
pmid: 23321020
Systemic sclerosis (SSc; scleroderma), a rare disorder of connective tissue characterized by widespread organ dysfunction due to fibrosis and ischemia, is marked by the overproduction and abnormal deposition of collagen. The brain parenchyma itself is largely collagen-free and, thus, primary central nervous system (CNS) involvement in SSc is believed to be rare, although peripheral neuropathy is common. However, SSc is known to cause widespread microvascular damage, which may be a plausible mechanism for the neuropsychiatric symptoms of mood, anxiety, and cognitive disorders that have been documented in SSc patients. Thus, SSc may be a systemic cause of vascular cognitive impairment and should be considered in the diagnosis of vascular dementia. The current case report illustrates a potential vascular connection between SSc and neuropsychiatric symptoms. The classification of SSc includes limited and diffuse forms. The limited form includes the CREST variant, an acronym for Calcinosis, Raynaud phenomena, Esophageal dysmotility, Sclerodactyly, and Telangiectasias. The diffuse form features widespread collagen deposition resulting in dermatological thickening and fibrosis in the lungs, heart, kidneys, and gastrointestinal tract. However, CNS involvement has been less often documented. Although microangiopathy is the principal pathogenic marker of the disease, more recently some attention has also been paid to macrovascular abnormalities. Therefore, CNS manifestations in SSc may be due to primary cerebrovascular changes or hypertension. It is known that vascular dementia is a frequent consequence of small and/or large vessel disease. Many subtypes of vascular dementia have been described; however, regardless of the subtype, hypertension is the most common cause. Less common causes include hypoperfusion due to large vessel and/or cardiac disease and other systemic vasculopathic syndromes (e.g., polyarteritis nodosa) leading to multiple infarcts that can lead to vascular dementia. SSc, although perhaps even less common cause, may also manifest clinically as vascular cognitive impairment.
CREST Syndrome, Bipolar Disorder, Dementia, Vascular, Raynaud Disease, Neuropsychological Tests, Medication Adherence, Diagnosis, Differential, Hospitalization, Antibodies, Anticardiolipin, Antibodies, Antinuclear, Humans, Female, Cognition Disorders, Aged
CREST Syndrome, Bipolar Disorder, Dementia, Vascular, Raynaud Disease, Neuropsychological Tests, Medication Adherence, Diagnosis, Differential, Hospitalization, Antibodies, Anticardiolipin, Antibodies, Antinuclear, Humans, Female, Cognition Disorders, Aged
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