Encefalopatía subaguda con respuesta a esteroides sin evidencia de enfermedad autoinmune tiroidea: un caso de meningoencefalitis autoinmune no vasculítica / Sub-acute encephalopathy that responds to sterorids without any evidence of autoinmune thyroid disease a case of non-vasculitic autoimmune nm

Aim. To report a case of sub-acute encephalopathy with all the extension study negative and with response to steroid therapy. Case report. The study involves a 22-year-old female with no relevant past history who presented symptoms of sub-acute encephalopathy consisting in behavioural disorders, generalised seizures and bradypsychia, which gradually progressed to a state of low-level consciousness. While she was in hospital all kinds of diagnostic tests were conducted, the results of which were either normal or negative; the electroencephalogram was repeatedly abnormal and detection of protein 14-3-3 in cerebrospinal fluid was positive. Empirical corticoid therapy was begun with clinical and electrophysiological improvements and the patient recovered completely without any sequelae. Conclusions. With no evidence of autoimmune thyroid disease, although non-specific autoimmunity was present, the patient was diagnosed as having non-vasculitic autoimmune meningoencephalitis (AU)

Objetivo. Presentar un caso de encefalopatía subaguda con todo el estudio de extensión negativo y con respuesta a terapia esteroidea. Caso clínico. Mujer de 22 años sin antecedentes de interés que presentó un cuadro de encefalopatía subaguda consistente en alteraciones del comportamiento, crisis generalizadas y bradipsiquia, que evolucionó progresivamente a un estado de bajo nivel de conciencia. Durante su ingreso se realizaron todo tipo de pruebas diagnósticas, que fueron normales o negativas; el electroencefalograma fue repetidamente anormal y se detectó positividad de la proteína 14-3-3 en el líquido cefalorraquídeo. Se inició corticoterapia empírica con mejoría clínica y electrofisiológica, y recuperación ad integrum sin secuelas. Conclusiones. Sin la evidencia de enfermedad autoinmune tiroidea, aunque con presencia de autoinmunidad no específica, se diagnosticó a la paciente meningoencefalitis autoinmune no vasculítica (AU)

[Sub-acute encephalopathy that responds to steroids without any evidence of autoimmune thyroid disease: a case of non-vasculitic autoimmune meningoencephalitis]. / Encefalopatía subaguda con respuesta a esteroides sin evidencia de enfermedad autoinmune tiroidea: un caso de meningoencefalitis autoinmune no vasculítica.

AIM: To report a case of sub-acute encephalopathy with all the extension study negative and with response to steroid therapy. CASE REPORT: The study involves a 22-year-old female with no relevant past history who presented symptoms of sub-acute encephalopathy consisting in behavioural disorders, generalised seizures and bradypsychia, which gradually progressed to a state of low-level consciousness. While she was in hospital all kinds of diagnostic tests were conducted, the results of which were either normal or negative; the electroencephalogram was repeatedly abnormal and detection of protein 14-3-3 in cerebrospinal fluid was positive. Empirical corticoid therapy was begun with clinical and electrophysiological improvements and the patient recovered completely without any sequelae. CONCLUSIONS: With no evidence of autoimmune thyroid disease, although non-specific autoimmunity was present, the patient was diagnosed as having non-vasculitic autoimmune meningoencephalitis.

[Mitochondrial encephalopathy due to complex I deficiency. Brain tissue biopsy findings and clinical course following pharmacological]. / Encefalopatia mitocondrial por deficit del complejo I. Hallazgos en la biopsia cerebral y evolucion clinica tras tratamiento farmacologico.

INTRODUCTION: Mitochondrial encephalomyopathies belong to a heterogeneous group of diseases with a range of neurological symptoms caused by a dysfunction somewhere in the nervous system. They may arise from mutations of the mitochondrial DNA or nuclear DNA in the genes that code for the subunits of the respiratory chain. The results obtained from using different drugs to treat these diseases vary widely. CASE REPORT: A 33-year-old female with a history of migraine with aura, who was admitted to hospital because of epileptic seizures. Neuroimaging tests showed left-side occipital insult and a biopsy study of a sample of brain tissue revealed gliosis and vacuolisation of the white matter. Lactic acid levels in blood were normal. No ragged red fibres were seen in the muscle biopsy, but there was evidence of a complex I deficiency in the respiratory chain. After establishing treatment with coenzyme Q and riboflavin, the patient had no further episodes of neurological disorders. CONCLUSIONS: The absence of elevated levels of lactate, ragged red fibres in the muscle biopsy or the negative results for mutations in the genetic study do not rule out the possible existence of a mitochondrial disease. The gliosis and vacuolisation of the white matter with respect to the neurons that were found in the results of the brain tissue biopsy must lead us to consider a mitochondrial disease.

