[L-2 hydroxyglutaric aciduria: presentation of a family diagnosed in adulthood]. / Aciduria L-2-hidroxiglutárica: presentación de una familia con diagnóstico en la edad adulta.

Introduction. Organic acidurias are a group of hereditary metabolic disorders characterized by an increase in excretion of organic acids in urine. L-2 hydroxyglutaric aciduria is a neurodegenerative disorder with insidious onset after infancy, which is likely inherited in an autosomal recessive mode, characterized by mental retardation, progressive ataxia, epilepsy, macrocephaly, pyramidalism and extrapyramidal symptoms in variable combinations, with subcortical encephalopathy and cerebral atrophy in neuroimaging studies. Biochemical diagnosis was based on the detection of high levels of L-2 hydroxyglutaric acid in body fluids. Clinical case. We present the case of a 42 year old male patient with psychomotor development delay, generalized tonic epileptic crisis, and ataxia and pyramidal syndrome after the age of 18 months. Neuroimaging study findings revealed subcortical leukoencephalopathy. Diagnosis of the disease was reached after measuring the level of L-2 hydroxyglutaric acid in body fluid (blood, urine and cerebrospinal fluid). This diagnosis was also confirmed in three of the patient's brothers who were affected by a non-filial neurological disease by measurement of this acid level in urine. The genetic study was performed in all the cases. Discussion. As with the majority of patients who reach adulthood without having been diagnosed of this disease during infancy, we believe that this disorder should be considered as a possibility in adults presenting a combination of the symptoms described and subcortical encephalopathy in magnetic resonance imaging, regardless of whether there is a family background of it. Thus, it should be included in the differential diagnosis of leukodystrophy in adult patients.

Calambres musculares y mialgias secundarias a zolmitriptán / Muscle cramps and myalgia related to zolmitriptan

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Heterotopia subcortical / Subcortical heterotopia

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[Neurotoxic effects induced by the topical administration of cycloplegics. A case report and review of the literature]. / Efectos neurotóxicos inducidos por administración tópica de ciclopléjicos. Caso clínico y revisión de la bibliografía.

INTRODUCTION: We report a patient who developed an acute confusional state with hallucinations after exposure to cycloplejic eye drops, and review the current literature regarding neurotoxicity due to this type of eye drops. CASE REPORT: A 61 year-old man who developed in two occasions confusion, disorientation and vivid visual hallucinations following exposure to a cyclopejic eye drop containing atropine 2%, scopolamine 0.5% and phenylephrine 4%. We performed a literature search regarding neurological complications of cycloplegic eye drops using the PubMed Database and the services of the Virtual Library 'Agencia Lain Entralgo'. The clinical features of all reports in which the original document was obtained are analyzed and summarized. We have summarized the clinical features of 29 patients with neurotoxicity due to cyclopentolate, 19 to atropine, 18 to scopolamine, 7 to homatropine, and 2 to tropicamide. Our patient should be the fourth reported in Spain, being the offending drug in the four cases the same eye drop. The most commonly reported symptoms are visual hallucinations, behavioral disorders/acute psychosis, alterations of consciousness/confusion, restlessness/hyperactivity, ataxia and speech disorders. Many of the patients reported are children and elder. There have been reported some fatal cases, specially related with atropine. CONCLUSIONS: Neurotoxicity related with anticholinergic effects of cycloplegic agents is not infrequent, although it is not well known in our setting; and can cause death in some cases. Exposure to these drugs should be taken in account in the differential diagnosis of acute confusional syndromes.

Efectos neurotóxicos inducidos por administración tópica de ciclopléjicos. Caso clínico y revisión de la bibliografía / Neurotoxic effects induced by the topical administration of cycloplegics. A case report and review of the literature

Introducción. Se describe el caso de un paciente que presentóun cuadro confusional agudo con alucinaciones visuales trasla exposición a un colirio ciclopléjico y se revisa además la bibliografíaexistente con respecto a esta complicación del uso de dichotipo de colirios. Caso clínico. Varón de 61 años que sufrió en dosocasiones un cuadro de confusión, desorientación temporoespacialy alucinaciones visuales vívidas tras exposición a un colirio ciclopléjicoque contenía atropina al 2%, escopolamina al 0,5% y fenilefrinaal 4%. Se realizó una búsqueda bibliográfica de complicacionesneurológicas de los colirios ciclopléjicos utilizando las basesde datos PubMed y los servicios de la Biblioteca Virtual de laAgencia Laín Entralgo. Se analizan los datos clínicos de todasaquellas publicaciones en las que se tuvo acceso al documento original.Hemos documentado los datos clínicos correspondientes a29 casos debidos a ciclopentolato, 19 a atropina, 18 a escopolamina,7 a homatropina y 2 a tropicamida. Nuestro caso sería el cuartoregistrado en España; en todos éstos es responsable el mismotipo de colirio. Los síntomas que se han descrito con mayor frecuenciason alucinaciones visuales, trastornos de conducta/psicosisaguda, alteración de nivel de conciencia/confusión, inquietudmotora/hiperactividad, ataxia/incoordinación motora y alteracionesdel lenguaje. La mayoría de los casos publicados correspondea niños y ancianos. En algunas ocasiones se ha descrito mortalidad,especialmente relacionada con atropina. Conclusiones. Laneurotoxicidad debida al efecto anticolinérgico de los agentes ciclopléjicosno es infrecuente, si bien es poco conocida en nuestromedio; puede llegar a causar la muerte. La exposición a dichos fármacosdebe tenerse en cuenta en el diagnóstico diferencial de loscuadros confusionales

