Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier
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Fecha
2023
Autores
Sanguino-Caneva, Ciro
Carrillo-Chapman, Carlos
Luque-Llano, Melissa
Bermúdez, Valmore
Vargas-Manotas, José
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Taylor and Francis
Facultad de Ciencias de la Salud
Facultad de Ciencias de la Salud
Resumen
Introduction: Linear scleroderma en coup de sabre is a subtype of
scleroderma hallmarked by cutaneous and extracutaneous
manifestations in which neurological symptoms can be a predominant
feature of this condition.
Case presentation: We report a case of a previously healthy 47-year old male who developed neuropsychiatric symptoms and right-sided
cephalalgia for two months. Clinical examination revealed a right
frontoparietal cutaneous lesion and neurological findings suggesting
a medial prefrontal syndrome. The neuroimaging evaluation identified
scalp and bone thinning adjacent to the skin lesion and cortical subcortical white matter hyperintensity due to vasogenic oedema at
the right frontal and parietal region. A biopsy from the affected area
revealed reactive gliosis.
Conclusion: To our knowledge, this is the first linear scleroderma en
coup de sabre report associated with a neurological involvement
typical of a medial prefrontal syndrome. This case highlights the
importance of clinical acuity in recognising atypical phenotypes within
the spectrum of this uncommon disease.
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Sanguino-Caneva C, Carrillo-Chapman C, Luque-Llano M et al. Case Report: Medial prefrontal syndrome in a coup de sabre scleroderma carrier [version 1; peer review: 1 approved with reservations, 1 not approved] F1000Research 2023, 12:1254 https://doi.org/10.12688/f1000research.141188.1
