The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series ...of anti-NMDAR encephalitis.
We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously.
A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode.
Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.
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?
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.
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.
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.
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.
Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) and post-COVID condition can present similarities such as fatigue, brain fog, autonomic and neuropathic symptoms.
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.
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.
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.
•Wireless sensor nodes for generic signal conditioning are presented.•The wireless signal acquisition of the presented sensor nodes is synchronous, achieving a maximum synchronization jitter of ...±1μs.•The generic signal conditioning circuit enables the use of the nodes for a wide range of measurements from strain to vibration.•The presented sensor nodes operation is demonstrated for SHM of a wind turbine, where the vibrational modes of the structure are detected.
Condition monitoring, fault diagnosis and Structural Health Monitoring (SHM) are an essential part of predictive maintenance. In order to provide early warning of damage, wireless sensor nodes are located at structures, which are often in hostile and hard to access environments. Therefore, there is a need of using sensor nodes with low power signal conditioning electronics, wide range measurement, synchronous data acquisition and reliable signal transmission within large scale-networks.
The sensor nodes developed in this work contain a transducer, a signal conditioning circuit and a signal processing software, and are powered by a small rechargeable lithium-ion polymer battery. The signal conditioning circuit is generic and configurable, enabling the conditioning of accelerometers and strain gages, and the signal acquisition of the nodes is performed through wireless synchronized communication.
The performance of the presented device is demonstrated for SHM of wind turbines. The commonly used system for SHM of wind turbines is installed during the construction of the structure and consists of wired sensors connected to a central data acquisition unit via cables. In this respect, the system developed in this work, which forms a wireless sensor network (WSN) for SHM, is compared to the commonly used system for SHM of wind turbines. The developed WSN offers two main advantages with respect to commonly used systems for SHM: installation and maintenance cost, and time, high reduction.
BACKGROUND AND OBJECTIVEMultiple system atrophy is a rare and fatal neurodegenerative disorder, characterized by autonomic dysfunction in association with either parkinsonism or cerebellar signs. The ...pathologic hallmark is the presence of alpha-synuclein aggregates in oligodendrocytes, forming glial cytoplasmic inclusions. Clinically, it may be difficult to distinguish form other parkinsonisms or ataxias, particularly in the early stages of the disease. In this case series we aim to describe in detail the features of MSA patients.MATERIAL AND METHODSUnified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease.RESULTSUMSARS proved to be useful to perform a follow-up being longitudinal examination essential to stratify risk of poor outcome. Neuropathological diagnosis showed an overlap between parkinsonian and cerebellar subtypes, with some peculiarities that could help to distinguish from other subtypes.CONCLUSIONA better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.
The aim of the present study is to analyse the influence that motor and non-motor symptoms have on the quality of life (QoL) of patients with Parkinson's disease (PD), and to study the relationship ...between the two types of symptoms.
This cross-sectional study included 103 patients with PD (55 men and 48 women). Quality of life was measured on the PDQ-39 scale. The UPDRS scale (I-IV) was also used, and different items were grouped to analyse the presence of tremor, rigidity, bradykinesia, and axial symptoms. The non-motor symptoms scale (NMSS) was administered to assess non-motor symptoms. We performed correlation analyses between different scales to analyse the influence of motor and non-motor symptoms on QoL.
Correlations were observed between the PDQ-39 summary index (PDQ39_SI) and the NMSS (correlation coefficient cc, 0.56; p<.001), UPDRS III (cc, 0.44; p< .001) and UPDRS IV (cc, 0.37; p<.001) scores. The strongest correlation was between cognitive symptoms and mood. The analysis pointed to a direct relationship between the NMSS score and axial symptoms (cc, 0.384; p<.01), bradykinesia (cc, 0.299; p<.01), and to a lesser extent, rigidity (cc, 0.194; p<.05). No relationship was observed between presence of tremor and the NMSS score.
Cognitive symptoms and mood exert the most influence on QoL of patients with PD. We found at least two phenotypes; one with predominantly axial symptoms, with significant involvement of non-motor symptoms, and a tremor-associated phenotype in which these symptoms are less prevalent.
