1 - Serviço de Neurorradiologia do Hospital de Braga, Braga, Portugal
2 - Serviço de Neurorradiologia do Centro Hospitalar Universitário do Porto, Porto, Portugal
3 - Serviço de Neurorradiologia do Hospital Dona Estefânia, Lisboa, Portugal
- XVII Congresso da SPNR, 25 e 26 de Novembro de 2022, Lisboa
Introduction: Scleroderma is a rare pediatric disease characterized by collagen deposition and fibrosis of the skin and soft tissues. Central nervous system involvement in scleroderma has been increasingly recognized and brain MRI may reveal cerebral findings ipsilateral to the skin abnormality.
Clinical cases: A 10-year-old girl, from central America, was referred to the emergency department due to a 2-month progressive headache associated with nausea and photophobia. The patient also reported two previous suspected episodes of seizures with recovery after 5 minutes. On the physical examination, facial asymmetry was evident with right hemifacial atrophy. Neurological examination showed no abnormalities. Brain MRI revealed multiple microbleeds in the right cerebral hemisphere with ipsilateral subcortical white matter lesions, temporal leptomeningeal enhancement and effacement of parietooccipital sulci. In the extracranial compartment, a right hemifacial atrophy was also notorious. Clinical presentation and MRI findings were compatible with Parry-Romberg syndrome. An 8year-old girl, caucasian, was referred for consultation in an outpatient setting to study the etiology of a previous stroke episode, which resulted in only slight face asymmetry. A small linear hyperpigmented cutaneous lesion was observed in the parasagittal left frontal region with associated alopecia. Brain MRI depicted a chronic left striatum-capsular infarction and additional ipsilateral cerebral multiple microbleeds and subcortical white matter lesions. Furthermore, it was confirmed a slight reduction on the left frontal parasagittal subcutaneous tissue thickness. The patient was positive for Scl-70 antibodies. Clinical presentation, laboratory and MRI findings were suggestive of linear scleroderma “en coup de sabre”, with an associated stroke episode potentially related to vasculitis.
Conclusion: These cases depict a rare entity that should be remembered in the differential diagnosis of headache, seizures or focal neurological deficits, especially in pediatric female patients. Presence of multiple cerebral microbleeds ipsilateral to atrophic cutaneous lesions should prompt the inclusion of scleroderma in the differential diagnosis.
Keywords: Unilateral microbleeds; Scleroderma; Brain; MRI; Parry-Romberg