Adult metachromatic leukodystrophy: Three cases with normal nerve conduction velocities in a family


Cengiz N., Özbenli T., Onar M., Yıldız L., Ertaş B.

Acta Neurologica Scandinavica, cilt.105, sa.6, ss.454-457, 2002 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 105 Sayı: 6
  • Basım Tarihi: 2002
  • Doi Numarası: 10.1034/j.1600-0404.2002.01257.x
  • Dergi Adı: Acta Neurologica Scandinavica
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.454-457
  • Ondokuz Mayıs Üniversitesi Adresli: Evet

Özet

Here, we report three cases of late onset metachromatic leukodystrophy (MLD) within a family. The patients presented with psychiatric disturbances and dementia. The arylsulphatase A (ASA) level in leucocytes was zero in all the patients. The cranial magnetic resonance imaging (MRI) revealed bilateral symmetrical demyelination but the nerve conduction velocities were normal in all three cases. The clinical, biochemical, imaging and electrophysiological data of the family has been discussed.