Encefalopatía mitocondrial por déficit del complejo I. Hallazgos en la biopsia cerebral y evolución clínica tras tratamiento farmacológico / Mitochindrial encephalopathy due to complex I. deficiency brain tissue biopsy findings and clinical course following pharmacological

Introducción. Las encefalomiopatías mitocondriales constituyen un grupo heterogéneo de enfermedades con sintomatología neurológica diversa por disfunción de cualquier parte del sistema nervioso. Pueden producirse por mutaciones del ADN mitocondrial o del ADN nuclear en los genes que codifican las subunidades de la cadena respiratoria. Los fármacos utilizados en estas enfermedades han tenido resultados muy dispares.Caso clínico. Mujer de 33 años con antecedentes de migraña con aura que ingresa por crisis epilépticas. En las pruebas de neuroimagen se observa una lesión occipital izquierda cuya biopsia cerebral muestra gliosis y vacuolización de la sustancia blanca. No presenta elevación del ácido láctico en sangre. Enla biopsia muscular no hay fibras rojo-rasgadas, pero se evidencia un déficit del complejo I de la cadena respiratoria. Tras iniciar tratamiento con coenzima Q y riboflavina, la paciente no vuelve a tener nuevos episodios de afectación neurológica.Conclusiones. La ausencia de lactato elevado, fibras rojo-rasgadas en la biopsia muscular o la negatividad de mutaciones en el estudio genético no excluyen la existencia de una enfermedad mitocondrial. Los hallazgos en la biopsia cerebral de gliosis y vacuolización de la sustancia blanca con relativo respeto de las neuronas nos deben hacer pensar en una enfermedad mitocondrial

Introduction. Mitochondrial encephalomyopathies belong to a heterogeneous group of diseases with a range ofneurological symptoms caused by a dysfunction somewhere in the nervous system. They may arise from mutations of the mitochondrial DNA or nuclear DNA in the genes that code for the subunits of the respiratory chain. The results obtained from using different drugs to treat these diseases vary widely. Case report. A 33-year-old female with a history of migraine with aura, who was admitted to hospital because of epileptic seizures. Neuroimaging tests showed left-side occipital insult and a biopsy study of a sample of brain tissue revealed gliosis and vacuolisation of the white matter. Lactic acid levels in blood werenormal. No ragged red fibres were seen in the muscle biopsy, but there was evidence of a complex I deficiency in the respiratory chain. After establishing treatment with coenzyme Q and riboflavin, the patient had no further episodes of neurological disorders. Conclusions. The absence of elevated levels of lactate, ragged red fibres in the muscle biopsy or thenegative results for mutations in the genetic study do not rule out the possible existence of a mitochondrial disease. The gliosis and vacuolisation of the white matter with respect to the neurons that were found in the results of the brain tissue biopsy must lead us to consider a mitochondrial disease

Parálisis de las cuerdas vocales como presentación inicial de una miastenia grave / Paralysis of the vocal cords as the presenting symptom of myasthenia gravis

No disponible

No disponible

[Chronic progressive external ophthalmoplegia: clinical and electromyographic manifestations in a series of cases]. / Oftalmoplejía externa progresiva crónica: manifestaciones clínicas y electromiográficas en una serie de casos.

INTRODUCTION: Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. The different conditions in this group of diseases overlap clinically, enzymatically and genetically. There is no effective treatment. Ptosis improves with corrective surgery involving tarsorrhaphy as a palliative measure. CASE REPORTS: Code numbers were examined in a retrospective study conducted in order to search for patients with ptosis or ophthalmoplegia who had either visited or been admitted to the neurology department over the last 10 years. Data concerning these patients' clinical features and results of complementary tests were collected. Six patients with CPEO were identified, five of whom were females. Ages ranged from 44 to 72 years. All the patients had ptosis, although 50% were asymmetric. Half of them reported mild dysphagia while swallowing liquids. Levels of creatine phosphokinase and acetylcholine antireceptor antibodies were normal. Half the patients showed increased jitter and a muscle biopsy revealed that five of them had ragged red fibres. The most frequent enzyme deficit was complex I and IV deficiency. There were no familial forms; the most common genetic anomaly was single deletion in the mitochondrial deoxyribonucleic acid. CONCLUSIONS: In cases of ptosis and ophthalmoplegia that do not respond to anticholinesterases, knowledge of this condition makes it possible to avoid the use of immunosuppressant drugs, which have important side effects.

