Which route of administration of acid tranexamic, intravenous or intra-articular, is more effective in the control of post-surgical bleeding after a total hip arthroplasty? A prospective, controlled and randomized study. / Aplicación del tranexámico intravenoso o intraarticular en el control del sangrado posquirúrgico tras una artroplastia total de cadera. Estudio prospectivo, controlado y aleatorizado.

INTRODUCTION: One of the most frequent complications after a total hip arthroplasty (THA) is bleeding, intravenous tranexamic acid (TXA) is used to reduce it. We considered it necessary to carry out a study to clarify which administration route is superior. MATERIAL AND METHOD: Prospective, controlled and randomized study in 2 arms carried out between February 2017 and February 2018. 15mg/kg of intravenous TXA were administered in group-A and 2gr of intra-articular TXA in group-B. The values of haemoglobin and haematocrit were evaluated at 24h-72h, blood loss volume, drained blood volume, transfusions and complications. RESULTS: 78 patients were included, 31 with intravenous treatment and 47 with intra-articular. The decrease of haemoglobin in the intravenous group was 3.15±1.64g/dl in 24h and 3.75±1.56g/dl in 72h, the haematocrit decreased by 10.4±4.17% in 24h and 11.85±4.15% in 72h. In the intra-articular group there was a haemoglobin fall of 3.03±1.30g/dl in 24h and 3.22±1.2g/dl in 72h and the haematocrit fell by 10.66±3.6% and 12,11±3.29% in 24 and 72h (P>.05). The mean drainage in 24h was 195.80ml in group-A versus 253.93ml in group-B (P>.05) and in 48h it was 225.33ml in group-A and 328.19ml in group-B (P=.009). The intravenous group lost an average of 1,505ml of blood compared to the 11,280ml of the intra-articular group. In 5.1% of the cases, transfusions were necessary. We had no secondary complications. CONCLUSIONS: The different routes of administration of TXA in THA have a similar effect in the reduction of postoperative bleeding. There was no evidence of an increase in complications.

Lung adenocarcinoma during pregnancy: clinical case and literature review.

Lung cancer during pregnancy is a very rare disease with less than 70 cases published to date. Information about specific lung cancer as adenocarcinoma is even more limited. Genetic techniques can detect oncogene mutations which seem to be more frequent among cases of lung cancer in pregnant women and could determine both the treatment and prognosis. We present a recent case seen at our hospital and a literature review.

Cobalt toxicity after revision total hip replacement due to fracture of a ceramic head. / Toxicidad por cobalto después de la revisión a una artroplastia total de cadera posterior a fractura de cabeza cerámica.

Symptomatic cobalt toxicity from a failed total hip replacement is a rare, but devastating complication. Potential clinical findings include cardiomyopathy, hypothyroidism, skin rash, visual and hearing impairment, polycythaemia, weakness, fatigue, cognitive impairment, and neuropathy. The case is presented of a 74year-old man in whom, after a ceramic-ceramic replacement and two episodes of prosthetic dislocation, it was decided to replace it with a polyethylene-metal total hip arthroplasty (THA). At 6months after the revision he developed symptoms of cobalt toxicity, confirmed by analytical determination (serum cobalt level=651.2µg/L). After removal of the prosthesis, the levels of chromium and cobalt in blood and urine returned to normal, with the patient currently being asymptomatic. It is recommended to use a new ceramic on ceramic bearing at revision, in order to minimise the risk of wear-related cobalt toxicity following breakage of ceramic components.

Secuencia Moebius. Análisis retrospectivo de 30 pacientes / Moebius syndrome. A retrospective analysis of 30 patients.

