CASE OF THE MONTH ABSTRACT: A 49-year-old woman developed acute left facial, hypoglos-sal, and phrenic nerve palsies, as well as dysphagia and weakness in theneck and arms. Electrophysiologic studies showed an acute motor axonalneuropathy. Serum anti-GM1 IgG antibody was positive. Intavenous immu-noglobulin treatment resulted in good clinical recovery. The present reportindicates that the cranial and phrenic nerves may be affected unilaterally inGuillainBarre syndrome, and that there is clinical variability in the axonalsubtype of this syndrome.
2002 John Wiley & Sons, Inc. Muscle Nerve 25: 297299, 2002DOI 10.1002/mus.10041
UNILATERAL CRANIAL AND PHRENICNERVE INVOLVEMENT IN AXONALGUILLAINBARR SYNDROME
YUMI SAKAKIBARA, MD,1 MASAHIRO MORI, MD,2 SATOSHI KUWABARA, MD,2
KAORU KATAYAMA, MD,1 TAKAMICHI HATTORI, MD,2 MICHIAKI KOGA, MD, PhD,3 andNOBUHIRO YUKI, MD, PhD3
1Department of Neurology, Narita Red Cross Hospital, Narita, Japan2Department of Neurology, Chiba University School of Medicine, 1-8-1 Inohana,Chuo-ku, Chiba, 260-8670 Japan3Department of Neurology, Dokkyo University School of Medicine, Tochigi, Japan
Accepted 21 September 2001
GuillainBarre syndrome (GBS) is an acute inflam-matory polyneuropathy characterized by relativesymmetric progressive weakness and areflexia1 thatnow is classified as demyelinating or axonal based onclinical, electrophysiologic, and pathologic crite-ria.4,5,11,12 Asymmetric cranial nerve palsy has notcommonly been noted in GBS. We describe a patientwith axonal GBS who had unilateral, multiple cra-nial, and phrenic nerve palsies as the initial manifes-tations.
A 49-year-old Japanese woman developed acute neckmuscle weakness 10 days after an upper respiratorytract infection and was unable to raise her head offthe bed. She also had difficulty in swallowing, weak-ness and numbness in her upper extremities, andrespiratory distress. On day 9, she was referred toNarita Red Cross Hospital. On admission, neurologi-cal examination showed unilateral facial and hypo-glossal nerve palsies on the left, in addition to pha-
ryngeal palsy, dysarthria, and dysphagia. Eyemovements and pupillary reactions were normal.Muscle strength on the Medical Research Councilscale was grade 2 for the neck flexors and rotators,grade 4 for the proximal arms, and grade 2 for thesmall hand muscles. There was no weakness in thelegs or trunk. Weakness of the upper limbs was ap-proximately symmetric. There was no muscle atro-phy or fasciculation. Tendon reflexes were dimin-ished in the upper extremities but normal in thelower ones. She was able to walk steadily despite theprofound weakness in her neck and upper extremi-ties. Plantar responses were flexor. The sensory ex-amination was normal. Although GBS was suspected,the presence of unilateral multiple cranial involve-ment confused the diagnosis.
In the cerebrospinal fluid, cell count was 1/mm3
and protein content was 45 mg/dl on day 16. Com-puted tomography and magnetic resonance imagingof the brain detected no abnormalities. A chestroentgenogram showed an elevated left diaphrag-matic level, indicative of unilateral phrenic nervepalsy (Fig. 1). Nerve conduction studies were per-formed on days 10, 23, and 302 (Table 1). Medianand ulnar motor nerve conduction studies showedmarked decreases in the compound muscle actionpotential amplitude after wrist stimulation, but distallatencies and nerve conduction velocities were nor-
Abbreviations: AMAN, acute motor axonal neuropathy; GBS, GuillainBarre syndromeKey words: acute motor axonal neuropathy; anti-ganglioside antibody;cranial neuropathy; GuillainBarre syndrome; pharyngeal-cervical-brachial variantCorrespondence to: M. Mori; e-mail firstname.lastname@example.org
2002 John Wiley & Sons, Inc.
Cranial Nerve Palsies in GBS MUSCLE & NERVE February 2002 297
mal. No F wave was evoked by the stimulation of themedian and ulnar nerves, whereas minimal F-wavelatency was normal with tibial nerve stimulation.These findings met the electrodiagnostic criteria ofacute motor axonal neuropathy (AMAN).5 Unfortu-nately, neither phrenic nerve conduction studies norsensory nerve conduction studies were performed.Serology was negative for herpes simplex virus, vari-cella-zoster virus, cytomegalovirus, EpsteinBarr vi-
rus, Mycoplasma pneumoniae, and Campylobacter jejuni.On day 8, enzyme-linked immunosorbent assay ofthe patients serum was performed to test for thepresence of IgG and IgM antibodies against GM2,GM1, GM1b, GD1a, GalNAc-GD1a, GD1b, GT1a,GT1b, GQ1b, galactocerebroside, and sulfated gluc-uronyl lactosaminyl paragloboside, as described else-where.21,22 Only anti-GM1 IgG antibody was positive(1:2,000). Its titer had decreased to1:500 by day 38.