Oftalmoplejía externa progresiva crónica: manifestaciones clínicas y electromiográficas en una serie de casos / Chronic progressive external ophthalmoplegia: clinical and electromyographic manifestations in a series of cases

Introducción. La oftalmoplejía externa progresiva crónica(CPEO) es una enfermedad mitocondrial común. Este grupode enfermedades presenta solapamiento clínico, enzimático y genéticoentre las diferentes entidades. No existe un tratamiento eficaz.La ptosis mejora con cirugía correctora de tarsorrafia comouna medida paliativa. Casos clínicos. Estudio retrospectivo en elque se busca por codificación a pacientes con ptosis u oftalmoplejíaen consultas o ingresados en neurología durante los últimos 10años. Se recogieron datos de la clínica y pruebas complementariasde estos pacientes. Se identificó a seis pacientes con CPEO; cincode ellos fueron mujeres. Sus edades estaban comprendidas entrelos 44 y los 72 años. Todos los pacientes presentaban ptosis, aunqueel 50% era asimétrica. La mitad refería disfagia leve paralíquidos. Los niveles de creatinfosfocinasa y de anticuerpos antirreceptoresde acetilcolina fueron normales. Existía un aumentodel jitter en la mitad de los pacientes y fibras rojas rasgadas en labiopsia muscular de cinco de ellos. El déficit enzimático más frecuentefue el de los complejos I y IV. No existieron formas familiares;la anomalía genética más común fue la deleción única en elácido desoxirribonucleico mitocondrial. Conclusión. El conocimientode esta entidad permite, en casos de ptosis y oftalmoplejíaque no responden a anticolinesterásicos, evitar el uso de medicacionesinmunosupresoras con efectos secundarios importantes

Introduction. Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. Thedifferent conditions in this group of diseases overlap clinically, enzymatically and genetically. There is no effective treatment.Ptosis improves with corrective surgery involving tarsorrhaphy as a palliative measure. Case reports. Code numbers wereexamined in a retrospective study conducted in order to search for patients with ptosis or ophthalmoplegia who had eithervisited or been admitted to the neurology department over the last 10 years. Data concerning these patients' clinical featuresand results of complementary tests were collected. Six patients with CPEO were identified, five of whom were females. Agesranged from 44 to 72 years. All the patients had ptosis, although 50% were asymmetric. Half of them reported mild dysphagiawhile swallowing liquids. Levels of creatine phosphokinase and acetylcholine antireceptor antibodies were normal. Half thepatients showed increased jitter and a muscle biopsy revealed that five of them had ragged red fibres. The most frequentenzyme deficit was complex I and IV deficiency. There were no familial forms; the most common genetic anomaly was singledeletion in the mitochondrial deoxyribonucleic acid. Conclusions. In cases of ptosis and ophthalmoplegia that do not respondto anticholinesterases, knowledge of this condition makes it possible to avoid the use of immunosuppressant drugs, which haveimportant side effects

[Neurological sequelae following electrocution. A case report and review of the literature]. / Secuelas neurológicas tras electrocución. Presentación de un caso y revisión de la bibliografía.

INTRODUCTION: Electrocution is the cause of a large number of accidents and, of these, a considerable percentage result in death. Several factors affect the severity and distribution of the injuries. It is known that for low voltages the characteristics of alternating current make it three times more dangerous than continuous current. The high percentage of neurological sequelae can be accounted for by the fact that nerves are the tissue with the lowest resistance in the body and electricity tends to follow the path that offers the least resistance. CASE REPORT: A 16-year-old male who was accidentally electrocuted by an electric guitar and later suffered a cardiorespiratory arrest; the patient required intubation. After withdrawing sedation, the patient was found to be blind in both eyes and could not remember what had happened. FLAIR MR imaging revealed oedema in both occipital lobes and, to a lesser extent, in the junction between the parietooccipital and temporooccipital lobes. Treatment with steroids was established for three days. After approximately one month, the patient had recovered his sight and the brain injuries had disappeared. CONCLUSIONS: The neurological manifestations secondary to accidents caused by electricity are usually divided into two types -immediate and delayed. Blindness has rarely been reported as a sequela in those who have survived fulguration due to a lightning strike. The patient's recovery after administering treatment with steroids and the reversibility of the oedema could lead us to think that the mechanism producing this sequela was the damage to the nervous system caused directly by the electricity, which is known as the 'phenomenon of electroporation'.

Secuelas neurológicas tras electrocución. Presentación de un caso y revisión de la bibliografía / Neurological sequelae following electrocution. A case report and review of the literature

Introducción. La electrocución genera un gran númerode accidentes y, de éstos, un porcentaje no desestimable termina enmuerte. Existen factores que influyen en la gravedad y la distribuciónde las lesiones. Se conoce que, debido a sus características, lacorriente alterna para voltajes bajos es tres veces más peligrosa quela continua. El alto porcentaje de secuelas neurológicas se explicadebido a que los nervios son el tejido que menor resistencia poseedentro del organismo y la electricidad tiende a escoger el camino demenor resistencia. Caso clínico. Varón de 16 años que sufrió unaelectrocución accidental por una guitarra eléctrica, con posteriorparada cardiorrespiratoria; precisó intubación. Tras la retirada dela sedación, el paciente presentaba una ceguera bilateral con amnesiade lo sucedido. La resonancia magnética mostró, en la secuenciaFLAIR, edema en ambos lóbulos occipitales y, en menor grado,en la unión parietooccipital y temporooccipital. Se instauró empíricamenteun tratamiento esteroideo durante tres días. Aproximadamenteal cabo de un mes, el paciente había recuperado totalmentela visión y habían desaparecido las lesiones cerebrales. Conclusiones.Las manifestaciones neurológicas secundarias a los accidentespor electricidad se suelen dividir en inmediatas y tardías. La ceguerase ha descrito raramente como secuela de los supervivientes a lafulguración por rayos. La recuperación tras la administración deltratamiento esteroideo y la reversibilidad del edema podrían hacerpensar que el mecanismo de producción de esta secuela fuera el dañodirecto de la electricidad en el sistema nervioso, conocido como ‘fenómenode electroporación

