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Facial diplegia with paraesthesias: facial nerve enhancement in three dimensional MRI
  1. AKINORI HOZUMI,
  2. NOBUHIRO YUKI,
  3. KAORU YAMAZAKI,
  4. KOICHI HIRATA
  1. Department of Neurology, Dokkyo University School of Medicine, Tochigi, Japan
  1. Dr Akinori Hozumi, Department of Neurology, Dokkyo University School of Medicine, Kitakobayashi 880, Mibu, Shimotsuga, Tochigi 321–0293, Japan. Telephone +81 282 86 1111 ext 2720; fax +81 282 86 5884; email ahozumi{at}ibm.net

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Facial diplegia (bilateral facial paralysis) is a rare clinical finding that can be the presenting symptom in a wide range of diseases.1 It occurs in about 50% of patients with Guillain-Barré syndrome (GBS). Guillain-Barré syndrome causes regional and functional variants with unusual features. Ropper described four patients with facial diplegia and distal limb paraesthesias, and he defined them as having a rare variant form of GBS because of shared clinical, electrophysiological, and CSF features.2 The aetiology and nosological position of facial diplegia presenting in this variant form is still controversial. We experienced a patient who had bilateral facial paralysis, distal limb paraesthesias, and diminished reflexes whose contrast enhanced three dimensional MRI (3-D MRI) showed enhancing lesions in the bilateral facial nerves.

A 27 year old woman had nasal discharge and coughing. One week later she noticed paraesthesias in her fingers and toes. Nine days after the onset of her neurological symptom, she developed bilateral facial weakness. On admission (day 12) she showed moderate, bilateral facial paralysis that caused her difficulty in moving her forehead, in approximating her eyelids, and in lifting the corners of her mouth. Tearing and taste were preserved. The other cranial nerves were normal. A motor examination showed normal strength in her limbs. Superficial and deep senses were normal even though she had distal limb paraesthesias. Deep tendon reflexes were absent in all her limbs and her plantar responses were flexor type. Cerebellar ataxia and autonomic nervous dysfunction were excluded. Chest radiography was normal. Laboratory studies of the identifiable causes of facial diplegia (sarcoidosis, Lyme disease, syphilis, infectious mononucleosis, herpes simplex virus, diabetes mellitus, and connective tissue disease) were all negative. On day 12, the CSF examination detected mild increases in protein concentration (57 mg/dl) without pleocytosis. The blink reflex was elicited and both the R1 and R2 components were reduced, but their latencies were not prolonged on day 15. Motor and sensory nerve conduction velocities, and median and tibial nerve fresponses were all normal on day 18. Auditory brainstem responses were normal. On Day 19, when her facial diplegia was moderate, conventional brain MRI detected no abnormality. A contrast enhanced 3-D MRI, which was obtained by spoiled gradient recalled acquisition in the steady state sequence using a 1.5 tesla system after injection of gadolinium-diethylenetriamine penta-acetic acid (Gd-DTPA), was performed. The intracanalicular segments of the bilateral facial nerves were remarkably enhanced by Gd-DTPA (figure). On Day 45, when her symptoms were no longer present, there were no abnormal enhancements of her facial nerves.

(A) Before and (B) after contrast enhanced transverse reformatted three dimensional MRI. The lateral intracanalicular segments of both facial nerves are enhanced (arrows) in B.

After showing symptoms of upper respiratory infection, the patient experienced the acute onset of facial diplegia, distal limb paraesthesias, and areflexia but no other neurological deficits. The CSF examination showed albuminocytological dissociation, and clinical and laboratory examinations excluded the possibility of viral or bacterial infection, Lyme disease, tumour, sarcoidosis, cerebrovascular disease, diabetes mellitus, bilateral Bell’s palsy, and congenital and familial disorders. The patient’s illness followed a monophasic course. We therefore diagnosed this case as having “facial diplegia with paraesthesias”, which should be included for the differential diagnosis whenever sudden bilateral facial paresis occurs. Routine brain MRI showed no abnormalities, whereas contrast enhanced 3-D MRI showed Gd enhancement of the bilateral facial nerves. The MRI findings indicate the involvement of the peripheral facial nerves in our patient. Fulbright et al 3reported an additional case of GBS with multiple cranial nerve enhancements seen on Gd enhanced MRI. The mechanism of abnormal enhancement of the cranial nerves in the patients with GBS is not entirely understood; however, it is widely regarded as disruption of the blood nerve barrier by the inflammatory infiltrate. Ramseyet al 4 evaluated the MRI findings obtained with Gd contrast enhancement in five patients who had acute bilateral facial paralysis: GBS (n=1), herpes simplex polyneuritis (n=1), meningeal lymphoma (n=1), and bilateral Bell’s palsy (n=2). Gd enhanced MRI has been shown to be the procedure of choice for demonstrating inflammatory lesions of the facial nerves. Nagaoka et al 5 showed oculomotor nerve enhancement on 3-D MRI in Fisher’s syndrome, the best known variant of GBS. Ours is the first report of facial nerve enhancement in “facial diplegia with paraesthesias”. These findings suggest that 3-D MRI with Gd-DTPA can be used to identify inflammatory conditions that produce peripheral lesion of the cranial nerves in GBS variants.

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