On day 9, the patient required mechanical ven-tilation. Once treatment with intravenous immuno-globulin (500 mg/kg/day for 5 days) was begun onday 14, she gradually recovered. One month afterthe start of treatment, she was taken off the respira-tor. Two months after onset, she was discharged withmild pharyngeal-cervical-brachial weakness and lefthypoglossal nerve palsy, but without left diaphrag-matic elevation.
This case was characterized by (1) unilateral mul-tiple cranial and phrenic nerve palsies; (2) pharyn-geal-cervical-brachial weakness; (3) pure motor axo-nal GBS (AMAN); and (4) the presence of anti-GM1antibody and absence of anti-GT1a antibody in theserum.
Pure motor axonal GBS, termed AMAN, is char-acterized by electrophysiologic and pathologic evi-dence of axonal degeneration of the motor nervesand possible association with anti-GM1 antibodies, C.jejuni infection, or both.10,14,16,20 Antibody activityagainst C. jejuni, however, was not found in our pa-tient; an antecedent agent other than C. jejuni there-fore may have been associated with her disorder.
Involvement of cranial motor or mixed nerves,such as the facial, oculomotor, glossopharyngeal, va-gal, or hypoglossal nerves, is common in GBS.17,18
Although less frequent in AMAN than in the demy-elinating type of GBS, such involvement has beenfound in 2661% of AMAN patients.12,20 Moreover,the report of unilateral cranial nerve palsies togetherwith GBS or Miller Fisher syndrome is rare.19 It hasbeen stated that persistent asymmetric cranial nervepalsy (except facial nerve palsy) is atypical and in-dicative of diagnoses other than GBS.1,18 Prolongedphrenic nerve conduction2,23 as well as asymmetri-cal2 involvement has been reported in GBS patients.To our knowledge, however, unilateral phrenicnerve palsy, which causes unilateral diaphragm el-evation that is visible on a chest roentgenogram, hasnot been described in GBS.
In 1986, Ropper17 described three patients whodeveloped prominent weakness of the pharyngeal-cervical-brachial regions with neither lower limb
Table 1. Nerve conduction study results.
Motor nerve study
limit10 23 302
MedianDistal latency (ms) 2.9 3.3 3.0 5.5Conduction velocity (m/s) 51 55 54 >47.0F-wave latency (ms) NE NE 23.0 50.0F-wave latency NE NE 25.2 41.0F-wave latency 46.6 NE
weakness nor sensory disturbance. He consideredthis a variant of GBS, and called it the pharyngeal-cervical-brachial variant of GBS. Our patientshowed bilateral pharyngeal and cervical weakness,and fulfilled the clinical criteria for this variant.18
Since the recent detection of anti-GT1a and anti-GD1a IgG antibodies in pharyngeal-cervical-brachialvariant of GBS by Mizoguchi et al.,13 antibodiesagainst the gangliosides GT1a, GQ1b, GD1a, andGM1b have been detected in some patients with GBSwho have oropharyngeal palsy.68,15 In particular,anti-GT1a IgG, which does not cross-react withGQ1b, has been associated with the pharyngeal-cervical-brachial variant of GBS and implicated in itspathophysiology.68 In our case, the patients serumhad elevated IgG antibody against GM1, but no an-tibody against any of the other gangliosides.
Although the electrophysiological features ofpharyngeal-cervical-brachial variant of GBS have yetto be fully described, in some cases AMAN might bediagnosed.3,13 Mizoguchi et al.13 reported conduc-tion block of the ulnar nerve at the elbow with noslowing of motor nerve conduction. Conductionblock at this common entrapment site is a frequentfinding in anti-GM1 positive cases associated withAMAN.9 Mild slowing of motor nerve conduction,less than 80% of the lowest limit of the normalrange, has been reported in some patients with thepharyngeal-cervical-brachial variant of GBS.7,17 Lossof the fastest fibers also can cause conduction slow-ing. AMAN therefore may be the main electrophysi-ologic pattern in, and axonal involvement of thelower cranial and cervical motor nerves may be thecause of, the pharyngeal-cervical-brachial variant ofGBS.
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