Pediatric Neurology 54 (2016) 95e96

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Visual Diagnosis

A Girl With “Weakness”: Can Hyperventilation From Crying Give a Clue to the Diagnosis? Debopam Samanta MD * Division of Child Neurology, University of Arkansas for Medical Sciences, Little Rock, Arkansas

This 5-year-old girl presented with reduced use of her dominant right hand, intermittent dragging of her right leg, clumsiness, and frequent falling for 3 weeks without any preceding trauma or illness. Medication exposure history, birth history, and developmental history were unremarkable. She was alert, fussy, and slightly dysarthric with no cranial nerve deficit. Muscle strength and sensation were normal. Reflexes and tone were mildly decreased in her right lower extremity. Choreiform movements occurred in her right hand, most prominently during examination for pronator drift. A clinical

diagnosis of right hemichorea was made. Complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, thyroid function test, streptococcal antibody tests, antinuclear antibody, and echocardiography were all normal. Noncontrast magnetic resonance imaging was also unremarkable. She cried intensely during electroencephalography (EEG), then developed high-amplitude left-sided slowing which reappeared after she stopping crying. This phenomenon is known as the rebuild phenomenon (Fig 1), which is classically present in moyamoya syndrome and suggests a localized

FIGURE 1. Electroencephalography (bipolar longitudinal montage; blue ¼ left hemisphere and red ¼ right hemisphere). Lateralized rebuild-updleft lateralized highamplitude 1.5-Hz slowing seen 45 seconds after patient stopped crying. (The color version of this figure is available in the online edition.)

* Communications should be addressed to: Dr. Samanta; 1 Children’s Way; Little Rock, AR 72202, USA. E-mail address: [email protected] 0887-8994/$ e see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2015.08.006

decrease in cerebral perfusion reserve. Repeat cranial magnetic resonance imaging with contrast and magnetic resonance angiography confirmed the diagnosis (Fig 2).

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D. Samanta / Pediatric Neurology 54 (2016) 95e96

Children with moyamoya syndrome usually present with ischemic stroke, but hemichorea can be a rare initial manifestation of unilateral moyamoya syndrome. Hong et al. speculated that the underactivity of the indirect pathway due to hemodynamic and functional compromise of the striatum could be responsible for hemichorea.1 Hyperventilation, commonly used during EEG, produces generalized slow waves in children. Localized or lateralized slow waves represent focal abnormality. The characteristic EEG rebuild-up phenomenon (reappearance of high-amplitude slow waves at 20-60 seconds after cessation of hyperventilation) occurs in about 65% of pediatric patients with moyamoya disease.2 This child highlights several important points: moyamoya can have unilateral involvement resulting in asymmetric clinical findings; hemichorea can be misdiagnosed as hemi-body weakness (though objective evidence of weakness was not particularly evident during physical examination, subtle or intermittent weakness cannot be completely excluded); hemichorea can be the initial manifestation of moyamoya; hyperventilation during crying can provide an important clue during EEG in children uncooperative for standard hyperventilation; and late building of slow waves in EEG during hyperventilation, although underappreciated, can be a useful finding in the diagnosis of moyamoya syndrome.

References FIGURE 2. Cranial magnetic resonance imaging. (A) Magnetic resonance angiography reveals a paucity of distal branches of left middle cerebral artery compared with the other side. (B) Fluid-attenuated inversion recovery sequence shows an increased number of discrete cortical blood vessels on the left side due to collateral formation from the left external carotid artery.

1. Hong YH, Ahn TB, Oh CW, Jeon BS. Hemichorea as an initial manifestation of moyamoya disease: reversible striatal hypoperfusion demonstrated on single photon emission computed tomography. Mov Disord. 2002;17:1380-1383. 2. Cho A, Chae JH, Kim HM, et al. Electroencephalography in pediatric moyamoya disease: reappraisal of clinical value. Childs Nerv Syst. 2014;30:449-459.

A Girl With "Weakness": Can Hyperventilation From Crying Give a Clue to the Diagnosis?

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