Malformación de Arnold Chiari / Arnold Chiari malformation

La malformación de Arnold Chiari es una enfermedad rara que consiste en una alteración anatómica de la base del cráneo, en la que se produce herniación del cerebelo y del tronco del encéfalo a través del foramen magnum hasta el canal cervical. Muchos niños afectados nunca llegan a tener síntomas. En el caso de que cause síntomas, estos no suelen aparecer hasta la infancia tardía o adolescencia. Presentamos el caso clínico de un paciente de 2 años con malformación de Arnold Chiari tipo I. (AU)

Arnold Chiari malformation is a rare disease that consists of an anatomical alteration of the base of the skull, in which herniation of the cerebellum and brainstem occurs through the foramen magnum to the cervical canal. Many affected children never develop symptoms. If it does cause symptoms, they usually don’t appear until late childhood or adolescence. We present the clinical case of a 2-year-old patient with Arnold Chiari malformation type I. (AU)

How sensitive and specific is the visual diagnosis of sarcoptic mange in free-ranging Iberian ibexes?

BACKGROUND: Sarcoptic mange is a broadly distributed parasitic disease caused by Sarcoptes scabiei that affects wild mammals from all over the world, including the Iberian ibex (Capra pyrenaica). Selective culling of the scabietic individuals is the main management measure for disease control in Iberian ibex populations. Although visual identification of mange-compatible lesions is the reference method to decide the target individual, both false negative and positive cases are common in the wild. The aim of this work is to determine the sensitivity (SE), and the specificity (SP) of selective culling after evaluating 403 ibexes hunted in the Sierra Nevada Nature Space for sarcoptic mange control between 2002 and 2015. METHODS: A combination of skin scrapings and potassium hidroxide (KOH) skin digestion was used for sarcoptic mange diagnosis. Generalized linear models (GLM) were used to assess the effects of sex, age (juveniles and adults) and period of the year (wet and dry periods) on the SE and SP of the visual diagnosis method. RESULTS: The SE obtained for the visual determination of scabietic ibexes was 87.14%, whereas the SP was 60.71%. According to our model selection, SE of the visual diagnosis was explained by the additive effects of age and the period of the year. In fact, SE was lower in juveniles (64.76%) than in adults (94.26%) and during the dry period (73.44%) as compared to the wet period (92.09%). On the other hand, SP was best explained by the GLM including the additive effects of sex and the period of the year. The visual diagnosis of sarcoptic mange resulted less specific in females (22.73%) than in males (74.19%) and during the wet (55.22%) than in the dry period (82.35%). CONCLUSIONS: Maximizing SE and SP is essential to achieving a high rate of removal of affected individuals from the environment without eliminating potentially resistant individuals. Selective culling must be conservative during the wet period and with females due to the lower SP. Conversely, visual diagnosis of scabietic juveniles and during the dry period has to be improved, due to the lower SE.

Evaluation of three enzyme-linked immunosorbent assays for sarcoptic mange diagnosis and assessment in the Iberian ibex, Capra pyrenaica.

BACKGROUND: Sarcoptic mange is a contagious skin disease caused by the mite Sarcoptes scabiei, affecting different mammalian species worldwide including the Iberian ibex (Capra pyrenaica), in which mortalities over 90 % of the population have been reported. No efficient diagnostic methods are available for this disease, particularly when there are low mite numbers and mild or no clinical signs. In this study, three enzyme-linked immunosorbent assays (ELISA) developed for dog (ELISA A), Cantabrian chamois (Rupicapra pyrenaica parva) (ELISA B) and Alpine chamois (Rupicapra rupicapra) (ELISA C), were evaluated to detect specific antibodies (IgG) to sarcoptic mange in Iberian ibex sera. METHODS: Serum samples from 131 Iberian ibexes (86 healthy and 45 scabietic) were collected from 2005 to 2012 in the Sierra Nevada Natural and National Parks (southern Spain). Based on visual inspection, ibexes were classified into one of three categories, namely healthy (without scabietic compatible lesions), mildly affected (skin lesions over less than 50 % of the body surface) and severely affected (skin lesions over more than 50 % of the body surface). The optimal cut-off point, specificity, sensitivity and the area under the curve (AUC) were calculated, and the agreement between tests was determined. Moreover, differences in the optical density (OD) related to scabies severity have been evaluated for the best test. RESULTS: ELISA C showed better performance than the two other tests, reaching higher values of sensitivity (93.0 %) and specificity (93.5 %) against the visual estimation of the percentage of affected skin, chosen as the gold standard. Significantly higher concentrations of specific antibodies were observed with this test in the mildly and severely infested ibexes than in healthy ones. CONCLUSIONS: Our results revealed that ELISA C was an optimal test to diagnose sarcoptic mange in the Iberian ibex. Further studies characterizing immune response during the course of the disease, including spontaneous or drug induced recovery, should follow in order to better understand sarcoptic mange in Iberian ibex populations.

Acute phase proteins increase with sarcoptic mange status and severity in Iberian ibex (Capra pyrenaica, Schinz 1838).

Sarcoptic mange is a contagious skin disease caused by Sarcoptes scabiei, affecting both domestic and wild mammals, including the Iberian ibex (Capra pyrenaica), a medium-sized mountain ungulate almost endemic to the Iberian Peninsula. Acute phase proteins (APPs) could be an indicator of sarcoptic mange disease and severity in Iberian ibex. Serum samples from 131 healthy and sarcoptic mange-affected Iberian ibexes were collected from 2005 to 2012 in Sierra Nevada Natural Space in southern Spain. Serum alpha-1-acid glycoprotein (AGP), serum amyloid A (SAA) and haptoglobin (Hp) concentrations were quantified, and statistically significant differences according to sarcoptic mange disease and severity were assessed. Both AGP and SAA were significantly higher in the sarcoptic mange-affected ibexes than in the healthy ones as well as in the severely affected ibexes as compared to those with less than 50 % of the body surface affected. For the first time, changes in APP are reported in relation to sarcoptic mange in Iberian ibex. It is also reported for the first time that the intensity of APP increase depends on the severity of sarcoptic mange, which could be related with the pathological secondary amyloidosis, leading to organ dysfunction in severely mange-affected animals. Species and population differences in the increase of APP in response to sarcoptic mange could indicate individual and population differences in the immune capability of each population to deal with mange, population prevalence and mortality being the last indicators of such sensitivity.