Parry Romberg Syndrome: 7 Cases and Literature Review

Abstract

SUMMARY:Parry Romberg syndrome is a rare progressive hemiatrophy of the face that typically occurs in children and young adults and has a peculiar progression that ceases without apparent cause after a highly variable period. Only a subset of patients with Parry Romberg syndrome will develop secondary neurologic or ophthalmologic symptoms, and prognosis is highly variable. Inconsistency in the pattern of atrophy and the development of associated symptoms in patients with Parry Romberg syndrome has made it challenging to diagnose, prognosticate, and treat. The precise etiology of this disease remains unknown, but some authors have implicated sympathetic cervical ganglion dysfunction, abnormal embryogenesis, autoimmune and inflammatory mechanisms, or vasculopathy as potential causes. We present 7 cases of Parry Romberg syndrome and their associated clinical and imaging findings with specific attention to the radiographic characteristics of this disease. ABBREVIATION: PRS ϭ Parry Romberg syndrome

Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome

“…Cerebral atrophy was accompanied by contralateral cerebellar atrophy (F). Intrathecal IgG-synthesis as well as parenchymal and prominent perivascular T-cell infiltration in a brain biopsy (G) confirmed neuroinflammation as a potential pathophysiological correlate of PRS 1 2. Glucocorticoid treatment subsequently stabilised the late clinical progression.…”

Parry-Romberg syndrome in a patient with scleroderma

“…The proposed mechanisms of disease include autoimmune processes, trauma, hypoactivity or hyperactivity of the sympathetic nervous system, disorders of the trigeminal nerve and infections. As it presents with similar features to localised scleroderma, it had been considered an autoimmune disease 6. The autoimmune theory was suggested by its coexistence with other autoimmune conditions such as localised scleroderma, and its tendency to present with raised autoimmune antibodies 7.…”

Extra-Cephalic Involvement in the Syndrome of Parry Romberg

“…left side [2]. However, other sites have also been documented, including the neck, shoulders, chest, and lower limbs [4]. Our patient had a right hemifacial lesion (V3 territory) with an extension of the atrophy towards the contralateral lower limb, which was rarely previously described in the literature [1,5,6].…”