Multiple system atrophy is a rare and fatal neurodegenerative disorder, characterized by autonomic dysfunction in association with either parkinsonism or cerebellar signs. The pathologic hallmark is ...the presence of alpha-synuclein aggregates in oligodendrocytes, forming glial cytoplasmic inclusions. Clinically, it may be difficult to distinguish form other parkinsonisms or ataxias, particularly in the early stages of the disease. In this case series we aim to describe in detail the features of MSA patients.
Unified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease.
UMSARS proved to be useful to perform a follow-up being longitudinal examination essential to stratify risk of poor outcome. Neuropathological diagnosis showed an overlap between parkinsonian and cerebellar subtypes, with some peculiarities that could help to distinguish from other subtypes.
A better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.
La atrofia multisistémica es un trastorno neurodegenerativo raro y letal que se caracteriza por una disfunción autonómica en asociación con parkinsonismo o signos cerebelosos. La marca anatomopatológica es la presencia de agregados de α-sinucleína en los oligodendrocitos, que forman inclusiones citoplasmáticas gliales. Desde un punto de vista clínico, puede ser difícil de distinguir de otros parkinsonismos o ataxias, particularmente en las primeras etapas de la enfermedad. En esta serie de casos, nuestro objetivo es describir en detalle las características de los pacientes con atrofia multisistémica.
Se resumen los datos objetidos de la puntuación de la Escala de calificación unificada de la atrofia multisistémica (UMSARS), imágenes estructurales y funcionales y las pruebas autonómicas cardiovasculares realizadas desde las primeras etapas de la enfermedad.
La escala UMSAR demostró ser útil para hacer un seguimiento: el examen longitudinal esencial fue para estratificar el riesgo de peor evolución. El diagnóstico neuropatológico mostró un solapamiento entre los subtipos parkinsoniano y cerebeloso, con algunas peculiaridades que podrían ayudar a distinguir los subtipos.
Una mejor descripción de las características de la atrofia multisistémica en casos confirmados mediante neuropatología podría ayudar a aumentar la sensibilidad del diagnóstico.
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.
5 CPRS cases (2 women/3 men) that met the Budapest criteria for the diagnosis of CRPS.
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.
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.
Proponemos un protocolo para el estudio del síndrome de dolor regional complejo (SDRC) basado en una batería de medidas cuantitativas (termografía cutánea, conductancia electroquímica cutánea y umbrales sensoriales en la prueba sensorial cuantitativa QST) y aplicamos dicho protocolo a cinco casos representativos de SDRC.
Se presentan cinco casos de SDRC (dos mujeres/tres hombres) que cumplieron con los criterios de Budapest para el diagnóstico de SDRC.
Todos los pacientes presentaron dolor espontáneo y alodinia. Dos casos corresponden a un estadio I, en ambos, la temperatura basal de reposo se incrementó en el miembro afectado. Tres casos muestran estadios más avanzados con disminución de la temperatura de reposo y retraso en la recuperación de la temperatura, en comparación con la extremidad contralateral, que reflejan fases más avanzadas de la enfermedad.
Estas pruebas funcionales cuantitativas no invasivas no solo mejoran la precisión diagnóstica del SDRC sino que también nos ayudan a estratificar las diferentes fases y comprender los procesos patológicos de la enfermedad.
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.
5 CPRS cases (2 women/3 men) that met the Budapest criteria for the diagnosis of CRPS.
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.
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.
Proponemos un protocolo para el estudio del síndrome de dolor regional complejo (SDRC) basado en una batería de medidas cuantitativas (termografía cutánea, conductancia electroquímica cutánea y umbrales sensoriales en la prueba sensorial cuantitativa QST) y aplicamos dicho protocolo a cinco casos representativos de SDRC.
Se presentan cinco casos de SDRC (dos mujeres/tres hombres) que cumplieron con los criterios de Budapest para el diagnóstico de SDRC.
Todos los pacientes presentaron dolor espontáneo y alodinia. Dos casos corresponden a un estadio I, en ambos, la temperatura basal de reposo se incrementó en el miembro afectado. Tres casos muestran estadios más avanzados con disminución de la temperatura de reposo y retraso en la recuperación de la temperatura, en comparación con la extremidad contralateral, que reflejan fases más avanzadas de la enfermedad.
Estas pruebas funcionales cuantitativas no invasivas no solo mejoran la precisión diagnóstica del SDRC sino que también nos ayudan a estratificar las diferentes fases y comprender los procesos patológicos de la enfermedad.