Alpha-methyl acetyl-coA racemase deficiency. Magnetic resonance imaging findings of three patients with encephalopathy, epilepsy, and stroke-like episodes.

Alpha-methyl acyl-CoA racemase deficiency (AMACRD) is a rare peroxisomal disorder that results in the accumulation of pristanic acid and 16 cases have been reported in the literature. Here, we present three additional patients, two confirmed by genomic study and one suspected. Three siblings who were born to healthy unrelated parents developed recurrent episodes of encephalopathy, seizures, and behavioral disturbances. In all 3, brain MRI showed lesions in the thalami, cerebral peduncles, and mesencephalic tegmentum, as well as brain volume loss. In addition, one patient had a chronic hemispheric infarct and an acute contralateral infarct, and another had a subacute infarct involving multiple vascular territories without abnormalities on MR angiography.

Global application of an unoccupied aerial vehicle photogrammetry protocol for predicting aboveground biomass in non-forest ecosystems.

Non-forest ecosystems, dominated by shrubs, grasses and herbaceous plants, provide ecosystem services including carbon sequestration and forage for grazing, and are highly sensitive to climatic changes. Yet these ecosystems are poorly represented in remotely sensed biomass products and are undersampled by in situ monitoring. Current global change threats emphasize the need for new tools to capture biomass change in non-forest ecosystems at appropriate scales. Here we developed and deployed a new protocol for photogrammetric height using unoccupied aerial vehicle (UAV) images to test its capability for delivering standardized measurements of biomass across a globally distributed field experiment. We assessed whether canopy height inferred from UAV photogrammetry allows the prediction of aboveground biomass (AGB) across low-stature plant species by conducting 38 photogrammetric surveys over 741 harvested plots to sample 50 species. We found mean canopy height was strongly predictive of AGB across species, with a median adjusted R 2 of 0.87 (ranging from 0.46 to 0.99) and median prediction error from leave-one-out cross-validation of 3.9%. Biomass per-unit-of-height was similar within but different among, plant functional types. We found that photogrammetric reconstructions of canopy height were sensitive to wind speed but not sun elevation during surveys. We demonstrated that our photogrammetric approach produced generalizable measurements across growth forms and environmental settings and yielded accuracies as good as those obtained from in situ approaches. We demonstrate that using a standardized approach for UAV photogrammetry can deliver accurate AGB estimates across a wide range of dynamic and heterogeneous ecosystems. Many academic and land management institutions have the technical capacity to deploy these approaches over extents of 1-10 ha-1. Photogrammetric approaches could provide much-needed information required to calibrate and validate the vegetation models and satellite-derived biomass products that are essential to understand vulnerable and understudied non-forested ecosystems around the globe.

Diagnostics and updated catalogue of Acalyptris Meyrick, the second largest genus of Nepticulidae (Lepidoptera) in the Americas.

We list all 56 currently known Acalyptris Meyrick species from North and South America, designate five new species groups, and provide pictorial diagnostics for all nine revised species groups of the American fauna. We describe seven new species: A. marmor Stonis Diskus, sp. nov., A. barbudo Stonis Remeikis, sp. nov., A. jareki Stonis Diskus, sp. nov., A. hilli Stonis Diskus, sp. nov., A. mortalis Diskus Stonis, sp. nov., A. hyacinthum Stonis Vargas, sp. nov., and A. extremus Stonis Diskus, sp. nov. We provide new data on morphology, biology or distribution for the following species: A. murex Diskus Stonis, A. hispidus Puplesis Robinson, A. trifidus Puplesis Robinson, A. bifidus Puplesis Robinson, A. terrificus Simkeviciute Stonis, and particularly A. yucatani Remeikis Stonis. We transfer Fomoria miranda Diskus Stonis to Acalyptris and provide the first photographic documentation of A. novenarius Puplesis Robinson, A. fortis Puplesis Robinson, A. martinheringi Puplesis Robinson, A. basihastatus Puplesis Diskus, A. pseudohastatus Puplesis Diskus, A. articulosus Puplesis Diskus, A. bovicorneus Puplesis Diskus, and A. insolentis Puplesis Diskus. We also comment on the re-deposition of some type series to the collection of the Zoological Museum of the Natural History Museum of Denmark, Copenhagen.

