[Argentinean consensus guidelines on the identification and clinical care of secondary progressive multiple sclerosis]. / Consenso sobre la identificación y seguimiento de la esclerosis múltiple secundaria progresiva en Argentina.

INTRODUCTION: The identification, diagnosis, follow-up, and treatment of patients with secondary progressive multiple sclerosis (SPMS) show significant differences between health care professionals in Argentina. AIM: To provide consensus recommendations on the management of patients with SPMS in Argentina to optimize patient care. DEVELOPMENT: A panel of expert neurologists from Argentina dedicated to the diagnosis and care of multiple sclerosis patients gathered during 2019 and 2020 to carry out a consensus recommendation on the diagnosis and treatment of SPMS patients in Argentina. To achieve consensus, the methodology of 'formal consensus-RAND/UCLA method' was used. Recommendations were established based on published evidence and the expert opinion. Recommendations focused on how to define SPMS and how to follow SPMS patients. CONCLUSION: The recommendations of this consensus guidelines attempt to optimize the care of SPMS patients in Argentina.

TITLE: Consenso sobre la identificación y seguimiento de la esclerosis múltiple secundaria progresiva en Argentina.Introducción. Existen diferencias significativas en el diagnóstico, la identificación y el seguimiento de pacientes con esclerosis múltiple secundaria progresiva (EMSP) entre los profesionales de la salud a cargo de su tratamiento. Objetivo. Proveer recomendaciones sobre el tratamiento de los pacientes con EMSP en Argentina con el fin de optimizar su cuidado. Desarrollo. Un grupo de neurólogos expertos en esclerosis múltiple de Argentina elaboró un consenso para el tratamiento de pacientes con EMSP en la región mediante metodología de ronda de encuestas a distancia y reuniones presenciales. Se establecieron 33 recomendaciones basadas en la evidencia publicada y en el criterio de los expertos que participaron. Las recomendaciones se enfocaron en el diagnóstico y el seguimiento de los pacientes con EMSP. Conclusión. Las recomendaciones establecidas en el presente consenso permitirían optimizar el cuidado y el seguimiento de los pacientes con EMSP en Argentina.

[Cerebral vasculitis]. / Vasculitis cerebrales.

INTRODUCTION: Cerebral vasculitis embraces a wide range of conditions that are characterised by involvement of the vessels of the central nervous system (CNS) due to inflammation of their walls, which in turn leads to occlusion or the formation of aneurysms with the ensuing ischaemic-haemorrhagic disorders this produces. DEVELOPMENT: In cases of cerebral vasculitis perhaps only the vessels of the CNS (isolated angiitis of the CNS) are involved, or they may be just another of the affected territories to be found in primary or secondary systemic angiitis (due to infection, collagen diseases, drugs, tumours). Neurological symptoms and lab tests are usually rather unspecific. The latest neuroimaging techniques are more useful, and the gold standard among them is digital angiography, although its sensitivity and specificity are limited. Brain tissue biopsy allows for confirmation of the diagnosis and is the gold standard for the diagnosis of isolated angiitis of CNS. A large group of conditions (which may be metabolic, demyelinating, vascular, infectious, and other peripheral vascular diseases) have similar clinical and imaging features, which makes it necessary to consider the differential diagnosis. CONCLUSIONS: Involvement of the nervous system casts a shadow over the prognosis in most cases of vasculitis and can be severe, as in isolated vasculitis of large vessels or in Takayasu's disease, or more benign, as in isolated vasculitis of small vessels and in other primary vasculitis. Treatment with corticoids and immunosuppressant agents, as well as anticoagulant and/or antiaggregating therapy, must be considered in each particular case according to the clinical condition and the progression of each patient.

[Mesial temporal sclerosis syndrome in adult patients]. / Síndrome de la esclerosis mesial temporal en pacientes adultos.

