Metachromatic leukodystrophy: about 04 cases and review of the literature

  • Leila kartout University Mohammed Vth Souissi, Rabat, Morocco.
  • Fatima zohra ouadghiri University Mohammed Vth Souissi, Rabat, Morocco.
  • Nour Mekaou University Mohammed Vth Souissi, Rabat, Morocco.
  • Lamya Karboubi University Mohammed Vth Souissi, Rabat, Morocco.
  • Badr Sououd Benjelloun Dakhama University Mohammed Vth Souissi, Rabat, Morocco.
Keywords: Metachromatic, leukodystrophy, arylsulphatase A, demyelinisation

Abstract

Metachromatic leukodystrophy (MDL) is a hereditary lysosomal inheritance disorder with autosomal recessive transmission. It is due to the deficiency of the enzyme arylsulphatase A or its activating protein soposin B. It can occur at any age, but the infant form is the most frequent. The diagnosis is based mainly on data from brain magnetic resonance imaging and the arylsulfatase A dosage. We report a serie of 04 cases collected in the pediatric department at Rabat's children's hospital, allowing us to know the clinical and paraclinical characteristics of the disease, as well as the current situation in the field of therapeutic.

Author Biographies

Leila kartout, University Mohammed Vth Souissi, Rabat, Morocco.

Pediatric Medical Emergency Department, Rabat Children's Hospital, Morocco
Faculty of Medicine and Pharmacy

Fatima zohra ouadghiri, University Mohammed Vth Souissi, Rabat, Morocco.

Pediatric Medical Emergency Department, Rabat Children's Hospital, Morocco
Faculty of Medicine and Pharmacy,

Nour Mekaou, University Mohammed Vth Souissi, Rabat, Morocco.

Pediatric Medical Emergency Department, Rabat Children's Hospital, Morocco
Faculty of Medicine and Pharmacy, 

Lamya Karboubi, University Mohammed Vth Souissi, Rabat, Morocco.

Pediatric Medical Emergency Department, Rabat Children's Hospital, Morocco
Faculty of Medicine and Pharmacy, 

Badr Sououd Benjelloun Dakhama, University Mohammed Vth Souissi, Rabat, Morocco.

Pediatric Medical Emergency Department, Rabat Children's Hospital, Morocco
Faculty of Medicine and Pharmacy, 

Published
2017-08-31
How to Cite
kartout, L., ouadghiri, F. zohra, Mekaou, N., Karboubi, L., & Dakhama, B. S. B. (2017, August 31). Metachromatic leukodystrophy: about 04 cases and review of the literature. EPH - International Journal of Medical and Health Science (ISSN: 2456 - 6063), 3(8), 19-25. Retrieved from https://ephjournal.com/index.php/mhs/article/view/229