Neurological involvement in localized scleroderma: a systematic review of clinical manifestations and neuroimaging findings
Abstract
Background: Localized scleroderma (LoS) is an autoimmune disorder leading to skin sclerosis and subcutaneous tissue atrophy. Apart from the disfiguring craniofacial lesions, the extracutaneous involvement in LoS has been increasingly recognized and studied. Among all, neurological involvement is common and worth special attention.
Objectives: This systematic review summarizes the neurological symptoms, neuroimaging findings, and other complementary examination results of LoS.
Methods: Records were retrieved using keywords related to the disease, neurological symptoms, and neurological examinations. After excluding irrelevant literature, case reports, case series, and cohort studies were included. A statistical analysis was performed, focusing on central and peripheral nervous system symptoms, neuroimaging manifestations, and other complementary examination results.
Results: A total of 242 topic-related publications reporting data on nearly 3,500 patients were finally included. The top ranked central nervous system symptoms of LoS were headache and migraine, epilepsy, and hemiparesis. Headache/migraine were more frequent in Parry-Romberg syndrome than in en coup de sabre, whereas epilepsy was broadly comparable across subtypes. The top peripheral nervous system symptoms were paresthesia, cranial nerve involvement, and facial pain. The top five most common manifestations on MRI were T2 white matter hyperintensity lesion, brain atrophy, abnormal gyral pattern, gliosis, and…
How strong is the evidence?
Evidence Hub reads this as a systematic review, done in people. Its evidence level is 1 of 5 (Highest · pooled evidence). Pooled evidence from several studies sits at the top of the evidence pyramid. Reported size: 3,500 participants or records.
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