1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-7-48-a 10.15844/pedneurbriefs-7-6-10 Metabolic and Degenerative Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Alternating Hemiplegia: Mitochondrial Dysfunction http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 06 1993 01 07 2016 7 6 48 48 Copyright: © 1993 The Author(s) 1993 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Evidence for mitochondrial dysfunction in patients with alternating hemiplegia of childhood

Mitochondrial function in 4 patients with alternating hemiplegia (AH) was evaluated by magnetic resonance spectroscopy of resting muscle at the Montreal Neurological Institute, McGill University, Montreal, Canada.

 Alternating Hemiplegia Inorganic Phosphate Cytosolic Phosphorylation Potential

Mitochondrial function in 4 patients with alternating hemiplegia (AH) was evaluated by magnetic resonance spectroscopy of resting muscle at the Montreal Neurological Institute, McGill University, Montreal, Canada. All patients had abnormally high resonance intensities from inorganic phosphate and an abnormally low calculated cytosolic phosphorylation potential. These changes were consistent with mitochondrial dysfunction. [1]

COMMENT. Alternating hemiplegia of childhood may represent a phenotype of mitochondrial disease. Mitochondrial diseases share with AH the occurrence of alternating hemiplegia, seizures, and neurodevelopmental deterioration. The clinical characteristics and differential diagnosis of AH are reviewed in a report of 22 cases from the Hopital des Enfants Malades, Paris, France [2]. SPECT images of two patients showed focal areas of decreased uptake of the radiotracer, representing impaired regional blood flow during and between hemiplegic episodes and a possible mechanism of the neurologic deficits. [3]

 Arnold DL Silver K Andermann F Evidence for mitochondrial dysfunction in patients with alternating hemiplegia of childhood Ann Neurol 1993 Jun 33 6 604 7 8498840 10.1002/ana.410330608 Bourgeois M Aicardi J Goutières F Alternating hemiplegia of childhood J Pediatr 1993 May 122 5 Pt 1 673 9 8496742 10.1016/S0022-3476(06)80003-X Siemes H Cordes M Single-photon emission computed tomography investigations of alternating hemiplegia of childhood Dev Med Child Neurol 1993 Apr 35 4 346 50 8335150 10.1111/j.1469-8749.1993.tb11647.x