Publications by authors named "C Elizabeth Pringle"

208 Publications

Autologous Hematopoietic Stem Cell Transplantation for Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Can J Neurol Sci 2021 Feb 26:1-7. Epub 2021 Feb 26.

Division of Neurology, The Ottawa Hospital, University of Ottawa, Ottawa, Canada.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to conventional therapy can lead to marked disability and represents a therapeutic challenge.

Objective: To report five cases of treatment-refractory disabling CIDP treated with autologous hematopoietic stem cell transplantation (AHSCT).

Methods: This was a retrospective cohort study from a tertiary care referral center for both neuromuscular disease and AHSCT. Patients with CIDP treated with AHSCT between 2008 and 2020 were included. All patients had major persistent and disabling neuropathic deficits despite combinations of intensive immunosuppressive therapy. The primary outcome measures were: Medical Research Council sum score, Overall Neuropathy Limitations Scale and requirement for ongoing CIDP immunotherapy after transplantation. We also analyzed safety outcomes by documenting all severe AHSCT-related complications.

Results: Five patients with refractory CIDP underwent AHSCT. Three were classified as manifesting a typical syndrome, two were classified as the multifocal Lewis Sumner variant. The mean age at time of CIDP diagnosis was 33.4 years (range 24-46 years), with a median delay of 46 months (range 21-135 months) between diagnosis and AHSCT. The median follow-up period was 41 months. All five patients were able to wean off CIDP-related immunotherapy. Marked improvements in Medical Research Council scale and overall Neuropathy Limitations Scale were noted in 4/5 patients. One patient with longstanding neurogenic atrophy showed no improvement in disability scales. There were no treatment-related deaths or critical illnesses.

Conclusions: AHSCT can achieve marked sustained clinical improvement of refractory CIDP and may allow for weaning off long-term complex immunotherapies.
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http://dx.doi.org/10.1017/cjn.2021.30DOI Listing
February 2021

Spinal muscular atrophy type III complicated by spinal superficial siderosis: a case report with molecular and neuropathological findings.

Acta Neuropathol Commun 2020 11 9;8(1):188. Epub 2020 Nov 9.

Department of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, Canada.

Spinal muscular atrophy (SMA) is largely linked to deletion or mutation of the Survival motor neuron 1 (SMN1) gene located on chromosome 5q13. Type III (Kugelberg-Welander disease) is the mildest childhood form and patients may become ambulatory and have a normal life expectancy. We report the clinical history and morphological findings of a 55-year-old woman who began to experience motor problems at the age of two. She was never fully ambulatory, and her severe scoliosis required the insertion of surgical rod at age 19. Unexpectedly, around 35 years of age, she began to experience sensory symptoms best characterized as a myelo-radiculo-neuropathy with pain as the dominant symptom. Investigations never clarified the etiology of these symptoms. Molecular confirmation of SMA type III was done post-mortem. Neuropathological examination showed classic changes of lower motor neuron neurodegeneration, in line with those reported in the single molecularly confirmed case published so far, and with findings in rare cases reported prior to the discovery of the gene defect. A key autopsy finding was the presence of a severe superficial siderosis of the lower half of the spinal cord. In recent years, the concept of duropathy was put forward, associating superficial siderosis of the spinal cord with various spinal abnormalities, some of which were present in our patient. The presence of significant hemosiderin deposits in the spinal cord and sensory nerve roots with associated tissue and axonal damage provide a plausible explanation for the unexpected sensory symptomatology in this mild lower motor neurodegeneration.
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http://dx.doi.org/10.1186/s40478-020-01063-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7653749PMC
November 2020

Simultaneous Eulerian-Lagrangian velocity measurements of particulate pipe flow in transitional regime.

Rev Sci Instrum 2020 Sep;91(9):095110

Fluid and Complex Systems Centre, Coventry University, Coventry CV1 5FB, United Kingdom.

We present a unique pipe flow rig capable of simultaneous particle tracking and flow velocity measurements in a dilute, neutrally buoyant particulate pipe flow in regimes of transition to turbulence. The flow consists of solid glass spheres for the disperse phase and a density-matching fluid for the carrier phase. The measurements are conducted using a bespoke, combined two-dimensional particle image velocimetry and particle tracking velocimetry technique. The technique takes advantage of a phase discrimination approach that involves separating the disperse and carrier phases based on their respective image characteristics. Our results show that the rig and the technique it implements can effectively be employed to study transitional particulate pipe flows at dilute concentrations.
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http://dx.doi.org/10.1063/1.5129062DOI Listing
September 2020

Emphysematous Cystitis Following Bone Marrow Transplant.

J Pediatr Hematol Oncol 2021 Jul;43(5):193-194

UFHealth Shands Children's Hospital, Gainesville, FL.

Emphysematous cystitis is an exceedingly rare complication of urinary tract infection in children and adults. Characterized by air within the bladder wall, this life-threatening condition most often impacts diabetic women. The inciting pathogen is typically Escherichia coli or Klebsiella pneumoniae, though many other organisms have also been reported. Diagnosis is most commonly made via computed tomography scan; however, plain radiographs have also been described to be diagnostic. Medical management is the mainstay of therapy, consisting of bladder decompression and treatment of the underlying infection. Urologic surgery is required in 10% of cases. We report a 10-year-old child with a recent history of bone marrow transplantation complicated by gut-associated graft-versus-host disease who was diagnosed with emphysematous cystitis following a change in his abdominal pain.
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http://dx.doi.org/10.1097/MPH.0000000000001912DOI Listing
July 2021

Manchester Arena Attack: management of paediatric penetrating brain injuries.

Br J Neurosurg 2021 Feb 17;35(1):103-111. Epub 2020 Jul 17.

Department of Paediatric Neurosurgery, Royal Manchester Children's Hospital, Manchester, UK.

Purpose: The Manchester Arena bombing on 22 May 2017 resulted in 22 deaths and over 160 casualties requiring medical attention. Given the threat of modern- era terrorist attacks in civilian environments, it is important that we are able to anticipate and appropriately manage neurological injuries associated with these events. This article describes our experience of managing paediatric neurosurgical blast injuries, from initial triage and operative management to longer-term considerations.

Materials And Methods: Case study and literature review.

Results: Paediatric traumatic and penetrating brain injury patients often make a good neurological recovery despite low GCS at time of injury; this should be accounted for during triage and operative decision making in major trauma, mass casualty events. Conservative management of retained shrapnel is advocated in view of low long-term infection rates with retained shrapnel and worsened neurological outcome with shrapnel retrieval. All penetrating brain injuries should receive a prolonged course of broad-spectrum antibiotics and undergo long term follow-up imaging to monitor for the development of cerebral abscesses. MRI should never be utilised in penetrating brain injury cases, even in the absence of macroscopically visible fragments, due to the effect of MRI ferromagnetic field torque on shrapnel fragments. Anti-epileptic drugs should only be prescribed for the initial seven days after injury, as continuing beyond this does not incur any benefit in the reduction of long term post-traumatic epilepsy.

Conclusion: All receiving neurosurgical units should become familiar with optimum management of these thankfully rare, but complex injuries from their initial presentation to long term follow up considerations. All neurosurgical units should have well-rehearsed local plans to follow in the event of such incidents, ensuring timely deliverance of appropriate neurosurgical care in such extreme settings.
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http://dx.doi.org/10.1080/02688697.2020.1787339DOI Listing
February 2021
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