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.
After the first report of the syndrome given by Dyke, Davidoff and Masson in 1933, Alpers and Dear defined two types of cerebral hemiatrophy
in 1939, according to the probable etiology and the time of the insult .
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
, homolateral cervical linear sclerodema and thyroid disease.
Computed tomographic findings in cerebral hemiatrophy
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.
The diagnosis of DDMS was based on facial asymmetry, hemiplegia, mental retardation, and cerebral hemiatrophy
Abstract: Progressive facial hemiatrophy
, also known as Parry-Romberg syndrome (PRS), is characterized by slowly progressive atrophy of one side of the face, primarily involving the subcutaneous tissues and fat.
Similarly, the use of pieces of adipose tissue and artificial tissue substitutes such as silicon, collagen and hydroxyapatite for soft tissue augmentation in facial hemiatrophy
, hypomastia, mammoplasty and other deformities again face safety issues.