Differential levels of Neurofilament Light protein in cerebrospinal fluid in patients with a wide range of neurodegenerative disorders.

Cerebrospinal fluid (CSF) biomarkers are useful in the diagnosis and the prediction of progression of several neurodegenerative diseases. Among them, CSF neurofilament light (NfL) protein has particular interest, as its levels reflect neuroaxonal degeneration, a common feature in various neurodegenerative diseases. In the present study, we analyzed NfL levels in the CSF of 535 participants of the SPIN (Sant Pau Initiative on Neurodegeneration) cohort including cognitively normal participants, patients with Alzheimer disease (AD), Down syndrome (DS), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). We evaluated the differences in CSF NfL accross groups and its association with other CSF biomarkers and with cognitive scales. All neurogenerative diseases showed increased levels of CSF NfL, with the highest levels in patients with ALS, FTD, CBS and PSP. Furthermore, we found an association of CSF NfL levels with cognitive impairment in patients within the AD and FTD spectrum and with AD pathology in DLB and DS patients. These results have implications for the use of NfL as a marker in neurodegenerative diseases.

[Video-EEG monitoring and simultaneous ECG for the differential diagnosis of transient consciousness disorders. A case report]. / Monitorización con vídeo-EEG y ECG simultáneo para el diagnóstico diferencial de trastornos de conciencia transitorios. A propósito de un caso.

We present the case of a 36 year-old woman, with history of transient consciousness disorders with vegetative state, interpreted as epileptic crises and treated with valproate for two years. After nine asymptomatic years, they reappeared associated with migraine, vomiting and some generalized convulsions. Electroencephalogram and cerebral magnetic resonance turned out normal, and treatment with zonisamide was started, without beneficial results. Later cardiological studies objectified a blockage of the left branch that coincided with dizziness. The study was completed with Video-EGG monitoring, where there was an episode that showed temporary right epileptiform activity, with a diagnosis established of focal epilepsy of unknown cause. At present, she remains asymptomatic with oxycarbazepine.

Hombro doloroso hemipléjico en pacientes con ictus: causas y manejo / Painful hemiplegic shoulder in stroke patients: causes and management

Introducción: El hombro doloroso hemipléjico es frecuente después de un ictus. Su aparición conlleva además del dolor, una limitación para las actividades de la vida diaria, así como para la participación en programas específicos de neurorrehabilitación. Todo este conjunto determina un peor resultado funcional. El buen manejo de los pacientes puede reducir tanto la frecuencia de aparición de hombro doloroso, como la intensidad del mismo, mejorando así el pronóstico funcional.Desarrollo: Entre los años 1980 y 2008 se llevó a cabo una búsqueda de la literatura en diferentes bases de datos. La evaluación de los artículos se realizó con el sistema de puntuación PEDro. Se establecieron 5 niveles de evidencia para obtener las conclusiones.Conclusiones: La subluxación del hombro, ocurre de manera precoz tras el ictus y se asocia con subluxación de la articulación del hombro y con espasticidad (subescapular y pectoral mayor principalmente). Los cabestrillos previenen la subluxación del hombro. Es preferible realizar movimientos con un menor rango de movimiento y sin agresividad, para evitar la aparición del hombro doloroso. La inyección de corticoides no mejora el dolor ni el rango de movimiento de los pacientes hemipléjicos, mientras que la toxina botulínica combinada con fisioterapia parece reducir el dolor del hombro hemipléjico (AU)

Introduction: The hemiplegic shoulder pain is common after a stroke. Its appearance brings pain and limits daily living activities as well as participation in specific Neuro-rehabilitation programs. All this leads to a worse functional outcome. Good management of patients can reduce both the frequency and intensity of shoulder pain, improving functional outcome.Development: We conducted a literature search of various databases between 1980 and 2008. The articles were evaluated using the PEDro scoring system. Five evidence levels were established for the conclusions.Conclusions: Shoulder subluxation, occurs at an early stage after stroke and is associated with subluxation of the shoulder joint and spasticity (mainly subscapularis and pectoralis). Slings prevent subluxation of the shoulder. It is preferable to move within a lower range of motion and without aggression to prevent the occurrence of shoulder pain. The injection of corticosteroids does not improve pain and range of motion in hemiplegic patients, while botulinum toxin combined with physical therapy appears to reduce hemiplegic shoulder pain (AU)

