1/20. Total decompression of facial nerve for melkersson-rosenthal syndrome.melkersson-rosenthal syndrome (MRS) is a rare condition characterized by recurrent facial paralysis in addition to various orofacial manifestations. The condition appears to be a granulomatous disorder causing oedema and inflammation of the soft tissues of the face, lips, oral cavity and particularly, the facial nerve. There is general agreement that the symptoms and signs resolve spontaneously, aided perhaps by an empirical course of oral steroids. However, in some patients the condition may be progressive, leading to disfiguring facial synkinesis and increasing residual paralysis. There is anecdotal evidence in the literature to suggest that surgical decompression of the facial nerve in its entire intratemporal course may prevent further attacks of facial paralysis and its sequelae. We present here our experience with surgical decompression of the facial nerve in a 27-year-old woman with MRS who had sufferent recurrent left-sided facial palsy since the age of four. A review of the literature pertaining to facial nerve decompression for melkersson-rosenthal syndrome is presented.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/20. cheilitis granulomatosa of melkersson-rosenthal syndrome: treatment with intralesional corticosteroid injections.BACKGROUND: melkersson-rosenthal syndrome may manifest as the classical triad (orofacial edema, facial nerve palsy and stable lingua plicata) but monosymptomatic manifestations or combinations of typical symptoms are not infrequent. The available therapeutic options provide only limited success or temporary benefit. CASE REPORT: A 20-year-old man presented with a 7-month history of recurrent episodes of swelling of the upper lip without pain, burning or local pruritus. No causative factors, such as food, drugs or latex, or physical, chemical or emotional conditions could be identified. The patient had been treated with oral antihistamines and corticosteroids with no clinical improvement. physical examination showed firm edema without fovea, limited to the central area of the upper lip without epidermal changes or symptoms on palpation. The patient had a previous history of facial palsy 6 years previously and recurrent episodes of herpes simplex labialis. skin prick tests with inhalant aeroallergens, food, latex and anisakis allergens were negative. Laboratory investigation revealed normal complete blood count, erythrocyte sedimentation rate, thyroid hormones, biochemistry, complement components (C3, C4 and C1-esterase inhibitor) and CH50, rheumatoid factor, antinuclear antibodies, immune complexes, protein electrophoresis and immunoglobulins. thorax and paranasal sinus radiographs were clear. biopsy of the involved area of the lip showed edema with lymphocytic and plasma cell infiltration and mononuclear perivascular infiltrates without granulomas, suggesting initial granulomatous cheilitis. Because the patient showed lack of response and/or poor tolerance to prior treatments (deflazacort, clofazimine and metronidazole), intralesional triamcinolone injections were administered with satisfactory response from the first session. CONCLUSIONS: Response to available treatments for melkersson-rosenthal syndrome is highly variable. In the present case, intralesional triamcinolone injections were effective.- - - - - - - - - - ranking = 0.125keywords = nerve (Clic here for more details about this article) |
3/20. Upper eyelid oedema in melkersson-rosenthal syndrome.melkersson-rosenthal syndrome is an uncommon disorder of uncertain aetiology characterized by orofacial oedema, facial nerve palsy and lingua plicata. The triad is seldom seen in its complete form, and oligo-symptomatic or mono-symptomatic forms are more common. An unusual case of melkersson-rosenthal syndrome involving the left upper eyelid is presented. The pathology, clinical features and management of this disorder are discussed.- - - - - - - - - - ranking = 0.125keywords = nerve (Clic here for more details about this article) |
4/20. Persistent unilateral orbital and eyelid oedema as a manifestation of melkersson-rosenthal syndrome.melkersson-rosenthal syndrome (MRS) is a complex neuromucocutaneous disorder characterized by localized orofacial oedema and cranial nerve dysfunction, frequently associated with minor signs, including furrowed tongue. Complete forms are rare whereas mono- and oligosymptomatic variants are more common. A 71-year-old man presented with a 2-year history of relapsing and progressively persistent oedema of the right eyelids and periorbital region. A fissured tongue and telangiectatic rosacea had been present since the age of 50 and 60 years, respectively. The patient was also affected by essential hypertension and diabetes mellitus. A skin biopsy showed a marked upper dermal oedema, and small epithelioid cell granulomas arranged in perivascular and perilymphatic location. collections of small epithelioid cells were occasionally observed within lymphatic spaces. No acid-fast bacteria, fungi or foreign bodies were detected. Intralesional corticosteroids induced transient improvement, whereas minocycline, clofazimine and dapsone have been ineffective. MRS may present with unilateral eyelid and periorbital swelling. Differential diagnoses of such cases may include a variety of cutaneous, ophthalmic and systemic diseases.- - - - - - - - - - ranking = 0.125keywords = nerve (Clic here for more details about this article) |