Introducción: La secuencia Moebius se caracteriza por el compromiso congénito de los nervios motor ocular externo y facial, y se puede asociar al compromiso de otros pares craneales y a otros defectos congénitos. Su etiología es multifactorial y no bien definida, actualmente la teoría más aceptada es la disrupción vascular durante el desarrollo del romboencéfalo. Su incidencia exacta se desconoce, pero impresiona estar en aumento y asociada a la exposición prenatal a teratógenos. Objetivos: Analizar las historias clínicas de 30 pacientes con secuencia Moebius y las características de su compromiso ocular. Materiales y métodos: Estudio retrospectivo, transversal, observacional y descriptivo. Se analizaron 30 historias clínicas de pacientes con secuencia Moebius atendidos por vez primera entre el mes de Julio de 1999 y Junio de 2012 por el servicio de Oftalmología del Hospital Garrahan. Resultados: Se estudiaron 30 pacientes 15 de sexo femenino y 15 de sexo masculino, dentro de los antecedentes maternos 7 madres refirieron ingesta de misoprostol y 4 tuvieron metrorragias durante el primer trimestre de embarazo. Todos los pacientes tuvieron compromiso del VII nervio; en 20 pacientes fue bilateral y simétrico; y en los restantes asimétrico. Todos los pacientes tuvieron compromiso del VI nervio bilateral, a algunos de los cuáles se les efectuó cirugía de estrabismo otros están en plan de cirugía y unos pocos no la requirieron por presentar fijación de ambos ojos en posición primaria de la mirada. Conclusión: la secuencia Moebius es una rara patología genética y congénita multifactorial y de compromiso multisistémico que ha visto incrementada su frecuencia desde el uso de ciertos fármacos teratógenos y que obliga a una intervención quirúrgica precoz de neuroortopedistas, oftalmólogos, cirujanos plásticos y control clínico multidisciplinario para brindarles a estos niños las mejores posibilidades de desarrollo funcional y estético reparador (AU)

Introduction: Moebius syndrome is characterized by congenital palsy of the external and facial oculomotpr nerves, and may be associated with involvement of other cranial nerves and congenital defects. The etiology is multifactorial and not well defined. Currently, the most widely accepted theory is a rhombencephalic maldevelopment. The true incidence of Moebius syndrome is unknown, but it seems to be increasing associated with prenatal exposure to teratogenic factors. Objectives: To analyze the clinical charts of 30 patients with Moebius syndrome assessing ocular involvement. Material and methods: A retrospective, cross-sectional, observational study. Thirty clinical charts of patients with Moebius syndrome that were first seen at the Department of Ophthalmology of Hospital Garrahan between July 1999 and June 2012 were assessed. Results: Of the 30 patients 15 were female and 15 male. Maternal history showed seven mothers that received misoprostol and four that had metrorrhagia in the first trimester of pregnancy. All patients had VII cranial nerve involvement; the involvement was bilateral and symmetric in 20 and asymmetric in the remaining patients. All patients had bilateral VI nerve involvement, some of whom underwent surgery for strabismus, others are on the list for surgery, and a few do not require surgery because of fixation of both eyes in primary gaze position. Conclusion: Moebius syndrome is a rare multifactorial genetic and congenital pathology with multisystemic involvement and increased incidence because of the use of teratogenic drugs requiring early surgical intervention by neuroorthopedic and plastic surgeons, and ophthalmologists, and a multidisciplinary follow-up to provide these children with the best possibilities for functional development and aesthetic repair (AU)

Esophageal atresia and prenatal exposure to mycophenolate.

Mycophenolate mofetil is a widely prescribed immunosuppressive agent for transplant patients and autoimmune diseases. Potential teratogenic effects after in utero exposure to mycophenolate mofetil has been described in human clinical observations. The complete clinical pattern is still being delineated. We present four newborns with esophageal atresia and other congenital anomalies, prenatally exposed to mycophenolate mofetil during the first trimester. Two of the cases had other defects related to the embryopathy: microtia, eye abnormalities and oral clefts. Two cases did not show major craniofacial anomalies. We propose that esophageal atresia with or without tracheoesophageal fistula is a feature of mycophenolate embryopathy even without the presence of other major craniofacial anomalies. The human teratogenicity of MMF is reinforced by this report, and the current contraceptive recommendations about its use in fertile women are stressed.

Precise determination of strontium isotope ratios by TIMS to authenticate tomato geographical origin.

Thermal Ionisation Mass Spectrometry (TIMS) was applied to discriminate a total of 118 tomato samples (berries, "passata", tinned tomatoes, sauce, double and triple concentrate) coming from two different countries. The TIMS technique gave significantly different results for the (87)Sr/(86)Sr ratios and δ‰ values between Chinese and Italian tomato samples, irrespective of the treatment type. This technique proved to be a "robust" method, suitable for a precise discrimination of the two geographical origins. TIMS was able, within the Italian samples, to discriminate different geographical production areas, by virtue of different (87)Sr/(86)Sr ratios and δ‰ values. This technique could be employed in the field of food safety and quality, as a profitable tool for authenticating tomato geographical origin.

