[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.

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.

[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.

Vasculitis cerebrales / Cerebral vasculitis

Introducción. Las vasculitis cerebrales comprenden un amplio grupo de entidades caracterizadas por la afectación de los vasos del sistema nervioso central (SNC) ocasionado por la inflamación de su pared, que conduce a la oclusión o a la formación de aneurismas, con las consiguientes alteraciones isquémicas-hemorrágicas. Desarrollo. En las vasculitis cerebrales pueden afectarse exclusivamente los vasos del SNC (vasculitis aislada del SNC), o ser otro de los territorios afectados en las vasculitis sistémicas primarias o secundarias (infecciosas, enfermedades del colágeno, por drogas, tumorales). Los síntomas neurológicos y el laboratorio suelen ser poco específicos. De mayor utilidad son las nuevas técnicas de neuroimágenes, y la angiografía digital es el patrón oro dentro de ellas; sin embargo, su sensibilidad y especificidad es limitada. La biopsia cerebral permite confirmar el diagnóstico y esel patrón oro para el diagnóstico de las vasculitis aisladas del SNC. Un amplio grupo de entidades (metabólicas, desmielinizantes, vasculares, infecciosas y otras vasculopatías) tienen manifestaciones clínicas e imaginológicas similares, por lo que se necesita plantear el diagnóstico diferencial. Conclusiones. La afectación del sistema nervioso ensombrece el pronóstico en la mayoría de las vasculitis y puede ser grave, como en las vasculitis aisladas de grandes vasos o en la enfermedad de Takayasu, o más benigno, como en la vasculitis aislada de pequeños vasos y en otras vasculitis primarias. El tratamiento con corticoides e inmusupresores debe considerarse encada caso particular según la entidad clínica y la evolución de cada paciente, así como el tratamiento con anticoagulantes y/o antiagregantes (AU)

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 cerebralvasculitis 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 (AU)

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

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(AU)

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(AU)

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(AU)

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(AU)

[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).

Preservación de la memoria musical en un síndrome amnésico / Musical memory preserved in a amnesic syndrome

Introducción. Los síndromes amnésicos presentan frecuentes disociaciones entre la memoria episódica, semántica y procedural. Sin embargo, escasean las descripciones acerca del procesamiento de un tipo especial de memoria, llamada 'memoria musical', y su relación con los otros subsistemas clásicos de memoria. Objetivo. Describir un paciente amnésico con preservación de la memoria musical y discutir las posibles bases neurobiológicas y neuropsicológicas implicadas. Caso clínico. Mujer de 35 años, diestra, universitaria, con conocimiento musical previo, desarrolló un síndrome amnésico secundario a una intoxicación con monóxido de carbono. Tras permanecer 10 días en estado de coma con daño bitemporal y en ganglios de la base, presentó graves fallos de memoria episódica y semántica, con indemnidad de la memoria procedural y de sus habilidades musicales, tanto la percepción (discriminación de sonidos, reconocimiento de melodías y lectura), la producción (el canto y la ejecución instrumental) y la memoria musical. Conclusiones. El caso de refleja la existencia de múltiples formas de memoria y aprendizaje que se mediarían por activación de diferentes sistemas. Esta doble disociación de los sistemas mnésicos de la paciente permite inferir que los circuitos neuronales involucrados en la memoria musical son diferentes de aquellos implicados en los otros subsistemas clásicos de memoria declarativa (episódica y semántica) (AU)

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 encephalopathy 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) (AU)

[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.

