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Case Report
Progressive facial hemiatrophy (Parry-Romberg syndrome), a rare cause of temporal lobe epilepsy: Case report
1 Undergraduate student of Medicine at Universidade do Estado de Mato Grosso (UNEMAT), Cáceres, Mato Grosso, Brazil
2 Programa de Mestrado em Neurologia - Universidade de Vassouras e Escola de Medicina - Universidade Iguaçu - UNIG, RJ, Nova Iguaçu, Brazil
3 Adjunct Professor of the Medicine course at the Iguaçu University, Iguaçu University - UNIG/RJ, Nova Iguaçu, Brazil
4 Physician, Neurologist, Hospital Samer Rede D’or São Luiz, Resende, RJ, Brazil
5 Physician, Neurologist, PhD student in Neurology at the Federal University of the State of Rio de Janeiro - UNIRIO, Adjunct Professor of the Medicine course at the Iguaçu University, Iguaçu University - UNIG, RJ, Nova Iguaçu, Brazil
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Antônio Marcos da Silva Catharino
Rua Gavião Peixoto 70, Room 811, CEP 24.2230-100, Icaraí, Niterói-RJ,
Brazil
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Article ID: 101252Z01FS2021
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How to cite this article
Souza FDS, Orsini M, Martins Jr GC, Namen M, Mayall ACGD, da Silva Catharino AM. Progressive facial hemiatrophy (Parry-Romberg syndrome), a rare cause of temporal lobe epilepsy: Case report. Int J Case Rep Images 2021;12:101252Z01FS2021.ABSTRACT
Introduction: Parry-Romberg syndrome is also known as progressive hemifacial atrophy. It, which is characterized by a unilateral atrophy of the skin, soft tissue, muscles, and/or bones of the face, is a variant of linear morphea or an independent disorder. It has sporadic neurocutaneous involvement, occasionally it can even involve the central nervous system (CNS). In this condition, facial atrophy may be accompanied by classic linear morphea lesions on the face or elsewhere. Parry-Romberg syndrome is an uncommon disorder, more common in females, usually manifested in the first decade of life, but its etiology remains unknown.
Case Report: A woman with 49 years old, seeking neurological care due to epileptic seizures that started 21 years ago. The crises described were mostly focal, with compromised consciousness, symptomatology related to the temporal lobe with automatisms and sensory hallucinations. Physical examination: right hemifacial atrophy was observed with hemilingual atrophy and right enophthalmos.
Conclusion: The patient was diagnosed with Parry-Romberg syndrome after semiological evaluation of the neurology and investigation of the patient’s clinical history. The electroencephalogram showed marked signs of right temporal cortico-subcortical dysfunction with 2–4 Hz waves, slow wave bursts. The conduct is clinical and the prognosis restricted to each patient.
Keywords: Epilepsy, Facial hemiatrophy, Neurocutaneous syndromes, Temporal lobe
SUPPORTING INFORMATION
Author Contributions
Felipe dos Santos Souza - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Marco Orsini - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Gilberto Canedo Martins Jr - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Marcelo Namen - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Antonio Carlos Garcia Dias Mayall - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Antônio Marcos da Silva Catharino - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Felipe dos Santos Souza et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