Magnetic resonance imaging (MRI) has become an essential tool in the work-up of epilepsy. Since its appearance it has been possible to identify pathologies, such as hippocampal sclerosis (HS), that had previously only been detected by histopathological assays. The aim of this study was to analyze the clinical manifestations, EEG and the outcome of patients with HS as shown by MRI. We revised the clinical histories of 384 outpatients from the Epilepsy Center, Ramos Mejía Hospital, who had been studied by MRI. Thirty five of them (15.5%) had a diagnosis of HS, based on the structural changes observed on the images. Six patients were excluded because of incomplete clinical data. Therefore, we studied 29 patients including 15 men. The mean age was 32.7 +/- 10.2 years (range: 19-58). All of them had partial seizures. Ten subjects had had febrile convulsions (34.5%) in childhood. Neurological examination was normal in all subjects. Interictal EEG showed focal abnormalities that were coincident in their location with the MRI abnormalities in 16 patients (55.1%). Fourteen patients (48.3%) showed right side hippocampal lesions on MRI, thirteen on the left side (44.9%) and 2 bilateral HS (6.8%). Twenty-seven patients (93.1%) had intractable epilepsy. Anterior temporal lobectomy was performed in 3 subjects with good outcome. The identification of these patients who present certain clinical and MRI characteristics, provides an opportunity to define the mesial temporal sclerosis syndrome. This could benefit patients in their prognosis and for specific treatments.

[Superficial haemosiderosis of the central nervous system, neuro otological manifestations and images from a magnetic resonance brain scan: a case report]. / Hemosiderosis superficial del sistema nervioso central, manifestaciones neurootológicas e imágenes de resonancia magnética cerebral: a propósito de un caso.

INTRODUCTION: Superficial haemosiderosis of the central nervous system (SHCNS) is an infrequent clinical entity; it is produced by the formation of clinically silent haemosiderin deposits in the leptomeninges, the subpial tissue, the cranial nerves and spinal cord, secondary to chronic bleeding in the subarachnoid space. Aetiology is idiopathic in half the cases or secondary to a vascular malformation or other structural abnormalities. At least 100 cases have been reported in the literature, most of which were diagnosed post mortem. In these descriptions there is a predominance of cerebellar ataxia, progressive hypoacusis, nystagmus, recurring headaches, pyramidal signs, an absence of caloric responses and xanthochromic cerebrospinal fluid. Magnetic resonance (MR) brain scanning in T2 revealed hypointensity in the brain stem, the cerebellum and the Sylvian fissure, with atrophy of the cerebellum and the brain stem. CASE REPORT: A 64 year old male with bilateral tinnitus and progressive neurosensory hipoacusis, who visited because of loss of balance, urinary incontinence and oscillopsia. The patient s personal history included an episode of unbearable headache at the age of 53. The neurological and neuro otological examination was pathological and the MR brain scan confirmed the diagnosis of SHCNS. CONCLUSIONS: We wish to highlight how extremely rare this entity is, and especially so when there is a predominance of progressive neuro otological manifestations; we also want to emphasize the importance of carrying out specific imaging studies to enable a diagnosis to be made while the patient is still alive.

[Musical memory preserved in an amnesic syndrome]. / Preservación de la memoria musical en un síndrome amnésico.

INTRODUCTION: In amnesic syndromes, it's usually to see dissociation between episodic, semantic and procedural memory. However, a few reports have been found about musical memory's processing and the relation with classic memory systems. AIM: To describe the musical's abilities preserved in a patient with amnesic syndrome and discuss possible neuropsychological and neurobiological bases implicated. CASE REPORT: A 28-years-old woman presenting with amnesic syndrome is reported. Following a carbon monoxide encephalophaty and subsequent hypoxia she remained in coma for 10 days with evidence of bilateral temporal changes, mainly affecting basal ganglia areas. The patient showed anterograde amnesia and semantic memory impairment, with disproportionately spared musical abilities' performance, either music perception (discrimination and recognition of tonal melodies, musical sight-reading) or music production (song and instrumental performance) or musical memory. CONCLUSIONS: This case suggests that the music require elaborate bihemispheric processing and may implicate different forms of information processing. The neural network involved in musical memory can be different that the declarative memory systems (episodic and semantic).