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

Introducción. La hemosiderosis superficial del sistema nervioso central (HSSNC) es una entidad rara, infrecuente; la producen depósitos de hemosiderina en las leptomeninges, el tejido subpial, los nervios craneanos y la médula espinal, secundarios al sangrado crónico y clínicamente silentes, en el espacio subaracnoideo. La etiología es idiopática en la mitad de los casos, o secundaria a una malformación vascular u otras anormalidades estructurales. En la bibliografía médica se han comunicado menos de 100 casos, diagnosticados la mayoría por autopsia. Predomina en esas descripciones la ataxia cerebelosa, la hipoacusia progresiva, el nistagmo, la cefalea recurrente, los signos piramidales, la ausencia de respuestas calóricas y el líquido cefalorraquídeo xantocrómico. La resonancia magnética (RM) del cerebro en T2 revela una hipointensidad en el tronco, el cerebelo y la cisura de Silvio con atrofia del cerebelo y el tronco. Caso clínico. Varón de 64 años, que presentó acúfenos bilaterales y una progresiva hipoacusia neurosensorial, que consultó por pérdida de equilibrio, incontinencia urinaria y oscilopsia. Los antecedentes personales revelaron un episodio de `cefalea insoportable' a los 53 años. El examen neurológico y neurootológico fue patológico, y la RM del cerebro confirmó el diagnóstico de HSSNC. Conclusiones. Deseamos destacar lo infrecuente de esta entidad y, en particular el predominio de las manifestaciones neurootológicas progresivas y puntualizar una vez más la importancia de realizar estudios por imágenes específicas para poder realizar el diagnóstico en vida del paciente (AU)

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 (AU)

[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.

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

La embolia fibrocartilaginosa (EFC) a partir del material de un disco intervertebral representa una causa excepcional de infarto medular. Hasta la fecha sólo han sido reportados 33 casos en humanos, en su mayoría con diagnóstico post mórtem. Presentamos el caso de un joven de 14 años que desarrolló dolor dorsal de instalación brusca luego de un esfuerzo físico, presentando a las pocas horas paraparesia de instalación progresiva. La resonancia magnética (RM) mostró una discopatía D10-11 sin alteración de la luz del canal espinal asociada a un nódulo de Schmorl en la plataforma superior del cuerpo vertebral de D11. Una segunda RM, obtenida una semana más tarde, mostró una lesión intramedular a nivel D7-8 afectando el territorio de la arteria espinal anterior. También mostró una señal anormal en el tercio posterior del cuerpo vertebral de D8. Estos hallazgos clínicos y neurorradiológicos son similares a los referidos en la literatura y contribuyen al diagnóstico presuntivo de infarto espinal anterior de posible origen embólico fibrocartilaginoso. El presente caso resalta la importancia de considerar esta etiología entre las causas de infarto medular, especialmente en individuos jóvenes, y destaca la utilidad de la RM para su diagnóstico in vivo. (AU)

[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.

Hidrosiringomielia en enfermedades desmielinizantes / Hydrosyringomyelia in demyelinating diseases

La cavitación de la médula espinal es un hallazgo frecuente en la neuromielitis óptica de Devic (NMO); sin embargo, su ocurrencia es excepcional en la esclerosis múltiple (EM). El objetivo del presente trabajo fue comparar mediante resonancia magnética (RM) las características de las cavidades hidrosiringomiélicas detectadas en 6 individuos con diagnóstico de NMO y en tres con EM. Todos los pacientes con NMO presentaron una evolución clínica "multifásica" y RM cerebral normal. La RM espinal mostró la presencia de cavidades centrales que no reforzaban luego de la inyección de gadolinio, se extendían a lo largo de más de tres segmentos vertebrales y permanecieron invariables durante el período de observación; en dos casos las cavitaciones fueron múltiples. Los pacientes con EM presentaron formas a brotes y remisiones; en todos ellos la RM cerebral mostró lesiones de sustancia blanca, hiperintensas en la secuencia T2.La RM de médula espinal evidenció lesiones hiperintensas en T2 que reforzaban con gadolinio; estas lesiones se asociaron en todos los casos con cavidades caudales no comunicantes con extensión inferior a dos segmentos vertebrales. En el seguimiento de los pacientes con EM se observó una reducción del tamaño de las lesiones y de la cavidad hidrosiringomiélica. Si bien aún permanece en discusión la existencia de la NMO como una entidad específica diferente de la EM, estos hallazgos contribuirían al diagnóstico diferencial de ambas patologías facilitando el inicio de diferentes esquemas terapéuticos. (AU)

[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.