Discovery of leaf-mining Tischeriidae (Lepidoptera) in Colombia and their distribution in the Neotropics.

This is the first report of the family Tischeriidae in Colombia. We describe two new species recently discovered in the department of Valle del Cauca in southwestern Colombia: Astrotischeria ochrimaculosa Diskus, Stonis Vargas, sp. nov., and A. colombiana Stonis Vargas, sp. nov. The latter is a leaf miner of Asteraceae, while A. ochrimaculosa is trophically associated with Malvaceae. Astrotischeria colombiana is known only from Colombia, but A. ochrimaculosa is more widely distributed from Colombia to Peru. The new species are illustrated with photographs of their habitats, adults, male and female genitalia, and leaf mines. We comment on the rarity of tischeriids in collections and their current known distribution in the Neotropics.

ß-Tubulinopathy caused by a mutation of the TUBB2B gene: magnetic resonance imaging findings of the brain.

Patients with mutations in tubulin-related genes usually present with brain malformations, intellectual disability, epilepsy, microcephaly and ocular abnormalities. In these patients the diagnosis can be suggested by neuroimaging findings. We report a 5-year-old patient with characteristic magnetic resonance imaging findings including malformation of cortical development, fused basal ganglia, large head of the caudate nuclei, absent anterior limbs of the internal capsules, corpus callosum dysgenesis and dysplastic cerebellar vermis. Sequencing of the TUBB2B gene confirmed a heterozygous mutation: c. 260C>A (p. Pro87Gln).

A new leafmining pest of guava: Hesperolyra guajavifoliae sp. nov., with comments on the diagnostics of the endemic Neotropical genus Hesperolyra van Nieukerken (Lepidoptera, Nepticulidae).

We describe a new pest of guava (Psidium guajava L.), Hesperolyra guajavifoliae Stonis & Vargas, sp. nov., that was recently discovered in western Colombia. Hesperolyra van Nieukerken is a small, Neotropical genus of pygmy moths (Nepticulidae). We re-examine and document the complex morphology of the male genitalia of the generic type species, H. diskusi (Puplesis & Robinson). We discuss the diagnostics and composition of the genus and provide a simple pictorial differentiation scheme for all currently known representatives of the genus. The new species is illustrated with photographs of the adults, some of the immature stages, male and female genitalia, and leaf mines. A link to the COI barcodes of H. guajavifoliae sp. nov. is provided and the relationship of Hesperolyra to other genera is discussed.

Contrast-enhanced T2-FLAIR MR imaging in patients with uveitis.

To report MRI findings which reflect a pathological inflammatory condition of the uveal tract. This study includes single-center retrospective case series of five patients with clinical diagnosis of uveitis. There were 1 male (20 %) and 4 female patients (80 %). The average age was 29.6 years (range 25-38 years). Patients and 50 age-range-matched control subjects were scanned using a 1.5 T scanner. Ten additional control subjects scanned at 3 T were evaluated to have reference images at that high field. All patients (n = 5, 100 %) presented uveal tract enhancement on post-contrast T2-FLAIR fat-suppressed images and only 2 (40 %) had enhancement on T1-weighted images. The enhancement was anterior in 2 (40 %), pan-uveal in 2 (40 %), and posterior in 1 patient (20 %). Two patients (40 %) had unilateral increased vitreous signal on T2-FLAIR. One patient (20 %) had bilateral retrobulbar fat enhancement in both post-contrast T2-FLAIR and T1-weighted images. Post-contrast T2-FLAIR images can reveal abnormal enhancement of the uveal tract and retrobulbar fat as well as increased vitreous signal in patients with uveitis. In our small series, the sensitivity of post-contrast T2-FLAIR was higher than the conventional post-contrast T1-weighted images. Nonetheless, when bilateral uveal tract enhancement is present, there should be discretion before calling uveitis because the finding has been reported in different eye conditions as well as in a small percentage of healthy subjects at 1.5 T. In addition, it should be noted that post-contrast T2-FLAIR enhancement of the uveal tract is a normal finding at 3 T imaging.

