Neurologic Manifestations in

Neurologic Complications of Progressive Systemic Sclerosis

“…1 ' Microangiopathy due to PSS affecting intracerebral arteries producing a rupture and resultant intracerebral hemorrhage bas also been reported. 11 Undoubtedly confusion and disorientation occurring in episodic form can be considered TIA, but in our report here, the personality change and delusional behavior seem unlike the TIA syndrome. It is true that such mental symptoms do occur in PSS, and these symptoms of delusion or hallucinations, etc., respond to steroid administration.…”

“…Generalized muscle wasting is frequently observed in patients with PSS, 11 and chronic myopathy seems to be clinically apparent. However, electrodiagnostic studies and biopsies were performed less frequently and on many occasions negative reports were observed.…”

Scleroderma and central nervous system vasculitis.

“…Discussion Primary CNS involvement in scleroderma is rare and unusual, 3 -6 leading some to argue that CNS dysfunction is either secondary to end-organ failure or, if vasculitis is present, it is associated with evidence of systemic vasculitis. 4 - 5 The rarity of CNS vasculitis associated with scleroderma in addition to the underestimated significance of the angiographic data resulted in diagnostic uncertainty and delayed appropriate medical intervention for our patient. Further uncertainty resulted from the subsequent normal findings on leptomeningeal biopsy, which had been recommended to confirm the angiographic findings.…”

“…1 -3 When cerebral ischemia is a manifestation of scleroderma, it is generally associated with evidence of renal failure and severe hypertension. 4 - 5 We describe a patient with CREST syndrome (a variant of scleroderma characterized by calcinosis cutis, Raynaud's phenomenon, esophageal reflux, sclerodactyly, and telangiectasia) and CNS vasculitis who was treated with high-dose immunosuppressive therapy, resulting in dramatic clinical improvement.…”