Abstract
Reverse Shapiro's syndrome was first described by Hirayama et al. in a girl with periodic
hyperthermia associated with complete agenesis of the corpus callosum. Here we report another such case in a 9-month-old girl presenting with fever of
unknown origin since the age of 7 months. On examination, she had mild hypotonia with
delayed developmental milestones. No other neurological or physical abnormalities
were noted. The cause of her prolonged fever of unknown origin was investigated and
all results were negative. Her brain magnetic resonance images showed agenesis of
the corpus callosum. On the basis of the previous literature, we suggest that the periodic hyperthermia
of this girl was caused by dopaminergic denervation of the hypothalamic thermoregulatory
center. Treatment with dopamine agonists (levodopa plus carbidopa) failed to control
the hyperthermia.
Keywords
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Article info
Publication history
Accepted:
November 18,
2004
Received in revised form:
May 11,
2004
Received:
June 3,
2003
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
