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Neurological involvement in localized scleroderma includes central and peripheral nervous system symptoms and imaging findingsNeurological Symptoms and Imaging Findings in Localized Scleroderma

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Key Takeaway
Note that localized scleroderma involves both central and peripheral nervous system symptoms and specific neuroimaging markers.

This systematic review synthesizes data from 242 publications involving 3,500 patients to characterize neurological involvement in localized scleroderma (LoS). The review identifies central nervous system symptoms, with headache, migraine, epilepsy, and hemiparesis as top-ranked findings. Peripheral nervous system symptoms frequently include paresthesia, cranial nerve involvement, and facial pain. Notably, headaches and migraines were reported more frequently in Parry-Romberg syndrome than in en coup de sabre, while epilepsy was broadly comparable across subtypes.

Neuroimaging findings were also synthesized. MRI findings commonly included T2 white matter hyperintensity lesions, brain atrophy, abnormal gyral patterns, gliosis, and ventricular dilatation. CT findings commonly included brain calcification, skull atrophy, and white matter hypodensity.

Clinical implications suggest that MRI scanning should be performed for all craniofacial LoS patients at the time of diagnosis. The utility of EEG and CSF tests is noted as selective. The findings are based on a systematic review of existing literature rather than primary clinical trials.

This systematic review looked at neurological involvement in patients with localized scleroderma (LoS). The researchers analyzed data from 3,500 patients across 242 different publications to identify common symptoms and imaging results.

Findings show that central nervous system symptoms often include headaches, migraines, epilepsy, and hemiparesis. Specifically, headaches and migraines were found more frequently in patients with Parry-Romberg syndrome than in those with en coup de sabre. Peripheral nervous system issues were also common, including facial pain, paresthesia, and cranial nerve involvement.

Imaging tests like MRI and CT scans revealed common findings such as white matter lesions, brain atrophy, and brain calcification. Because of these findings, the review suggests that doctors should perform MRI scans for all craniofacial LoS patients at the time of diagnosis. Because this is a review of existing literature rather than a new clinical trial, these results show common patterns rather than new treatments.

What this means for you:
Patients with localized scleroderma may experience various neurological symptoms and common brain imaging changes.

Common questions

What neurological symptoms are common in localized scleroderma?

Patients with localized scleroderma may experience several central nervous system symptoms, including headaches, migraines, epilepsy, and hemiparesis. Additionally, peripheral nervous system symptoms such as paresthesia, cranial nerve involvement, and facial pain are frequently reported in these patients.

What do imaging tests like MRI and CT show for these patients?

MRI scans commonly show T2 white matter hyperintensity lesions, brain atrophy, abnormal gyral patterns, gliosis, and ventricular dilatation. CT scans of these patients often reveal brain calcification, skull atrophy, and white matter hypodensity.

Are there differences between types of localized scleroderma?

The review found that headaches and migraines were more frequent in patients with Parry-Romberg syndrome than in those with en coup de sabre. However, symptoms like epilepsy were found to be broadly comparable across different subtypes of the condition.

Study Details

Study typeMeta analysis
EvidenceLevel 1
PublishedSep 2026
View Original Abstract ↓
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.
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