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Background and objective Acute transverse myelitis (TM) is an infrequent neurological complication of systemic lupus erythematosus (SLE). Short-term outcome varies widely between cohorts. Little is known about the epidemiology and long-term functional outcome of TM associated to SLE. Methods Patients with SLE and acute TM were identified during hospital admission, visits to the Emergency Room or the Neurology Outpatient Clinic. We evaluated ambispectively those patients with SLE presenting with clinical myelopathy and corroborated with spinal MRI. Cases were divided as partial (non-paralyzing) or complete (paralyzing). We determined long-term functional outcome as well as mortality in those patients with follow-up periods of at least five years. Results We identified 35 patients (partial, n = 15; complete, n = 20) in which complete clinical and imaging data were available (26 with follow-up ≥ 5 years). Patients with complete TM were significantly older than those with partial forms. Positive antiphospholipid antibodies were observed in 80% of patients, suggesting a possible mechanistical role. Surprisingly, functional recovery at one year was in general good; however, we observed a five-year mortality of 31% because of sepsis (in 10 cases) or pulmonary embolism (in one case). Conclusions Short-term outcome of SLE-related TM is generally good, and recurrence rate is low. However, we observed a long-term fatality rate of 31% for reasons unrelated to TM, suggesting that TM is a manifestation of severe immune dysregulation and a predictor of severity and mortality in patients with SLE.
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Lupus Eritematoso Sistémico/complicaciones , Mielitis Transversa/diagnóstico por imagen , Mielitis Transversa/mortalidad , Adulto , Azatioprina/uso terapéutico , Femenino , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Imagen por Resonancia Magnética , Masculino , México , Mielitis Transversa/etiología , Prednisona/uso terapéutico , Centros de Atención Terciaria , Adulto JovenRESUMEN
INTRODUCTION: Neurally-mediated syncope (NMS) is defined as a transient loss of consciousness due to an abrupt and intermittent drop in blood pressure (BP). OBJECTIVES: This study describes the putative pathophysiological mechanisms giving rise to NMS, the role of baroreflex (BR), and the interaction of its main haemodynamic variables: heart rate (HR) and BP. DEVELOPMENT: Episodic dysregulation affects control over the haemodynamic variables (HR and BP) mediated by baroreflex mechanisms. During active standing, individuals experience a profound transient drop in systolic BP due to the effect of gravity on the column of blood and probably also because of reflex vasodilation. Abnormalities in the BR in NMS could be due to a more profound drop in BP upon standing, or to delayed or incomplete vasoconstriction resulting from inhibited or delayed sympathetic activity. CONCLUSIONS: Sympathetic hyperactivity is present in patients with NMS at rest and before syncope. During active standing or passive tilting, excessive tachycardia may be followed by bradycardia and profound hypotension. Recovery of systolic BP is delayed or incomplete.
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Barorreflejo , Postura , Reflejo , Síncope/fisiopatología , Sistema Nervioso Autónomo/fisiopatología , Presión Sanguínea , Frecuencia Cardíaca , HumanosRESUMEN
INTRODUCTION: When studying patients with dementia the search of reversible or treatable entities has a primordial role. Many patients do not recover the previous cognitive level; however, that does not signify that the disease is not treatable with partial recovery or prevention of secondary progression. DEVELOPMENT: The prevalence of reversible dementias is around 15% in most series. Rutine studies include complete blood cell count, erythrocyte sedimentation rate, electrolytes and chemistry panel, liver function tests, vitamin B12 and folate serum levels, urianalysis and syphilis serology. Special tests such as thyroid function tests, protein electrophoresis, human immunodeficiency virus serology, heavy metals serum levels, electrocardiography, cerebrospinal fluid analysis, and chest roentnography should be performed according to the patient clinical profile. Neuroimaging should be done in all cases. Non-contrasted computed tomography must be performed first. Magnetic resonance imaging is most useful for searching for more subtle structure abnormalities. Electroencephalogram, positron emission tomography and single photon emission computed tomography are not recommended as routine studies. Brain biopsy is indicated in few cases. CONCLUSION: Most common causes of reversible and treatable dementias include: medications, obstructive hydrocephalus, infectious, vascular, immunological, psychiatric, toxic and metabolic disorders, among others.
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Demencia , Demencia/tratamiento farmacológico , Demencia/etiología , Demencia/patología , Demencia/fisiopatología , Diagnóstico Diferencial , Progresión de la Enfermedad , Humanos , Imagen por Resonancia MagnéticaRESUMEN
INTRODUCTION: The veno-arteriolar reflex (VAR) is triggered by an increase in the transmural venous pressure on placing a part of the body in the same direction as the gravitational acceleration below the heart. AIM: To assess the VAR in healthy subjects on raising a part of the body above the level of the heart. SUBJECTS AND METHODS: VAR was studied in 16 healthy subjects (20-65 years old) by means of changes in the blood flow in the skin detected using a digital infrared photoplethysmograph attached to the fingertip under the following conditions: right arm at the height of the heart, right arm below the heart and right arm below the level of the heart. The variables measured were: amplitude of the blood flow in the skin with the arm raised to the height of the heart (baseline amplitude), percentage decrease of the blood flow in the skin with the arm below the heart and percentage increase in blood flow with the arm above the heart. RESULTS: The percentage of vasoconstriction with the right arm below the heart was 35%, and that of vasodilation, 50%. CONCLUSIONS: Evaluation of the VAR with the arm below the heart causes vasoconstriction, and elevation of the arm causes an important degree of vasodilation. Vasoconstriction and vasodilation are maintained while the limb is kept above or below the heart. This is an economical and potentially very useful way of studying the innervation of the microcirculation in a number of different peripheral neuropathies of thin and mixed fibres.
