ABSTRACT
INTRODUCTION: Epicrania fugax is a recently reported condition consisting in brief painful paroxysms that begin in the posterior regions of the brain and irradiate towards the ipsilateral eye, nose or temple. AIMS: To present 18 cases of epicrania fugax from a monographic headache centre in a tertiary hospital and to analyse their demographic and clinical features, as well as the indication and response to prophylactic treatment. PATIENTS AND METHODS: Between March 2008, when epicrania fugax was first reported, and March 2011, of a total of 1210 patients who were attended in that service (1.48%), 18 (12 females and 6 males) were diagnosed as suffering from this condition. Six of these cases had been published earlier. RESULTS: The mean age at onset was 42.5 ± 17.7 years (range: 23-82 years). They presented painful paroxysms that began in the occipital (n = 11; 61.1%), parietal (n = 6; 33.3%) or parieto-occipital (n = 1; 5.6%) regions and irradiated towards the ipsilateral eye (n = 12; 66.6%) or temple (n = 6; 33.3%); the whole process lasted less than 15 seconds. Most of them described the pain as lancinating or stabbing. In 10 cases (55.5%) a pain remained in the area where the paroxysms began, which in 6 cases (33.3%) was limited to a well-defined circular area and met the criteria for classification as nummular headache. In 12 cases (66.6%), prophylactic treatment was used, above all lamotrigine and gabapentin, with varying results. CONCLUSION: Our aim is to back the proposal of epicrania fugax as a new syndrome with a well-defined clinical spectrum. It does not appear to be an exceptional condition and further knowledge about it will probably give rise to the description of new series. Treatment is often necessary and, although further information and experience are needed, gabapentin and lamotrigine both play a promising role.
Subject(s)
Headache , Adult , Aged , Aged, 80 and over , Female , Headache/diagnosis , Headache/drug therapy , Humans , Male , Middle Aged , Young AdultABSTRACT
INTRODUCTION: A severe form of acute encephalitis associated to antibodies against the N-methyl D-aspartate (NMDA) receptor has recently been reported. This clinical picture occurs in young people, not always with an underlying tumour and, despite the initial severity, if identified and treated at an early stage, complete recovery without any kind of sequelae can be achieved. We report on a new case and review the body of knowledge currently available on this recently identified condition. CASE REPORT: A 22-year-old female who visited our centre due to a progressive conduct disorder. Over the days that followed, deterioration in the level of consciousness made it necessary to put her on assisted respiration. Magnetic resonance imaging of the head showed hyperintense lesions mainly in deep temporal regions. A study of the cerebrospinal fluid revealed pleocytosis with a predominance of lymphocytes and antibodies against the NMDA receptor. In the complementary study no underlying tumour was observed. Treatment with corticosteroids and immunoglobulins brought about a slow but steady improvement. After a one-year follow-up there have been no recurrences, no tumours have appeared and the patient has gone back to her usual day-to-day activities. Even a significant temporal atrophy that developed at the beginning of the clinical picture has gradually been seen to resolve itself in neuroimaging studies performed as a control measure. CONCLUSION: Encephalitis due to anti-NMDA antibodies is a disease that has only recently been characterised, but which, according to currently available data, is relatively frequent. Clinically well defined, suspicion of this condition will make it possible to reach a definitive diagnosis and to establish early treatment.
Subject(s)
Antibodies/adverse effects , Antibodies/immunology , Limbic Encephalitis/etiology , Receptors, N-Methyl-D-Aspartate/immunology , Female , Humans , Limbic Encephalitis/immunology , Limbic Encephalitis/physiopathology , Young AdultABSTRACT
Introducción. Recientemente se ha descrito una forma grave de encefalitis aguda asociada a anticuerpos contra el receptor N-metil-D-aspartato (NMDA). Este cuadro ocurre en jóvenes, no siempre con un tumor subyacente y, pese a la gravedad inicial, su identificación y tratamiento precoz pueden llevar a recuperaciones sin secuelas. Presentamos un nuevo caso y revisamos los conocimientos disponibles acerca de esta nueva entidad. Caso clínico. Mujer de 22 años de edad que acudió a nuestro centro como consecuencia de un trastorno progresivo del comportamiento. Durante los días siguientes, un deterioro del nivel de conciencia hizo necesario el soporte respiratorio. Una resonancia magnética craneal mostró lesiones hiperintensas principalmente en regiones temporales profundas. El estudio de líquido cefalorraquídeo reveló pleocitosis con predominio linfocitario y anticuerpos contra el receptor NMDA. En el estudio complementario no se apreció tumor subyacente. El tratamiento con corticoesteroides e inmunoglobulinas dio lugar a una lenta pero continua mejoría. Al cabo de un año de seguimiento no ha presentado recidivas, no ha aparecido tumor alguno y la paciente se ha reintegrado a sus actividades habituales. Incluso una atrofia temporal llamativa desarrollada al inicio del cuadro se ha ido resolviendo en estudios de neuroimagen de control. Conclusión. La encefalitis por anticuerpos anti-NMDA es una enfermedad recientemente caracterizada, pero, de acuerdo con los datos disponibles hasta la fecha, relativamente frecuente. Clínicamente bien definida, la sospecha de esta entidad hará posible un diagnóstico definitivo y la instauración de un tratamiento precoz (AU)
Introduction. A severe form of acute encephalitis associated to antibodies against the N-methyl D-aspartate (NMDA) receptor has recently been reported. This clinical picture occurs in young people, not always with an underlying tumour and, despite the initial severity, if identified and treated at an early stage, complete recovery without any kind of sequelae can be achieved. We report on a new case and review the body of knowledge currently available on this recently identified condition. Case report. A 22-year-old female who visited our centre due to a progressive conduct disorder. Over the days that followed, deterioration in the level of consciousness made it necessary to put her on assisted respiration. Magnetic resonance imaging of the head showed hyperintense lesions mainly in deep temporal regions. A study of the cerebrospinal fluid revealed pleocytosis with a predominance of lymphocytes and antibodies against the NMDA receptor. In the complementary study no underlying tumour was observed. Treatment with corticosteroids and immunoglobulins brought about a slow but steady improvement. After a one-year follow-up there have been no recurrences, no tumours have appeared and the patient has gone back to her usual day-to-day activities. Even a significant temporal atrophy that developed at the beginning of the clinical picture has gradually been seen to resolve itself in neuroimaging studies performed as a control measure. Conclusion. Encephalitis due to anti-NMDA antibodies is a disease that has only recently been characterised, but which, according to currently available data, is relatively frequent. Clinically well defined, suspicion of this condition will make it possible to reach a definitive diagnosis and to establish early treatment (AU)