Monitorización con vídeo-EEG y ECG simultáneo para el diagnóstico diferencial de trastornos de conciencia transitorios. A propósito de un caso / Video-EEG monitoring and simultaneous ECG for the differential diagnosis of transient consciousness disorders. A case report

Se presenta el caso de una mujer de 36 años, con antecedentes de trastornos de conciencia transitorios con cortejo vegetativo en la adolescencia, interpretados como crisis epilépticas y tratados con ácido valproico durante dos años. Tras permanecer nueve años asintomática, reaparecieron asociados a migraña, vómitos y alguna convulsión generalizada. El electroencefalograma y la resonancia magnética cerebral resultaron normales e inició tratamiento con zonisamida, sin beneficio. Estudios cardiológicos posteriores objetivaron bloqueo de rama izquierda, que coincidía con mareo. Se completó el estudio mediante monitorización con vídeo-EEG, donde tuvo un episodio que evidenció actividad epileptiforme temporal derecha, estableciéndose el diagnóstico de epilepsia focal de causa desconocida. Actualmente, permanece asintomática con oxcarbacepina. La anamnesis es fundamental para diferenciar trastornos de conciencia transitorios, especialmente síncopes y crisis epilépticas, pero no siempre es suficiente. La monitorización con vídeo-EEG y registro simultáneo de electrocardiograma resulta muy útil para distinguirlos(AU)

We present the case of a 36 year-old woman, with history of transient consciousness disorders with vegetative state, interpreted as epileptic seizures and treated with valproic acid for two years. After nine asymptomatic years, they reappeared associated with migraine, vomiting and some generalized convulsions. Electroencephalogram and cerebral magnetic resonance turned out normal, and treatment with zonisamide was started, without beneficial results. Later cardiological studies objectified a blockage of the left branch that coincided with dizziness. The study was completed with Video-EEG monitoring, where there was an episode that showed right temporal epileptiform activity, with a diagnosis established of focal epilepsy of unknown etiology. At present, she remains asymptomatic with oxycarbazepine(AU)

[Painful hemiplegic shoulder in stroke patients: causes and management]. / Hombro doloroso hemipléjico en pacientes con ictus: causas y manejo.

INTRODUCTION: The hemiplegic shoulder pain is common after a stroke. Its appearance brings pain and limits daily living activities as well as participation in specific Neuro-rehabilitation programs. All this leads to a worse functional outcome. Good management of patients can reduce both the frequency and intensity of shoulder pain, improving functional outcome. DEVELOPMENT: We conducted a literature search of various databases between 1980 and 2008. The articles were evaluated using the PEDro scoring system. Five evidence levels were established for the conclusions. CONCLUSIONS: Shoulder subluxation, occurs at an early stage after stroke and is associated with subluxation of the shoulder joint and spasticity (mainly subscapularis and pectoralis). Slings prevent subluxation of the shoulder. It is preferable to move within a lower range of motion and without aggression to prevent the occurrence of shoulder pain. The injection of corticosteroids does not improve pain and range of motion in hemiplegic patients, while botulinum toxin combined with physical therapy appears to reduce hemiplegic shoulder pain.

[Paraneoplasic polyneuropathy with anti-amphiphysin antibodies]. / Polineuropatía paraneoplásica con anticuerpos anti-anfifisina.

Paraneoplastic neurological syndromes consist of a dysfunction of any part of the nervous system, in isolation or in combination, caused by a malign neoplasia, but not by the direct tissular or metastasic invasion of the tumour. Their pathogeny is explained by immunological mechanisms and they are characterised by the presence of high rates of antibodies in serum and cerebrospinal fluid. We present the case of a patient with a sensitive neuropathy that produced ataxia, and who suffered from a poorly differentiated adenocarcinoma of the lung, in whom the search for antineuronal antibodies was positive for antiamphiphysin antibodies, supporting the diagnosis of paraneoplastic polyneuropathy.

