Preoperative assessment of neurovesical function in children with anorectal malformation: association with vertebral and spinal malformations.

PURPOSE: We preoperatively assessed neurovesical function and spinal cord function in children with anorectal malformations. In cases of neurovesical dysfunction we looked for an association with vertebral malformation or myelodysplasia. MATERIALS AND METHODS: We prospectively evaluated 80 children with anorectal malformations via preoperative urodynamics and magnetic resonance imaging of the spine. Bladder compliance and volume, detrusor activity and vesicosphincteric synergy during voiding allowed urodynamic evaluation. Results were reported according to Wingspread and Krickenbeck classifications of anorectal malformations. RESULTS: Urodynamic findings were pathological in 14 children (18%). Pathological evaluations did not seem related to type of fistula or level of anorectal malformation. Vertebral anomalies were seen in 34 patients (43%) and myelodysplasia in 16 (20%). Neither vertebral anomaly nor myelodysplasia seemed associated with type of fistula or severity of anorectal malformation. Of 14 children with pathological urodynamics no vertebral anomaly or myelodysplasia was found in 7. Of 66 children with normal urodynamics 40 presented with vertebral or spinal malformation. CONCLUSIONS: Lower urinary tract dysfunction is common in patients with anorectal malformations. Normal spine or spinal cord does not exclude neurovesical dysfunction. Myelodysplasia or vertebral anomaly does not determine lower urinary tract dysfunction. Thus, we recommend preoperative urodynamic assessment of the bladder and magnetic resonance imaging of the spine in children with anorectal malformations.

ANÁLISE DE IMAGENS NA DETERMINAÇÃO DA FORMA E TEXTURA DE AREIAS

A forma dos grãos tem importante papel na avaliação da distribuição granulométrica das partículas de um solo. Entretanto, a maioria das técnicas de peneiramento evidencia que os grãos são esféricos e descritos por um único parâmetro, o diâmetro. Este trabalho teve por objetivo estudar a aplicação da análise de imagens, aliada ao grau de esfericidade e à teoria dos fractais, como ferramenta para mensurar parâmetros de forma e textura superficial de duas areias de características supostamente distintas da região da Grande Vitória, Espírito Santo, Brasil. Os resultados dos parâmetros de forma e textura superficial das amostras são apresentados e discutidos. As análises dos resultados para a variável esfericidade mostram diferenças estatísticas significativas entre as duas areias de origens diferentes. A areia de jazida de cordão litorâneo (AJ) apresentou grau de esfericidade ligeiramente superior e textura superficial mais lisa do que a de rio (AR), indicando que as areias AJ sofreram maior intemperismo em razão do transporte.

Paralyzed neonatal larynx in adduction. Case series, systematic review and analysis.

OBJECTIVE: Bilateral vocal cord abductor paralysis (BVCAbP) is considered a rare cause of stridor in the newborn. The goal of this work is to present a case series and to review systematically the literature on bilateral vocal cord abductor paralysis in the newborn to better characterize the current knowledge on this entity. METHODS: We performed a systematic literature review with Medline (1950-2011). The authors screened all cases of BVCAbP reported and selected those affecting newborns. RESULTS: Out of the 129 articles screened, 16 were included. A total of 69 cases could be retrieved and analyzed. Associated co-morbidities were found in 54% of the patients, most notably malformative conditions (intracranial or other), or a positive perinatal history (trauma/asphyxia, prematurity). Tracheostomy placement was required in 59% of children, and of these 44% were successfully decannulated. In terms of functional outcome full recovery or improvement were seen in 61% of patients. Major underlying co-morbidities affected negatively the functional outcome (p=.004), but not the need for tracheostomy (p=.604) or the decannulation success rate (p=.063). CONCLUSION: BVCAbP in the newborn is a serious cause of airway obstruction. It can be seen either in a context of multisystem anomalies or as an isolated finding. Newborns with major co-morbidities affecting their normal development are more likely to have poor functional outcomes and to remain tracheostomy-dependant.

Left recurrent laryngeal nerve palsy associated with silicosis.

Left recurrent laryngeal nerve palsy usually results from invasion or compression of the nerve caused by diseases localized within the aortopulmonary window. This study reports the case of a 76-yr-old male with vocal cord paralysis due to lymph node involvement by silicosis. This rare entity was identified by video-mediastinoscopy, which revealed a granulomatous and fibrosed recurrent lymph node encasing the nerve. The nerve was dissected and released from scar tissues. Progressive clinical improvement was observed followed by total and durable recovery of the voice after 15 weeks follow-up.

