Rapid Communication| Volume 163, ISSUE 1, P86-89, February 01, 1999

Download started.


Localized scleroderma associated with progressing ischemic stroke


      We present a 73 year-old Japanese woman with localized scleroderma involving the right side of the scalp accompanied by continuous tingling pain, who developed insidiously progressive left hemiparesis. In magnetic resonance imaging of the brain, an infarct first appeared in the watershed region of the right middle cerebral artery territory and subsequently extended to deep white matter accompanied by scattered hemorrhages. Focal stenosis in the M2 portion of the right middle cerebral artery was revealed on magnetic resonance angiography, and the distal vessels were only shown faintly. A biopsy specimen from the sclerotic scalp lesion showed obvious thickening of vessel walls and mild mononuclear cell infiltration. We believe that the progressing ischemic stroke was caused by hemodynamic disturbances from localized sclerotic obstruction of the middle cerebral artery, with an autoimmune pathogenesis.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Adams H.P
        • Brott T.G
        • Crowell R.M
        Guidelines for the management of patients with acute ischemic stroke.
        Circulation. 1994; 90: 1588-1601
        • Baguley E
        • Hughes G.R.V
        Antiendothelial call antibodies.
        J Rheumatol. 1989; 16: 716-717
        • Black C.M
        The aetiopathogenesis of systemic sclerosis.
        J Int Med. 1993; 234: 3-8
        • Black C.M
        • Denton C.P
        • Korn J.H
        • Crombrugghe B
        Systemic sclerosis: Current pathogenetic concepts and future prospects for targeted therapy.
        Lancet. 1996; 347: 1453-1458
        • Chung M.H
        • Sum J
        • Morrell M.J
        • Horoupian D.S
        Intracerebral involvement in scleroderma en coup de sabre: Report of a case with neuropathological findings.
        Ann Neurol. 1995; 37: 679-681
        • Fieschi C
        • Rasura M
        • Anzini A
        • Beccia M
        Central nervous system vasculitis.
        J Neurol Sci. 1998; 153: 159-171
        • Gordon R.M
        • Silverstein A
        Neurologic manifestations in progressive systemic sclerosis.
        Arch Neurol. 1970; 22: 126-134
      1. Jablonska S, Lovell CR. Localized scleroderma. In: Jayson MIV, Black CM (Eds.), Systemic Sclerosis: Scleroderma, Wiley: Chichester, 1988:303–318.

        • Lee J.E
        • Haynes J.M
        Carotid arteritis and cerebral infarction due to scleroderma.
        Neurology. 1967; 17: 18-22
        • Lüer W
        • Jockel D
        • Henze T
        • Schipper H.I
        Progressive inflammatory lesions of the brain parenchyma in localized scleroderma of the head.
        J Neurol. 1990; 237: 379-381
        • Yoshio T
        • Masuyama J
        • Sumiya M
        • Minota S
        • Kano S
        Antiendothelial cell antibodies and their relation to pulmonary hypertension in systemic lupus erythematosus.
        J Rheumatol. 1994; 21: 2058-2063