Parry-Romberg syndrome

Carolei

et al. 2016

The Neurohospitalist

A 65-year-old man came to our hospital for an outpatient neurological assessment. He had a 20-year history of hemifacial atrophy associated with hemitongue atrophy ( Figure 1A), intermittent unilateral jaw spasms leading to frequent tongue bites, unilateral alopecia ( Figure 1B), parotid and submandibular gland atrophy, and tooth loss. Despite the extreme discomfort experienced by the patient, he had never received a full evaluation and diagnosis. On neurological assessment, myokymias of the jaw-closing muscles (massetez and temporalis) were detected (video) and confirmed by the presence of myokymic discharges during an electromyographic study. No other neurological signs were recognized. Laboratory and neuroimaging investigations were normal. A diagnosis of Parry-Romberg syndrome was made. This is a rare neurological disorder, mostly sporadic, and characterized by hemifacial atrophy involving the skin, the fat, the connective tissue, and sometimes the bone.

mentioning

confidence: 50%

Facial Myokymias In Parry-Romberg Syndrome

Carolei

et al. 2016

The Neurohospitalist

“…[3] this desorder can affect skin-soft tissue, muscles and bones, which were present in our case. [12] More strict definitions of the syndrome include contralateral jacksonian epilepsy, trigeminal neuralgia, and changes in the eyes and hair. [1] This rare entity is more commonly seen in women with a ratio of 3:2.…”

Section: Discussionmentioning

confidence: 99%

Parry Romberg Syndrome: A Rare Entity With Possible Association With Periodontitis

Elghoulbzouri¹,

Erraji²,

Ennibi³

2018

AIMDR

Background: Parry Romberg syndrome (PRS) or progressive hemifacial atrophy is a rare entity uncommon, degenerative, poorly understood with unknown etiology, but possible factors that are involved in the pathogenesis in this disorder, like trophic malfunction of sympathetic system, disturbance of fat metabolism, trauma, viral infections, heredity, endocrine disturbances and auto-immunity. PRS is seen most generally unilateral, in facial tissues including muscles, bones and skin. This alteration is characterized by the "coup de sabre"; this is clear line of demarcation between the normal and abnormal structures, results in psychological disturbance and communication defects like speech defects, and also dental malformations. It needs multidisciplinary approach including physician, dentist and psychiatric. The objective of this work is to report a rare case of PRS, concerning a young female patient, with persistence of moderate periodontal destruction with severe gingival inflammation. Extensive investigations and a multidisciplinary approach are necessary for successful management of the case.

“…The disease may occur at every age, however, it most often affects children below 10 y/o, in whom it leads to atrophy of soft and osseous tissues, oral structures and teeth (deformation of the face and mandible, unilateral tongue atrophy, masseter muscle fatigue, soft and hard palate atrophy, mandible hypoplasia, atrophy of alveolar processes, malocclusion, delayed teeth eruption, teeth loss) [4,27]. In 20% of cases PFH may be accompanied by neurological symptoms (including headaches, migraines, convulsions, trigeminal nerve paralysis, paresthesia, hemiplegia, anomalies of intracranial vessels), and in 15% of case by ocular symptoms (including enophthalmos, strabismus, eyelid atrophy, uveitis, glaucoma, diplopia, Horner's syndrome, mydriasis) [27][28][29][30]. Guerrerosantos et al proposed a 4-grade classification of PFH based on the level of tissue atrophy: type 1 -minimal soft tissue atrophy in a severe phase of the disease, usually between the age of 10-20; type 2 -moderate soft tissue atrophy with no influence on osseous and cartilaginous tissues; type 3 -moderately severe soft tissue atrophy with atrophy of the osseous and cartilaginous tissues, typical for patients who developed the disease before the age of 10; type 4 -severe soft tissue atrophy with atrophy of the osseous and cartilaginous tissues, and disorders of affected organs (nose, oral cavity, eye) [31].…”

Section: Progressive Facial Hemiatrophymentioning

confidence: 99%

“…bóle głowy, migrena, drgawki, porażenie nerwu trójdzielnego, parestezje, hemiplegia, anomalie naczyń śródczaszkowych) oraz w 15% przypadków objawy okulistyczne (m.in. enoftalmia, zez, atrofia powiek, zapalenie błony naczyniowej oka, jaskra, diplopia, zespół Hornera, mydriaza) [27][28][29][30]. Guerrerosantos i wsp.…”

Section: Postępujący Zanik Połowiczy Twarzyunclassified

Localized scleroderma (morphea). Diagnostic and therapeutic recommendations of the Polish Dermatological Society

Krasowska¹,

Rudnicka²,

Dańczak‐Pazdrowska³

et al. 2019

Localized scleroderma (morphea) is a chronic autoimmune disease of the connective tissue. Its etiopathogenesis is unknown. Most often the disease affects the skin, but may also involve the subcutis, muscles, and the osteoarticular system. The clinical presentation and the course of disease may be diverse. This article presents clinical characteristics of various types of the disease, the classification of its subtypes, recommendations for differential diagnosis and treatment. STReSzCzenie Twardzina ograniczona (morphea) jest przewlekłą chorobą zapalną tkanki łącznej o podłożu autoimmunologicznym i o niewyjaśnionej etiopatogenezie. Najczęściej zajęta jest skóra, ale proces chorobowy może zajmować tkankę podskórną, mięśnie i układ kostno-stawowy. Zróżnicowany obraz kliniczny choroby wymaga prawidłowego rozpoznania i zastosowania odpowiedniej terapii. W artykule przedstawiono charakterystykę kliniczną oraz zalecenia dotyczące klasyfikacji, postępowania diagnostycznego i leczenia.