Introduction. Electrocution is the cause of a large number of accidents and, of these, a considerable percentage resultin death. Several factors affect the severity and distribution of the injuries. It is known that for low voltages the characteristicsof alternating current make it three times more dangerous than continuous current. The high percentage of neurologicalsequelae can be accounted for by the fact that nerves are the tissue with the lowest resistance in the body and electricity tendsto follow the path that offers the least resistance. Case report. A 16-year-old male who was accidentally electrocuted by anelectric guitar and later suffered a cardiorespiratory arrest; the patient required intubation. After withdrawing sedation, thepatient was found to be blind in both eyes and could not remember what had happened. FLAIR MR imaging revealed oedema inboth occipital lobes and, to a lesser extent, in the junction between the parietooccipital and temporooccipital lobes. Treatmentwith steroids was established for three days. After approximately one month, the patient had recovered his sight and the braininjuries had disappeared. Conclusions. The neurological manifestations secondary to accidents caused by electricity are usuallydivided into two types –immediate and delayed. Blindness has rarely been reported as a sequela in those who have survivedfulguration due to a lightning strike. The patient’s recovery after administering treatment with steroids and the reversibility ofthe oedema could lead us to think that the mechanism producing this sequela was the damage to the nervous system causeddirectly by the electricity, which is known as the ‘phenomenon of electroporation’

[Recurrent aseptic meningitis secondary to taking ibuprofen and ketorolac]. / Meningitis recurrente aséptica secundaria a toma de ibuprofeno y ketorolaco.

INTRODUCTION: Aseptic meningitis is a process that is characterised by an inflammatory reaction of the meninges that is not due to any infectious agent. Its aetiology is varied and is most frequently caused by rheumatologic and/or autoimmune processes, chemical or medication-induced meningitis, the most notable drugs involved being antibiotics and non-steroidal anti-inflammatory drugs (NSAI). CASE REPORT: We report the case of a 70-year-old male, with no relevant history, who was admitted to hospital five times over a period of 16 months because of acute meningitis with polymorphonuclear pleocytosis, high protein levels in cerebrospinal fluid and normal glucose in cerebrospinal fluid. No evidence of an infectious causation, chemical meningitis, carcinomatosis or autoimmune disease was found and the patient was diagnosed with recurrent aseptic meningitis. It was found that the patient had taken ibuprofen or ketorolac on several occasions, a few hours before the appearance of symptoms. These episodes were quickly resolved after withdrawal of this medication. CONCLUSIONS: A number of NSAI have been reported as inducers of aseptic meningitis, one of the most notable being ibuprofen. We report the case of a patient who, as a consequence of taking ibuprofen and ketorolac, presented episodes of recurrent aseptic meningitis. To our knowledge this side effect of ketorolac has not been reported before. Its clinical features are impossible to differentiate from those of infectious meningitis. Diagnosis is reached by exclusion and a careful pharmacological study, including over-the-counter drugs like some of the NSAI, must be performed in patients with this condition, since it is a problem that can easily be solved by withdrawing the drug that causes it.

Meningitis recurrente aséptica secundaria a toma de ibuprofeno y ketorolaco / Recurrent aseptic meningitis secondary to taking ibuprofen and ketorolac

Introducción. La meningitis aséptica es un proceso que se caracteriza por una reacción inflamatoria meníngea no causada por ningún agente infeccioso. Su etiología es variada, y la más frecuente es la secundaria a procesos reumatológicos y/o autoinmunes, meningitis química e inducida por fármacos, entre los que destacan los antibióticos y los antiinflamatorios no esteroideos (AINE). Caso clínico. Varón de 70 años de edad, sin antecedentes relevantes, que en el período de 16 meses ingresó en cinco ocasiones por meningitis aguda con pleocitosis polimorfonuclear, hiperproteinorraquia y glucosa normal en el líquido cefalorraquídeo, en las que se descartó una etiología infecciosa, meningitis química, carcinomatosis o enfermedad autoinmune y se le diagnosticó meningitis aséptica recurrente. Se comprobó la toma de ibuprofeno o ketorolaco en distintas ocasiones, unas pocas horas antes de la aparición de la clínica. Estos episodios se resolvieron rápidamente tras la retirada de dicha medicación. Conclusiones. Se han implicado varios AINE como inductores de meningitis aséptica, entre los que destaca el ibuprofeno. Presentamos un paciente que, como consecuencia de la toma de ibuprofeno y ketorolaco, sufrió episodios de meningitis recurrente aséptica. En nuestro conocimiento no se ha comunicado previamente este efecto secundario con el ketorolaco. Su clínica es indistinguible de la meningitis infecciosa; el diagnóstico se efectúa por exclusión, y debe realizarse una cuidadosa historia farmacológica, que incluya fármacos de libre dispensación como algunos AINE, en pacientes con esta patología, puesto que se trata de un problema fácilmente solucionable con la retirada de la medicación que lo causa (AU)

Introduction. Aseptic meningitis is a process that is characterised by an inflammatory reaction of the meninges that is not due to any infectious agent. Its aetiology is varied and is most frequently caused by rheumatologic and/or autoimmune processes, chemical or medication-induced meningitis, the most notable drugs involved being antibiotics and non-steroidal anti-inflammatory drugs (NSAI). Case report. We report the case of a 70-year-old male, with no relevant history, who was admitted to hospital five times over a period of 16 months because of acute meningitis with polymorphonuclear pleocytosis, high protein levels in cerebrospinal fluid and normal glucose in cerebrospinal fluid. No evidence of an infectious causation, chemical meningitis, carcinomatosis or autoimmune disease was found and the patient was diagnosed with recurrent aseptic meningitis. It was found that the patient had taken ibuprofen or ketorolac on several occasions, a few hours before the appearance of symptoms. These episodes were quickly resolved after withdrawal of this medication. Conclusions. A number of NSAI have been reported as inducers of aseptic meningitis, one of the most notable being ibuprofen. We report the case of a patient who, as a consequence of taking ibuprofen and ketorolac, presented episodes of recurrent aseptic meningitis. To our knowledge this side effect of ketorolac has not been reported before. Its clinical features are impossible to differentiate from those of infectious meningitis. Diagnosis is reached by exclusion and a careful pharmacological study, including over-thecounter drugs like some of the NSAI, must be performed in patients with this condition, since it is a problem that can easily be solved by withdrawing the drug that causes it (AU)

[Aphasia and parietal syndrome as the presenting symptoms of a demyelinating disease with pseudotumoral lesions]. / Afasia y síndrome parietal como forma de presentación de una enfermedad desmielinizante con lesión pseudotumoral.

INTRODUCTION: Multiple sclerosis (MS) often presents with sensory symptoms, which are usually due to spinothalamic or spinal cord disorders; parietal syndrome is, however, very rare as the initial symptom. Likewise, aphasia is also an infrequent symptom of MS; in the few cases that have been reported, it is usually linked to the existence of important pseudotumoral lesions. CASE REPORT: We describe the case of a 31-year-old female with a 48-hour history of a progressive clinical picture consisting in nominal aphasia and a sensory parietal syndrome. Magnetic resonance imaging showed a lesion 3.6 cm in diameter that was hyperintense in T2 with perilesional edema and minimal gadolinium uptake, along with other images that revealed increased signal intensity in the periventricular subcortical white matter on the right-hand side and in the left-hand frontal subcortical region. A spectroscopic analysis of the largest lesion revealed that this lesion showed evidence of inflammation, with cell destruction and replacement, although it was not possible to distinguish between a demyelinating disease and a high grade glioma. Hence, a brain biopsy was required in order to reach the final diagnosis of demyelinating pseudotumoral lesion. CONCLUSIONS: Giant pseudotumoral plaques are a rare form of presenting symptom in MS; use of the clinical features, simple images and spectroscopy is not a very reliable means of reaching a differential diagnosis with a tumour and this often makes it necessary to conduct a biopsy study of the lesion.

Afasia y síndrome parietal como forma de presentación de una enfermedad desmielinizante con lesión pseudotumoral / Aphasia and parietal syndrome as the presenting symptoms of a demyelinating disease with pseudotumoral lesions

Introducción. Las manifestaciones sensitivas son frecuentes como síntomas iniciales de la esclerosis múltiple (EM), generalmente por alteración espinotalámica o medular; pero es excepcional un síndrome parietal como forma de inicio. Igualmente, la afasia es una manifestación infrecuente en la EM; en los pocos casos descritos suele asociarse a la existencia de grandes lesiones pseudotumorales. Caso clínico. Mujer de 31 años con un cuadro progresivo de 48 horas de evolución, consistente en afasia nominal y un síndrome sensitivo parietal. En la resonancia magnética se observó una lesión de 3,6 cm de diámetro hiperintensa en T2 con edema perilesional y con mínima captación de gadolinio, además de otras imágenes de aumento de intensidad de señal en sustancia blanca subcortical periventricular derecha y en zona subcortical frontal izquierda. El análisis espectroscópico de la lesión de mayor tamaño reveló que dicha lesión presentaba datos de inflamación, con destrucción y recambio celular, sin poder distinguir entre enfermedad desmielinizante o glioma de alto grado; se precisó para ello la realización de una biopsia cerebral para alcanzar el diagnóstico final de lesión pseudotumoral desmielinizante. Conclusiones. Las placas gigantes pseudotumorales constituyen una forma infrecuente de inicio de la EM; su diagnóstico diferencial con un tumor por la clínica, la imagen simple y la espectroscopia son poco fiables, por lo que, con frecuencia, es preciso recurrir a la biopsia de la lesión

