Sevoflurane anaesthesia for a patient with adult polyglucosan body disease

• Published in: Canadian journal of anesthesia Vol. 43; no. 12; pp. 1257 - 1259
• Main Authors: INOUE, S, ISHII, R, FUKUDA, H, SAITOH, K, SHIMIZU, R
• Format: Journal Article
• Language: English
• Published: Toronto, ON Canadian Anesthesiologists' Society 01-12-1996
• Subjects: Anesthesia; Anesthesia depending on patient's condition; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Anesthetics, Inhalation - pharmacology; Animals; Biological and medical sciences; Dogs; Ethers - pharmacology; Glucans - metabolism; Humans; Male; Medical sciences; Methyl Ethers; Middle Aged; Motor Neuron Disease - physiopathology; Volatile compound; Glycogenosis IV Andersen; Nervous system diseases; Metabolic diseases; General anesthesia; Enzymopathy; Genetic disease; Case study; Sevoflurane; General anesthetic; Central nervous system disease; Carbohydrate; Fluorine Organic compounds
• ISSN: 0832-610X; 1496-8975
• DOI: 10.1007/BF03013436

Adult polyglucosan body disease (APBD) is a rare neurological disorder of unknown cause characterized by four manifestations: upper motor neuron signs, peripheral neuropathy with motor and sensory loss, urinary incontinence, and dementia. The purpose of this report is to present a patient with APBD anaesthetized successfully with sevoflurane and nitrous oxide.
A 51-yr-old man with APBD was scheduled for haemorrhoidectomy. Paraesthesia, dysaesthesia, distal muscular atrophy and fasciculation were recognized in the extremities. Dementia, bulbar paralysis and respiratory insufficiency were basent. Anaesthesia was induced with inhalation of sevoflurane and nitrous oxide, and the trachea was intubated without the use of muscle relaxants. Maintenance of anaesthesia was performed with sevoflurane (inspired concentration: 1.5-2.5%) and nitrous oxide (50%). Emergence from anaesthesia and the postoperative course were uneventful, and no exacerbation of neurological signs and symptoms was recognized. No postoperative analgesia was required.
General anaesthesia and tracheal intubation with sevoflurane and nitrous oxide provided safe anaesthesia for a patient with APBD.