Infection and immunity for human parvovirus B19 in patients with febrile exanthema.

The contribution of parvovirus B19 (B19V) as a causative agent of febrile exanthema (FE) in Cordoba, Argentina, was analysed by detection of viral DNA, and specific IgM and IgG. Serum from 141 patients with FE who were negative for measles and rubella, collected during 2005-2009, plus serum from 31 healthy individuals, were assayed. B19V was the aetiological agent in 14·9% of all FE cases, and in 39·1% in an epidemic year (2007). B19V DNA was detected in 47·6% of IgM-positive FE patients, 30·2% of IgM-negative/IgG-positive FE patients, and 9·7% of healthy controls, indicating B19V long-term infection in ~10% of immunocompetent individuals. Persistent B19V DNA was significantly more frequent in children than adults and in males than females. All patients with acute B19V infection had rash and fever, 85·7% had adenopathy, and only 14·3% had arthropathy. This is the first follow-up study of markers of infection and immunity for B19V infection in Argentina.

Neutralizing antibodies in multiple sclerosis patients treated with 375 micrograms interferon-beta-1b.

BACKGROUND: Neutralizing antibodies (NAbs) to IFN-beta may have a detrimental effect on treatment response, but increasing IFN-beta dose could reduce their occurrence. The OPTimization of Interferon for MS (OPTIMS) study was a multicenter trial investigating clinical and MRI outcomes with the approved IFN-beta-1b dose (250 microg) and a higher dose (375 microg), s.c. every other day. OBJECTIVE: To analyze the occurrence of NAbs and their effect on clinical and MRI response over a long-term (4-year) follow-up using cross-sectional and longitudinal statistical analysis. METHODS: Relapses or disease progression was assessed open-label and MRI scans were performed serially during the first year of the study. Neutralizing antibodies were measured using the MxA protein production neutralization assay. RESULTS: A total of 145 patients with relapsing-remitting multiple sclerosis from 14 centers participated in the study. Neutralizing antibody frequency was negatively associated with MRI treatment response, but no detrimental effect of NAbs on the clinical response was observed. Results obtained using cross-sectional or longitudinal statistical approaches were similar. Over the 4-year period, NAb-positive patients treated with 375 microg had a significantly greater probability of NAb disappearance (hazard ratio: 3.41; 95% confidence interval: 1.78 - 6.43; p < 0.01). CONCLUSION: Use of an IFN-beta-1b dose higher than the currently approved 250-microg dose is associated with an increased probability of NAb disappearance. The OPTIMS study was registered at ClinicalTrials.gov: NCT00473213.

MRI activity and neutralising antibody as predictors of response to interferon beta treatment in multiple sclerosis.

OBJECTIVE: To prospectively validate MRI activity and neutralising anti-interferon antibody (NAb) during the first 6 months of interferon beta treatment as response indicators in multiple sclerosis (MS). METHODS: Patients with relapsing-remitting MS were followed during the first 2 years of treatment. Neurological assessments were performed every 3 months or when a relapse was suspected. MRI scans performed at baseline and at 3, 4, 5 and 6 months after the start of treatment were assessed centrally for disease activity: new T2 or gadolinium enhancing T1 lesions. NAb were assessed using the MxA protein assay; positivity was defined as two consecutive titres >or=20 NU/ml. We evaluated the predictivity of an active scan, NAb positivity, or both, during the first 6 months of treatment, on the occurrence of clinical disease activity in the following 18 months. RESULTS: 147 patients were assessed at 16 centres. Predictivity parameters (with confidence intervals) were as follows: active scan, sensitivity (SN) 52% (34-69%), specificity (SP) 80% (65-91%), negative predictive value (NPV) 73% (58-77%), positive predictive value (PPV) 62% (42-79%), p = 0.002; NAb positivity, SN 71% (45-88%), SP 66% (55-76%), NPV 92% (82-97%), PPV 29% (16-45%), p = 0.01; active scan and NAb positivity, SN 71% (38-91%), SP 86% (73-94%), NPV 94% (86-98%), PPV 50% (29-70%), p = 0.0003. CONCLUSIONS: MRI activity and NAb occurrence during the first 6 months of interferon beta treatment were reliable predictors of long term clinical response, particularly when combined. Patients with negative predictors showed a less than 10% risk of developing clinical activity. Patients with positive predictors showed a 50% risk of further clinical activity. These patients need to be followed carefully with further MRI and NAb tests.

