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PubMed1995

Progressive facial hemiatrophy with multiple benign tumors and hamartomas.

We report the unusual association of Progressive Facial Hemiatrophy (Parry-Romberg syndrome) with multiple benign tumors (orbital neurinoma, mandibular odontogenous fibroma) and hamartomas. The neurological clinical features were infantile hemiplegia, mild mental retardation and focal seizures. Brain CT-scan and MRI showed porencephaly and cerebral calcifications ipsilateral to hemifacial atrophy. Immunological investigations proved negative. The etiology of the disease and the bridging of this case to phakomatoses are discussed.

Adolescent↗
PubMedDate not supplied

[Facial hemiatrophy].

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Facial Hemiatrophy↗
PubMed1992

Progressive facial hemiatrophy: report of five cases and biochemical analysis of connective tissue.

Five cases of progressive facial hemiatrophy (PFH) are reported. A nonindurated depression on normal-colored skin was observed in the cheeks of 3 subjects, and 2 patients showed indurated, pigmented atrophic lesions associated with linear scleroderma or generalized morphea. Lipoatrophy with mild subcutaneous fibrosis was observed histologically in the patients with nonindurated depressions. In contrast, the patients with indurated lesions exhibited a marked dermal fibrosis and the disappearance of appendices in the dermis. When compared with unaffected skin used as a control, collagen and glycosaminoglycan contents were not different in diseased areas. However, the dermatan sulfate/hyaluronic acid ratio was increased 1.5- to 3.2-fold in PFH patients regardless of their clinical and histological differences. These results suggest that both types of PFH may be based on a similar connective tissue disorder.

Adolescent↗
PubMed1991

[A case of unilateral lingual atrophy and ipsilateral muscular atrophy supplied by trigeminal nerve--in relation to progressive facial hemiatrophy].

A 39-year-old man was admitted to our hospital because of atrophy of the right side of the tongue of 6 years' duration. Neurological examination showed atrophy and fasciculations in the right side of the tongue. Magnetic resonance imaging showed atrophy and high signal intensity areas on T1 and T2 weighted images in the right masseter, temporal, lateral and medial pterygoid muscles. Electromyography showed fibrillations and positive sharp waves at rest, high amplitude polyphasic motor unit potentials with weak contraction in the right tongue and masseter muscles. Examination of autonomic functions suggested postganglionic lesion of right facial sympathetic nerves. We believe that this case may be related to progressive facial hemiatrophy.

Adult↗
PubMed2004

Progressive facial hemiatrophy: a complex disorder not only affecting the face. A report in a monozygotic male twin pair.

UNLABELLED: Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of skin followed by unilateral craniofacial atrophy of subcutaneous tissues, including fat, muscle and bone. Hereditary factors have been postulated to be involved in the aetiology of PFH. Yet, the occurrence of PFH in one of two identical male twins reported here makes this possibility unlikely. PFH usually occurs in the first two decades of life, and the clinical presentation resembles linear scleroderma. PFH may be complicate by autoimmune, neurological, ocular and dental disorders. Management of PFH comprises a long term follow-up of somatic disorders, and prevention of psychological problems. Treatment of PFH is symptomatic and consists of plastic surgery after the disease activity has stopped. CONCLUSION: The occurrence of PFH in one of monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosis of PFH and accurate follow-up is essential to disclose the occurrence of complications.

Child↗
PubMed1999

Progressive facial hemiatrophy (Parry-Romberg syndrome).

A 48-year-old white woman presented with a 2-year history of progressive facial hemiatrophy involving the right side of the chin and tongue, associated with mastigatory spasm. Neurological examination showed no abnormalities. Computed tomography and magnetic resonance imaging of the brain and electrophysiological investigations were normal. Histopathological examination of a skin biopsy specimen from the chin revealed atrophy of the subcutaneous fat with homogenization of dermal collagen fibres. Phenytoin 100 mg t.d.s. relieved the mastigatory spasm.

Facial Hemiatrophy↗
PubMed2003

Progressive facial hemiatrophy: central nervous system involvement and relationship with scleroderma en coup de sabre.

