic, 15.44% aterothrombotic, 8.44% infarct of undetermined cause, 0.24% unusual aetiology) and 19.78% corresponded to haemorrhagic strokes (13.99% intraparenchymatous hemorrhage, 5.79% subarachnoid hemorrhage). Intra-hospital mortality was 5.3%. 11% suffered from complications while in hospital, and average length of stay was 10.4 days, being much longer for those patients discharged to a medium-long stay centre (17.5 days). Compared to other series, the incidence of cardioembolic and aterothrombotic subtypes of stroke is low. However, because of the inclusion of neurosurgical patients, an increase of cerebral haemorrhages is observed. CONCLUSIONS: Intra-hospital morbidity and mortality and average length of stay in our series are consistent with those from other centres of similar characteristics. A better coordination with medium-long stay centres along with the presence of neurologists on call, would certainly improve these variables.

Patología cerebrovascular en un hospital terciario. Situación actual y puntos de mejora / Cerebrovascular disease in a tertiary care hospital current situation and issues to be imporved

Introduction. Caring for patients suffering from a cerebrovascular diseases requires a large quantity of resources which must be optimised. The aim of this study is to analyse the management of stroke in a tertiary care hospital. Patients and methods. All admissions with a diagnosis of stroke were analysed retrospectively for the year 2003. Length of stay, computed tomography in the Emergency Room, origin, previous admissions during the last year, presence of vascular risk factors, stroke subtype, complications and mortality during admission and destination when discharged from hospital, were all recorded. Results. 936 patients were admitted to hospital with a diagnosis of stroke. 80,22% corresponded to acute ischaemic strokes (27,14% lacunar, 18,57% transient ischaemic attacks, 10,25% cardioembolic, 15,44% aterothrombotic, 8,44% infarct of undetermined cause, 0,24% unusual aetiology) and 19,78% corresponded to haemorrhagic strokes (13,99% intraparenchymatous hemorrhage, 5,79% subarachnoid hemorrhage). Intra-hospital mortality was 5,3%. 11% suffered from complications while in hospital, and average length of stay was 10,4 days, being much longer for those patients discharged to a medium-long stay centre (17,5 days). Compared to other series, the incidence of cardioembolic and aterothrombotic subtypes of stroke is low. However, because of the inclusion of neurosurgical patients, an increase of cerebral haemorrhages is observed. Conclusions. Intra-hospital morbidity and mortality and average length of stay in our series are consistent with those from other centres of similar characteristics. A better coordination with medium-long stay centres along with the presence of neurologists on call, would certainly improve these variables (AU)

Introducción. La atención a los pacientes con enfermedad cerebrovascular consume gran cantidad de recursos que se necesitan usar óptimamente. El objetivo del presente estudio es analizar la situación de la atención a los ictus en un hospital terciario. Pacientes y métodos. Se analizaron retrospectivamente todos los ingresos con diagnóstico de ictus durante el año 2003. Se registraron la duración de la estancia, la realización de tomografía axial computarizada en Urgencias, procedencia, ingresos previos en el último año, presencia de factores de riesgo vascular, subtipo de ictus, complicaciones y mortalidad durante el ingreso y destino al alta. Resultados. Ingresaron un total de 936 pacientes con diagnóstico de ictus. El 80,22% correspondían a ictus isquémicos (27,14% lacunares, 18,57% accidente isquémico transitorio, 10,25% cardioembólicos, 15,44% aterotrombóticos, 8,44% clasificación incierta, 0,24% causa inhabitual) y el 19,78% a formas hemorrágicas (13,99% hematomas, 5,79% hemorragia subaracnoidea). La mortalidad intrahospitalaria fue del 5,3%, el 11% presentaron algún tipo de complicación durante el ingreso y la estancia media fue de 10,4 días, muy superior en aquellos pacientes con destino al alta a un centro de media-larga estancia (17,5 días). Por subtipo de ictus se aprecia una menor incidencia de ictus cardioembólicos y aterotrombóticos que en otras series. Al incluir pacientes neuroquirúrgicos hay, en cambio, un aumento de hemorragias cerebrales. Conclusiones. La morbimortalidad intrahospitalaria y la estancia media de nuestra serie son acordes a las de los centros de similares características. La mejor coordinación con centros de media-larga estancia junto con la presencia de neurólogos de guardia y/o la creación de unidades de ictus, sin duda, podrían mejorar estos resultados (AU)

A novel mutation (K317M) in the MAPT gene causes FTDP and motor neuron disease.

BACKGROUND: Frontotemporal dementia with parkinsonism is often linked to chromosome 17 and is related to mutations in the MAPT gene. In some families the genetic basis is still unknown. The authors report two pedigrees with FTDP-17 harboring a novel mutation (K317M) in exon 11 in the MAPT gene. METHODS: The authors identified two apparently unrelated pedigrees with an autosomal dominant neurodegenerative condition. Thirteen patients were examined and eight autopsies were performed. RESULTS: Mean age at onset was 48 years. Mean disease duration was 6 years. Dysarthria often heralded the disease. All cases had parkinsonism and pyramidalism and half of them had amyotrophy. Behavioral or personality changes were not a prominent feature. Cognitive decline appeared late in the evolution. Neuropathologically, a massive degeneration of the substantia nigra without Lewy bodies was a constant finding. A variable degree of frontotemporal atrophy was found. Corticospinal tract degeneration and anterior horn neuron loss were present in six of seven autopsies in which the spinal cord was examined. An extensive deposition of abnormal tau protein in a mixed pattern (neuronal, glial) was observed. Pick's bodies were not seen. Biochemical analysis of tau revealed two bands of 64 and 68 kDa. CONCLUSION: Genetic analysis revealed the same novel mutation (K317M) in exon 11 of the MAPT gene in both pedigrees. A common haplotype between members of the two pedigrees suggests that they belong to the same family.

