A new respiratory scoring system for evaluation of respiratory outcomes in children with spinal muscular atrophy type1 (SMA1) on SMN enhancing drugs.

Nusinersen (NUS), the first treatment approved for Spinal Muscular Atrophy type 1 (SMA1), was made available in the UK for SMA1 through the Expanded Access Program (EAP) in 2017. The Great Ormond Street Respiratory (GSR) score was developed as an objective respiratory assessment for children with SMA1 during their treatment. Aims: Track respiratory status of SMA1 children over the course of Nusinersen treatment and compare GSR scores amongst SMA1 sub-types. Single centre study on SMA1 patients using the GSR score at set time points: prior to first NUS dose; 2 weeks post end of loading doses; 2 weeks post-subsequent doses. GSR score ranges 1-28, being 1-9 = Stable minimal support, thorough to 23-28 = Poor reserve with maximum support. 20 SMA1 children underwent NUS treatment between January 2017 - November 2018. Median age of diagnosis was 5.0 months. NUS started at median of 9.57 months. From 5th dose onwards, GSR scores were significantly lower for Type 1C patients compared to Type 1B By month 18, irrespective of subtypes, the whole cohort appears to stabilise GSR Scores. As treatment duration increases, an overall stabilisation of respiratory status across the cohort was observed. Further longitudinal studies are needed to validate the GSR.

[Post-traumatic L5-S1 root avulsion. Myelographic and x-ray computed tomographic study. Apropos of a case. Review of the literature]. / Avulsion radiculaire L5-S1 post-traumatique. Exploration myélographique et tomodensitométrique. A propos d'un cas. Revue de la littérature.

A patient presented an avulsion of left L5-S1 roots, with S1 meningocele, following a road accident. Saccoradiculography and CT scan imaging, even without contrast, allowed identification of total lesion in a suggestive clinical context. The characters of the CT scan images are emphasized. Images showed also the presence of pseudo-meningoceles and absence of roots.

[The silent kidney. Evolving ultrasonic picture of emphysematous pyelonephritis: the vanishing kidney. Apropos of a case]. / Le rein sourd. Aspect évolutif échographique de la pyélonéphrite emphysémateuse: l'évanescence rénale. A propos d'un cas.

The authors report the progressive disappearing of the sonographical image of a previously visualized kidney in a case of emphysematous pyelonephritis. They have called the sonographic vanishing kidney "the sign of the Deaf Kidney". The non-visualisation of a kidney with advanced emphysematous pyelonephritis is due to the infiltration of the perirenal environment by a gas envelope and the filling of the excretory cavities by gas. The perirenal gas-bag forms a shield against ultrasonic waves. The sonographer must be aware of the eventuality of not being able to individualize a renal structure when wrapped in gas. He must then ask for a zonography of the renal area in order to confirm the gas-containing collection in the kidney.

Spinal subdural metastasis. A case report.

A case of metastasis to the thoracic spinal subdural space is reported. Metrizamide myelography showed narrowing of the upper thoracic subarachnoid space. A tumor in the spinal subdural space is unusual; only a few cases have been reported. The myelographic appearance is not specific; an epidural metastasis can produce the same myelographic picture.

The madness of art.

The author finds a constant thread of what Benjamin Rush termed "tristimania" in works of great authors. The madness of art, Henry James's description of the need to transform and metamorphose personal experience by means of prose, poetry, or other artistic expression, is illustrated in the lives and works of many great writers. Psychotherapy as a means of dealing with depression in artists can be either rejected or used to advantage. The author posits that the most enduring works may be those of artists who have lived through their sadness to experience and control their rage against aging.