Introduction. We report a patient who developed an acute confusional state with hallucinations after exposure tocycloplejic eye drops, and review the current literature regarding neurotoxicity due to this type of eye drops. Case report. A 61year-old man who developed in two occasions confusion, disorientation and vivid visual hallucinations following exposure toa cyclopejic eye drop containing atropine 2%, scopolamine 0.5% and phenylephrine 4%. We performed a literature searchregarding neurological complications of cycloplegic eye drops using the PubMed Database and the services of the VirtualLibrary ‘Agencia Laín Entralgo’. The clinical features of all reports in which the original document was obtained areanalyzed and summarized. We have summarized the clinical features of 29 patients with neurotoxicity due to cyclopentolate,19 to atropine, 18 to scopolamine, 7 to homatropine, and 2 to tropicamide. Our patient should be the fourth reported in Spain,being the offending drug in the four cases the same eye drop. The most commonly reported symptoms are visual hallucinations,behavioral disorders/acute psychosis, alterations of consciousness/confusion, restlessness/hyperactivity, ataxia and speechdisorders. Many of the patients reported are children and elder. There have been reported some fatal cases, specially relatedwith atropine. Conclusions. Neurotoxicity related with anticholinergic effects of cycloplegic agents is not infrequent, althoughit is not well known in our setting; and can cause death in some cases. Exposure to these drugs should be taken in account inthe differential diagnosis of acute confusional syndromes

Coma inducido por sobredosis de baclofeno intratecal / Coma induced by an overdose of intrathecal baclofen

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[Neurobrucellosis: four case reports]. / Neurobrucelosis: presentación de cuatro casos.

INTRODUCTION: Brucellosis is a form of anthropozoonosis that is found the world over. It has a particularly high incidence rate in Spain, above all in rural areas such as Extremadura, Castile and Leon, Castile-La Mancha and Aragon, largely due to the consumption of non-pasteurised milk and cheese. Neurobrucellosis can be defined as a set of either early or late neurological complications caused by Brucella. It is difficult to determine its frequency, although it oscillates between 2-18% of all cases of brucellosis. CASE REPORTS: We report the cases of four patients diagnosed as having neurobrucellosis. The different presenting clinical symptoms, the complementary explorations, treatment and duration are all described. CONCLUSIONS: The clinical features of neurobrucellosis vary greatly and, in general, tend to be chronic. Many of the laboratory procedures usually employed in the diagnosis of brucellosis frequently give negative results. For these reasons, and because it is a disease that is both treatable and curable, the degree of suspicion must be high, especially in endemic areas, so that an early diagnosis can be made to allow suitable treatment to be established.

[Brain abscesses due to Listeria monocytogenes]. / Abscesos cerebrales por Listeria monocytogenes.

INTRODUCTION: Listeria monocytogenes is a gram-positive bacillus which causes sporadic infections in immunocompromised humans, with a special propensity for the central nervous system, in the form of acute, subacute or chronic meningitis, rhombencephalitis or abscesses in the brain or spinal cord. The final diagnosis is established by germ culture in blood or in cerebrospinal fluid (CSF). Preferred treatment is ampicillin in association with aminoglycosides. CASE REPORT: We report the case of a 70-year-old male patient with a history of arterial hypertension and chronic lymphatic leukaemia with no specific treatment, who suffered meningoencephalitis and brain abscesses caused by L. monocytogenes. Symptoms were a 48-hour history of headache and a febrile condition. The CSF showed lymphocytic pleocytosis with hypoglycorrhachia. Magnetic resonance scans of the brain revealed areas of cerebritis and multiple brain abscesses in the right frontal lobe. Specific treatment was established with ampicillin for 13 weeks, associated with gentamicin and vancomycin during the first few weeks, until x-rays showed the lesions to be resolved. CONCLUSIONS: L. monocytogenes infections must be investigated in all patients with cellular immunosuppression who present febrile symptoms. The central nervous system may be the only area of the body infected. Moreover, this site will need studying in patients who present neurological focus data or an alteration in the state of consciousness and bacteraemia due to L. monocytogenes. Establishing suitable treatment as early as possible can improve the prognosis.

[A relapse of multiple sclerosis manifesting as acute delirium]. / Trastorno delirante agudo como manifestación de un brote de esclerosis múltiple.

Psychotic symptoms are infrequent in multiple sclerosis (MS) and their relationship to cerebral lesions has not been clearly documented. The case of a 58 year old woman with secondary progressive MS is presented. She had acute delirium with persecutory delusions associated to paresis of her left leg. Magnetic resonance imaging of the brain disclosed an active lesion in the left hippocampus. The patient was treated with risperidone and megadoses of methylprednisolone, with dramatic improvement. Clinical and radiological data in this patient suggest that psychotic disorders can be symptomatic of a relapse in MS and, therefore, susceptible to be treated with steroids.