Abstract We report a case with symptoms of facial swelling, bilateral facial paralysis, dysphagia and aspiration. On electrophysiological studies, the right facial nerve was not excitable. The left facial nerve compound muscle action potential (CMAP) amplitude was severely dispersed and latency was mildly prolonged, consistent with demyelina- tion. Cerebrospinal fluid examinations were normal. Anti- ganglioside antibodies and tumor markers were negative. Bickerstaff brainstem encephalitis, stroke, diabetes melli- tus, vasculitis, sarcoidosis, Sjögren’s syndrome, Melkers- son-Rosenthal Syndrome, trauma, infectious diseases, toxicity, neoplasm, facial onset sensory and motor neuronopathy (FOSMN) and other degenerative diseases were excluded. Intravenous immunoglobulin therapy resolved symptoms of lower cranial nerve dysfunction. Clinically incomplete improvement of bilateral facial paralysis was observed. We conclude that IVIg therapy may improve the symptoms of multiple cranial nerve palsies due to pharyngo-facial variant of Guillain-Barré syndrome. Key words: Bilateral facial diplegia; Guillain-Barré syndrome; dysphagia; neuropathy; intravenous immuno - globulin therapy. Introduction Facial diplegia is characterized by loss of volun- tary movements of bilateral facial muscles. Although unilateral facial or lower cranial nerve palsies may frequently be seen, multiple cranial neuropathies, in- volving bilateral facial and lower cranial nerves are rarely reported (Shuaib and Becker, 1987; Spillane et al., 1991). Clinical localization may be due to dis- eases affecting the supranuclear structures, subarach- noid space, skull base, nuclear lesions, cranial nerves, the neuromuscular junction or the muscle. Usually post-infectious autoimmune diseases such as Guillain-Barré Syndrome, diabetes mellitus, stroke, vasculitis, neoplasm, trauma, infectious dis- eases, toxicity, congenital or degenerative diseases may be the cause (Weintraub, 1976; Gorman et al., 1977; Beal, 1990; Keane, 1994). Case report An 82 years old housewife noticed swelling and weakness on her right eyelid and the right side of her face. Within 3 days she noticed the same symptoms on the left hemiface and started to have difficulty swallowing with occasional aspiration. Her medical history disclosed hypothyroidism. She denied any infection or taking any new medication within the last month. On examination she had facial swelling, facial drooping, bilateral ptosis and tongue was fis- sured. The patient was alert and oriented. Bilateral facial paresis prominent on the right and weakness of bilateral cranial nerves IX and X, with decreased gag reflex and dysarthria was noted. There was no sensory or motor deficit in her extremities. Deep ten- don reflexes were normal. There was no pathological reflex. Brain MRI revealed multiple, non-specific, ischemic-gliotic lesions in the periventricular white matter. On electrophysiological studies, sensory potentials were absent in both sural nerves. Sensory nerve conduction velocities were slow in median and ulnar nerves (Table 1a). Distal motor latencies were prolonged and motor nerve conduction velocities were slow. The compound muscle action potential (CMAP) amplitude was low in the right peroneal nerve. The right facial nerve was inexcitable. CMAP amplitude was low in the left facial nerve (Table 1b). The left facial nerve CMAP amplitude was severely dispersed and latency was mildly prolonged (Fig. 1). There was total denervation of the right facial mus- cles and partial denervation of the left facial muscles. Acta Neurol. Belg., 2009, 109, 317-321 IVIG- Responsive Multiple Cranial Neuropathy: a pharyngo-facial variant of Guillain-Barré Syndrome Isin UNAL-CEVIK 1 , Mehmet Zulkuf ONAL 1 , Zeki ODABASI 2 and Ersin T AN 3 1 Ufuk University, Faculty of Medicine, Department of Neurology, Ankara; 2 Gulhane Military Medical Academy, Department of Neurology, Ankara and 3 Hacettepe University, Faculty of Medicine, Department of Neurology, Ankara, Turkey ————