[Usefulness of oral glucose tolerance test (OGTT) in output patients with advanced chronic renal failure (CRF)]. / importancia del test de sobrecarga oral a la glucosa (TSOG) en una consulta específica de IRC avanzada estadio IV y V.

UNLABELLED: A pathological Oral Glucose Tolerance test (OGTT) is early marker of peripheral insulin resistance. Nevertheless, its utility in nondiabetic patients with CRF stage IV-V is undetermined. AIM: We wanted to detect, in a population of non diabetic patients with CRF, the presence of carbohydrates metabolism anomalies, by means of the OGTT and to relate it with metabolic, anthropometric, cardiovascular parameters and renal function. We studied 45 non diabetic patients with advanced CRF (stage IV-V), 26 men, mean age 66.5 years, with average Cockroft-Gault of 23.6 ml/min. We measured weight, height, waist and BMI. BIOCHEMICAL: glucose, insulin, OGTT, C peptide, lipid profile, HbA1C and Hto. Cardiovascular comorbidity, mean proteinuria and systolic and diastolic blood pressure (6 months pre and post analytical measure) were measured. Pulse pressure was also calculated. RESULTS: 47% of the patients presented normal fasting glucose, whereas 53% had isolated impaired fasting glucose (IFG). After the OGTT, 36% of the patients presented impaired glucose tolerance (IGT) and 14% diabetes (>200 mg/dl). Of the patients with normal fasting glucose, 38% had IGT after OGTT and 5% diabetes. Patients with abnormal OGTT were older (71+/-13.6 versus 60+/- 18.8 years, p=0.03), had greater HbA1C (5.6+/-0.5 versus 5.2+/-0.3%, p=0.02), total cholesterol (193+/-37.7 versus 169.8+/-44.9 mg/dl, p=0.03), pulse pressure (63.4+/- 14.5 versus 52.3+/-9.7 mmHg, p=0.0001) and greater prevalence of ischemic heart disease (28% versus 5%, p=0.05). Creatinine Clerance negatively correlated with the OGTT (r=-0.39, p=0.01) and plasma creatinine positively with fasting insulin (r=0.33, p=0.02) and C-peptide (r=0.42, p=0.006). Urinary Proteins were correlated with fasting glucose (r=0.30, p=0.04), C-peptide (r=0.52, p=0.001), triglycerides (r=0.36, p=0.01) and with the HOMA-IR index (r=0.30, p=0.05). CONCLUSION: Fasting Glucose did not predict OGTT results in patients with CRF. For this reason, we think that the OGTT can be very usefull tool to identify states of "prediabetes" and diabetes in patients with CRF, specially in those whose present an elevated Pulse Pressure, age greater than 65 years, hyperlipidaemia and HbA1C above 5.2%. The early detection of these metabolic anomalies, may lead to intensify dietetic and pharmacological measures directed to delay or to attenuate the appearance of diabetes and its serious complications in a population in which the cardiovascular risks factors are very elevated.

Every-other-day interferon beta-1b versus once-weekly interferon beta-1a for multiple sclerosis (INCOMIN Trial) II: analysis of MRI responses to treatment and correlation with Nab.