5/20. Bilateral upper eyelid edema in melkersson-rosenthal syndrome.melkersson-rosenthal syndrome is an uncommon disorder characterized by a triad of facial nerve palsy, orofacial edema, and fissured tongue. A 42-year-old woman with melkersson-rosenthal syndrome presented with painless, nonpitting, bilateral asymmetric upper eyelid edema. The left eyelid was a bit larger than the right eyelid. CT and MRI demonstrated periorbital soft tissue thickening compatible with the microscopic findings of infiltration of lymphocytes, edema, and cystic dilatation of lymphatic vessels. After treatment by systemic doxycycline and corticosteroid, she showed some improvement of the eyelid edema. Isolated bilateral eyelids swelling may be observed in melkersson-rosenthal syndrome. In the case of unexplained nonpitting eyelid edema, biopsy should be performed.- - - - - - - - - - ranking = 0.125keywords = nerve (Clic here for more details about this article) |
6/20. melkersson-rosenthal syndrome with diffuse facial swelling and multiple cranial nerve palsies.We report an unusual case of melkersson-rosenthal syndrome presenting as diffuse facial swelling with facial, glossopharyngeal and vagus nerve palsies. A skin biopsy revealed non-caseating epithelioid cell granulomas throughout the dermis. The disease responded poorly to systemic steroids, antihistamines, antibiotics and potassium iodide.- - - - - - - - - - ranking = 0.625keywords = nerve (Clic here for more details about this article) |
7/20. A monosymptomatic melkersson-rosenthal syndrome in an 8-year old boy.melkersson-rosenthal syndrome (MRS) is a systemic neuro-mucocutaneous granulomatous disease, characterized in its classical form by a triad of recurrent facial nerve paralysis, swelling of the lips and lingua plicata. However, this classical triad is rarely present, while the monosymptomatic or oligosymptomatic forms are more frequent. The presence of two or one of the manifestations mentioned above, with granulomatous cheilitis in the biopsy, is sufficient to make the diagnosis of monosymptomatic or oligosymptomatic form of MRS. This syndrome is very rare in childhood, instead, it is more frequent in young adults between the second and third decades of life. We present the case of an 8 years old boy who was brought to us because of a non painful swelling of the upper lip, associated with gingival hypertrophy, that had persisted for more than two months. Given the negative results of the hemato-chemical and instrumental assessments, we performed an upper lip biopsy whose histological study showed granulomatous cheilitis. We diagnosed this case as a monosymptomatic MRS and administered an intralesional steroid therapy using triamcinolone, with complete recovery.- - - - - - - - - - ranking = 0.125keywords = nerve (Clic here for more details about this article) |
8/20. Management of cheilitis granulomatosa.Peripheral facial nerve palsy, recurrent or persistent oral or facial swelling, and fissured tongue constitute a triad of symptoms known as Melkersson-Rosenthal syndrome. Granulomatous labial enlargement, known as cheilitis granulomatosa, is considered the single most important diagnostic feature of this syndrome. This lesion has been difficult to treat. This article describes a case of 8 months' duration of cheilitis granulomatosa of the lower lip, which was successfully managed with intralesional steroid injections.- - - - - - - - - - ranking = 0.125keywords = nerve (Clic here for more details about this article) |
9/20. Total facial nerve decompression for recurrent facial paralysis: an update.Recurrent facial paralysis (RFP) is a rare disorder that in some individuals may lead to worsening sequelae. melkersson-rosenthal syndrome is a variant of RFP that is associated with recurrent facial edema. In the past, decompression of the mastoid segment of the facial nerve has not been successful in preventing recurrences. In 1981 we began performing total facial nerve decompression for RFP and in 1986 reported its efficacy in one patient with melkersson-rosenthal syndrome and in another in whom both nerves were decompressed for alternating bilateral paralysis. An additional four cases with 3 to 8 years of followup demonstrate no recurrences in any patient. Total facial nerve decompression for RFP in selected patients appears efficacious in preventing recurrences. decompression will remain investigational until further followup is obtained. Furthermore, its salutary effect should not be extrapolated to Bell's palsy without further study.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/20. melkersson-rosenthal syndrome: elevations in serum angiotensin converting enzyme and results of treatment with methotrexate.We have presented the case of a 37-year-old white man who had recurrent, unilateral, facial edema, temporary partial facial nerve palsy, sensory loss along the ophthalmic and maxillary divisions of the fifth cranial nerve, ageusia bilaterally on the anterior two thirds of the tongue, right extraocular muscle palsies, and intractable migraine-type headaches, leading to the diagnosis of melkersson-rosenthal syndrome. The serum angiotensin converting enzyme levels, which are postulated to derive from undiscovered granulomas, were found to be elevated. methotrexate therapy mitigated the clinical course of the disease, and may prove to be useful in the treatment of this rare and recalcitrant condition.- - - - - - - - - - ranking = 0.25keywords = nerve (Clic here for more details about this article) |
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