Introduction. Multiple sclerosis (MS) often presents with sensory symptoms, which are usually due to spinothalamic or spinal cord disorders; parietal syndrome is, however, very rare as the initial symptom. Likewise, aphasia is also an infrequent symptom of MS; in the few cases that have been reported, it is usually linked to the existence of important pseudotumoral lesions. Case report. We describe the case of a 31-year-old female with a 48-hour history of a progressive clinical picture consisting in nominal aphasia and a sensory parietal syndrome. Magnetic resonance imaging showed a lesion 3.6 cm in diameter that was hyperintense in T2 with perilesional edema and minimal gadolinium uptake, along with other images that revealed increased signal intensity in the periventricular subcortical white matter on the right-hand side and in the left-hand frontal subcortical region. A spectroscopic analysis of the largest lesion revealed that this lesion showed evidence of inflammation, with cell destruction and replacement, although it was not possible to distinguish between a demyelinating disease and a high grade glioma. Hence, a brain biopsy was required in order to reach the final diagnosis of demyelinating pseudotumoral lesion. Conclusions. Giant pseudotumoral plaques are a rare form of presenting symptom in MS; use of the clinical features, simple images and spectroscopy is not a very reliable means of reaching a differential diagnosis with a tumour and this often makes it necessary to conduct a biopsy study of the lesion

[Recurrent Tolosa-Hunt syndrome with normal neuroimaging. A report of three cases]. / Síndrome de Tolosa-Hunt de repetición con neuroimagen normal. Descripción de tres casos.

INTRODUCTION: Tolosa-Hunt syndrome is caused by an unspecific inflammation in the cavernous sinus or superior orbital fissure. Recurrence occurs in 40% of cases. Magnetic resonance (MR) of the head and orbital phlebography can reveal characteristic data, although diagnosis is reached by exclusion according to International Headache Society criteria. Corticoids are the preferred treatment, since their use gives rise to the disappearance of the clinical condition in a few days. CASE REPORT: We describe the cases of three patients with recurrent Tolosa-Hunt syndrome and MR images of the head with normal contrast. Case 1: a 52-year-old male with paralysis of the oculomotor nerve with recurrence on the contralateral side. Case 2: a 42-year-old female with recurrent paralysis of the abducent nerve. Case 3: a 34-year-old male with involvement of the oculomotor nerve, optic nerve and the first branch of the trigeminal nerve with recurrence of the ophthalmoplegia. In all cases the clinical symptoms disappeared with the use of corticoid therapy. CONCLUSIONS: The oculomotor disorder is produced by nerve infiltration and not by compression. The time for recurrence varies largely, and the clinical condition in the recurrence is usually milder owing to the fact that diagnosis is reached and treatment is started earlier. The absence of alterations in the MR of the head does not rule out the existence of Tolosa-Hunt syndrome, even in cases of recurrence, and these disorders therefore should not be included in the diagnostic criteria.

Síndrome de Tolosa-Hunt de repetición con neuroimagen normal. Descripción de tres casos / Recurrent tolosa-hunt syndrome with normal neuroimaging. A report of three cases

Introducción. El síndrome de Tolosa-Hunt está causadopor una inflamación inespecífica en el seno cavernoso o fisura orbitariasuperior. La recidiva ocurre en un 40% de los casos. Laresonancia magnética (RM) cerebral y la flebografía orbitaria puedenmostrar datos característicos, aunque el diagnóstico es deexclusión, según los criterios de la International Headache Society.Los corticoides son el tratamiento de elección, ya que producen ladesaparición de la clínica en pocos días. Casos clínicos. Se describentres casos de pacientes con síndrome de Tolosa-Hunt de repeticióny RM cerebral con contraste normal. Caso 1: varón de 52 añoscon parálisis del III par con recurrencia en el lado contralateral.Caso 2: mujer de 42 años con parálisis del VI par de repetición.Caso 3: varón de 34 años con afectación del III par, nervio óptico yprimera rama del trigémino con recurrencia de oftalmoplejía. Entodos los casos la clínica desapareció tras la instauración de corticoides.Conclusiones. La afectación oculomotora se produce porinfiltración nerviosa y no por compresión. El tiempo para la recurrenciaes muy variable y la clínica en la recurrencia suele ser másleve, debido al diagnóstico y a la instauración de tratamiento mástemprana. La ausencia de alteraciones en la RM cerebral no descartala existencia de un síndrome de Tolosa-Hunt, incluso en casosde recurrencia, por lo que no se deberían incluir estas alteracionesen los criterios diagnósticos

