Cranial nerve involvement in typical and atypical chronic inflammatory demyelinating polyneuropathies

Abstract

Background and purpose Cranial nerve palsy is occasionally present in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), but its prevalence, characteristics and relations with the CIDP subtypes have rarely been investigated. The aim of this study was to systematically assess cranial nerve involvement in typical and atypical CIDP. Methods Clinical data were reviewed in 132 consecutive patients with CIDP, including typical CIDP (n = 89), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) (n = 31), distal acquired demyelinating symmetric (DADS) (n = 9) and others (n = 3). Results The frequency of cranial nerve palsy was 11% in typical CIDP, 48% in MADSAM and 11% in DADS. Facial and bulbar palsy was most frequently present (9%), followed by ocular motor nerve palsy (5%). Bilateral involvement was seen in all typical CIDP and DADS patients, whereas 80% of MADSAM patients had unilateral palsy. The presence of cranial nerve involvement was associated with more severe limb muscle weakness in typical CIDP, but not in MADSAM. Cranial nerve palsy fully recovered in 90% of typical CIDP and in 67% of MADSAM patients. Conclusion Amongst the CIDP subtypes, cranial palsy is frequent and unilateral in MADSAM, and less frequent and bilateral in typical CIDP and DADS. In typical CIDP, facial and bulbar palsy reflects more severe and extensive inflammation.

Chronic inflammatory demyelinating polyneuropathy with hypoglossal nerve involvement and inverted Beevor’s sign: case report

“…One notable feature in the present case was cranial nerve involvement, which included the facial and hypoglossal nerves. Cranial neuropathy is clinically uncommon in patients with CIDP, although it has been described in several case series or reports, including in cranial nerves II to X, and XII [ 2 , 3 , 8 – 11 ] (Table 2 ). A review of the literature revealed a reported incidence of cranial nerve involvement in 5% to 20% of patients with CIDP.…”

“…A review of the literature revealed a reported incidence of cranial nerve involvement in 5% to 20% of patients with CIDP. According to a retrospective study, cranial palsy is frequent in MADSAM (48%), but less frequent in typical CIDP (11%) and distal acquired demyelinating symmetric (11%) [ 3 ]. Facial paralysis is the most common involved, followed by bulbar involvement and oculomotor nerve paralysis [ 3 , 8 – 11 ].…”

“…According to a retrospective study, cranial palsy is frequent in MADSAM (48%), but less frequent in typical CIDP (11%) and distal acquired demyelinating symmetric (11%) [ 3 ]. Facial paralysis is the most common involved, followed by bulbar involvement and oculomotor nerve paralysis [ 3 , 8 – 11 ]. Typical CIDP patients usually have bilateral cranial nerve involvement, while MADSAM patients frequently have unilateral paralysis [ 3 ].…”

“…Facial paralysis is the most common involved, followed by bulbar involvement and oculomotor nerve paralysis [ 3 , 8 – 11 ]. Typical CIDP patients usually have bilateral cranial nerve involvement, while MADSAM patients frequently have unilateral paralysis [ 3 ]. However, reports of hypoglossal neuropathy in CIDP are especially rare [ 2 , 12 ].…”

“…Typical CIDP is a symmetric sensorimotor polyneuropathy that causes both proximal and distal limb weakness, sensory impairment, and areflexia. Cranial nerve involvement is not commonly encountered; this is especially true for the involvement of the hypoglossal nerve [ 2 , 3 ]. Beevor's sign is an upward umbilicus deviation when a supine patient tries to sit up, and indicates predominant weakness of the lower rectus abdominis muscle.…”