Progressive Hemifacial Atrophy with Localized Scleroderma

Tan, Ersin; Kürkçüoğlu, Nazif; Atalağ, Mehmet; Gököz, Aytaç; Zileli, Turgut

doi:10.1159/000116369

Cited by 9 publications

(4 citation statements)

References 10 publications

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“…There is one report of HFA with symptomatic masticatory spasms requiring treatment and trigeminal neuropathy was disclosed by nerve conduction studies [33]. Only one study in HFA affecting predominantly the tongue, failed to demonstrate neurogenic or myogenic process [31]. Neuroimaging was explored in a pediatric case of PR syndrome [9], speculating vasomotor disturbance and sympathetic dysfunction, but EMG was not performed.…”

Section: Discussionmentioning

confidence: 99%

A cross-sectional electromyography assessment in linear scleroderma patients

2014

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BackgroundMuscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS.MethodsWe conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997–2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern.ResultsNine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement.ConclusionIn our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed.

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Section: Discussionmentioning

confidence: 99%

A cross-sectional electromyography assessment in linear scleroderma patients

2014

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“…Several reports of intracerebral lesions such as infl ammation, calcifi cation and demyelination were shown on MRI and CT in patients with localised scleroderma leading to epilepsy and functional motor impairments. 7,8 Progressive hemifacial atrophy (ParryRomberg Syndrome) associated with localised scleroderma has also been reported, 2,9,10 but the relationship to these two conditions remains controversial. In most cases the term Parry-Romberg Syndrome should be used for progressive hemifacial atrophy alone.…”

Section: Discussionmentioning

confidence: 99%

A rare case of frontal linear scleroderma (en coup de sabre) with intra-oral and dental involvement

2010

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“…Muscle thinning can occur but it remains functional. At times there is ipsilateral atrophy of tongue, sweat and salivary glands [15,19,22,71,80,83]. However, in the simple cases of PHA (progressive hemifacial atrophy), the skin is normally pliable and movable [56,72].…”

Section: Soft Tissue Involvementmentioning

confidence: 99%

Progressive hemifacial atrophy: historical review and actual features related to a new classification of facial hypoplasia and atrophy

et al. 1994

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progressive hemifacial atrophy, described initially by Parry and Romberg, is reviewed in terms of etiopathogenesis and clinical features. The purpose is to clarify the variegated nature of this syndrome and to distinguish it from other forms of atrophy affecting the face, principally the soft tissues. The authors propose a new general classification of hypoplasia and atrophy of the face.Progressive hemifacial atrophy (PHA) is an acquired syndrome affecting peculiarly the subcutaneous fatty tissue together with skin, mimic musculature, bone and cartilage in that order of frequency. The etiology is unknown but neurodystrophic and sclerodermic theories have been suggested. The incidence in the population is at present unknown. Female/male ratio is 3 : 2 according to Rogers, while Archambault and Fromm, Mussinelli et al. and Rees reported ratios less than 1.2:1. Other authors, mainly in early papers, reported a very high occurrence in women [2,56,71,73]. Nerobyeyev et al., in 1990, noted a ratio higher than 4:1 [59]. The left side of the face is not significantly more affected than the right side; bilateral incidence is almost 5% [56, 72], but according to Rees bilateral involvement in Scleroderma or Romberg's atrophy is "'more unique than rare" [71]. The disease starts in the first or second decade of life (mean age: 5 15 yrs. ; range of 1-70 yrs.); and progresses slowly for 2-10 yrs. with long periods of remission. HistoryThe clinical features were described by Parry and Romberg [64,74]. It was called "progressive facial hemiCorrespondence to .' Dr. R. Roddi,

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Progressive Hemifacial Atrophy with Localized Scleroderma

Cited by 9 publications

References 10 publications

A cross-sectional electromyography assessment in linear scleroderma patients

A cross-sectional electromyography assessment in linear scleroderma patients

A rare case of frontal linear scleroderma (en coup de sabre) with intra-oral and dental involvement

Progressive hemifacial atrophy: historical review and actual features related to a new classification of facial hypoplasia and atrophy

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