TITLE: Las dos caras del reflejo venoarteriolar: vasoconstriccion y vasodilatacion cutanea al bajar y subir el brazo.Introduccion. El reflejo venoarteriolar (RVA) lo provoca un incremento en la presion venosa transmural al colocar una parte del cuerpo en el sentido de la aceleracion gravitatoria por debajo del corazon. Objetivo. Evaluar el RVA en sujetos sanos al levantar una parte del cuerpo por encima del corazon. Sujetos y metodos. En 16 sujetos sanos (20-65 años) se estudio el RVA mediante cambios en el flujo sanguineo de la piel con un fotopletismografo digital infrarrojo colocado en el pulpejo en sujetos sanos durante las siguientes condiciones: brazo derecho a la altura del corazon, brazo derecho 40 cm por debajo del corazon y brazo derecho 40 cm por encima del corazon. Las variables medidas fueron: amplitud del flujo sanguineo de la piel con el brazo a la altura del corazon (amplitud basal), porcentaje de disminucion del flujo sanguineo de la piel con el brazo por debajo del corazon y porcentaje de aumento del flujo sanguineo de la piel con el brazo por encima del corazon. Resultados. El porcentaje de vasoconstriccion con el brazo derecho por debajo del corazon fue del 35%, y el de vasodilatacion, del 50%. Conclusiones. La evaluacion del RVA con el brazo por debajo del corazon provoca vasoconstriccion, y la elevacion del brazo produce una importante vasodilatacion. La vasoconstriccion y la vasodilatacion se mantienen mientras la extremidad se mantenga por encima o por debajo del corazon. Este es un estudio potencialmente muy util y economico para estudiar la inervacion de la microcirculacion en diversas neuropatias perifericas de fibras delgadas y mixtas.
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Brazo/fisiología , Reflejo , Vasoconstricción , Vasodilatación , Adulto , Anciano , Humanos , Persona de Mediana Edad , Flujo Sanguíneo Regional , Piel , Adulto JovenRESUMEN
Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.
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Anticuerpos Antinucleares/análisis , Antígenos HLA-D/inmunología , Síndromes Paraneoplásicos/epidemiología , Síndromes Paraneoplásicos/inmunología , Formación de Anticuerpos/fisiología , Comorbilidad , Femenino , Antígenos HLA-D/análisis , Humanos , Inmunidad/fisiología , Masculino , Neoplasias/epidemiología , Neoplasias/inmunología , Proteínas Oncogénicas/análisis , Proteínas Oncogénicas/inmunología , Sensibilidad y EspecificidadRESUMEN
INTRODUCTION: Acute peripheral neuropathy represents a medical emergency. The causes of it are diverse and plentiful. The most common cause of acute paralytic peripheral neuropathy is the Guillain-Barré syndrome (GBS). As many as 85% of those affected can be expected to make an excellent recovery. OBJECTIVE: To describe the principal risk factors associated, clinical manifestations, treatment, evolution and complications of 28 cases of Guillain-Barré syndrome (GBS) in the "Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán". METHODS: We search in our computer records all files under diagnostic of GBS, during the last ten years. We found 28 cases that were available to study. RESULTS: Mean age was 37 years old (SD 17.2). Fifteen patients were female (54%) and 13 were male (46%). Nine patients (32%) were preceded by a superior via infection, 5 (18%) by a diarrhea illness and 14 patients had not a predisposing factor. The duration of symptoms before diagnostic has a median of 7 days (2-15). Twenty-six patients (93%) had an ascending paralysis and 18 had paresthesias (64%). The most frequent subtype was acute inflammatory-demyelinating polyneuropathy (AIDP) in 18 patients (64%), acute motor-sensory axonal neuropathy (AMSAN) in 5 (18%), acute motor axonal neuropathy (AMAN) in 3 (11%) and 2 patients (7%) had the Fisher-Miller syndrome. Fifteen patients (54%) developed respiratory involvement requiring mechanical ventilation. Twenty-four patients (86%) had cerebrospinal fluid proteins elevated. Twenty patients (72%) had a total recovery, 6 (21%) had a partial recovery and 2 had not any response (7%). DISCUSSION: GBS is a particularly highstakes illness in that its onset is sudden and paralysis is frequently extreme (requiring assisted respiration), however, as many as 85% of those affected can be expected to make an excellent recovery. In our study the majority of patients (54%) develop respiratory involvement requiring mechanical ventilation but in this group the majority had a favorable outcome (71%).
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Síndrome de Guillain-Barré , Adulto , Femenino , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/epidemiología , Síndrome de Guillain-Barré/terapia , Humanos , Masculino , Factores de RiesgoRESUMEN
Central neurogenic hyperventilation in patients with a normal level of consciousness is uncommon. This condition occurs in bilateral pontine tegmental lesions, particularly tumors such as CNS lymphomas and glioma, as well as traumatic lesions. The physiopathological mechanisms are unknown and no there is no effective treatment for this entity. We report a case of central neurogenic hyperventilation associated to a unilateral basal pontine infarction.
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Infartos del Tronco Encefálico/complicaciones , Hiperventilación/etiología , Puente , Humanos , Hiperventilación/fisiopatología , Masculino , Persona de Mediana EdadRESUMEN
Electroencephalography is an important tool in the diagnosis of primary or secondary disorders of central nervous system, epilepsy is one of the most important. Sometimes normal electroencephalographic activity simulates epileptiform activity. This activity does not have pathological value and is considered a variant of normal brain activity. The main groups based on the classification proposed by Blum and Cervone are: rhythmic patterns; epileptiform patterns; lambda waves, and age dependent changes. These changes are frequently seen during drowsiness, sleep and during activation maneuvers and more common in children and adolescents. The aim of this review is to present the most important characteristics of each of these variants, in order to prevent them being confused with abnormal brain activity.