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A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy

We dont know what exactly causes Bells palsy. We report two adolescent girls with facial nerve palsy and serological evidence ofR conorii infection. Well, hope what I found below will be able to shed some light on the confusion. Here, we report the first case of unilateral facial nerve paralysis developed after 4 wks of intermittent cervical traction therapy. This results in paralysis of the affected part of the face, which can affect movement of the eye(s) and/or the mouth, as well as other areas.But sudden weakness that occurs on one side of your face should be checked by a doctor right away to rule out these more serious causes. There appear to be different pathways for voluntary and emotional movement. Wegener’s granulomatosis (WG) is an idiopathic systemic form of vasculitis characterized by the presence of necrotizing granulomas and vasculitis in the upper airways, lower airways, and kidneys.

Bells palsy is a diagnosis of exclusion; we must first rule out more serious etiologies, such as an upper motor neuron lesion from a stroke, tumor or intracranial hemorrhage. Chest auscultation, examination of the remaining cranial nerves, and tympanoscopy were normal. Tumors: acoustic neuroma, schwannoma, cholesteatoma, parotid tumors, glomus tumors. A 50-year-old nondiabetic, nonhypertensive male patient was referred from a primary health centre to our institute with a provisional diagnosis of bell’s palsy of left side. Difficulty in chewing food, food may get stuck within the mouth on the affected side. Incidence 20 per 100,000 between ages 10-40 years but 59 per 100,000 over age 65 years. Patient’s past history was not significant except for 2-3 episodes of upper respiratory tract infection with persistent rhinorrhea for 2 months in the last one year.

It involves the entire side of the faceforehead, eyelids, facial muscles of expression and mouthan important sign when differentiating Bells palsy from a true stroke. IgM and IgG antibodies against R conorii at titres of 1/960 and 1/3840 respectively were detected by indirect immunofluorescence test. The incidence is 15-40 new cases per 100,000 people per year in the US. Otoscopic examination did not show any abnormality and no hearing loss was detected on audiogram. There is no need to panic, as it is a benign condition, and often improves. The rest of the routine hematological and biochemical investigations including blood sugar and thyroid profile were normal. Blink reflex study was performed which showed absent ipsilateral R1 and R2 responses on left side stimulation while normally presenting contralateral R2 response.

Initiation of antiviral agents such as Zovirax (acyclovir, Glaxo SmithKline) at the first signs of disease probably would not cure Bells palsy. Facial palsy subsided three months later. There is a preceding viral illness in 60% of patients. Viral markers for HIV, Hepatitis B, and Hepatitis C were nonreactive. Neuro doctor will diagnose Facial palsy by asking you questions, such as about how your symptoms developed. Routine microscopy of urine revealed 3+ proteinuria and red blood cells (RBCs) casts. Accordingly 24-hour urinary protein was sent which came out to be 2.2 g/day.

It can progress, but reaches maximal involvement in two to three weeks.8,9 Men and women are affected equally, and even though age may not be a factor, most cases occur in individuals ages 20-40.9 Bells palsy affects the left and right sides of the face with equal frequency.10 There is a higher incidence of the condition during pregnancy, especially with concurrent preeclampsia.11 To date, the literature does not indicate a strong familial predilection to Bells. The acoustic reflexes, measured by ipsilateral and contralateral stimulation, were absent in the right and normal in the left ear. The virus reproduces and travels along the nerve. However, c-ANCA was detected positive against proteinase-3 (titre 1 : 640). Rare genetic syndromes such as Moebius syndrome or CHARGE syndrome. Based on these investigations, patient was diagnosed as a case of Wegener’s granulomatosis. Treatment was started (1 week after presentation) according to EUVAS (European Vasculitis Study Group) protocol for the management of primary small and medium vessel vasculitis.