BACKGROUND: In RRMS, clinical exacerbations are usually associated with different types of active lesions at MRI, including: hyperintense lesions on T1-weighted post-gadolinium sequences; new hyperintense lesions or enlarging old lesions on PD/T2-weighted scans; or new hypointense lesions on T1-weighted pre-Gd sequences. OBJECTIVE/METHODS: Primary outcome was the occurrence of patients with at least one active MRI lesion of the different types indicated above during treatment with 250 microg every other day (EOD) interferon beta (IFNbeta)-1b or 30 microg once weekly (OW) IFNbeta-1a in outpatients with RRMS (INCOMIN Trial). RESULTS: The number of patients with at least one 'active' lesion, evaluated over the two-year follow-up, was significantly (P = 0.014) lower in the EOD IFNbeta-1 b arm (1 3/76, 17%) then in the OW IFNbeta-1a arm (25/73, 34%). NAb frequency over two-year follow-up was 22/65 (33.8%) in the EOD IFNbeta-1b arm and 4/62 (6.5%) in the OW IFNbeta-1a arm, significantly greater in the EOD IFNbeta-1b arm. CONCLUSIONS: The development of MRI active lesions is strongly reduced by EOD-IFNbeta-1b compared with OW-IFNbeta-1a, indicating that EOD-IFNbeta-1b is more effective than OW-IFNbeta-1a in reducing ongoing inflammation and demyelination in MS. Logistic regression showed that NAb status did not affect the risk of MRI activity.

[Intrathecal baclofen and Botulinum toxin in infantile cerebral palsy]. / Baclofén intratecal y toxina botulínica en la parálisis cerebral infantil.

INTRODUCTION: Infantile cerebral palsy is considered to be a motor disorder affecting both posture and movement. It is the manifestation of a cerebral lesion that took place during the maturing process of the brain. Spastic cerebral palsy is the most frequent variety. The spasticity presented by these patients strongly influences their functioning and gives rise to several complications that affect their quality of life. AIMS AND DEVELOPMENT: The main aim of this study is to determine a set of suitable and effective therapeutic steps that can be used to improve patients' spasticity and to prevent the complications that stem from them, such as contractures, deformities, surgery, etc. Multiple treatments were used, including physiotherapy, rehabilitation and oral pharmacotherapy, with few satisfactory results. At present there are two very promising therapeutic alternatives for patients with infantile cerebral palsy: Botulinum toxin, which we already have a great deal of experience with, and intrathecal treatment with baclofen. We report on a series of 10 patients aged between 8 and 15 years who had had an intrathecal baclofen infusion pump implanted. To date (maximum follow-up: 12 months), their clinical course has been satisfactory in most cases, with no severe complications related to the surgical technique, except in one individual who presented a fistula that resolved spontaneously. CONCLUSIONS: Intrathecal baclofen seems to be a very useful alternative in the treatment of spasticity to improve the quality of life of these patients and those around them.

Choanal atresia associated with prenatal methimazole exposure: three new patients.

We report three patients with bilateral choanal atresia in children prenatally exposed to methimazole (MMI) in order to define a MMI embryopathy clinical pattern. The combination of choanal atresia and other specific malformations strongly resembles previously reported patients exposed to MMI in utero. At present, propylthiouracil is considered the best treatment in pregnancies. However in Argentina and some other countries MMI is the only antithyroid drug, possibly posing a significant risk to the unborn fetus.

[Unusual forms of clinical presentation of attention deficit hyperactivity disorder]. / Formas clinicas inhabituales de presentacion del trastorno por deficit de atencion con hiperactividad.

INTRODUCTION AND DEVELOPMENT: Attention deficit hyperactivity disorder (ADHD) is one of the most frequently observed processes in our Neuropaediatric unit, and in recent years has become one of the leading reason for visits, together with headaches. On occasions, the diagnosis that should be made, in accordance with the criteria included in the DSM IV, is more difficult because initially they are patients who visit because of some other problem that becomes the centre of our clinical attention. Headaches, in our experience, are the main reason for visiting in these cases. A careful review of the patient's history and a physical examination, together with their observation during the interview, lead us to suspect a genuine case of ADHD underlying the problem that presents with other non specific symptoms. These patients are mainly boys and, less frequently, girls who see how the chances of academic and social failure increase and then become worried to such an extent that they start suffering from important tension type headaches. Concentrating the study and the therapy on just solving the problem of the headaches sometimes gives rise to therapeutic failure, which occasionally goes on for months. Other symptoms, such as tics, unspecific dizziness, pains in the abdomen and visual disorders, can also be the reason that brings ADHD patients to visit. CONCLUSIONS: When faced with these symptoms we should always bear in mind (mainly in the case of headaches) the possibility referred to above and special care must be taken not to diagnose ADHD in excess, and always base our judgment on objective proof that meets the criteria required for its diagnosis. Therapy based on pharmacological treatment and a suitable psychopedagogical intervention solves the problem in these cases and significantly reduces the symptomatology that led to the visit.

