Complete Melkersson-Rosenthal syndrome with multiple cranial nerve palsies

Zeng, W.; Geng, Shizhong; Niu, Xiaoting; Yuan, Jingyi

doi:10.1111/j.1365-2230.2009.03418.x

Cited by 16 publications

(15 citation statements)

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“…Severity of palsy also may vary from mild to severe, which could be unilateral or bilateral. Involvement of other cranial nerves also has been noted such as olfactory auditory; glossopharyngeal and hypoglossal nerves [8]. Numbness can involve other body parts also.…”

Section: Discussionmentioning

confidence: 99%

Melkerrson-Rosenthal Syndrome, a rare case report of chronic eyelid swelling

2013

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Melkerrson-Rosenthal syndrome is a rare disorder of unknown etiology. The classical triad of recurrent facial paralysis, swelling of the face, lips and deep furrowed tongue (Lingua Plicata) is seen in very few cases, majority of the patients often present with one or two symptoms only, which often leads to misdiagnosis and mismanagement. Clinically these symptoms vary from days to years, which further delay the definitive diagnosis and symptoms may eventually become permanent. The cause of this entity is not very well understood, but thought to be attributable to multiple entities including genetic and Infectitious. As this entity has been associated with numerous other clinical entities, diagnosis often remains an exclusion process. Methods: A middle age male with a chronic history of heavy eyelids with skin indurations predominately around left eye was presented to ophthalmology clinic. Physical examination revealed a deep furrowed tongue. The skin biopsy from left eyelid revealed a non-specific granulomatous lesion. The clinical correlation of facial swelling and deep plicated tongue prompted the differential of MRS Results: Histologically a non-specific granulomatous lesion was seen in dermis. As a rule, other causes of granulomatous diseases were ruled out especially Crohn’s disease and sarcoidosis. Polarization failed to reveal any foreign body. Conclusions: The finding of granulomatous lesion and clinical picture led to the definite diagnosis of Melkerrson-Rosenthal syndrome. Association with rosacea was other findings. Clinically his sign and symptoms are under control and no occurrence of symptoms has been noted so far.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1647494495993706

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Section: Discussionmentioning

confidence: 99%

Melkerrson-Rosenthal Syndrome, a rare case report of chronic eyelid swelling

2013

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“…Ancak MRS'nun temel üç bulgusu her zaman birlikte bulunmayabilmekte; her bir bulgu farklı zamanlarda tek tek de ortaya çıkabilmektedir. Klasik triadın tekrarlayan PFP ve orofasiyal ödem gibi major bulgularından en az birinin olması halinde tanı için yüzdeki ödemden cilt biyopsisi yapılarak histopatolojik olarak granülamatöz keilitisin gösterilmesi gerekmektedir (12).…”

Section: Melkerssonunclassified

“…Literatürde, tedavide kortikosteroidlerin kullanılmasıyla ilgili olarak farklı sonuçlar bulunmaktadır. Kortikosteroidlerin ödemi ve doku zedelenmesini önlediğini, bu hastalarda kullanımının yarar sağladığı yönünde yayınlar olduğu gibi tedavinin kısmen yararlı veya hiç yararlı olmadığına dair yayınlar da mevcuttur (4,7,8,12,13). Kortikosteroidler tedavide topikal, lezyon içi veya sistemik yolla kulanılabilmektedir (13).…”

Section: Melkerssonunclassified

“…Ayrıca klofazimin, minoksilin, metotreksat, danazol, dapson, sülfasalazin, hidroklorokin, difenhidramin, penisilin, tetrasiklin, eritromisin ve klindamisin gibi ilaçlar da steroidlere alternatif veya steroidlerle kombine olarak denenmiştir (8). Medikal tedaviye yanıt vermeyen olgularda nadiren de olsa fasiyal sinir dekompresyonu ve keiloplasti gibi cerrahi yöntemlerde uygulanabilir (3,12).…”

Section: Melkerssonunclassified

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Melkersson Rosenthal Syndrome: Report of Two Cases

Bayındır¹,

Kablan²,

Çiçek³

et al. 2012

J Turgut Ozal Med Cent

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ÖzetMelkersson Rosenthal Sendromu, tekrarlayan fasiyal paralizi, ağrısız ve gode bırakmayan orofasiyal ödem, fissürlü dil triadı ile karakterize, etiyolojisi net olarak bilinmeyen ve nadir görülen bir hastalıktır. Klasik triadın görülmesi nadirdir, genellikle tek ya da iki semptom birlikte görülür. Bu sunumda, öykü ve klinik özellikleri ile Melkersson Rosenthal Sendromu tanısı konan iki olgu tartışılacaktır. Otuzbir yaşında bir erkek hastada ve 16 yaşında bir kız hastada öykü ve klinik özellikleri ile Melkersson Rosenthal Sendromu'nun tanısı konulmuştur. Hastaların her ikisi de 1 mg/kg/gün oral prednizolon ile tedavi edilmiştir. Tekrarlayan fasiyal paralizi olgularında Melkersson Rosenthal Sendromu düşünülmelidir. Anahtar Kelimeler: Tekrarlayan Fasiyal Paralizi; Orofasiyal Ödem; Melkersson Rosenthal Sendromu. Melkersson Rosenthal Syndrome: Report of Two Cases AbstractMelkersson-Rosenthal Syndrome is a rare disease of unknown etiology, being characterized by the triad of recurrent facial nerve palsy, gode-free orofacial edema and fissured tongue. Classical triad of this syndrome is rarely present, especially single or two symptoms can be seen. In this report, two cases who diagnosed as Melkersson Rosenthal Syndrome according to the history and clinical features will be discussed. Melkersson Rosenthal Syndrome was diagnosed in two patients; thirty-one year old male and 16 year old girl patient, according to the history and clinical features. Both of them were treated with 1 mg/kg/day oral prednisolone. We will discuss two cases, which were diagnosed as Melkersson-Rosenthal Syndrome with history and clinical features.

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“…Involvement of other cranial nerves also has been noted such as olfactory, auditory, glossopharyngeal and hypoglossal nerves. [ 5 ] Numbness can involve other body parts also. Our patient had evidence of sensory-neural deafness in association with facial palsy which recovered on treatment with steroids.…”

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confidence: 99%