Pediatric Neurology 50 (2014) 117e118
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
Unilateral Pupillary Dilatation in an 11-Year-Old Girl Amy Eapen BA, Lalitha Sivaswamy MD * Beaubien Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan
An 11-year-old girl with a 4-day history of left-sided pounding headache, phonophobia, and intermittent double vision was referred to the emergency department. Physical exam revealed ptosis and a left dilated pupil (Fig 1). Extraocular movements and the remaining neurological examination were normal. A partial cranial nerve III palsy was suspected. A similar episode had occurred in the past. Brain computed tomography and spinal fluid analysis were unremarkable. Magnetic resonance imaging of the brain showed enhancement and thickening of the left cranial nerve III in the cisternal segment (Fig 2). The patient was diagnosed with ophthalmoplegic migraine. Ophthalmoplegic migraine has an incidence of 0.7 per million.1 The average age of onset is 8 years. The International Classification of Headache Disorders II classifies ophthalmoplegic migraine as a cranial neuralgia. Clinical criteria for diagnosis include at least two attacks of
“migraine-like” headache accompanied or followed by paresis of cranial nerves III, IV, and/or VI. The headache is often retro- or periorbital. The episode may last a couple of weeks before complete resolution. Magnetic resonance imaging shows oculomotor nerve enhancement at the exit point from the midbrain in more than 80% of cases.2 The most commonly proposed explanation of ophthalmoplegic migraine is that it is a demyelinating cranial neuropathy caused by inflammatory neuropeptides released during a migraine attack. The unusually porous nature of the bloodenerve barrier in the subarachnoid space might account for the demyelination-remyelination cycle that manifests as thickening and enhancement of the nerve in that location.3 The other potential mechanism may be vasoconstriction of the vasa nervosum of the oculomotor nerve, resulting in localized transient ischemia and disruption of the blood nerve barrier at the site of nerve exit from the brain stem.4 The beneficial effect of steroids, in some studies, has lent further credence to the notion of an inflammatory basis. Migraine-specific treatments are rarely effective. Our patient was unusual because she did not have external ophthalmoplegia. She was treated with analgesics and her headache resolved over 48 hours. The ptosis lasted for 8 weeks. Her imaging findings were had improved
FIGURE 1. Left ptosis and mydriasis at the time of presentation.
* Communications should be addressed to: Dr. Sivaswamy; Associate Professor of Pediatrics and Neurology; Children’s Hospital of Michigan; Wayne State University School of Medicine; 3901 Beaubien; Detroit, MI 48201. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.pediatrneurol.2013.09.015
FIGURE 2. Coronal T1-weighted postcontrast magnetic resonance image of the brain depicts enhancement of the left third cranial nerve at the point of exit from the brain stem.
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considerably on repeat magnetic resonance imaging 12 weeks after presentation. References 1. Bharucha DX, Campbell TB, Valencia I, Hardison HH, Kothare SV. MRI findings in pediatric ophthalmoplegic migraine: a case report and literature review. Pediatr Neurol. 2007;37:59-63.
2. Mark AS, Casselman J, Brown D, et al. Ophthalmoplegic migraine: reversible enhancement and thickening of the cisternal segment of the oculomotor nerve on contrast-enhanced MR images. Am J Neuroradiol. 1998;19:1887-1891. 3. Carlow TJ. Oculomotor ophthalmoplegic migraine: is it really migraine? J Neuroophthalmol. 2002;22:215-221. 4. Lal V, Sahota P, Singh P, et al. Ophthalmoplegia with migraine in adults: is it ophthalmoplegic migraine? Headache. 2009;49: 838-850.
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
Unilateral Pupillary Dilatation in an 11-Year-Old Girl Amy Eapen BA, Lalitha Sivaswamy MD * Beaubien Children’s Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan
An 11-year-old girl with a 4-day history of left-sided pounding headache, phonophobia, and intermittent double vision was referred to the emergency department. Physical exam revealed ptosis and a left dilated pupil (Fig 1). Extraocular movements and the remaining neurological examination were normal. A partial cranial nerve III palsy was suspected. A similar episode had occurred in the past. Brain computed tomography and spinal fluid analysis were unremarkable. Magnetic resonance imaging of the brain showed enhancement and thickening of the left cranial nerve III in the cisternal segment (Fig 2). The patient was diagnosed with ophthalmoplegic migraine. Ophthalmoplegic migraine has an incidence of 0.7 per million.1 The average age of onset is 8 years. The International Classification of Headache Disorders II classifies ophthalmoplegic migraine as a cranial neuralgia. Clinical criteria for diagnosis include at least two attacks of
“migraine-like” headache accompanied or followed by paresis of cranial nerves III, IV, and/or VI. The headache is often retro- or periorbital. The episode may last a couple of weeks before complete resolution. Magnetic resonance imaging shows oculomotor nerve enhancement at the exit point from the midbrain in more than 80% of cases.2 The most commonly proposed explanation of ophthalmoplegic migraine is that it is a demyelinating cranial neuropathy caused by inflammatory neuropeptides released during a migraine attack. The unusually porous nature of the bloodenerve barrier in the subarachnoid space might account for the demyelination-remyelination cycle that manifests as thickening and enhancement of the nerve in that location.3 The other potential mechanism may be vasoconstriction of the vasa nervosum of the oculomotor nerve, resulting in localized transient ischemia and disruption of the blood nerve barrier at the site of nerve exit from the brain stem.4 The beneficial effect of steroids, in some studies, has lent further credence to the notion of an inflammatory basis. Migraine-specific treatments are rarely effective. Our patient was unusual because she did not have external ophthalmoplegia. She was treated with analgesics and her headache resolved over 48 hours. The ptosis lasted for 8 weeks. Her imaging findings were had improved
FIGURE 1. Left ptosis and mydriasis at the time of presentation.
* Communications should be addressed to: Dr. Sivaswamy; Associate Professor of Pediatrics and Neurology; Children’s Hospital of Michigan; Wayne State University School of Medicine; 3901 Beaubien; Detroit, MI 48201. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.pediatrneurol.2013.09.015
FIGURE 2. Coronal T1-weighted postcontrast magnetic resonance image of the brain depicts enhancement of the left third cranial nerve at the point of exit from the brain stem.
118
A. Eapen, L. Sivaswamy / Pediatric Neurology 50 (2014) 117e118
considerably on repeat magnetic resonance imaging 12 weeks after presentation. References 1. Bharucha DX, Campbell TB, Valencia I, Hardison HH, Kothare SV. MRI findings in pediatric ophthalmoplegic migraine: a case report and literature review. Pediatr Neurol. 2007;37:59-63.
2. Mark AS, Casselman J, Brown D, et al. Ophthalmoplegic migraine: reversible enhancement and thickening of the cisternal segment of the oculomotor nerve on contrast-enhanced MR images. Am J Neuroradiol. 1998;19:1887-1891. 3. Carlow TJ. Oculomotor ophthalmoplegic migraine: is it really migraine? J Neuroophthalmol. 2002;22:215-221. 4. Lal V, Sahota P, Singh P, et al. Ophthalmoplegia with migraine in adults: is it ophthalmoplegic migraine? Headache. 2009;49: 838-850.
