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Linear scleroderma associated with progressive brain atrophy.

Salvatore Grosso1, Antonella Fioravanti, Giovanni Biasi

  • 1Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Viale M. Bracci - Le scotte, 53100 Siena, Italy.

Brain & Development
|January 22, 2003
PubMed
Summary
This summary is machine-generated.

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Linear scleroderma (LS) can cause severe neurological issues, including epilepsy and brain atrophy, even without facial symptoms. Neuroimaging is recommended for LS patients experiencing neurological problems.

Area of Science:

  • Neurology
  • Dermatology
  • Pediatrics

Background:

  • Linear scleroderma (LS) typically presents with unilateral skin lesions.
  • Neurological complications are sometimes seen, particularly with 'en coup de sabre' and Parry-Romberg syndrome, often involving facial structures.

Observation:

  • A child with LS developed severe epilepsy and progressive mental deterioration.
  • The patient exhibited rapid atrophy of the ipsilateral cerebral hemisphere.
  • Crucially, this neurological involvement was not associated with facial or skull abnormalities.

Findings:

  • Brain imaging revealed reduced diameters of the left internal carotid and middle cerebral arteries.
  • This suggests a potential vascular component to the neurological manifestations in LS.

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Implications:

  • Neuroimaging studies are crucial for all patients diagnosed with linear scleroderma.
  • Brain imaging becomes essential when patients present with any neurological symptoms, regardless of facial involvement.