Characteristic brain MRI findings in ataxia-neuropathy spectrum related to POLG mutation.

Patients with mutations in the polymerase gamma gene (POLG) may present with progressive ataxia and in such situations neuroimaging findings may suggest the diagnosis. Herein we report a patient with a POLG gene W748S homozygous mutation and characteristic lesions in the thalamus, cerebellum and inferior olivary nucleus seen on magnetic resonance imaging.

Intracranial Aneurysms in Children: The Role of Stenting and Flow-Diversion.

OBJECTIVE: To report a single-center experience in the endovascular management of cerebral aneurysms in children with stenting and flow-diversion techniques. METHODS: During a 7-year period, 5 male patients with six intracranial aneurysms (IA) were treated by endovascular therapy with stenting or flow-diversion. The average age was 11 years (range 6-18 years). RESULTS: The etiology of the aneurysms was vasculopathic in 3 cases (50%), traumatic in 2 patients (33.3%), and idiopathic in 1 case (16.7%). Two-thirds of the aneurysms were giant in size. The aneurysms were most frequently located in the anterior circulation (66.7%). Fifty percent of the aneurysms were treated with stenting and coiling, and 50% were treated with flow-diversion stents alone. After treatment, occlusion was graded as: partial in five aneurysms (88.3%) and complete in 1 case (16.7%). Most cases (83.3%) had a good outcome after the procedure. During follow-up, most aneurysms had progressive occlusion (80%), while the rest were unchanged (20%). Most stents and flow-diverter devices remained patent (80%). However, one Pipeline flow-diverter device in the Anterior cerebral artery (ACA) A2 segment had an asymptomatic occlusion. CONCLUSIONS: In this series, device-assisted endovascular techniques were a relatively safe and effective method of treatment of pediatric aneurysms. However, continued follow-up is required after treatment, because there are unsolved issues regarding the durability of flow-diverters and stents.

Congenital and Acquired Conditions of the Mesial Temporal Lobe: A Pictorial Essay.

PURPOSE: Our goal is to pictorially review a wide spectrum of congenital and acquired conditions affecting the medial aspect of the temporal lobe. CONCLUSION: After completing this article, the reader will have knowledge of the imaging appearance of diverse developmental, malformative, and acquired lesions of the mesial temporal lobe, which will be useful when evaluating pathology in this location.

Traumatic carotid-cavernous fistula with pontomesencephalic and cervical cord venous drainage presenting as tetraparesis.

A 27-year-old male presented with progressive ascending myelopathy leading to tetraparesis. Magnetic resonance imaging of the cervical spine showed dilated perimedullary veins and spinal cord edema. Catheter angiography demonstrated a direct carotid-cavernous fistula (CCF) with prominent pontomesencephalic and perimedullary venous drainage. Successful coil embolization of the fistula was performed with improvement of the patient's symptoms. To our knowledge, no case of a direct CCF with perimedullary drainage has been previously reported.

Endovascular treatment of penetrating traumatic injuries of the extracranial carotid artery.

PURPOSE: To describe the clinical and angiographic results of endovascular therapy for traumatic injuries of the extracranial carotid artery. MATERIALS AND METHODS: The clinical and angiographic features of 36 traumatic injuries of the carotid artery during a 12-year period were reviewed. There were 35 male patients (97.2%) and 1 female patient (2.8%) with an average age of 28.8 years (range 13-60 years). Of the 36 lesions of the carotid artery, 29 (80.6%) were the result of gunshot injury, and 7 (19.4%) were secondary to stab wounds. In 24 (66.7%) instances, the injury resulted in a pseudoaneurysm; in 7 (19.4%), in an arteriovenous fistula (AVF); in 4 (11.1%), in a dissection; and in 1 (2.8%), in inactive bleeding. All patients were treated with an endovascular approach using different techniques (balloon occlusion, embolization, or stent deployment). RESULTS: Endovascular therapy resulted in documented lesion occlusion in 34 (94.4%) patients. Two patients declined any follow-up postprocedural imaging; however, they have remained asymptomatic. Clinical improvement was documented in 35 (97.2%) patients, and there was one procedure-related complication with fatal consequences. CONCLUSIONS: In this series, endovascular techniques were an effective method of treatment. It was possible to use different endovascular reconstructive techniques or parent artery occlusion depending on the degree of vessel damage, with resolution of clinical symptoms and avoidance of surgery in most cases.

