Isolated facial diplegia in Guillain–Barré syndrome: Bifacial weakness with paresthesias

Abstract

Bifacial weakness with paresthesias (BFP) is a subtype of Guillain-Barré syndrome defined by rapidly progressive bilateral facial weakness in the absence of other cranial neuropathies, ataxia, or limb weakness. Many patients also complain of distal limb paresthesias and display diminished or absent deep tendon reflexes. BFP is a localized form of Guillain-Barré syndrome and is thought to be caused exclusively by demyelinating- rather than axonal-type neuropathy. Patients with BFP do not display anti-ganglioside IgG antibodies. Since it is rare, many physicians are unfamiliar with BFP, as bilateral facial weakness is more commonly associated with sarcoidosis, Lyme disease, or meningeal pathology. Many patients diagnosed with bilateral Bell palsy may instead have BFP. In this review, we highlight the clinical features of BFP and outline diagnostic criteria.

A case of bilateral facial nerve palsy in a patient with diabetes mellitus

“…Bilateral facial nerve palsy is rare and an association with diabetes mellitus is even rarer, compared to the other common causes like Guillain-Barre syndrome, sarcoidosis, Lyme disease and leprosy. , presence of nerve conduction evidence of facial nerve demyelination 5,6 , involvement of other cranial nerves 5 , prolonged recovery 5 and CSF showing albuminocytological dissociation 4 which were absent in our patient. This case highlights the fact that physicians should be aware of the various diagnostic possibilities of facial diplegia and should thoroughly investigate for all possible causes, some of which are life-threatening and potentially fatal.…”

Mitochondrial damage and “plugging” of transport selectively in myelinated, small-diameter axons are major early events in peripheral neuroinflammation

“…Although most patients survive, about half retain a residual neurological deficit, which can be severe [ 1 ]. Sensory symptoms have been under-emphasized [ 2 ], but recent studies reveal that these are clinically relevant [ 3 – 5 ] and prevalent, with paresthesia being reported by 75% [ 2 ] of patients and pain by 89% [ 6 ]. Interestingly, these symptoms often precede muscle weakness.…”

Bifacial weakness with paresthesias: Serial nerve conduction studies indicate diffuse demyelinating neuropathy

“…[1][2][3] Of these, the presynaptic form of CMS is rare, comprising only approximately 6% of cases. 4,5 Mutations of the choline acetyltransferase (CHAT) gene, located on chromosome 10q11.2, affect the function of the enzyme, choline acetyltransferase (ChAT), which catalyzes the synthesis of acetylcholine from acetyl coenzyme A and choline in neurons. [6][7][8][9] This leads to impaired resynthesis and depletion of acetylcholine in synaptic vesicles, and impaired neuromuscular transmission.…”

“…A localized form of GBS characterized by isolated facial diplegia in the absence of ophthalmoplegia, ataxia, or limb weakness has been described, but is rare . Typically, these patients also complain of distal paresthesias, and this subtype has been named bifacial weakness with paresthesias (BFP) . In keeping with other GBS subtypes, the majority of patients with BFP also complain of antecedent infection; have a monophasic disease course; and display evidence of cerebrospinal fluid (CSF) albuminocytological dissociation (raised protein with a normal cell count) and neurophysiological neuropathy.…”