[Neuromuscular disorders in critically ill patients]. / Alteraciones neuromusculares del paciente crítico.

INTRODUCTION AND OBJECTIVE: Critically ill patients admitted to the Intensive Care Unit (ICU) often develop neuromuscular disorders. The objective of this study was to diagnose these and determine the causes. MATERIAL AND METHODS: We present a series of 13 critically ill patients who developed weakness or paresia, reduced or absent ROT and normal brain stem reflexes, in whom ENG and EMG studies were done in EESS and II which were considered together with data from general laboratory analysis, radiological and microbiological examinations, medication given and posterior clinical course of the patient. Muscle biopsy was not done in any patient. RESULTS: All the patients were intubated, with signs of sepsis, multiple-organ failure and malnutrition. All had received cortico-steroids and amino-glucosides and 8/13 neuromuscular blockers. Neurophysiological study showed that in all cases there was axon type neuropathy, mainly motor and in the lower limbs. Fifty four percent of the patients died. The neuropathy improved in the others. CONCLUSIONS: Critically ill patients often have axon type neuropathy. In our series, the causes of this were sepsis and multiple organ failure. It is important that this pathology be ruled out in the critically ill patient whom it is difficult to disintubate and/or has generalized muscle weakness.

[Conduction and origin of evoked potentials in the somesthetic pathway (author's transl)]. / Conducción y origen de los potenciales evocados en la vía somestésica.

In 24 normal subjects, sensory conduction velocity increased progressively from the distal to the proximal segments of the peripheral nervous system. In the central somatosensory pathway, sensory impulses slowed down at a rate much greater than what could be expected from just the three known synaptic delays (1 ms each) in medulla, thalamus and somatosensory cortex. The present data point to the special characteristic of conduction in the thalamo-cortical axons as the main cause for the deceleration observed in the central somatosensory pathway. A conduction velocity of 14 m/s has been calculated for this last segment. However it is also possible that sensory conduction in the medial lemniscus might be reduced in relation to the conduction values in the peripheral segments.

[Alterations of previous potentials of somatosensory cerebral evoked potential after lesions of the brain-stem (author's transl)]. / Alteración de los potenciales previos del potencial evocado somatosensorial en lesiones de tronco encefálico.

The characteristics of previous potentials of somatosensory evoked potential in 12 patients with lesions of the brain-stem and in 24 normal individuals have been studied. Lesions of the brain-stem were confirmed by means of computerized axial tomography, arteriography or necropsy. According to data reported by other authors somatosensory evoked potential is anteceded by three previous potentials with central latencies of 10, 12, and 14 milliseconds. Brain-stem lesions which do not involve the medial lemniscus showed normal potencials, while extensive lesions of the protuberance abolish P-12 potential and increase central latencies of P-14 and somatosensory evoked potential. P-12 potential originates in the medial lemniscus and P-14 potential in the thalamic nuclei. The present results confirm the interest of previous potentials of somatosensory evoked potential, as well as other neurophysiologic techniques, in the evaluation of lesions of the brain-stem.

Neurophysiological assessment of alpha pattern coma.

Somatosensory evoked potentials, blink reflexes, and H wave reflexes, were recorded on several days from three patients with alpha pattern coma. Coma was secondary to cardiac arrest in two cases and to brainstem infarction in one. Results are compatible with damage to the brainstem reticular formation with sparing of thalamo-cortical circuits as the main physiopathological characteristic of alpha pattern coma. This condition should not be regarded as a discrete entity when establishing the prognosis of patients in coma, since they only differ from other patients in coma from the point view of the EEG record.

Conduction velocity through the somesthetic pathway in chronic renal failure.

The latencies of evoked potentials recorded at different levels of the peripheral and central nervous system by median nerve stimulation were measured before and after dialysis, and compared with similar recordings in normal volunteers. Abnormally slow conduction velocity was more often found in proximal rather than in distal segments of the peripheral pathway in patients with chronic renal failure. This abnormality was not correlated with the presence of clinical signs of neuropathy or low values of motor conduction velocity, neither was it affected by dialysis. These findings support the hypothesis of peripheral nerve dysfunction secondary to metabolic derangement in uraemic patients.