Thalidomide neuropathy: clinical, electrophysiological and neuroradiological features.

OBJECTIVE: Thalidomide is a promising therapy for multiple myeloma. Sensory neuropathy is a side effect of thalidomide and resulted to be partially reversible in 50% of cases, suggesting a sensory ganglionopathy. Spinal cord magnetic resonance imaging (MRI) was found to be useful in the diagnosis of sensory ganglionopathies and we use it to determine if thalidomide neuropathy has features of a ganglionopathy. MATERIAL AND METHODS: Six patients with multiple myeloma developed thalidomide-induced polyneuropathy. Nerve conduction studies, somatosensory-evoked potentials (SEPs) and cervical and dorsal spinal cord MRI were obtained in all. RESULTS: All patients had a sensory neuropathy, with clinical or electrophysiological abnormalities involving all four limbs. Spinal cord MRI showed high signal intensity in the posterior columns in only one patient, with abnormal central conduction time at SEPs. CONCLUSION: Our results suggest that thalidomide can induce either an axonal length-dependent neuropathy or, less frequently, a ganglionopathy.

Conocimientos de tocoginecólogos y pediatras acerca de la etiología y los factores de riesgo de los defectos congénitos / Knowledge of tocogynecologists and pediatricians about the etiology and risk factors for congenital defects

Introducción.Los defectos congénitos incluyen todas aquellas alteraciones morfológicas o funcionales presentes desde el nacimiento,de causa genética o ambiental.Objetivos.El propósito del presente estudio fue evaluar los conocimientos de tocoginecólogos y pediatras acerca de la etiología y los factores de riesgo de los defectos congénitos y analizar la asociación entre tales conocimientos y diferentes variables demográficas.Población,material y métodos.Se interrogó a 312 médicos de 12 hospitales públicos de la Ciudad de Buenos Aires con un cuestionario voluntario,anónimo e individual constituido por 33 preguntas.Resultados.La proporción de respuestas correctas fue,en promedio,de 22/33(67 por ciento,rango:18 por ciento-97 por ciento)Mas del 30 por ciento de los entrevistados no reconocio a los defectos congénitos como una de las principales causas de mortalidad infantil.Prácticamente todos los entrevistados reconocieron la asociación entre la edad materna avanzada y el síndrome de down.La talidomida fue identificada como agente teratogénico por mas del 90 por ciento de la muestra,sin embargo,aún se observo cierto desconocimiento acerca de los ácidos valproico y retinoico,A su vez mas del 30 por ciento contestó erroneamente que el consumo global de alcohol no implica un mayor riesgo durante la gestación.Las respuestas acerca del diagnóstico prenatal revelaron que existen dificultades para distinguir etiologías de las enfermedades genéticas.En algunas preguntas se observaron diferencias según la especialidad,la edad y el sexo de los entrevistados.Conclusiones.Se hace énfasis en la importancia de incorporar la enseñanza de la genética en las carreras de medicina,así como llevar a cabo acciones entre genetistas,pediatras y tocoginecólogos a fin de desarrollar programas de capacitación adecuados

[Neuropsychological problems of cerebrovascular pathology in children]. / Problemas neuropsicológicos de la patología vascular cerebral en el niño.

Cerebrovascular pathology in infancy is, unlike in adults, infrequent. However, this must not lead us to think it is unimportant. There are a number of different vascular processes that exert an influence to varying degrees on the neurological and psychological future of our young patients. These sequelae can be of more or less importance and, on some occasions, can be lesions that will accompany them throughout the rest of their lives, in spite of the therapeutic methods we have available today. We basically differentiate vascular organic lesions that are pre-established or due to malformations from other causes acquired as a consequence of an anomalous situation inherent to other pathologies and that have an extravascular origin. The most striking of these, because of their frequency, gravity and cellular pathology, are peri/intraventricular haemorrhages (PIVH) in those born premature and those weighing very little at birth. The causes from malformations remain and are relatively rare with a stable frequency, while the number of cases of PIVH has risen sharply in recent years due to different causes, the most notable of which is the increase in preterm multiple births, at around 30-32 weeks of gestation. This group of the population requires early and continuous neuropaediatric monitoring. Some of the sequelae are detected and dealt with quickly; others may appear later and will need the joint, coordinated attention of neuropaediatricians, psychologists, educators and physiotherapists.

