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Difficulties in differentiation of Parry–Romberg syndrome, unilateral facial sclerodermia, and Rasmussen syndrome

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Abstract

Introduction

Parry–Romberg syndrome (progressive facial hemiatrophy) is a unilateral, slowly progressive atrophy affecting the skin, subcutaneous tissues, muscles, and bones. The relationship between Parry–Romberg syndrome and connective tissue disorders, especially scleroderma en coup de sabre, is still unclear. The neurological symptoms, including epilepsy, migraine, and brain lesion, on neuroimaging may be similar. Rasmussen encephalitis (RE) is connected with chronic inflammation and damage of one hemisphere. Clinically, it is manifested by epileptic partial seizures and unilateral neurological symptoms.

Case Report

The authors present the case of a 10-year-old girl with features suggestive of RE, with refractory partial motor dextrolateral seizures followed by development of hemiparesis and with progressive intellectual deterioration. At the age of 2 years, some changes on the left part of the face typical of Parry–Romberg syndrome or a linear form of scleroderma were noticed.

Discussion

The authors discussed the difficulties in differential diagnosis in that patient. The presented girl constitute the case from the borderline zone of the aforementioned disorders.

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Authors and Affiliations

  1. Departament of Pediatric Neurology, Medical University of Silesia, Katowice, Poland

    Justyna Paprocka, Ewa Jamroz & Elzbieta Marszal

  2. Departament of Neuropathology, Institute of Neurology, Collegium Medicum Jagiellonian University, Kraków, Poland

    Dariusz Adamek

  3. Department of Pediatric Surgery, Division of Pediatric Neurosurgery, Medical University of Silesia, Katowice, Poland

    Marek Mandera

Authors
  1. Justyna Paprocka
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  2. Ewa Jamroz
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  3. Dariusz Adamek
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  4. Elzbieta Marszal
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  5. Marek Mandera
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Correspondence to Justyna Paprocka.

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Paprocka, J., Jamroz, E., Adamek, D. et al. Difficulties in differentiation of Parry–Romberg syndrome, unilateral facial sclerodermia, and Rasmussen syndrome. Childs Nerv Syst 22, 409–415 (2006). https://doi.org/10.1007/s00381-005-1262-x

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  • DOI: https://doi.org/10.1007/s00381-005-1262-x

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