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Anesthetic management for a patient with Jansky-Bielschowsky disease

Yuko Yamada1, Katsushi Doi, Shinichi Sakura

  • 1Department of Anesthesiology, Shimane Medical University, Izumo, Japan.

Canadian Journal of Anaesthesia = Journal Canadien D'Anesthesie
|January 10, 2002
PubMed

Insights

Anesthetic management for Jansky-Bielschowsky disease (JBD) involved thiamylal and sevoflurane. Careful attention to intractable hypothermia was crucial during the procedure.

Area of Science:

  • Anesthesiology
  • Neurology
  • Genetics

Background:

  • Jansky-Bielschowsky disease (JBD) is a severe, late-infantile neurodegenerative disorder.
  • Patients often present with dementia, intractable epilepsy, and blindness.
  • Anesthetic considerations for JBD patients require careful planning due to complex comorbidities.

Observation:

  • A 14-year-old female with JBD underwent surgical resection of a gingival tumor and sacral sinus infection.
  • Preanesthetic evaluation revealed severe muscle atrophy, dementia, frequent seizures, and risk of upper airway obstruction.
  • Anesthesia was induced with thiamylal and maintained with sevoflurane, N2O, and O2, avoiding muscle relaxants.

Findings:

  • General anesthesia with thiamylal and sevoflurane provided adequate surgical conditions.
  • Intractable intraoperative hypothermia was a significant challenge requiring management.
  • Emergence from anesthesia was smooth, though seizures persisted postoperatively.

Implications:

  • This case highlights the feasibility of general anesthesia in JBD patients.
  • Management of hypothermia and seizures are critical aspects of perioperative care for JBD.
  • Further research into anesthetic protocols for rare neurodegenerative diseases is warranted.
Abstract

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