Introduction. Tolosa-Hunt syndrome is caused by an unspecific inflammation in the cavernous sinus or superiororbital fissure. Recurrence occurs in 40% of cases. Magnetic resonance (MR) of the head and orbital phlebography can revealcharacteristic data, although diagnosis is reached by exclusion according to International Headache Society criteria.Corticoids are the preferred treatment, since their use gives rise to the disappearance of the clinical condition in a few days.Case report. We describe the cases of three patients with recurrent Tolosa-Hunt syndrome and MR images of the head withnormal contrast. Case 1: a 52-year-old male with paralysis of the oculomotor nerve with recurrence on the contralateral side.Case 2: a 42-year-old female with recurrent paralysis of the abducent nerve. Case 3: a 34-year-old male with involvement ofthe oculomotor nerve, optic nerve and the first branch of the trigeminal nerve with recurrence of the ophthalmoplegia. In allcases the clinical symptoms disappeared with the use of corticoid therapy. Conclusions. The oculomotor disorder is producedby nerve infiltration and not by compression. The time for recurrence varies largely, and the clinical condition in therecurrence is usually milder owing to the fact that diagnosis is reached and treatment is started earlier. The absence ofalterations in the MR of the head does not rule out the existence of Tolosa-Hunt syndrome, even in cases of recurrence, andthese disorders therefore should not be included in the diagnostic criteria

[Horner syndrome as the first symptom of nasopharyngeal cancer. Two case reports]. / Sindrome de Horner como primera manifestación del cáncer de cavum. A propósito de dos casos.

INTRODUCTION: Horner syndrome (HS) is produced by damage to the oculosympathetic pathway and gives rise to palpebral ptosis, enophthalmos and myosis. Anhidrosis also occurs in cases in which the lesion is located before the separation of the vasomotor and sudomotor fibres at the start of the internal carotid artery. Nasopharyngeal cancer may damage the cranial nerves, mainly in the cavernous sinus, and very few cases of HS due to infiltration of the tumour into the parapharyngeal space have been reported. CASE REPORTS: Case 1: a 67-year-old male with a 10-day history of HS on the left side without anhidrosis. The aetiological study revealed a nasopharyngeal lymphoepithelioma; the tumour became smaller and HS disappeared following treatment with chemotherapy. Case 2: a 48-year-old male with a 1-week history of HS without anhidrosis, and a 2-month history of deafness and pain in the right ear. The aetiological study showed a nasopharyngeal lymphoepithelioma and serous otitis. The tumour became smaller and HS disappeared following treatment with chemotherapy. CONCLUSIONS: It is important to carry out studies of the nasopharynx in patients with this syndrome to allow early diagnosis and treatment of a region that is not readily available for direct examination. The existence of an associated pathology in the ear or the paranasal sinuses strongly suggests that its origins lie in the nasopharynx.

Síndrome de Horner como primera manifestación del cáncer de cavum. A propósito de dos casos / Horner syndrome as the first symptom of nasopharyngeal cancer. Two case reports

Introducción. El síndrome de Horner (SH) se produce por lesión de la vía oculosimpática, y da lugar a ptosis palpebral, enoftalmos y miosis. Hay también anhidrosis en los casos en los que la lesión se localiza antes de la separación de las fibras vaso-motoras y sudomotoras en el inicio de la carótida interna. El cáncer de nasofaringe puede lesionar los pares craneales, fundamentalmente en el seno cavernoso, y son raros los casos de SH por infiltración del tumor en el espacio parafaríngeo. Casos clínicos. Caso 1: varón de 67 años con SH, sin anhidrosis en el lado izquierdo, de 10 días de evolución. En el estudio etiológico se demostró un linfoepitelioma de cavum; tras la quimioterapia disminuyó el tamaño tumoral y desapareció el SH. Caso 2: varón de 48 años con un SH derecho, sin anhidrosis, de una semana de evolución, con sordera y dolor en el oído derecho de dos meses de evolución; en el estudio etiológico se objetivó un linfoepitelioma de cavum y otitis serosa; tras la quimioterapia disminuyó el tamaño tumoral y desapareció el SH. Conclusiones. El estudio del cavum en pacientes con este síndrome es importante para el diagnóstico y el tratamiento precoces de una zona de difícil acceso a la exploración directa. La existencia de patología asociada en el oído o los senos paranasales nos debe orientar más hacia su origen en la nasofaringe

