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Landry’s 19th century report gives the impression that Guillain–Barré syndrome (GBS) is characterised by ascending weakness. This clinical picture now is called “Landry’s ascending paralysis.”1 Indeed, muscular weakness in GBS does usually begin in the legs, progressing to the trunk, arms, and cranial regions.2 However, several clinical variants are now recognised in which weakness initially begins in other areas.2 Four patients with acute polyneuropathy were reported initially to have had muscle weakness in the hands.3 In two of these, Campylobacter jejuni infection had preceded the neurological symptoms, and serum anti-GM1 antibody was detected in the others.
To determine the frequency and clinical features of hand onset GBS, we reviewed the medical records of 464 consecutive patients with the disease. Eleven had been treated at our hospital, the others were referred to our laboratory from other hospitals for antiganglioside antibody tests. Hand onset GBS was diagnosed when the first symptom that a GBS patient recognised was hand weakness. Paraesthesiae and other sensory symptoms may have preceded hand weakness, but patients who developed weakness in both the hands and legs on the first day of illness were excluded.
We found that 33 (7%) of the patients reviewed had hand onset GBS. Frequent initial symptoms were weak hand grip and clumsy fingers. Paraesthesiae in the hands or all four limbs had preceded hand weakness in eight of them. Three patients presented with facial palsy, diplopia, or blurred vision on the day of hand weakness onset. Weakness was limited to the hands and arms throughout the acute phase of illness in four patients (12%), while it spread to the legs in the others. Assisted ventilation was required for four patients (12%). Compared with the other patients, those with hand onset GBS more often had a history of preceding diarrhoea, had antiganglioside IgG antibodies, and, less frequently, had sensory disturbance (table 1). Of the autoantibodies present, anti-GM1, anti-GM1b, and anti-GD1a IgG were significantly associated with hand onset GBS. Antecedent C jejuni infection was proven by serological assay (n = 19) or stool culture (n = 2) in 20 (61%) of the patients with hand onset GBS.
Hand onset GBS patients (n = 25) who had antiganglioside IgG, evidence of preceding C jejuni infection, or both often had a history of previous gastrointestinal symptoms but rarely cranial nerve involvement or sensory disturbance. Although most patients had received plasmapheresis or intravenous immunoglobulin within two weeks of onset, seven showed irreversible neurological damage at the last consultation. Moderate or mild weakness remained in the arms and legs of four patients (from four to 22 months after onset), and weakness mainly remained in the upper limbs of three (from two to four months after onset). Results of nerve conduction studies done within two weeks of weakness onset were available for 10 patients: five had predominant axonal disturbance and the others showed unclassified findings. In contrast, the eight patients who had neither antiganglioside IgG nor evidence of a preceding C jejuni infection often had a history of previous respiratory infection and sensory disturbance. All had received plasmapheresis or intravenous immunoglobulin and, except for one, had no or only mild weakness 12 months after onset. The exception required assisted ventilation at nadir and still had moderate distal weakness and amyotrophy in the legs six months after onset. Results of nerve conduction studies were available for four patients who had neither antiganglioside IgG nor evidence of a preceding C jejuni infection; all of these had a primary demyelinating disturbance.
In the larger population, we confirmed previous findings that hand onset GBS is related to C jejuni enteritis and anti-GM1 antibody,3 although the cases reported had either C jejuni or anti-GM1 antibody. Furthermore, we found that hand onset GBS is characterised by pure motor symptoms and the presence of IgG antibodies against GM1b and GD1a, as well as those against GM1. Residual symptoms were frequent in the C jejuni or autoantibody related populations, but no statistical analysis was made of outcome. In contrast, one quarter of the patients with hand onset GBS had no evidence of previous C jejuni infection or antiganglioside IgG. Antecedent respiratory infection symptoms and sensory involvement were characteristic, and those patients tended to have better outcomes than the others.
It is noteworthy that motor deficit remained only in the arms during the course of the illness in the four hand onset GBS patients, two of whom were positive for C jejuni serology and antiganglioside IgG. Another patient who developed acute pure motor neuropathy following C jejuni enteritis was reported to have localised weakness in his hands and anti-GM1 IgG.4 Although that patient had preserved tendon reflexes in the four limbs, a serial electrophysiological study confirmed the diagnosis of an axonal variant of GBS, indicating that anti-GM1 IgG and C jejuni infection are related to hand-predominant weakness in GBS. It also is noteworthy that the six patients who had hand onset GBS had an initial diagnosis of cervical spondylosis (n = 4), lacunae infarction (n = 1), or brachial plexus neuritis (n = 1) on hospital admission. Frequent hand function problems have been reported even in mildly affected GBS patients who could walk unaided at nadir.5 Early treatment has been suggested in such cases. Recognition of the clinical characteristics of hand onset GBS may lead to a good prognosis because individuals can be given specific treatment as early as possible.
This research was supported in part by a grant-in-aid from the Uehara Memorial Foundation, a grant for scientific research (B) (KAKENHI 14370210 to NY) from the Ministry of Education, Culture, Sports, Science and Technology of Japan, and a research grant for neuroimmunological diseases from the Ministry of Health, Labour and Welfare of Japan.
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