Zespół Melkerssona Rosenthala jako rzadka przyczyna nawracającego porażenia nerwu twarzowego (2)

Correspondence to: Grzegorz Opala, MD, PhD, Department of Neurology, Aging, Degenerative and Cerebrovascular Diseases, Silesian Medical Academy, Central University Hospital, ul. Medyków 14, 40-752 Katowice, Poland, tel./fax +48 32 204 61 64, e-mail: neurowp@infomed.slam.katowice.pl
Pracę otrzymano: 19.03.2004; przyjęto do druku: 6.06.2005

Peripheral facial nerve palsy is a common reason for neurological consultation. The palsy is recurrent in about 3% of patients [1], and careful diagnosis is necessary in such patients. One of the rare causes of recurrent facial nerve palsy is Melkersson-Rosenthal syndrome.

The etiology of Melkersson-Rosenthal syndrome is unknown. In 1928, E. Melkersson described a patient with facial edema and facial nerve paralysis and suggested that these two findings were related. Three years later, C. Rosenthal added one more sign, fissured tongue (lingua plicata) [2]. Since then, recurrent orofacial edema, recurrent peripheral facial palsy, and fissured tongue have been considered the classic triad of Melkersson-Rosenthal syndrome. However, the triad is not always completely present [3,4], and many case reports of Melkersson-Rosenthal syndrome describe a monosymptomatic variant.
The most important and consistent sign of Melkersson-Rosenthal syndrome is orofacial edema, which occurs in 80-100% of patients and usually affects the lips [3]. The swelling may also involve the chin, cheeks, or periorbital tissues [4]. In 1945, Miescher reported that the orofacial edema was caused by cheilitis granulomatosa [5-7]. Peripheral facial paralysis occurs in one third of cases of Melkersson-Rosenthal syndrome and is indistinguishable from that of Bell's palsy. The paralysis has a tendency to become recurrent, usually affecting alternating sides of the face [4-6,8]. A fissured tongue is found only in some patients and is thought to be a genetic trait with dominant inheritance [4,9].

In this report, we describe the case of a patient with the classic triad of the Melkersson-Rosenthal syndrome.
Case report
A 24-year-old woman presented to the neurology clinic at our institution with right-sided peripheral facial nerve palsy that had occurred spontaneously the day before. Neurologic examination showed a painless, nonpruritic, firm facial edema on the right side.
A fissured tongue was also observed, with furrows radiating from the central raphe, deviation of the tongue to the left side, and weakened deep reflexes. The patient had experienced 5 episodes of peripheral facial nerve palsy within the past 15 years, the first occurring at the age of 8 years. Each of these episodes had involved the left side of the face. Now, for the first time, the symptoms were occurring on the right side, prompting a detailed diagnostic workup.

The results of basic laboratory tests, including blood morphology, sedimentation rate, glucose, urinalysis, and liver and kidney function tests, were normal. Results of electrocardiography were also normal. Serum electrophoresis, gamma-globulins (IgG, IgA, and IgG), and COMBI test (Indirect Immunofluorescence assay for detection
of IgG/IgM/IgA-Antibodies in human serum) were negative. Magnetic resonance imaging (MRI) of the head was normal. However, magnetic resonance angiography (MRA) of the cerebral vessels suggested compression of the right seventh and eighth cranial nerves by a vascular loop. The classic clinical symptoms of nerve compression were not apparent. No operative procedure was carried out.

To further substantiate this diagnosis, a biopsy
of upper lip was performed later in the course of the illness. Changes characteristic of cheilitis granulomatosa were not seen. However, because the biopsy was obtained after the patient had already been treated, it is possible that the histological findings were influenced by the treatment.

We administered a combination therapy consisting of dexamethasone, ketoprofen, galantamine, doxycycline, and vitamin B complex. Since the facial edema had partially subsided by the fourth day, physiotherapy and kinesitherapy of the facial muscles were initiated. From the sixth day, slow improvement of the facial weakness was observed. By 4 months, the symptoms had disappeared completely. There was no recurrence during one and a half years of observation.
Discussion
Peripheral facial nerve paralysis is a common disorder arising from various causes. In addition to Melkersson-Rosenthal syndrome and idiopathic Bell's palsy, conditions that may be associated with peripheral facial nerve paralysis include diabetes mellitus, connective tissue diseases, sarcoidosis, otitis media, otic herpes zoster, migraine, vascular pathology in the cerebellopontine angle or tumors of this region, or complications of trauma. Peripheral facial nerve paralysis also may occur in borreliosis, HIV infection, and Ramsay-Hunt syndrome.

