Parry Romberg syndrome: Imaging features in 4 consecutive cases and review of literature

Ahmed, Sabha; Tiwari, Sarbesh; Yadav, Taruna; Khera, Pushpinder Singh; Garg, Pawan Kumar; Sureka, Binit; Budania, Anil; Singh, Saurabh

doi:10.1016/j.jocn.2020.04.024

Cited by 6 publications

(10 citation statements)

References 20 publications

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“…2 A 2020 literature review includes the following additional treatment modalities for PRS: anticonvulsants for seizures and cosmetic procedures, such as autologous fat grafts, after stabilization of disease. 3…”

Section: Discussionmentioning

confidence: 99%

“…5 Typical MRI findings on T2-weighted imaging in PRS can demonstrate hyperintense white matter lesions usually ipsilateral to skin involvement but can be found bilaterally as well. 2,3 Other MRI findings include ipsilateral cerebral hemiatrophy and intraparenchymal calcifications. 3 Consistent with the literature, our patient's MRI demonstrated hyperintensities in the white matter on T2-weighted imaging.…”

Section: Discussionmentioning

confidence: 99%

“…1,6 Some authors advocate that PRS and LSCS are on a spectrum of the same pathology. [2][3][4] A wide array of neurological symptoms in PRS has been noted in the literature. In a 2015 review, the authors reviewed neurological symptoms in PRS and noted epilepsy as the most common.…”

Section: Introductionmentioning

confidence: 99%

“…Several theories have been proposed: trauma, infection, sympathetic dysfunction, vasculitis, and autoimmune. 2,3 Linear scleroderma en coup de sabre (LSCS) is described as a unilateral linear depression typically located between the crown and forehead. 4 The association of PRS with LSCS has been well described in the literature.…”

Section: Introductionmentioning

confidence: 99%

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A case of parasympathetic hyperactivity and associated Parry–Romberg syndrome

Clapp

Hohler

2021

SAGE Open Medical Case Reports

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This case report describes a 46-year-old female with a history of multiple endocrine neoplasia type 1 syndrome status post-parathyroidectomy, thymectomy via robotic video-assisted thoracoscopic surgery, and pituitary adenoma resection presenting with parasympathetic hyperactivity and Parry–Romberg syndrome. Parry–Romberg syndrome is a rare disorder that manifests as facial hemiatrophy. Reported symptoms include cognitive dysfunction, olfactory hallucinations, and parasympathetic hyperactivity: miosis of the right eye, anhidrosis, diarrhea, urinary incontinence, bowel incontinence, and orthostatic hypotension. Previous reports have noted associations between Parry–Romberg syndrome and sympathetic hyperactivity and dysregulation. This case report focuses on an association between Parry–Romberg syndrome and unopposed parasympathetic activity.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A case of parasympathetic hyperactivity and associated Parry–Romberg syndrome

Clapp

Hohler

2021

SAGE Open Medical Case Reports

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“…Computed tomography (CT) and magnetic resonance imaging (MRI) can help diagnose CTP and associated complications such as atrophy and calcinosis cutis [16]. In Parry-Romberg syndrome, the usefulness of CT and MRI in measuring subcutaneous tissue atrophy was described [36]. However, these techniques are usually not as accessible as HFUS, are more expensive, uncomfortable for the patient, and may require the use of a contrast medium and, in the case of CT, ionizing radiation [32].…”

Section: Discussionmentioning

confidence: 99%

High-Frequency Ultrasound to Assess Activity in Connective Tissue Panniculitis

Giavedoni

Podlipnik

Vega

et al. 2021

JCM

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Determining disease activity from clinical signs in patients with connective tissue panniculitis (CTP) is often challenging but is essential for therapeutic decision making, which largely relies on immunosuppressant treatment. High-frequency ultrasound (HFUS) may be useful in supporting such decisions by accurately determining CTP activity. This study aimed to investigate the accuracy of HFUS in identifying signs of CTP activity or inactivity and assess its usefulness in therapeutic decision making. A prospective cohort study of consecutive patients with biopsy-proven CTP receiving HFUS was conducted in a tertiary university hospital (2016–2020). HFUS was performed at inclusion and at each 3- or 6-month follow-up visit, depending on disease activity. Twenty-three patients with CTP were included, and 134 HFUSs were performed. In 59.7% (80) of the evaluations, the clinical presentation did not show whether CTP was active or not. In these cases, HFUS showed activity in 38.7% (31) and inactivity in 61.3% (49). In 71.25% (57) of the visits, HFUS was the determinant for therapeutic decisions. Further follow-up showed consistent clinical and HFUS responses in all unclear cases after treatment modification. HFUS appears to be a useful adjunct to the clinical examination for CTP to assess activity and make therapeutic decisions.

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Neuropathologic findings in a patient with Parry-Romberg syndrome: active cerebral vasculitis and brain injury

Wappler-Guzzetta,

Hanak,

Bannout

et al. 2024

J Rare Dis

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Purpose Parry-Romberg syndrome (PRS) is a rare disorder characterized by slowly progressive hemifacial atrophy. Although the underlying etiology is unknown, proposed disease mechanisms which include autoimmune, infection, trauma, and other causes have been proposed as underlying disease mechanisms. Approximately, 10–20% of PRS patients have neurologic manifestations such as epilepsy, headaches, or associated vascular malformations. There are reports of PRS responsive to immunosuppressive medications, supporting the autoimmune hypothesis. Currently, the neuropathologic findings in patients with PRS are not well documented. Methods Herein, we present a case of a 19-year-old female with PRS, who underwent partial frontal lobectomy for intractable epilepsy. Results The resection specimen showed multifocal active lymphocytic (T-cell mediated) arteritis in midsized cortical arterioles, with the adjacent meninges showing fibrosis involving both the meningeal tissue and its vascular network. In addition, neuronal loss and gliosis were evident in the cortex, likely due to the associated vascular injury. Conclusions This report is the first to demonstrate active cerebral vasculitis in a patient with PRS, supporting the previous suspicion of inflammatory etiology in this disease. In addition, the widespread vascular fibrosis in the meningeal vessels and an area of cortical ischemia support the presence of previous inflammatory vascular processes in the area.

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Parry Romberg syndrome: Imaging features in 4 consecutive cases and review of literature

Cited by 6 publications

References 20 publications

A case of parasympathetic hyperactivity and associated Parry–Romberg syndrome

A case of parasympathetic hyperactivity and associated Parry–Romberg syndrome

High-Frequency Ultrasound to Assess Activity in Connective Tissue Panniculitis

Neuropathologic findings in a patient with Parry-Romberg syndrome: active cerebral vasculitis and brain injury

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