Progressive Facial Hemiatrophy Revisited

Guo, Zhen‐Ni; Zhang, Hong‐Liang; Zhou, Hong-Wei; Wen-jing, Lan; Wu, Jiang; Yang, Yi

doi:10.1001/archneurol.2011.190

Cited by 10 publications

(2 citation statements)

References 12 publications

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“…[2,5,6] In this report, we describe a case of PHA who manifested serial changes in brain lesions on magnetic resonance imaging (MRI). The case suggests that PHA may have a “relapsing–remitting” disease course and that periphery hepatitis B virus (HBV) infection associated auto-immune inflammatory process may be one of the triggers for relapse of PHA.…”

Section: Introductionmentioning

confidence: 99%

Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

Zhang

Zhu

et al. 2016

Medicine

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Introduction:Progressive hemifacial atrophy (PHA) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, and fat, muscle, and osteocartilagenous structures creating a sunken hemiface appearance.Etiopathogenesis of PHA is poorly understood; no definitive treatment is currently available.Clinical Findings:We report a 41-year-old woman with PHA who showed an uncharacteristic “relapsing–remitting” evolution of brain lesions and was seropositive for hepatitis B virus (HBV). She presented with a history of recurrent tonic-clonic seizures. Magnetic resonance imaging (MRI) showed progressive atrophy and multiple white matter lesions in the left side of the brain. Interestingly, the serial MRI examination (4 MRI scans over a period of 9 years) showed a “relapsing–remitting” pattern of brain lesions akin to that observed in a subtype of multiple sclerosis. Autoimmune-related investigations revealed increased serum levels of immunoglobulin (Ig) G, anti-nuclear antibody (ANA), and γ-IgG. Infection is considered as one of the possible causes of PHA. However, the association of peripheral infection such as HBV infection with PHA has not been reported.Conclusion:Our experience with this case suggests that PHA may have a relapsing–remitting disease course. Autoimmune inflammatory response to chronic HBV infection may have triggered the relapse in this case. This case underlines a novel etiopathogenetic mechanism of PHA.

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Section: Introductionmentioning

confidence: 99%

Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

Zhang

Zhu

et al. 2016

Medicine

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“…Parry-Romberg syndrome, described by Parry and Romberg in the nineteenth century, is also known as progressive facial hemiatrophy (1)(2)(3)(4)(5)(6)(7)(8)(9)(10) . This syndrome is usually unilateral (2)(3)(4)6,10,11) , affecting the skin (1,3,5,(7)(8)(9)(10)12) , muscles (1,3,(5)(6)(7)(8)10,11) , fatty tissue (4,6,8,12) , cartilage (4,7) and bones (1,3,4,(6)(7)(8)(9)(10)(11)(12) , and can also present as idiopathic facial atrophy because its etiology remains unknown.…”

Section: Introductionmentioning

confidence: 99%