Hemifacial spasm
Year: 1999
Type: letter
Abstract: 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 diVerent reactivity from those in patients with Fisher's syndrome. 3 4IgG 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.
Authors: P. J JANNETTA, A. KASSAM;, R P. REIGOSA
Institution: University of Pittsburgh
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W4254184988