Clinical and serological characteristics of progressive facial hemiatrophy
: a case series of 12 patients.
MR imaging examinations revealed hemiatrophy
most prominently involving right-sided skin and subcutaneous fat, with lesser involvement of the muscles of mastication.
Neurologic examination showed left-sided hearing loss (50-60 dB, 500-4000 Hz), left-sided facial paralysis (Grade 3 according to the House-Brackmann classification), and deviation of the tongue to the left on protrusion with left hemiatrophy
of the tongue, without deficits of other cranial nerves.
Three-dimensional imaging of progressive facial hemiatrophy
(parry-romberg syndrome) with unusual conjunctival findings.
The patient had not experienced any trauma and did not have any endocrinological problems, developmental anomalies, and/or systemic diseases such as Wegener granulomatosis, orbital metastasis, osteomyelitis, progressive lipodystrophy, or facial hemiatrophy
. The possibility of carcinoma in the pterygopalatine fossa was excluded by a FDG-positron emission tomography study.
Classical imaging findings include hypoplasia of one brain hemisphere (hemiatrophy
), often with reduced volume of a corresponding cranial fossa, and consecutive thickening of nearby bony structures and enlargement of equilateral paranasal sinuses, the frontal sinus being most often involved [1-3].
Dyke-Davidoff-Masson syndrome (DDMS) is characterized by the presence of hemiatrophy
of one cerebral hemisphere, which is usually a consequence of an insult to the developing brain.
It provided descriptions of Parry's Disease, now known as Grave's Disease or thyrotoxicosis, enlargement of the thyroid gland; the first recorded cases of Hirschsprung's disease, a birth defect that affects the nerve cells in the large intestine; and Parry-Romberg Syndrome, or facial hemiatrophy
, a rare disease characterized by progressive shrinkage and degeneration of the tissues beneath the skin (Hull 338).
Dermatological examination revealed a linear atrophic depression of soft tissues, especially of the lower part of the right cheek and of the right side of the chin with labial asymmetry and tongue hemiatrophy
. No eyebrow or forehead alopecia was present (Fig 1).
, homolateral cervical linear sclerodema and thyroid disease.
of the tongue and floor of the mouth demonstrated by computed tomography.
Significant findings on physical examination included chronic hemiatrophy
involving his entire left face, as well as a Babinski sign on the right.