Infecciones por el virus EV-D68 en niños hospitalizados en un hospital de alta complejidad / EV-D68 virus infections in children admitted to a tertiary-care hospital

Introducción: Los reportes de infecciones por enterovirus D68 (EV-D68) han aumentado en los últimos años. Material y métodos: Cohorte prospectiva. Se realizó la búsqueda de EV-D68 en niños internados en el Hospital de Pediatría Juan P. Garrahan entre 1-5-2016 y 30-9-2016 con: infección respiratoria aguda baja (IRAB) que requirieran cuidados intensivos, parálisis aguda fláccida (PAF) asimétrica con compromiso de sustancia gris en resonancia magnética nuclear (RMN) o identificación de cualquier enterovirus con cuadro clínico compatible. La identificación de EV-D68 se realizó en el Servicio de Neurovirus, Instituto Nacional de Enfermedades Infecciosas INEI-ANLIS "Dr. CG. Malbrán". Resultados: n: 6. PAF: cuatro niños presentaron PAF asimétrica, con arreflexia y RMN compatible con mielitis. Requirieron ventilación mecánica en unidades de cuidados intensivos (UCI) dos de los 4 niños. Todos presentaron parálisis residual. Se identificó EV-D68 en secreciones nasofaríngeas (SNF) de todos ellos. En líquido cefalorraquídeo sólo en uno. Miocarditis: Una niña sana de 5 años se internó en UCI por disfunción miocárdica y fiebre. Presentaba además derrame pericárdico moderado. Recibió gamaglobulina e.v. con buena evolución. En SNF se identificaron virus sincicial respiratorio (VSR) y EV-D68. IRAB grave: se identificó EV-D68 en un paciente de 14 meses que permaneció en UCI por IRAB grave con requerimientos de ventilación no invasiva por 72 hs, con buena evolución posterior. Se constató coinfección VSR y EV-D68 en SNF. Conclusiones: Se reportan 6 pacientes internados con infección por EV-D68. La vigilancia epidemiológica activa es esencial para identificar la circulación, las características clínicas y el pronostico de las infecciones por virus emergentes (AU)

Introduction: Reports on enterovirus D68 (EV-D68) infections have increased over the past years. Material and methods: A prospective cohort study. A search for EV-D68 infection was conducted in children hospitalized at Hospital de Pediatría Juan P. Garrahan between 1-5-2016 and 30-9-2016 with: acute lower respiratory infection (ALRI) requiring intensive care unit (UCI) admission, acute flaccid paralysis (AFP), asymmetry with grey matter involvement on magnetic resonance imaging (MRI), or identification of any enterovirus associated with compatible features. The identification of EV-D68 was performed at the Department of Neuroviruses of the InstitutoNacional de EnfermedadesInfecciosas INEI-ANLIS "Dr. CG. Malbrán". Results: n: 6. AFP: four children had asymmetric AFP with areflexia and MRI compatible with myelitis. Two of four required mechanical ventilation in the ICU. All of them presented with residual paralysis. EV-D68 was identified in the nasopharyngeal swab (NPS) in all of them and in the cerebrospinal fluid in only one. Myocarditis: A 5-year-old healthy girl was admitted to the ICU because of myocardial dysfunction and fever associated with moderate pericardial effusion. She was put on IV gamma globulin with a good response. In the NPS respiratory syncytial virus (RSV) and EV-D68 were identified. Severe ALRI: EV-D68 was identified in a 14-month-old patient who was admitted to the UCU because of severe ALRI requiring non-invasive ventilation for 72 hours with a good outcome. A RSV and EV-D68 coinfection was found in the NPS. Conclusions: We report six inpatients with a EV-D68 infection. Active epidemiological surveillance is crucial to identify circulation of the virus, clinical features, and prognosis of emerging viruses (AU)

An Atlas of Infectious and Parasitic Diseases of the Central Nervous System. A Cooperative Study of SILAN (Sociedad Iberolatinoamericana de Neurorradiologia).

Infectious diseases of the central nervous system vary in frequency in different locations in America and Europe. What is common in Brazil can be a sporadic presentation in Europe. Cooperative work gathering experiences from neuroradiologists working in various places can be achieved and will help to identify uncommon cases that can present in our daily practice.

