Neurological involvement in localized scleroderma: a systematic review of clinical manifestations and neuroimaging...

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.

This systematic review summarizes the neurological symptoms, neuroimaging findings, and other complementary examination results of LoS.

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.

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 ventricular dilatation. The top three most common manifestations on CT were brain calcification, skull atrophy, and white matter hypodensity. Other complementary examinations, including electroencephalography and cerebrospinal fluid test should be selectively performed if necessary.

Neurological involvement was common among LoS patients. Attention should be paid to the neurological manifestations of LoS at their first visit. It is recommended to perform MRI scanning for all craniofacial LoS patients, with or without neurological symptoms, at the time of diagnosis.