3149 Real-world Australian experience with Ofatumumab in the MSBase registry

Objectives Iatrogenic cerebral amyloid angiopathy (iCAA) is a very rare but emerging cause of stroke.We present a case of possible iCAA secondary to childhood Lyodura use. Methods Case report.Results A 40-year-old female presented to our service with focal seizures.This occurred on a background of being a monozygotic twin with partial trisomy 17, previous lobar intracerebral haemorrhage (ICH) in 2021, and craniosynostosis with repair and ventriculoperitoneal shunt insertion in 1982.Her twin had no history of ICH or childhood neurosurgery.CT brain on admission revealed a new left frontal ICH.A review of a previous MRI brain from 2022 revealed lobar cerebral microhaemorrhages and foci of cortical superficial siderosis, all clustered towards the vertex, and evidence of the previous right paracentral ICH.Case notes from her 1982 childhood neurosurgery were obtained which revealed Lyodura grafting occurring to repair a dural tear, also at the vertex.Lyodura was a commercially available irradiated human dura mater sourced post-mortem, available in Australia between 1972-1987.Use globally has been implicated in iatrogenic Creutzfeldt-Jakob disease and more recently, in iCAA.Thus, in our case, iCAA was suspected, which met the 'possible' criterion on a recently proposed diagnostic criteria.Options to pursue amyloid PET or biopsy to strengthen diagnostic certainty were offered but were declined by the patient's family.Conclusion iCAA is an exceedingly rare but emerging entity.Cases, such as ours, may be caused by historic use of Lyodura.Interrogation of childhood neurosurgical records is important to ensure similar cases are not missed.

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Published
2024-08-01
DOI
https://doi.org/10.1136/bmjno-2024-anzan.127
Citations
1
Primary Topic
Systemic Lupus Erythematosus Research
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article
Field-Weighted Citation Impact
0.47
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article

3149 Real-world Australian experience with Ofatumumab in the MSBase registry

Richard Macdonell, Tim Spelman, William M. Carroll, Jeannette Lechner‐Scott et al.
1 citations
Systemic Lupus Erythematosus Research
0.47
article

3149 Real-world Australian experience with Ofatumumab in the MSBase registry

Richard Macdonell, Tim Spelman, William M. Carroll, Jeannette Lechner‐Scott, Nevin John, Allan G. Kermode, Tomáš Kalinčík, Helmut Butzkueven, Anneke van der Walt, Pamela McCombe, Marzena J. Fabis‐Pedrini, Suzanne Hodgkinson, Mark Slee, Michael Harry Barnett, Margaret Migocki, Birte Schmitt
article en
1 citations

Abstract

Objectives Iatrogenic cerebral amyloid angiopathy (iCAA) is a very rare but emerging cause of stroke.We present a case of possible iCAA secondary to childhood Lyodura use. Methods Case report.Results A 40-year-old female presented to our service with focal seizures.This occurred on a background of being a monozygotic twin with partial trisomy 17, previous lobar intracerebral haemorrhage (ICH) in 2021, and craniosynostosis with repair and ventriculoperitoneal shunt insertion in 1982.Her twin had no history of ICH or childhood neurosurgery.CT brain on admission revealed a new left frontal ICH.A review of a previous MRI brain from 2022 revealed lobar cerebral microhaemorrhages and foci of cortical superficial siderosis, all clustered towards the vertex, and evidence of the previous right paracentral ICH.Case notes from her 1982 childhood neurosurgery were obtained which revealed Lyodura grafting occurring to repair a dural tear, also at the vertex.Lyodura was a commercially available irradiated human dura mater sourced post-mortem, available in Australia between 1972-1987.Use globally has been implicated in iatrogenic Creutzfeldt-Jakob disease and more recently, in iCAA.Thus, in our case, iCAA was suspected, which met the 'possible' criterion on a recently proposed diagnostic criteria.Options to pursue amyloid PET or biopsy to strengthen diagnostic certainty were offered but were declined by the patient's family.Conclusion iCAA is an exceedingly rare but emerging entity.Cases, such as ours, may be caused by historic use of Lyodura.Interrogation of childhood neurosurgical records is important to ensure similar cases are not missed.

The Royal Melbourne Hospital (AU), The University of Queensland (AU), The University of Melbourne (AU), Flinders University (AU), Murdoch University (AU), Royal Brisbane and Women's Hospital (AU), John Hunter Hospital (AU), The Alfred Hospital (AU), Sir Charles Gairdner Hospital (AU), Karolinska Institutet (SE), Myer Foundation (AU), Novartis (Australia) (AU), Monash Health (AU), Teva Pharmaceuticals (Australia) (AU), Mind Australia (AU), Ingham Institute (AU), Perron Institute for Neurological and Translational Science (AU), Austin Health (AU), Monash University (AU), University of Newcastle Australia (AU)
Openalex Percentile: Top 33%
Systemic Lupus Erythematosus Research
0.47
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