Combined protocol of cell therapy for chronic spinal cord injury. Report on the electrical and functional recovery of two patients.

BACKGROUND: This is a preliminary report on successful results obtained during treatment of two patients with chronic spinal cord injury. The therapeutic approach was based on the generation of controlled inflammatory activity at the injury site that induced a microenvironment for the subsequent administration of autologous, BM-driven transdifferentiated neural stem cells (NSC). METHODS: BM mesenchymal stem cells (MSC) were cocultured with the patient's autoimmune T (AT) cells to be transdifferentiated into NSC. Forty-eight hours prior to NSC implant, patients received an i.v. infusion of 5 x 10(8) to 1 x 10(9) AT cells. NSC were infused via a feeding artery of the lesion site. Safety evaluations were performed everyday, from the day of the first infusion until 96 h after the second infusion. After treatment, patients started a Vojta and Bobath neurorehabilitation program. RESULTS: At present two patients have been treated. Patient 1 was a 19-year-old man who presented paraplegia at the eight thoracic vertebra (T8) with his sensitive level corresponding to his sixth thoracic metamere (T6). He received two AT-NSC treatments and neurorehabilitation for 6 months. At present his motor level corresponds to his first sacral metamere (S1) and his sensitive level to the fourth sacral metamere (S4). Patient 2 was a 21-year-old woman who had a lesion that extended from her third to her fifth cervical vertebrae (C3-C5). Prior to her first therapeutic cycle she had severe quadriplegia and her sensitive level corresponded to her second cervical metamere (C2). After 3 months of treatment her motor and sensitive levels reached her first and second thoracic metameres (T1-T2). No adverse events were detected in either patient. DISCUSSION: The preliminary results lead us to think that this minimally invasive approach, which has minor adverse events, is effective for the repair of chronic spinal cord lesions.

[Intracranial pressure monitoring and eclampsia]. / Monitorización de la presión intracraneal y eclampsia.

Intracranial pressure (ICP) monitoring has been shown to improve clinical-pharmacological treatment of intracranial hypertension (ICH) in a rising number of situations, assuring effective cerebral perfusion pressure (CPP) and, concomitantly, reducing the risk of brain ischemia. Although its use in entities such as eclampsia have been reported, the continuous use of ICP monitoring is restricted. We report the case of an eclampsic woman in whom ICP was monitored. Recordings allowed CPP to be correctly stabilized, with strict correlation between ICP and tomographic measurements of density. We also review the pathophysiologic mechanisms that have been proposed to cause ICH in eclampsia and emphasize the usefulness of ICP monitoring to manage this complication.

[Acute reversible paraparesis secondary to probable fibrocartilaginous embolism]. / Paraparesia aguda reversible secundaria a probable embolia fibrocartilaginosa.

Fibrocartilagenous embolism (FCE) of the intervertebral disc represents a very rare cause of spinal infarct. Up to now only 33 others cases in human beings have been reported in the literature, most of them diagnosed post mortem. We present a 14-year-old boy who developed acute dorsal back pain after lifting a heavy gate, followed by progressive paraparesis. An MRI of the spine showed a degenerative disc at D10-D11 without compromise of the spinal canal lumen associated with an acute Schmorl's nodule situated in the superior endplate of D11. A week later, a second MRI disclosed an intraxial spinal cord lesion at D7-D8 vertebral level involving the vascular territory of the anterior spinal artery. It also showed an abnormal signal located in the posterior third of the D8 vertebral body. These clinical and neuro-radiological findings are similar to those mentioned in the literature and support the diagnosis of an anterior spinal infarct secondary to a probable fibrocartilaginous embolism. This case highlights the importance of considering this etiology among the causes of spinal cord infarct, especially in young people, and underlines the utility of MRI in its diagnosis in vivo.

[Hydrosyringomyelia in demyelinating diseases]. / Hidrosiringomielia en enfermedades desmielinizantes.