[Alternating hemiplegia in young babies]. / Hemiplejía alternante del lactante.

INTRODUCTION. This paper review the state of the art of alternating hemiplegia of childhood. This entity is a rare neurologic disorder of infancy characterize by transient hemiplegic spells shifting from one side to another, and occasionally affecting to both hemispheres at the same time. Usually start before 18 months, many cases exhibit neonatal symptoms related with the disorder. Early symptomatology include abnormal ocular movements, mainly nystagmus, and tonic or dystonic attacks generally beginning before 6 months of age. These symptoms are frequently misdiagnosis as epilepsy. Typical hemiplegic fits which disappeared when infant felt asleep appeared by 12 months of age. Diagnosis is on clinical basis after excluding any other causes of fluctuating and transitory hemiplegic attacks. Complementary investigations, such as electroencephalograms, TAC, MRI, angiographic MRI, CFS are strictly normal. Cerebral SPECT show controversial abnormalities in some studies. We perform SPECT study in a alert 12 months old girl during an interictal period resulting in a non significant asymmetry, with lesser perfusion in left frontal basal and anterior temporal gyrus. Nevertheless, we observed an striking hyperperfusion in middle occipital area. Treatment is symptomatic with flunarizine. Our patient experience a dramatic decrease in frequency, duration and severity of hemiplegic attacks.

Thyroid function and anti-thyroid antibodies in MS patients screened for interferon treatment. A multicenter study.

Interferon beta (IFNB) treatment for multiple sclerosis (MS) has been associated with thyroid disorders (TD), in particular in patients with subclinical TD or anti-thyroid (AT) autoantibodies (autoAb) before starting treatment. TD and AT autoAb frequency was reported increased in MS. To determine whether MS patients have subclinical thyroid function abnormalities or anti-thyroid autoimmunity predisposing to develop TD, we performed a prospective multicenter screening of thyroid function and autoimmunity in 152 relapsing-remitting (RR) MS patients selected to receive IFNB treatment and in 437 healthy normothyroidal controls. Thyroid-related hormones and anti-thyroid microsomal antigen (anti-TMA) autoAb were tested with sensitive immunoradiometric or chromatographic assays. Cases were stratified for different progressively decreasing or increasing cutoff values of thyroid-stimulating hormone (TSH) (0.3, 0.2, 0.1, 3 and 5 mIU/l), and odds ratios (OR) with 95% confidence intervals (CI) calculated using logistic regression adjusted for gender, age, and anti-TMA autoAb positivity. The frequency of cases below or above the TSH cutoff values was not significantly different in MS patients and controls, and the risk to have an abnormal TSH level was not significantly increased in MS patients (OR ranging 0.37-0.84; CI, 0.05-3.01), even if anti-TMA autoAb positive (OR ranging 0.35-0.85; CI, 0.04-3.00). Frequencies of subclinical hypothyroidism and of anti-TMA autoAb positivity were, however, trending higher in MS men (ranging 5-7%) than in controls (3%). MS patients do not have an increased risk of subtle thyroid function abnormalities, subclinical TD, or anti-TMA autoAb positivity that may predispose to develop thyroid dysfunction during IFNB treatment. The positive trend for subclinical hypothyroidism and anti-TMA autoAb positivity, however, advises a longitudinal study of thyroid function and autoimmunity during IFNB treatment to see whether patients with baseline subclinical thyroid dysfunction develop clinically significant alteration during treatment.

Interferon-beta dose and efficacy: the OPTIMS study.

Interferon beta (IFN-beta) reduces exacerbation rates in patients with relapsing-remitting multiple sclerosis (MS), but some patients do not respond to treatment. Recent studies have shown a clear dose-response effect on the reduction of exacerbation rates, and on burden of disease accumulation and active lesion frequency seen on MRI. During treatment with 8 MIU IFN-beta we noticed a 30% rate of treatment failure. We then treated non-responders with 12 MIU IFN-beta and observed significant improvement in the clinical signs of disease activity. In order to compare the efficacy of two different doses of IFN-beta-1b, a multicenter study for the optimization of interferon for MS (OPTIMS) has been organized. The design of the study is presented here.