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Published on: September 20, 2018
Melkersson-Rosenthal syndrome.
Mervi Kanerva1, Kirsi Moilanen, Susanna Virolainen
1Department of Otorhinolaryngology, Helsinki University Central Hospital, Helsinki, Finland. mervi.kanerva@fimnet.fi <mervi.kanerva@fimnet.fi>
Melkersson-Rosenthal syndrome (MRS) with facial palsy (FP) presents differently than edema-dominated MRS. Further research on MRS with FP is needed to improve understanding of this rare neurological disorder.
Area of Science:
- Neurology
- Dermatology
- Otorhinolaryngology
Background:
- Melkersson-Rosenthal syndrome (MRS) is a rare neurological disorder characterized by facial palsy, swelling, and a fissured tongue.
- The clinical presentation of MRS can vary, with some cases dominated by edema and others by facial nerve involvement.
Purpose of the Study:
- To investigate the characteristics of Melkersson-Rosenthal syndrome (MRS) patients presenting with facial palsy (FP).
- To identify differences in MRS patients treated at Otorhinolaryngology and Dermatology departments.
Main Methods:
- Retrospective chart review of MRS patients from two hospital departments.
- Patient questionnaires and examinations for those with facial palsy.
- Histopathological analysis of tissue biopsies and genetic testing for UNC-93B1 mutations.
Main Results:
- All 18 MRS patients in the Otorhinolaryngology department had facial palsy; 9 presented with the triad form.
- Non-necrotizing granulomatous infiltrations were observed in two patients during acute edema episodes.
- Dermatology patients included 2 with triad MRS and 15 with monosymptomatic granulomatous cheilitis, featuring persistent edema and typical MRS histology.
- No UNC-93B1 mutations were detected in any patients.
Conclusions:
- The clinical presentation of MRS with facial palsy differs from the commonly described edema-predominant form.
- Further research into MRS with facial palsy is recommended to enhance understanding of the syndrome's diverse manifestations.
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