Spinal cord cavitation is a frequent finding in optic neuromyelitis (Devic's syndrome) (DS) but it is also, although rarely, observed in patients with multiple sclerosis (MS). The objective of our study was to compare the MRI characteristics of the syringomyelic cavities in 6 patients with DS and 3 patients with MS. All the patients with DS had a relapsing clinical form with normal brain MRI. Spinal MRI revealed unenhanced central cavities which extended more than 3 vertebral bodies and remained unchanged in follow-up studies. Two patients presented multiple cavities.MS patients suffered a relapsing remitting form of the disease, they all had hyperintense T2 enhancing lesions on their spinal MRI. Moreover spinal MRI also revealed non communicating cavities which extended less than 2 vertebral bodies. Follow-up studies in MS patients revealed a reduction of both the spinal lesions and the cavities. It is still debated whether DS represents a distinct clinical entity different from MS. These findings help distinguishing both disorders in cases when spinal cavities are present and also contribute to the therapeutic choice.

Pseudomigraña con pleocitosis linfocitaria / Pseudomigraine with pleocytosis

El síndrome de cefalea y déficit neurológico transitorio con pleocitosis en el liquido cefalorraquídeoo pseudomigraña con síntomas neurológicos temporarios y pleocitosis linfocítica (HaNLD), es uncuadro de cefaleas recurrentes, déficit neurológico reversible y pleocitosis linfocitaria en el líquido cefalorraquídeo(LCR), de duración variable y resolución espontánea. Si bien se han propuesto múltiples mecanismosetiopatogénicos (vascular, infeccioso, inmunológico y alteración de los canales de calcio), su etiología es aúndesconocida. Describimos el caso de una mujer de 28 años de edad, con episodios recurrentes de migraña conpleocitosis, síndrome confusional y déficit neurológico transitorio, con remisión clínica dentro de los dos meses.Si bien la etiología sigue siendo desconocida. Presentamos los diagnósticos diferenciales a tener en cuenta ante este síndrome

The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis or pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (HaNDL) is a syndrome consisting of recurrent headaches, reversible neurological deficit, lymphocytic pleocytosis in cerebrospinal fluid (CSF), variable duration over time and spontaneous resolution. Although several etiopathogenic mechanisms have been suggested (vascular, infectous, immunological and calciumchannelopthy), its etiology remains unknown. We describe a 28 year old female, with recurrent migrainewith pleocytosis, confusional syndrome and transient neurological deficit. The clinical remission was achievedwithin two months. Although its etiology remains unknown the differential diagnosis is discussed in order to keepin mind this syndrome

Hallazgos diagnósticos a través de la RM en una población de pacientes con epilepsia, seleccionados al azar / MRI diagnostic findings in a randomly selected epileptic population

A partir de los avances planteados con la RM, hemos realizado un estudio prospectivo en una población de 124 pacientes de consultorio externo con diagnóstico de epilepsia basado en datos clínicos y EEG, se aplicaron los criterios de la Liga Internacional de Epilepsia (ILAE, 1981, 1989) desde diciembre de 1993 a diciembre de 1994. El protocolo empleado incluyó imágenes en los planos sagital, coronal, axial y adquisición 3D coronal FFE, todos ellos paralelos y perpendiculares al eje mayor del hipocampo. Se completó con una secuencia IR axial. La RM fue normal en 67 pacientes (54 por ciento). En 57 pacientes (46 por ciento) la RM fue patológica con alteraciones corticales en 13 pacientes, tumores en 9 pacientes, esclerosis hipocampal en 8 pacientes, malformaciones vasculares en 4 pacientes, quistes aracnoideos en 5 pacientes y miscelánea en 18 pacientes. Hemos comparado los diferentes resultados hallados a través de la RM y la TC. El 33,8 por ciento de los pacientes que presentaron TC normal, tenía anormalidades en la RM, incluyendo 7 imágenes tumorales. La RM debería constituir parte de los análisis de rutina en los pacientes epilépticos, ya que representa una herramienta esencial para el diagnóstico de la epilepsia sintomática, resultando un método más específico y de mayor sensibilidad que la TC.