OBJECTIVE: To investigate the relationship of progressive facial hemiatrophy (PFH) and scleroderma en coup de sabre by establishing the presence and type of central nervous system (CNS) involvement in both diseases and the possible coexistence of PFH with scleroderma in other body sites. METHODS: We divided 19 cases of PFH into 2 groups: group 1 in which atrophies were preceded by cutaneous indurations (n = 10) and group 2 with no precedent indurations (n = 9). The third group consisted of 7 cases of scleroderma en coup de sabre with no PFH features. Clinical and laboratory investigations included indirect immunofluorescence for antinuclear antibodies, and routine neurological examination involved electroencephalography, magnetic resonance imaging (MRI) before and after contrast application to evaluate the integrity of blood-brain barrier, angio-MRI to evaluate intracranial blood vessel anomalies, and 99mTc-HM-PAO-SPECT to evaluate regional cerebral blood flow (CBF). RESULTS: We found similar anomalies in all 3 groups. MRI did not show abnormality in 2 out of 9 PFH cases preceded by indurations, in 5 out of 9 cases not preceded by indurations, and in all 7 cases of scleroderma en coup de sabre, including 5 patients, in whom the CBF was found to be diminished. In single cases of groups 1 and 2, SPECT was normal despite some MRI abnormalities. Angio-MRI was not contributory since the same abnormalities of Willis circle were found in normal controls. In single cases of both PFH groups, MRI with contrast disclosed some damage of the blood-brain barrier. CONCLUSION: Our results suggest frequent CNS involvement in PFH cases, regardless of the time of presentation of cutaneous indurations, with or without coexistent plaques of localized scleroderma in other locations. This indicates a close relationship between PFH and scleroderma en coup de sabre. The detection of abnormal SPECT by normal MRI in some cases of PFH and scleroderma en coup de sabre is of practical importance. This indicates the usefulness of SPECT in studying both PFH and scleroderma en coup de sabre.

Adult↗
PubMed1997

[Progressive facial hemiatrophy (Parry-Romberg disease). Report of two cases associated with trigeminal neuralgia and cramps].

The authors report two cases of progressive facial hemiatrophy (PFH) associated with cranial nerves hyperexcitability. One of them (Case 1), a 41-year-old-woman noticed a slight atrophy on the left side of her face at age of ten. For the last three months, she has been suffering from fits of neuralgic pains on the left side of her face which have not ceased by drug treatment In view of this, a surgery procedure was planned for the patient. The other patient (Case 2), a 33-year-old-woman has a 8 year history which started with cutaneous abnormalities (white and dark spots). Two years later she observed that her right cheek and right thigh had got a mild atrophy. Frequently, she has been complaining of cramps in the mandibular region which are precipitated, sometimes, by opening the mouth. A probable relation between neural excitability and PHF is discussed.

Adult↗
PubMed1989

[A syndrome of facial hemiatrophy, circumscribed scleroderma and focal epileptic seizures associated with bilateral uveitis].

A peculiar form of sclerodermia is described in a 10 year girl. Initially, the disease pronounced itself with focal epileptic spells involving lated the focal sclerodermia and facial hemiatrophy. The peculiar feature of the case is the bilateral uveitis and subclinical serose meningitis. Brain CT scans and Doppler investigation of extracranial carotid and vertebral arteries were performed.

Child↗
PubMed2000

Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy.

OBJECTIVES: To report a case and discuss the mechanism of hemimasticatory spasm. DESIGN: Case report. PATIENT: A 37-year-old woman had a 3-year history of involuntary spasms of the right masseter muscle in association with localized scleroderma and facial hemiatrophy. Electrophysiological studies revealed a normal blink reflex. However, the masseter reflex and silent period were absent on the affected side. Distal latency and compound muscle action potential of the masseter nerve were normal. Needle electromyography demonstrated irregular bursts of motor unit potentials similar to those described in hemifacial spasm. A magnetic resonance imaging scan of the head showed mild hypertrophy of the masseter muscle and atrophy of subcutaneous fatty tissues on the affected side. Local injection of botulinum toxin A into the masseter muscle resolved the patient's symptoms. CONCLUSION: On the basis of clinical and electrophysiological findings, focal demyelination of motor branches of the trigeminal nerve owing to deep tissue changes is suggested as the cause of abnormal excitatory electrical activities resulting in involuntary masticatory movement.

Action Potentials↗