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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 - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Marco Orsini - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Gilberto Canedo Martins Jr - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Marcelo Namen - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Antonio Carlos Garcia Dias Mayall - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Antônio Marcos da Silva Catharino - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
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.