Brain Injuries In Iowa, September 2001

Under Iowa law, hospitals treating persons with a brain or spinal cord injury which results in a hospital admission, patient transfer, or death must report that injury to the Central Registry for Brain and Spinal Cord Injuries of the Iowa Department of Public Health.

The BH4 domain of Bcl-X(L) rescues astrocyte degeneration in amyotrophic lateral sclerosis by modulating intracellular calcium signals.

Collective evidence indicates that motor neuron degeneration in amyotrophic lateral sclerosis (ALS) is non-cell-autonomous and requires the interaction with the neighboring astrocytes. Recently, we reported that a subpopulation of spinal cord astrocytes degenerates in the microenvironment of motor neurons in the hSOD1(G93A) mouse model of ALS. Mechanistic studies in vitro identified a role for the excitatory amino acid glutamate in the gliodegenerative process via the activation of its inositol 1,4,5-triphosphate (IP(3))-generating metabotropic receptor 5 (mGluR5). Since non-physiological formation of IP(3) can prompt IP(3) receptor (IP(3)R)-mediated Ca(2+) release from the intracellular stores and trigger various forms of cell death, here we investigated the intracellular Ca(2+) signaling that occurs downstream of mGluR5 in hSOD1(G93A)-expressing astrocytes. Contrary to wild-type cells, stimulation of mGluR5 causes aberrant and persistent elevations of intracellular Ca(2+) concentrations ([Ca(2+)](i)) in the absence of spontaneous oscillations. The interaction of IP(3)Rs with the anti-apoptotic protein Bcl-X(L) was previously described to prevent cell death by modulating intracellular Ca(2+) signals. In mutant SOD1-expressing astrocytes, we found that the sole BH4 domain of Bcl-X(L), fused to the protein transduction domain of the HIV-1 TAT protein (TAT-BH4), is sufficient to restore sustained Ca(2+) oscillations and cell death resistance. Furthermore, chronic treatment of hSOD1(G93A) mice with the TAT-BH4 peptide reduces focal degeneration of astrocytes, slightly delays the onset of the disease and improves both motor performance and animal lifespan. Our results point at TAT-BH4 as a novel glioprotective agent with a therapeutic potential for ALS.

Delayed priming promotes CNS regeneration post-rhizotomy in Neurocan and Brevican-deficient mice.

A wealth of literature has provided evidence that reactive tissue at the site of CNS injury is rich in chondroitin sulfate proteoglycans which may contribute to the non-permissive nature of the CNS. We have recently demonstrated using a murine model of human brachial plexus injury that the chondroitin sulfate proteoglycans Neurocan and Brevican are differentially expressed by two subsets of astrocytes in the spinal cord dorsal root entry zone (DREZ) following dorsal root lesion (Beggah et al., Neuroscience 133: 749-762, 2005). However, direct evidence for a growth-inhibitory role of these proteoglycans in vivo is still lacking. We therefore performed dorsal root lesion (rhizotomy) in mice deficient in both Neurocan and Brevican. Rhizotomy in these animals resulted in no significant increase in the number of sensory fibres regenerating through the DREZ compared to genetically matched controls. Likewise, a conditioning peripheral nerve lesion prior to rhizotomy, which increases the intrinsic growth capacity of sensory neurons, enhanced growth to the same extent in transgenic and control mice, indicating that absence of these proteoglycans alone is not sufficient to further promote entry into the spinal cord. In contrast, when priming of the median nerve was performed at a clinically relevant time, i.e. 7 weeks post-rhizotomy, the growth of a subpopulation of sensory axons across the DREZ was facilitated in Neurocan/Brevican-deficient, but not in control animals. This demonstrates for the first time that (i) Neurocan and/or Brevican contribute to the non-permissive environment of the DREZ several weeks after lesion and that (ii) delayed stimulation of the growth program of sensory neurons can facilitate regeneration across the DREZ provided its growth-inhibitory properties are attenuated. Post-injury enhancement of the intrinsic growth capacity of sensory neurons combined with removal of inhibitory chondroitin sulfate proteoglycans may therefore help to restore sensory function and thus attenuate the chronic pain resulting from human brachial plexus injury.

Kirjallisuutta

Virallisen vastaväittäjän dosentti Jukka Kemppisen Helsingin yliopiston oikeustieteelliselle tiedekunnalle antama 11.11.2002 päivätty lausunto

Supracerebellar arachnoid cyst - A rare cause of acquired Chiari I malformation.

Chiari I malformation (CM) associated with a cervico-thoracic syrinx due to supracerebellar arachnoid cyst has not been reported in the literature. We report such a case, managed by fenestration of the arachnoid cyst and foramen magnum decompression (FMD), aiming to reduce the inferiorly directed pressure on the cerebellum and eliminate the craniospinal pressure dissociation respectively. Imaging done post-operatively showed upward displacement of the cerebellar tonsils with a decompressed craniovertebral junction and disappearance of the syrinx.

