Esclerodermia lineal en coup de sabre. Manifestaciones neurológicas, imágenes y revisión / Linear scleroderma en coup de sabre. Neurological symptoms images and review
Rev. neurol. (Ed. impr.)
; 41(9): 534-537, nov. 2005. ilus
Article
in Es
| IBECS
| ID: ibc-128273
Responsible library:
ES1.1
Localization: BNCS
RESUMEN
Introducción. La esclerodermia lineal en golpe de sable, mejor conocida como esclerodermia lineal en coup de sabre (ELCS), es una enfermedad rara de causa incierta, caracterizada por atrofia focal progresiva craneofacial y, en parte, es diferente al síndrome de Parry-Romberg (SPR). Casos clínicos. Comunicamos tres pacientes con ELCS (2 mujeres y 1 hombre, con un promedio de edad de 40 años). Las manifestaciones neurológicas principales fueron cefalea y crisis convulsivas. Los alteraciones radiológicas, aunque diversas, fueron todas ipsilaterales al golpe de sable. En una paciente sometida a biopsia se obtuvo evidencia histopatológica de gliosis e infiltrado inflamatorio mixto perivascular. Destacamos en otro paciente la afectación cerebrovascular, dado el hallazgo de un infarto cerebeloso antiguo subclínico y oclusión de la arteria cerebelosa superior en ausencia de otra posible causa. Conclusiones. La ELCS, cuando afecta al sistema nervioso central es heterogénea en su presentación clínica y radiológica. Los estudios de imagen durante el control clínico y los hallazgos histopatológicos apoyan un proceso inflamatorio focal que puede ser progresivo. La afectación arterial se debe probablemente a una vasculopatía inflamatoria no ateroesclerosa, oclusiva y crónica (AU)
ABSTRACT
Introduction. Sword stroke linear scleroderma, which is better known as linear scleroderma en coup de sabre (LSCS), is a rare disease with an uncertain causation that is characterised by progressive craniofacial focal atrophy and is, at least in part, different from Parry-Romberg syndrome (PRS). Case reports. Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The main neurological symptoms were headache and seizures. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. Histopathological evidence for gliosis and mixed perivascular inflammatory infiltrate was found in the study of a biopsy specimen taken from one female. Cerebrovascular involvement was seen in another patient, as highlighted by the observation of an earlier subclinical cerebellar infarct and occlusion of the superior cerebellar artery in the absence of any other possible causation. Conclusions. When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. The arterial involvement is probably due to a non-atherosclerotic, occlusive and chronic inflammatory disease of the peripheral vessels (AU)
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Collection:
06-national
/
ES
Database:
IBECS
Main subject:
Scleroderma, Localized
/
Facial Hemiatrophy
Type of study:
Diagnostic_studies
Limits:
Adult
/
Female
/
Humans
/
Male
Language:
Es
Journal:
Rev. neurol. (Ed. impr.)
Year:
2005
Document type:
Article