Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Allogeneic bone marrow transplantation for Alexander's disease

M J Staba1, S Goldman, F L Johnson

  • 1Department of Pediatric Hematology/Oncology, University of Chicago Children's Hospital, IL 60637, USA.

Bone Marrow Transplantation
|August 1, 1997
PubMed
Summary

Allogeneic bone marrow transplantation (BMT) was evaluated for infantile Alexander's disease. This treatment did not alter the patient's neurological deterioration or prognosis.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Forest understory biomass heterogeneity is "moisture complex" or associated litter the cause?

Journal of chemical ecology·2013
Same author

Spinal anesthesia in vaginal delivery; a report of 1,547 cases.

American journal of obstetrics and gynecology·2010
Same author

Recurrent hydatidiform mole.

Canadian Medical Association journal·2010
Same author

Noninvasive discrimination of rejection in cardiac allograft recipients using gene expression profiling.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons·2006
Same author

A family with seizures and minor features of tuberous sclerosis and a novel TSC2 mutation.

Neurology·2003
Same author

Heart transplantation. An update and review.

Minerva cardioangiologica·2003

Area of Science:

  • Neurology
  • Genetics
  • Pediatrics

Background:

  • Alexander's disease is a rare leukodystrophy.
  • The exact enzyme deficiency causing Alexander's disease remains unidentified.
  • Infantile Alexander's disease presents with severe neurological deterioration.

Observation:

  • A case report details a 7-month-old female infant diagnosed with the infantile form of Alexander's disease.
  • The patient underwent allogeneic bone marrow transplantation (BMT) in an attempt to halt neurological decline.
  • Initial post-transplant recovery showed a temporary return to the patient's pre-illness neurological state.

Findings:

  • Despite initial improvements, the patient's neurological status progressively deteriorated following BMT.
  • Allogeneic bone marrow transplantation did not prove effective in reversing or arresting the neurodegenerative course of Alexander's disease.

Related Experiment Videos

  • The study concludes that BMT is not a viable therapeutic strategy for this condition.

    • Implications:

    • This case highlights the limitations of current therapeutic approaches for Alexander's disease.
    • It suggests that the pathological mechanisms of Alexander's disease may not be addressable by bone marrow transplantation.
    • Further research into the specific enzyme deficiency and alternative treatment strategies is warranted for Alexander's disease.