Coste del tratamiento bucodental en un grupo de tetrapléjicos institucionalizados

El objetivo del presente trabajo es calcular el coste de la rehabilitación bucodental de 20 pacientes afectos de tetraplejia por diferentes causas, y que se hallan ingresados en la 'Fundación Instituto Guttmann' de Barcelona. Se analizan todos los factores y se calcula el coste medio.

Molecular Mechanisms of Acute Brain Injury and Ensuing Neurodegeneration

Injury to the central nervous system (CNS), including stroke, traumatic brain injury andspinal cord injury, cause devastating and irreversible damage and loss of function. Forexample, stroke affects very large patient populations, results in major suffering for the patients and their relatives, and involves a significant cost to society. CNS damage implies disruption of the intricate internal circuits involved in cognition, the sensory-motor functions, and other important functions. There are currently no treatments available to properly restore such lost functions. New therapeutic proposals will emerge from an understanding of the interdependence of molecular and cellular responses to CNS injury, in particular the inhibitory mechanisms that block regeneration and those that enhanceneuronal plasticity...

Carotid sparing in t1-t2 no glottic cancer using helical tomotherapy

Objective: To study the dosimetric properties and clinical implementation of a carotid dose sparing irradiation protocol using helical Tomotherapy in early stage laryngeal cancer.Materials and Methods: We have developed a simple treatment planning algorithm for carotid sparing. We have compared carotid and critical organ doses and planned target volume (PTV) dose with our standard laryngeal irradiation protocol. Dose constraints were the following: maximum point dose to the carotids <35 Gy, to the spinal cord <30 Gy, and PTV was covered at >95% of the prescribed dose (70 Gy in 2 Gy per fraction). A daily megavoltage CT was done to account for patient movement and anatomy modification. To date, 7 patients have been treated with this protocol in our department.Results: Our early results showed a significant reduction in the carotid dose with an average maximum dose of 35.8 Gy. The average maximum spinal cord dose was 25.8 Gy. PTV was covered without important "hot spots". Average maximum dose in the PTV was 74.1 Gy with an average absolute maximum dose of 75.2 Gy. To date, the clinical outcomes have been excellent.Conclusion: Helical Tomotherapy allows a significant decrease of carotid dose without dangerous spinal cord overdose. There was no important overdose in the PTV that can potentially increase the late complication risks. Daily control imaging brings added security especially when working with such high-dose gradients. We think further studies and longer follow-up are needed to investigate the clinical outcomes such as the local control rate and the vascular late toxicities.

Protéinurie orthostatique et phénomène de compression de la veine rénale gauche (nutcracker syndrome) [Orthostatic proteinuria and compression of the left renal vein (nutcracker syndrome)]

INTRODUCTION: The pathogenic mechanism of orthostatic proteinuria has not yet been clearly established. OBSERVATION: In a tall, thin, 21 year-old man, isolated proteinuria was discovered during an urological control conducted one year after a bilateral orchidopexy following left testicular torsion. Proteinuria was orthostatic. Doppler examination of the kidney revealed an entrapment of the left renal vein (nutcracker phenomenon-NCP). COMMENTS: An NCP was diagnosed in a young patient presenting with orthostatic proteinuria. By provoking modifications in intraglomerular haemodynamics, the NCP may, in nearly half of the cases, be at the origin of orthostatic proteinuria. Doppler examination is the diagnostic method of choice in the screening for NCP.

Osteolytic metastasis detected by F18-FDG PET in a patient with lung carcinoma

We present a 53-year-old man with a vocal cord paralysis observed as a primary manifestation of lung carcinoma. Tc-99m MDP whole body bone scan were performed and resulted a normal scintiscan. The bone scan does not revealed suspicious foci of uptake. The possibility of bone metastasis was taken into consideration. A whole body F18-FDG-PET scan showed intense uptake in the left upper lung corresponding to the primary tumor. A bronchial biopsy confirmed infiltration by small cell lung carcinoma (SCLC). SCLC is composed of poorly differentiated, rapidly growing cells with disease usually occurring centrally rather than peripherally. It metastasizes early. The whole-body F18-FDG-PET scan clearly demonstrated a focus of increased uptake in the second lumbar vertebral body suspicious for osteolytic metastasis. A lytic bone metastasis was confirmed by MRI. The patient then received therapy and underwent follow up abdominal CT. The scan showed blastic changes in the L2 vertebra suggesting response to treatment.