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Correspondence |
Greenlane Hospital, Greenlane, Auckland, New Zealand, E-mail: rina_london{at}yahoo.com
To the Editor:
Alternating hemiplegia of childhood (AHC) is a rare neurological disorder, diagnosed clinically, usually after 18 months. It is characterised by attacks of unilateral hemiplegia, with variable duration and severity. Seizures, occulomotor disturbances and cognitive impairment, can occur. It was first described by Verret in 1971 and is a diagnosis of exclusion and of unknown etiology.1
A pregnant 37-yr-old woman with AHC presented at 13 weeks gestation for a termination of pregnancy for severe fetal malformation. Alternating hemiplegia of childhood had been diagnosed at age 14, after an initial misdiagnosis of myasthenia gravis. Her attacks regressed during adolescence, being able to control the triggering factors, namely stress, anxiety, exertion and extremes of temperature. Two previous volatile anesthetics were followed by extreme limb weakness, and difficulty swallowing. Symptoms in both cases resolved without intervention, although the cause was unclear. A general anesthetic performed in 2000 for a Cesarean section was performed with propofol as a target controlled infusion (TCI). A modified rapid sequence was performed with rocuronium and alfentanil. She recalled no weakness postoperatively.
At the time of the current presentation, she had brisk reflexes, and a positive Babinski sign on the right with no focal weakness. She was otherwise well, and had a normal airway examination.
She was fasted, and premedicated with ranitidine, metoclopramide and sodium citrate.
The anesthetic machine was prepared according to standard malignant hyperthermia protocol to avoid the potential risk of rhabdomyolysis. Anesthesia was induced with midazolam and fentanyl followed by a propofol TCI. A size 4 laryngeal mask air-way was inserted and she remained spontaneously breathing only oxygen for the ten-minute procedure. Routine observations including temperature were stable throughout. At the time of discharge three hours later, she walked unaided, easily.
Evidence suggests that AHC is a channelopathy, sometimes with mitochondrial abnormalities.2 Flunarizine, a selective calcium channel blocker, appears to have some success in reducing duration and frequency of attacks.3 There has only been one case report of AHC and anesthesia, describing this same patient, whilst she was having her Cesarean section.4 For this patient, total iv anesthesia appeared the safest option. She was fearful that the stress of a regional anesthetic could trigger an attack. As she was slim and well fasted, using an laryngeal mask airway was deemed appropriate, and muscle relaxants could thus be avoided.5 There are only 250 documented cases of alternating hemiplegia in the world, although under-diagnosis is probably common. Due to the rarity of the disease, and its unusual presentation, patient care would likely benefit from establishment of an international database.
References
1 Verret S, Steele JC. Alternating hemiplegia of child-hood: a report of eight patients with complicated migraine beginning in infancy. Paediatrics 1971; 47: 675–80.
2 Casaer P, Azou M. Flunarizine in alternating hemiplegia in childhood. Lancet 1984; 11: 579.
3 Mikati MA, Kramer U, Zupanc ML, Shanahan RJ. Alternating hemiplegia of childhood: clinical manifestations and long term outcome. Pediatr Neurol 2000; 23 134–141[Medline]
4 Parris-Piper TW. Caesarean section under general anaesthetic in a woman with alternating hemiplegia of childhood. Int J Obstet Anesth 2002; 11: 317–20.[Medline]
5 Han TH, Brimacombe J, Lee EJ, Yang HS. The laryngeal mask airway is effective (and probably safe) in selected healthy parturients for elective cesarean section: a prospective study of 1067 cases. Can J Anesth 2001; 48: 1117–21.
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