Introduction. Horner syndrome (HS) is produced by damage to the oculosympathetic pathway and gives rise to palpebral ptosis, enophthalmos and myosis. Anhidrosis also occurs in cases in which the lesion is located before the separation of the vasomotor and sudomotor fibres at the start of the internal carotid artery. Nasopharyngeal cancer may damage the cranial nerves, mainly in the cavernous sinus, and very few cases of HS due to infiltration of the tumour into the parapharyngeal space have been reported. Case reports. Case 1: a 67-year-old male with a 10-day history of HS on the left side without anhidrosis. The aetiological study revealed a nasopharyngeal lymphoepithelioma; the tumour became smaller and HS dis-appeared following treatment with chemotherapy. Case 2: a 48-year-old male with a 1-week history of HS without anhidrosis, and a 2-month history of deafness and pain in the right ear. The aetiological study showed a nasopharyngeal lympho-epithelioma and serous otitis. The tumour became smaller and HS disappeared following treatment with chemotherapy. Conclusions. It is important to carry out studies of the nasopharynx in patients with this syndrome to allow early diagnosis and treatment of a region that is not readily available for direct examination. The existence of an associated pathology in the ear or the paranasal sinuses strongly suggests that its origins lie in the nasopharynx

[Mumps meningitis: a case mix in a Neurology Department]. / Meningitis urliana: casuística en un servicio de Neurología.

INTRODUCTION: Parotiditis is a viral infection that produces a non-suppurative inflammation of the parotid glands, although it may affect other salivary glands, the testicles, meninges and the pancreas. Clinical meningitis appears in 1-10% of cases, normally between the 4th and the 10th day after infection of the parotid glands. In this study, we analyse the clinical and analytic characteristics of the patients admitted to our Neurology Department over the last 12 years. CASE REPORTS: A retrospective study was conducted by analysing the hospital discharge reports. The eligibility criterion was the existence of an acute meningeal syndrome linked to the presence of positive IgM titres for the parotiditis virus. In all, 13 cases were found, with a mean age of 16.5 years, and most of them were males (92.3%). Parotiditis preceded meningitis in all our patients with an average of 8.3 days, while orchiepididymitis, when it occurred, appeared at the same time or after meningitis. Analysis of the cerebrospinal fluid showed a lymphocyte profile with glycorrhachia above 40%, except in two cases that showed a non-persistent neutrophilic profile. There was a transient 30.7% reduction in prothrombin time in the patients. No sequelae were observed. CONCLUSIONS: Vaccination campaigns have drastically reduced the number of cases of meningitis due to this germ since the second half of the 90s. This virus must be borne in mind in cases of meningitis in young males who come from underdeveloped countries.

Meningitis urliana: casuística en un servicio de Neurología / Mumps meningitis: a case mix in a neurology department

Introducción. La parotiditis es una infección vírica que produce inflamación no supurada de las parótidas, aunque puede afectar a otras glándulas salivares, los testículos, las meninges y el páncreas. La meningitis clínica aparece en el 1-10% de los casos, normalmente entre el 4.º y el 10.º día de la inflamación parotídea. Se analizan las características clínicas y analíticas de los pacientes con esta meningitis ingresados en nuestro Servicio de Neurología en los últimos 12 años. Casos clínicos. Se ha realizado un estudio retrospectivo analizando los informes de alta. El criterio de inclusión es la existencia de un síndrome meníngeo agudo asociado a la presencia de una valoración positiva IgM frente al virus de la parotiditis. Se han encontrado 13 casos con una media de edad de 16,5 años; la mayoría son varones (92,3%). La parotiditis precede a la meningitis en todos nuestros pacientes en 8,3 días de media, mientras que la orquiepididimitis, cuando aparece, lo hace a la vez que la meningitis o después. El análisis del líquido cefalorraquídeo muestra un perfil linfocitario con glucorraquia por encima del 40%, excepto en dos casos que presentan un perfil neutrofílico no persistente. Existe un descenso transitorio del tiempo de protrombina en el 30,7% de los pacientes. No hemos encontrado secuelas. Conclusiones. Las campañas de vacunación han producido una drástica reducción de las meningitis por este germen a partir de la segunda mitad de los años 90. Se debe tener en cuenta este virus en los casos de meningitis en varones jóvenes procedentes de países subdesarrollados

Introduction. Parotiditis is a viral infection that produces a non-suppurative inflammation of the parotid glands, although it may affect other salivary glands, the testicles, meninges and the pancreas. Clinical meningitis appears in 1-10% of cases, normally between the 4th and the 10th day after infection of the parotid glands. In this study, we analyse the clinical and analytic characteristics of the patients admitted to our Neurology Department over the last 12 years. Case reports. A retrospective study was conducted by analysing the hospital discharge reports. The eligibility criterion was the existence of an acute meningeal syndrome linked to the presence of positive IgM titres for the parotiditis virus. In all, 13 cases were found, with a mean age of 16.5 years, and most of them were males (92.3%). Parotiditis preceded meningitis in all our patients with an average of 8.3 days, while orchiepididymitis, when it occurred, appeared at the same time or after meningitis. Analysis of the cerebrospinal fluid showed a lymphocyte profile with glycorrhachia above 40%, except in two cases that showed a non-persistent neutrophilic profile. There was a transient 30.7% reduction in prothrombin time in the patients. No sequelae were observed. Conclusions. Vaccination campaigns have drastically reduced the number of cases of meningitis due to this germ since the second half of the 90s. This virus must be borne in mind in cases of meningitis in young males who come from underdeveloped countries