Bilateral facial palsy with a history of a tick bite indicates Lyme disease, in which case oral antibiotics are indicated. A repeat serology for R conorii two weeks later revealed IgM antibody titre of 1/1600 and IgG antibody titre of 1/3840, thus confirming a recentR conorii infection. Patient’s clinical features were closely followed and a urine analysis performed 15 weeks after starting immunosuppressive therapy revealed only 1+ proteinuria (260 mg/24 hours) without any RBCs. By this time patient improved significantly from his systemic involvement with normalization of renal function parameters along with complete recovery from facial nerve paralysis. Wegener’s granulomatosis or granulomatosis with polyangiitis is an uncommon, systemic disease that mainly affects the head and neck region, the lungs, and the kidneys. Besides, a significant proportion of patients present with peripheral nervous system (PNS) involvement in the form of mononeuritis multiplex due to vasculitis or with central nervous system (CNS) involvement due to infiltrating granulomatous lesions (10–45%) [3]. The common presenting manifestations of the disease are recurrent sinusitis, nasal ulceration with discharge, and symptoms of lower respiratory tract infection.

Any evidence of nystagmus, eye muscle imbalance with the subjective complaint of diplopia, or limb paralysis or weakness suggests an abnormality of the brain stem or the subarachnoid space, where the cranial nerves are close together.4 Evaluate the involved eye for an adequate Bells phenomenon: the upward rolling of the globe during forced eyelid closure. Tick bite signs were not present in either patient. In absence of any systemic symptoms or signs, initially we thought about common possibilities of LMN facial nerve palsy including bell’s palsy, herpes infection, Guillain-Barre syndrome, and pontine small vessel stroke. MRI brain, turning out to be normal, ruled out the structural brain stem lesion. However, patient was evaluated further in view of peripheral edema and was established to have evidence of kidney disease on urine examination, further confirmed by renal biopsy. Positive serum c-ANCA suggested the diagnosis of Wegener’s granulomatosis, featuring with pauci-immune crescentic glomerulonephritis. Neurological involvement is commonly seen in Wegener’s granulomatosis.

The only laboratory tests needed for the diagnosis are complete blood count (CBC), erythrocyte sedimentation rate (ESR), urinalysis and plasma glucose analysis. Titres above 1/150 for IgM and above 1/400 for IgG specific antibodies are suggestive of acute infection. Notwithstanding the common neurological involvement, cranial neuropathy as the first manifestation of the disease preceding other organs involvement is uncommon. Unilateral facial nerve palsy in our case was a probable manifestation of vasculitis leading to ischemic cranial neuropathy. Alternatively, the cranial neuritis could be a result of immune-mediated inflammatory process. Cadoni et al. [5] substantiated the pathological role of antiendothelial cell antibodies (AECAs) in WG and hypothesized them to be useful for the early diagnosis of WG.

Also, advise patients about the risks of corneal exposure, and instruct them to instill non-preserved artificial tears hourly while awake and ophthalmic lubricating ointments at bedtime. Its diagnostic role in WG appears to be promising though and, in our opinion, further research work is needed to support the same. Isolated facial cranial neuritis as the first presentation of the disease is rarely reported. Although, there have been a few reports of bilateral facial nerve palsy. Nikolaou et al. reported a case of a young female who presented with bilateral serous otitis media followed by bilateral sensorineural hearing loss and bilateral facial nerve palsy [6]. Wegener’s disease presenting as bilateral facial nerve palsy has also been reported by Ferri et al.

Herpes simplex virus in idiopathic facial paralysis (Bells palsy). Wegener’s granulomatosis is an autoimmune disease and can be lethal if left untreated. Long term remission can be achieved in up to 90% of the cases with the help of therapeutic agents like steroids and other immunosuppressant drugs such as cyclophosphamide, azathioprine, and methotrexate. The presentation of Wegener’s granulomatosis can be varied and the patients may initially present as an isolated cranial neuritis. Physicians should be aware of the atypical and varied presentations of this disorder, which may thus help in early diagnosis and initiation of treatment. Lower motor neuron type of facial paralysis is not always synonymous with bell’s palsy and secondary causes should always be ruled out by a thorough clinical examination and a comprehensive investigation panel. Wegener’s granulomatosis can present with isolated cranial neuritis as the first manifestation of the disease.

Townsend JJ, Collins PK.