From the Guidelines
Neurologic involvement in scleroderma should be diagnosed through a combination of clinical evaluation, neuroimaging, and laboratory tests, as it occurs in approximately 10-15% of patients, with peripheral neuropathy being the most frequent presentation. Physicians typically perform a detailed neurological examination to assess for symptoms like headaches, seizures, cognitive changes, neuropathic pain, or sensory abnormalities. MRI of the brain and spine is the primary imaging modality to detect central nervous system involvement, while nerve conduction studies and electromyography help identify peripheral neuropathies. Laboratory tests including cerebrospinal fluid analysis, autoantibody testing, and inflammatory markers can support the diagnosis 1.
The frequency of neurologic involvement in scleroderma can vary, but peripheral neuropathy is the most common manifestation, affecting 5-67% of patients depending on diagnostic criteria. Central nervous system involvement is less common, seen in about 8% of cases. The neurologic complications result from various mechanisms including vasculopathy affecting small vessels in the nervous system, immune-mediated damage, and secondary effects from other organ involvement such as renal or cardiac disease.
Some studies have reported on the management of systemic lupus erythematosus with neuropsychiatric manifestations, which may be relevant to scleroderma, suggesting the use of glucocorticoids alone or with immunosuppressive therapy, with good results (60–75% response rate) 1. However, the most recent and highest quality study on scleroderma recommends a comprehensive approach to diagnosis and management, emphasizing the importance of early recognition and treatment to prevent irreversible damage 1.
Key points to consider in the diagnosis of neurologic involvement in scleroderma include:
- Clinical evaluation to assess for symptoms like headaches, seizures, cognitive changes, neuropathic pain, or sensory abnormalities
- Neuroimaging, particularly MRI of the brain and spine, to detect central nervous system involvement
- Laboratory tests, including cerebrospinal fluid analysis, autoantibody testing, and inflammatory markers, to support the diagnosis
- Nerve conduction studies and electromyography to identify peripheral neuropathies
- Early recognition and treatment to prevent irreversible damage, with a comprehensive approach to management, as recommended by the most recent and highest quality study 1.
From the Research
Diagnosing Neurologic Involvement in Scleroderma
To diagnose neurologic involvement in scleroderma, the following methods can be used:
- Complete neurologic examination to assess sensation, strength, and reflexes 2
- Nerve conduction studies (NCS) to evaluate peripheral nerve function 2
- Quantitative sensory testing (QST) to assess sensory thresholds 2
- Electroencephalography (EEG) to evaluate central nervous system involvement 3
- Visual evoked potentials (VEPs) to assess optic nerve function 3
- Imaging studies such as high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) to evaluate central nervous system abnormalities 4
Frequency of Neurologic Involvement in Scleroderma
The frequency of neurologic involvement in scleroderma varies depending on the study and the specific type of scleroderma:
- In systemic sclerosis (SSc), neurologic involvement occurs in approximately 23.73% of patients, with headache, seizures, and cognitive impairment being common manifestations 5
- In localized scleroderma (LoS), neurologic involvement occurs in approximately 60% of patients, with headaches, epilepsy, and pyramidal signs being common manifestations 4
- Peripheral neuropathy occurs in approximately 14.25% of SSc patients, with carpal tunnel syndrome and trigeminal neuropathy being common types of neuropathy 5
- Autonomic neuropathy involving the cardiovascular and gastrointestinal systems is also common in SSc patients 5
Clinical Features of Neurologic Involvement in Scleroderma
The clinical features of neurologic involvement in scleroderma include: