PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “FACIAL HEMIATROPHY”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources
PubMed1997

Clinical and radiologic findings in progressive facial hemiatrophy (Parry-Romberg syndrome).

We describe the clinical and radiologic changes related to progressive facial hemiatrophy (Parry-Romberg syndrome) occurring during a 20-month period in a child who presented with unilateral neurologic deficits and facial hemiatrophy. CT and MR findings included unilateral focal infarctions in the corpus callosum, diffuse deep and subcortical white matter signal changes, mild cortical thickening, and leptomeningeal enhancement with dense mineral deposition. Angiographic findings were normal. We hypothesize that a noninfectious, unilateral inflammatory process, possibly associated with a chronic vasomotor disturbance and sympathetic nerve chain inflammation, was a major factor in the pathogenesis of this syndrome.

Arachnoid↗
PubMed1975

Refractive change as the initial sign of progressive facial hemiatrophy.

Unilateral arrested myopia preceded other signs of progressive facial hemiatrophy in a 13-year-old girl. Facial signs including soft tissue atrophy, enophthalmos, pupillary dialation with decreased pupillary reaction, and extraocular muscle involvement later appeared on the same side as the eye with the arrested myopia.

Adolescent↗
PubMed1979

[Pathogenesis of symptomatic facial hemiatrophy].

The data on clinical and electroencephalographic examination of 19 patients are presented. The patients developed facial hemiatrophy after they had sustained ganglionitis of the superior cervical sympathetic ganglion, stem encephalitis, trifacial neuralgia (neuritic stage), tumours of the Gasserian ganglion and syringobulbia. A pathogenetic relationship between the development of symptomatic facial hemiatrophy and lesions of the vegetative and somatic apparatus in the cerebral (mesencephalotruncal area) and extracerebral (superior cervical sympathetic ganglion) structures of the nervous system is noted.

Arachnoiditis↗
PubMed1991

[Progressive facial hemiatrophy].

The authors describe a case of progressive facial hemiatrophy in a woman aged 26 years, coming from the Isfarin region of Tadzhikistan. The patient views herself as being ill for 14 years, since the moment of an epileptic attack with tonic and clonic convulsions. Approximately at the same time she noted a small dry ulcer on the left on the vertex. The ulcer slowly increased, followed by skin atrophy. The disease progressed for 4 to 5 years. At present to the left there are folds in the form of scars on the face. The skin is thinned, united with the bones in the frontal and parietal areas, the subcutaneous fat is atrophic. The lips and nose at the left are subatrophic. Negligible enophthalmos, hemiatrophy of the tongue at the left. Alopecia. A certain deterioration of memory and reduction of the critical attitude are recorded. The patient is in a state of euphoria. Left-sided anosmia. The left auricular floor is subatrophic, hearing is almost lacking. Diffuse elevation of the tendinous reflexes of the limbs on the left side. X-ray signs of osteoporosis of the bones of the cranial vault on the left.

Adult↗
PubMed1991

[Experimental study of progressive facial hemiatrophy: effects of cervical sympathectomy in animals].

Progressive facial hemiatrophy (Romberg's syndrome) is of unknown cause and uncertain pathogenesis. The main pathogenetic hypotheses are: sympathetic system alterations, localized scleroderma, trigeminal changes, possibly of genetic origin. To test the hypothesis of sympathetic system alterations, we designed an experimental model with ablation of the superior cervical sympathetic ganglion in rabbits, cats and dogs. All the animals were operated upon when 30 days old and were examined monthly for 1 year. During this period localized alopecia, corneal ulceration, keratitis, strabismus, enophthalmos, ocular atrophy, hemifacial atrophy and slight bone atrophy on the side of the sympathectomy were observed. Thus, cervical sympathectomy reproduces in animals the principal clinical alterations of Romberg's syndrome. Our data suggest that the sympathetic system is involved in the pathogenesis of this syndrome.

Animals↗
PubMed1981

Myopathy associated with sclerodermal facial hemiatrophy.

A patient who had linear scleroderma associated with ipsilateral hemiatrophy of the tongue and subsequent facial hemiatrophy was studied. Biopsy specimens of the plaque of scleroderma showed the skin changes of scleroderma as well as fascial and muscle changes. The fascia had an impressive plasma cell fasciitis with numerous plasma cells and scattered lymphohistiocytic cells. Histochemical study of the temporalis muscle underlying the plaque of circumscribed scleroderma disclosed severe localized atrophy of type 1 and type 2 fibers similar to the pathologic findings previously described in a patient with localized scleroderma.

Adult↗
PubMed2006

Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients.

BACKGROUND: Progressive facial hemiatrophy (PFH) is characterized by a slowly progressive atrophy of soft tissues and in some cases bony structures. Coexisting features of localized scleroderma (LS) are commonly observed, indicating the close nature of both disorders. OBJECTIVE: We sought to investigate clinical, serological, and radiographic findings in PFH from 278 patients with LS and to discuss the relationship to linear scleroderma en coup de sabre (LSCS). METHOD: A total of 12 patients with PFH were retrospectively evaluated on the basis of clinical, serological, and radiographic findings. RESULTS: Five patients (42%) presented with complete PFH, and 7 patients (58%) with partial PFH involving either cheek or forehead. Five of the patients (42%) had a coexisting LSCS lesion, and 3 of them (25%) had concomitant LS of the trunk. Intraoral involvement was present in 6 cases. Neurological involvement was common, in particular, epileptic seizures. Serologic investigations showed neither evidence for infection with Borrelia burgdorferi nor any other indication of underlying systemic immunological disorders. LIMITATIONS: There were a relatively small number of patients in a retrospective study. CONCLUSION: The reported cases suggest a close relationship between PFH and LSCS. PFH might appear as two different subtypes, one involving cutaneous structures and presenting with clinical features similar to LSCS, one being strictly restricted to subcutaneous structures, primarily affecting the cheek area. The manifold clinical features of central nervous system involvement indicate the pathogenetic importance of neurological involvement in the development of PFH. Magnetic resonance imaging should be included in the tools of standard diagnostic procedures in patients with PFH. The etiologic relevance of autoimmunity as well as preceding trauma should be investigated in larger collective studies.

Adolescent↗
PubMed1994

[Facial hemiatrophy--Romberg's disease].

Author present a care of acquired facial hemiatrophy--Romberg's disease, a sporadic disease of unclear etiology characterized by shrinking and deformation of one side of face, including superficial facial tissues, subcutaneous tissues, fatty tissue, musculature, cartilage and bones. The principle etiopahogenetic hypothesis suggests that Romberg's disease is the consequence of central and peripheral alterations in the sympathetic nervous system. The disease was first described by Romberg in 1846, and in 1963 Roger gave a detailed description and analysis of 1035 cases. In this study authors presented the latest information form literature (magnetic resonance image, biochemical investigations, experimental model of a sympathectomy at animals). A case of a patient treated with great number of available methods: computerized tomography, X-ray, Doppler spectral analyze, termovision, electromyography, electroencephalography, evoked visual potentials, brainstem's evoked potential is shown.

Adolescent↗
PubMedDate not supplied

[Parry-Romberg progressive facial hemiatrophy: pathogenic and evolutionary considerations with regards to a case followed up for a long period of time].

INTRODUCTION: The Parry-Romberg syndrome is a clinical condition in which there is progressive facial hemiatrophy which may be associated with neurological, ocular, cutaneous and auto-immune disorders. It is of unknown aetiology, although many theories have been put forward. CLINICAL CASE: We present a case of left facial hemiatrophy associated with ipsilateral lingual hemiatrophy and signs of cortico-cerebellar dysfunction, the neuroimaging findings (NMR) and clinico-radiological follow-up covered a period of over 10 years. CONCLUSION: The data on the clinical course and neuroimaging, together with our review of the literature, suggest that the Parry-Romberg syndrome may be considered to be a dysgenetic process which may originate during the first stages of CNS embryogenesis.

Atrophy↗
PubMed2000

Progressive facial hemiatrophy after epileptic seizures.

Intractable complex partial seizures developed in a 3-year-old female with normal intracranial findings on computed tomography. Frontal paramedian band-like depression of the skin gradually developed thereafter, and progressive facial hemiatrophy (Parry-Romberg syndrome) was diagnosed. Computed tomography scanning at 5 years of age revealed multiple parenchymal calcifications and low-density areas in the white matter of the frontoparietal lobes. Epileptic seizures, one of the major neurologic complications of progressive facial hemiatrophy, could precede the succeeding neurocutaneous changes.

Child, Preschool↗
PubMed2005

Progressive facial hemiatrophy complicated by sclerodactyly, Raynaud's phenomenon, anti-ribonucleoprotein antibody, and trigeminal nerve disturbance.

A Japanese woman was diagnosed as having progressive facial hemiatrophy when she was 26 years old. After 30 years, Raynaud's phenomenon and sclerodactyly suddenly appeared; at the same time, positive rheumatoid factor and anti-ribonucleoprotein (anti-RNP) antibody were noted on serological examinations. When she was 60 years old, trigeminal nerve disturbance also appeared. The associations between progressive facial hemiatrophy, systemic scleroderma, and trigeminal nerve disturbance are interesting and should be discussed.

Journal Article↗
PubMed1990

Surgical treatment of progressive facial hemiatrophy.

On the basis of multiple clinical observations (64 persons), classification of the defects in progressive facial hemiatrophy is proposed. Three degrees of atrophy of the facial tissues have been pointed out and the methods of surgical treatment depending on them have been described. In degree I-II atrophy, contour plasty using silicone implants and injections of elastosil are preferred. In degree III atrophy, autotransplantation of free skin-muscle and skin-fascia flaps is performed.

Adult↗
PubMed1980

Masticatory spasm in facial hemiatrophy.

Spontaneous spasms began in the left masseter muscle six years following the onset of facial hemiatrophy in a young woman. A dental procedure had preceded the original facial wasting by four weeks. The electromyographic findings were similar to those described in hemifacial spasm and consisted of brief bursts of one to four morphologically different potentials, each firing at rates of up to 200 Hz. The findings demonstrate that spontaneous activity due to nerve injury can arise in muscles innervated by the trigeminal nerve.

Adult↗
PubMed1982

[Scleroderma with progressive facial hemiatrophy and atrophy of the other side of the body].

The authors report the case of a 28 year old woman suffering from a chronic polyarthritis with a linear sclerodermia and presenting a crossed atrophy involving the left side of the body and the right side of the face and neck. The polyarthritis began at age 6, with signs of systemic illness, resulting early in important joint disability and proceeding with exacerbations and remissions. From the onset of this polyarthritis the patient experienced in the left side of the body highly painful and frequent muscular cramps which became rarer with the occurrence and progression of atrophy on the same side. At age 15, the patient experienced similar cramps in the right face, followed by progressive right hemiatrophy. At age 28, examination showed crossed atrophy involving the left side of the body and the right side of the face, tongue and neck, associated in these territories with several atrophic plaques on the skin, circumscribed alopecia, as well as numerous joint sequella. The laboratory date yielded immunologic abnormalities. On the CT scan the brain was normal but the right facial hemiatrophy involving orbital region and pharynx was visible. On the muscular biopsy there were inflammatory changes in the atrophic territory. Skin biopsies were consistent with sclerodermia. Two clinical points should be emphasized. Firstly, the remarkably crossed topography of the atrophy: the upper limit of the left body atrophy corresponded with the lower limit of the right face and neck atrophy; secondly, the unusual features of the muscular cramps. The relationship between sclerodermic facial hemiatrophy and Parry Romberg syndrome are then discussed: the study of this case and the literature do not provide sufficient criteria to allow description of two separate entities. Finally, the pathogenesis of localized sclerodermia is considered; in this case the association with the immunologic abnormalities and chronic polyarthritis is in favor of the hypothesis of a systemic disorder.

Adult↗
PubMed1992

Progressive facial hemiatrophy with localized scleroderma.

A 46-year-old woman who presented with progressive facial hemiatrophy (PFH) following localized scleroderma is described. The patient had a markedly deformed and depressed plaque surrounded by erythema on the right cheek. She also showed linear scleroderma with hair loss on the occipital area and morphea lesions on the neck and shoulder. Histological findings of the facial and other atrophic lesions were consistent with localized scleroderma. Therefore, the PFH and localized scleroderma may have had the same pathological background in this case.

Facial Hemiatrophy↗