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Diagnostic difficulties of paroxysmal symptoms in a boy with Parry-Romberg syndrome
Affiliations- Chair of Paediatric and Adolescent Neurology, Jagiellonian University , Kraków
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Abstract
Parry-Romberg syndrome is characterized by progressive unilateral facial atrophy affecting subcutaneous tissue, cartilage and bone structures. Headache attacks and epilepsy are commonly associated with this syndrome but the underlying pathophysiology is still unknown. A case of a 12-year-old boy with Parry-Romberg syndrome and syringomyelia suffering from severe headache attacks and epileptic seizures is reported herein.
Headache attacks were associated with bilateral autonomic symptoms and hyperventilation and were usually followed by complex partial and sometimes by secondary generalized tonic seizures. Detailed neuroimaging examinations were performed (magnetic resonance imaging [MRI] of the head, orbits, and spinal cord, MR angiography, and MR spectroscopy of the cerebellum). The EEG pattern revealed localized discharges contralaterally to the affected side. Antiepileptic treatment with carbamazepine was instituted with minimal effect. Modification of treatment (replacement with oxcarbazepine) was successful.
In the reported patient interesting correlation of headache attacks, autonomic symptoms and epileptic seizures was observed. Additionally we believe it is the first report of coincident syringomyelia and Parry-Romberg syndrome.
Abstract
Parry-Romberg syndrome is characterized by progressive unilateral facial atrophy affecting subcutaneous tissue, cartilage and bone structures. Headache attacks and epilepsy are commonly associated with this syndrome but the underlying pathophysiology is still unknown. A case of a 12-year-old boy with Parry-Romberg syndrome and syringomyelia suffering from severe headache attacks and epileptic seizures is reported herein.
Headache attacks were associated with bilateral autonomic symptoms and hyperventilation and were usually followed by complex partial and sometimes by secondary generalized tonic seizures. Detailed neuroimaging examinations were performed (magnetic resonance imaging [MRI] of the head, orbits, and spinal cord, MR angiography, and MR spectroscopy of the cerebellum). The EEG pattern revealed localized discharges contralaterally to the affected side. Antiepileptic treatment with carbamazepine was instituted with minimal effect. Modification of treatment (replacement with oxcarbazepine) was successful.
In the reported patient interesting correlation of headache attacks, autonomic symptoms and epileptic seizures was observed. Additionally we believe it is the first report of coincident syringomyelia and Parry-Romberg syndrome.
Keywords
Parry-Romberg syndrome, facial hemiatrophy, headache, epilepsy, syringomyelia
About this articleTitle
Diagnostic difficulties of paroxysmal symptoms in a boy with Parry-Romberg syndrome
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
297-303
Page views
267
Article views/downloads
409
DOI
10.1016/S0028-3843(14)60045-1
Bibliographic record
Neurol Neurochir Pol 2010;44(3):297-303.
Keywords
Parry-Romberg syndrome
facial hemiatrophy
headache
epilepsy
syringomyelia
Authors
Marek Kaciński
Agnieszka Biedroń
Anna Zając
Małgorzata Steczkowska