Post-transplantation HTLV-1 myelopathy in three recipients from a single donor.

OBJECTIVES: This paper reports for the first time three cases of infection by HTLV-I via organ transplantation; all the organs coming from the same asymptomatic infected donor. The need is considered for the implementation of compulsory screenings for HTLV antibodies on organ donors and on blood banks. METHODS: The determination of antibodies for HTLV-I/II on samples of serum and cerebral spinal fluid from the patients and the donor was performed by enzyme immunoassay and western blot. Analysis of proviral DNA was performed by polymerase chain reaction. To detect changes in the sequence of amino acids, the tax gene was sequentiated, amplified, and compared with ATK prototype stocks. Spinal cord magnetic resonance imaging, cerebral spinal fluid, and somatosensory evoked potential studies were carried out in all patients. RESULTS: All three transplanted patients developed a myelopathy within a very short period of time. In all three patients and donor the virus belonged to the Cosmopolitan A subtype. The homology of HTLV-I sequences recovered from the patients and donor was 100% in all four cases. Proviral load was high in all three patients. The factors that certainly contributed to the infection in the first place, and the development of the disease later, were on the one hand the high proviral load and their immunosuppressed condition, and on the other the virus genotype, which proved to be an aggressive variant. However, the analysis of the histocompatibility antigen showed that two of the patients carried an haplotype that has been associated with a lower risk of developing this disease. CONCLUSIONS: It is argued that, although in Spain and other European countries there is not compulsory screening for HTLV antibodies because of the studies that show a low seroprevalence, in view of the cases here reported, and to avoid the serious consequences that such infection has on transplanted patients, compulsory screenings, both on organ donors and on blood banks, should be implemented.

[Antiepileptic drugs used in childhood. New products and new concepts]. / Fármacos antiepilépticos utilizados en la infancia. Nuevos productos y nuevos conceptos.

INTRODUCTION: Eight new antiepileptic drugs (AED) have been marketed in Spain since 1990 and others will soon follow. OBJECTIVE: To review the concepts underlying the development of the new drugs, as well as their indications, efficacy and safety. DEVELOPMENT: Pharmacologic antiepileptic intervention is no longer solely directed towards an anticonvulsant effect, but also to epileptogenic prevention, disease modification and reversal of pharmacoresistance. The development of new AED, initially based on the screening of putative products in animal models, changed during the last half of the century to a rational design based on known facts about excitatory /inhibitory neuronal mechanisms. More recently, attention has focussed on pharmacogenetics. The new AED were initially indicated for partial epilepsies, but some have been shown to have a broader clinical spectrum. Some show the ideal pharmacokinetic mechanisms, avoiding hepatic metabolism and protein binding. Drug interactions and adverse effects, especially severe idiosyncratic adverse effects, are rare, although there are some exceptions. In most cases, however, seizure control does not seem to be better than with the classic AED. Because of the specific characteristics of childhood epilepsy and pharmacokinetics, as well as the regulations governing the development of clinical trials, the use of new products in children is circumspect, which in turn delays the access of such patients to a possible therapeutic benefit.

Fármacos antiepilépticos utilizados en la infancia. Nuevos productos y nuevos conceptos / Antiepileptic drugs used in childhood. New products and new concepts

Introducción: Desde 1990 se han comercializado en nuestro país ocho nuevos fármacos antiepilépticos (FAE), y otros más lo serán en un futuro próximo. Objetivo Revisar los conceptos que guían el desarrollo de los nuevos fármacos, indicaciones, mecanismo de acción, eficacia y tolerabilidad. Desarrollo La intervención farmacológica antiepiléptica no es ya puramente anticonvulsionante, sino que se dirige hoy en día a la prevención de la epileptogénesis, a la modificación del pronóstico de la enfermedad y a revertir la farmacorresistencia. El desarrollo de nuevos fármacos pasó desde la experimentación animal con diferentes productos, hacia el "diseño racional" de fármacos basado en los mecanismos conocidos de excitación/inhibición neuronal durante la segunda mitad de siglo, para encaminarse actualmente hacia la denominada farmacogenética. Los nuevos FAE estaban indicados inicialmente para las epilepsias focales, aunque algunos han demostrado poseer un espectro de acción más amplio. La farmacocinética de muchos de ellos posee las características ideales, eludiendo el metabolismo hepático y la unión a proteínas. Las interacciones medicamentosas y los efectos secundarios son menores, sobre todo en lo referido a reacciones idiosincrásicas graves, existiendo alguna excepción que conviene reseñar. La eficacia terapéutica, sin embargo, no es mayor que la de los fármacos clásicos en la mayoría de los casos. Las características específicas de la epilepsia y de la farmacocinética durante la infancia, además de las normas oficiales para el desarrollo de los ensayos clínicos, condicionan una utilización precavida de los nuevos FAE en la infancia, lo que simultáneamente retrasa la accesibilidad de los niños al posible beneficio terapéutico (AU)