Terapia endovascular en aneurismas vertebrobasilares: experiencia del grupo neurovascular (junio de 1996- octubre de 2004) / Endovascular therapy of vertebral basilar aneurysms: experience of a neurovascular group (june 1996-october 2004)

Este artículo describe las características demográficas y clínicas de una serie consecutiva de 71 pacientes diagnosticados con aneurismas del eje vertebrobasilar, sus características morfológicas y los resultados de la terapia endovascular realizada en cuatro instituciones colombianas durante ocho años.

¿Nuevo estadio de neurocisticercosis? Presentación de dos casos de reactivación / Neurocysticercosis

En este artículo se presentan los casos de dos niñas de diez años de edad, quienes consultaron por síndrome convulsivo. La TAC cerebral mostró lesiones únicas calcificadas en la región temporoparietal derecha, con edema perilesional. La RM con gadolinio de una de las pacientes mostró realce anular y edema rodeando la lesión. Ambas pacientes tenían serología Elisa positiva para cisticercosis. Luego del tratamiento con esteroides.anticonvulsivantes y antiparasitarios, en el control de RM desapareció el edema y el realce

Esclerosis Múltiple seudotumoral, diagnostico por imágenes de resonancia mágnetica / Tumefactive multiple sclerosis

Presentamos dos casos interesantes de esclerosis múltiple, comprobados, con manifestaciones atípicas clínicas y por imagen, similares a neoplasias intracraneanas. Esta forma de presentación de la enfermedad es conocida como esclerosis múltiple seudotumoral. El conocimiento de sus hallazgos por imágenes de resonancia magnética, así como por exámenes clínicos y de laboratorio puede evitar procedimientos invasivos. Se hace una revisión actualizada del tema, en la que se recalca en el diagnóstico diferencial

Statistical analyses of structural magnetic resonance imaging of the head of the caudate nucleus in Colombian children with attention-deficit hyperactivity disorder.

Magnetic resonance imaging (MRI) studies of the caudate nucleus have reported reversal asymmetry and alterations of its size, suggesting a striate cortical disorder related to attention-deficit hyperactivity disorder (ADHD). The objective was to evaluate whether alterations of the asymmetry and size of the caudate nucleus head exist in a sample of well-controlled 7- to 11-year-old Colombian children, with different types of ADHD. Two groups of cases-ADHD of the combined type and ADHD of the inattentive type-and one control group, were selected. Multiple methods for assessing ADHD (rating scales, psychologic.interview, neurologic history and examination, and neuropsychologic evaluation) were used to confirm the diagnoses. Participants with a history of language disorder, learning disabilities, depression, and other major neurologic and psychiatric conditions were excluded. Finally all groups had 15 children, matched by sex (7 male, 8 female), age, socioeconomic status, and grade. Height, weight, head circumference, and encephalic index were statistically controlled. Three T1-weighted volumetric (three-dimensional) MRI slides of the caudate nucleus head were obtained with a 1.5-Tesla Gyroscan apparatus. The control group had a significantly higher Wechsler Full-Scale IQ than the groups with ADHD of the combined type and ADHD of the inattentive type (P < .001). Volumes from the left caudate nucleus head were significantly larger than volumes from the right in all groups (P < .001). There were no group differences when volumes were compared between groups. All of the groups had left caudate nucleus head volumes significantly higher than right, although there were no between-group differences. The results in relation to previous studies are discussed.