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Guillain-Barré syndrome is an immune mediated peripheral neuropathy characterised by acute onset of symmetric limb weakness and areflexia. Patients with typical Guillain-Barré syndrome had greater leg weakness than arm weakness, with an ascending progression.1 Some patients with Guillain-Barré syndrome, however, present muscle weakness only at the oropharynx, neck, and proximal upper limb muscles, and a descending pattern of weakness appears as illness progresses.2Ropper2 proposed that the second group of patients had a variant of Guillain-Barré syndrome, “pharyngeal-cervical-brachial weakness (PCB)”. Ophthalmoplegia and cerebellar ataxia, however, are often noted in patients with PCB. Therefore, it is unclear whether PCB is an “atypical” Fisher’s syndrome or a distinct variant entity of Guillain-Barré syndrome. To clarify this, we investigated the relation of neck and limb weakness with cranial nerve involvements. We report here that oropharyngeal palsy in Guillain-Barré syndrome has a significant association with neck and arm-dominant weakness whereas ophthalmoplegia does not.
We made prospective examinations of 113 patients with Guillain-Barré syndrome and 39 patients with Fisher’s syndrome who had been referred to our neuroimmunological laboratory between December 1996 and February 1998 with cranial nerve dysfunction and weakness in the neck and limbs on the day of admission. All the patients fulfilled the accepted clinical criteria for these syndromes. Diagnosis of Fisher’s syndrome was also made in patients who initially presented with ophthalmoplegia, ataxia, and areflexia and later developed generalised muscle weakness. On admission, 53 patients with Guillain-Barré syndrome or Fisher’s syndrome (Guillain-Barré syndrome, 14 (12%); Fisher’s syndrome, 39 (100%)) had ophthalmoplegia, and 48 (Guillain-Barré syndrome, 41 (36%); Fisher’s syndrome, seven (18%)) had oropharyngeal palsy. Generalised muscle weakness was present in six (15%) patients with Fisher’s syndrome. Of the 48 patients with Guillain-Barré syndrome or Fisher’s syndrome who had oropharyngeal palsy, 36 (75%) and 20 (42%) respectively had neck and arm-dominant weakness, compared with 33 (32%) and 13 (13%) of the 104 patients without oropharyngeal palsy (table). Patients with oropharyngeal palsy showed a significant increase in the frequency of neck weakness (χ2 test,p<0.0001) and arm dominant weakness (p<0.0001). By contrast, leg dominant weakness was less common in patients with oropharyngeal palsy (11 (23%)) than in patients without (50 (48%)) (p=0.003). There was no significant association of ophthalmoplegia with neck or arm dominant weakness (p=0.5 and p=0.9 respectively).
Statistical analysis showed that muscle weakness in the neck and upper limbs was frequent in patients with Guillain-Barré syndrome or Fisher’s syndrome who had oropharyngeal palsy. This may account for the distribution of muscle weakness that occurs in PCB. According to the clinical criteria of Ropper et al for PCB,1 it should be diagnosed only in patients who have a restricted distribution of muscle weakness in the pharynx, neck, and proximal upper limbs but no weakness or areflexia in the legs. In his original report,2 however, one of the three patients with PCB had generalised areflexia. Moreover, the patient with Guillain-Barré syndrome described by Mizoguchi et al,3 whose initial symptoms were lower cranial nerve dysfunction and upper limb weakness, later developed generalised muscle weakness. These patients with PCB with generalised areflexia or weakness indicate that the preservation of the tendon reflex and muscle power in the legs depends on the severity of the involvement of the limbs. None of the patients in our study met the clinical criteria proposed by Ropper.1 However, the close association of weakness of the pharynx, neck, and upper limbs in Guillain-Barré syndrome and Fisher’s syndrome indicates that PCB is a distinct variant of Guillain-Barré syndrome, because ophthalmoplegia, a cardinal sign in Fisher’s syndrome, is not associated with oropharyngeal palsy, neck weakness, or arm dominant weakness.
Our finding is also supported by detection of serum antibodies against GT1a ganglioside in patients with PCB which show different reactivity from those in patients with Fisher’s syndrome.3 4 IgG anti-GT1a antibodies in patients with PCB are not absorbed by GQ1b ganglioside whereas those in patients with Fisher’s syndrome are.4 Because only GT1a is recognised by serum IgG from the patient who had a restricted distribution of muscle weakness in the pharynx, neck, and proximal upper limbs,4 we speculate that anti-GT1a and anti-GD1a antibodies respectively contributed to the development of PCB and generalised weakness in the patient described by Mizoguchi et al.3
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