Progressive facial hemiatrophy: MRI appearances

The cranial CT and MRI appearances of a 14‐year‐old girl with Parry‐Romberg syndrome and epilepsy are described. The findings are compared with the two published descriptions of MRI and CT in such patients. MRI appearances in our patient differ from those published and may be consistent with a vascular malformation. The one published report on the intracranial histopathology of a patient with Parry‐Romberg syndrome and epilepsy states that a microscopic vascular malformation was found. We discuss the relationship between radiological and pathological findings. The possible aetiologies of Parry‐Romberg syndrome (vascular malformation, immunological, trauma, sympathetic innervation, hereditary, slow virus) are discussed. We suggest that Parry‐Romberg syndrome could be regarded as a neurocutaneous syndrome, a component of which includes intracerebral vascular dysplasia. In this and the other three cases of Parry‐Romberg syndrome with epilepsy, the sensitivity of MRI in detecting intracranial lesions is demonstrated. Recommendations for imaging these patients are proposed.

[1]  M. Morrell,et al.  Intracerebral involvement in scleroderma en coup de sabre: Report of a case with neuropathologic findings , 1995, Annals of neurology.

[2]  M. Fritzler,et al.  Autoantibodies in Parry-Romberg syndrome: a serologic study of 14 patients. , 1995, The Journal of rheumatology.

[3]  S. Felber,et al.  MR of brain involvement in progressive facial hemiatrophy (Romberg disease): reconsideration of a syndrome. , 1994, AJNR. American journal of neuroradiology.

[4]  A. Larner,et al.  Some observations on the aetiology of progressive hemifacial atrophy ("Parry-Romberg syndrome") , 1993, Journal of neurology, neurosurgery, and psychiatry.

[5]  T. Lehman.  The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma en coup de sabre. Mistaken diagnosis or overlapping conditions? , 1992, The Journal of rheumatology.

[6]  C. Fink,et al.  Intracranial findings in progressive facial hemiatrophy. , 1992, The Journal of rheumatology.

[7]  T. Zileli,et al.  Progressive hemifacial atrophy with localized scleroderma. , 1989, European neurology.

[8]  R. Grossman,et al.  Occult cerebral vascular malformations: high-field MR imaging. , 1986, Radiology.

[9]  D. Norman,et al.  MR imaging of calcified intracranial lesions. , 1985, Radiology.

[10]  D. Norman,et al.  Intracranial vascular malformations: MR and CT imaging. , 1985, Radiology.

[11]  R. Lederman.  Progressive facial and cerebral hemiatrophy. , 1984, Cleveland Clinic quarterly.

[12]  B. Berg,et al.  Progressive hemifacial atrophy: report of three cases, including one observed over 43 years, and computed tomographic findings. , 1982, Archives of neurology.

[13]  S. Wolf,et al.  Neurological complications of progressive facial hemiatrophy , 1974 .

[14]  J. Hickman,et al.  PROGRESSIVE FACIAL HEMIATROPHY. REPORT OF A CASE WITH MARKED HOMOLATERAL INVOLVEMENT. , 1964, Archives of internal medicine.

[15]  M. Eadie,et al.  THE CLINICAL FEATURES OF HEMIFACIAL ATROPHY , 1963 .

[16]  M. Moss,et al.  Progressive facial hemiatrophy following cervical sympathectomy in the rat. , 1960, Archives of oral biology.

[17]  H. Bruch,et al.  Progressive facial hemiatrophy , 1937 .