Melkersson-Rosenthal syndrome may present at any age. However, it frequently occurs in young adults 20 to 30 years old [3] and rarely occurs in children [1,4,8]. Generally, there is no difference in prevalence between the sexes, but some authors suggest that women are more predisposed [3,9]. Although the cause still remains unclear, genetic factors, vasomotor disturbances, infectious agents, and allergies have all been implicated [9,10].

In the acute phase of Melkersson-Rosenthal syndrome, lip biopsy can demonstrate an inflammation process involving a noncaseating sarcoid granuloma consisting of aggregates of histiocytes, lymphocytes, and plasma cells [4,9,10]. According to some authors, such findings are mandatory for diagnosis of the syndrome, whereas others think they are not required [2,9]. Orofacial edema may occur as the only symptom of the monosymptomatic variant of Melkersson-Rosenthal syndrome [3,9,11]. However, some authors regard this condition as a different disease named cheilitis granulomatosa Miescher.

Besides the monosymptomatic and classic forms
of Melkersson-Rosenthal syndrome, oligosymptomatic forms have been reported in which one or more of the classic symptoms coexist with keratitis, paralysis of the medial rectus muscle, retrobulbar neuritis, hyperacusis, migraine-like headache, blepharospasm, trigeminal neuralgia, or dysesthesias of the face [3,9,12]. These combinations of signs occurring in different constellations make the final diagnosis difficult. Before the diagnosis of Melkersson-Rosenthal syndrome
is finally made, patients may have consulted many different specialists-laryngologists, neurologists, and dermatologists. Because of the unknown etiology of the syndrome, differential diagnosis is very important, mainly to exclude other diseases.

The prerequisite for ensuring the accurate diagnosis is a careful anamnesis, especially regarding the recurrent character of the symptoms and the tendency toward fixation in time. Melkersson-Rosenthal syndrome usually appears unilaterally, but it may affect the right and left sides alternately. With each episode, the peripheral facial nerve palsy may last longer and withdraw more slowly and not always completely.

Because the etiology is unknown, treatment remains symptomatic. Corticosteroids (methylprednisolone [9] or prednisone [4,6]) are most effective in reducing the facial edema. They may be taken orally, parenterally, or topically. There are cases of successful treatment with nonsteroidal anti-inflammatory agents (clofazimine [7], dapsone [4,12], danazol [4,12]) and broad-spectrum antibiotics (tetracycline [3,4], metronidazole [11]). Surgical procedures to decompress the facial nerve in the facial nerve canal have also been used [4,5]. Such procedures have been successful in treating disfiguring swelling [4]. One should not forget about physiotherapy and kinesitherapy to improve facial muscle function.

Our patient presented with the classic triad
of Melkersson-Rosenthal syndrome. Her predominant symptom had been recurrent left facial nerve paralysis. The occurrence of the facial nerve paralysis on the right side for the first time suggested the possibility that this episode was produced by a cause other than Melkersson-Rosenthal syndrome. MRA suggested neurovascular conflict in the cerebellopontine angle ipsilateral to the present facial nerve palsy.

Neurovascular conflict in the cerebellopontine angle may be simply an anatomical variant or
a pathologic condition. It may be subclinical, or, under certain conditions, it may produce clinical symptoms of cranial nerve dysfunction. These usually consist
of trigeminal neuropathy or facial hemispasm or, rarely, paroxysmal vertigo [13]. The diagnosis
of neurovascular conflict in the cerebellopontine angle is generally made on the basis of these classic clinical symptoms, but they were absent in our patient.

In the literature there are not many case reports
of Melkersson-Rosenthal syndrome, so it seems that the presentation of the classic triad of this syndrome
is interesting.
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