Reflexes in Guillain-Barré Syndrome
Abstract & Commentary
Synopsis: Normal or hyperactive reflexes do not necessarily rule out a diagnosis of Guillain-Barré' syndrome.
Source: Yuki N, et al. Guillain–Barré syndrome associated with normal or exaggerated tendon reflexes. J Neurol 2012;259:1181-1190.
Progressive symmetrical muscle weakness, associated with absent or hyporeflexia, are the cardinal clinical features of Guillain-Barré syndrome (GBS). Additional features are facial and oropharyngeal weakness in 50%, respiratory weakness necessitating ventilatory support in 10-30%, ophthalmoparesis in 15%, hand and feet paresthesiae in more than 80%, back and limb pain in 66%, and dysautonomia in 70%. Unusual features of GBS include mental status changes, meningeal signs, papilledema, facial myokymia, hearing loss, vocal cord paralysis, and the syndrome of inappropriate antidiuretic hormone secretion (SIADH). How often are deep tendon reflexes (DTRs) normal or overactive in GBS, and with which other features are they more likely to occur?
In a Japanese survey study of patients' sera sent for anti-ganglioside testing to the Dokkyo Medical University Neuroimmunology Laboratory between 2001-2006, clinical criteria for GBS were fulfilled in 48 who demonstrated normal or brisk DTRs on initial examination. Comparison was made to 1000 randomly chosen GBS patients with hypo- or areflexia, who served as controls. Subsequent retrospective analysis of clinical, serologic, and electrophysiologic features was performed on a total of 213 GBS patients culled from three hospitals, two in Japan and one in Chieti, Italy, seen between 1999-2010. Statistical analysis included the Mann–Whitney U test, and the x2 or Fisher's exact test, with P < 0.05 considered statistically significant.
Compared to 1000 GBS patients with hypo- or areflexia, the 48 GBS patients in the Japanese survey study with preserved DTRs were more likely to have experienced antecedent diarrhea, demonstrate limb weakness as opposed to distal paresthesiae as an initial symptom, demonstrate a mild functional grade at nadir, and have IgG antibodies to GM1, GM1b, GD1a, or GalNAc-GD1a. Ophthalmoparesis, facial weakness, and sensory dysfunction were less likely. Normal or hyperreflexia was maintained throughout the course in 32 of these patients.
Among the 213 GBS patients culled from three hospitals, three patients demonstrated hyperreflexia throughout their course, eight demonstrated normal DTRs throughout, and 12 demonstrated hyperreflexia, even at nadir, with return of normal reflexes upon recovery. These 23 patients more frequently demonstrated: 1) pure motor weakness, 2) independent ambulation even at nadir, 3) acute motor axonal, rather than acute inflammatory demyelinating, polyneuropathy, and 4) GM1, GM1b, GD1a, or GalNAc-GD1a antibodies. Approximately 10% of GBS patients retain normal or brisk DTRs, and their presence should not preclude the diagnosis of GBS.
Commentary
With an annual incidence of 0.81-1.89/100,000, GBS incidence increases with age, is more common in males, and remains a disabling disease despite the advent of plasma exchange and intravenous immunoglobulin infusions. Mortality within the first year is of approximately 4%, severe persistent disability at 1 year is seen in 14%, and 40% suffer continued weakness, pain, and require a change in professional career. Older age, preceding diarrhea, greater weakness on admission, short interval from symptom onset to admission, the need for mechanical ventilation, and absent or low compound muscle action potential amplitudes on nerve conduction studies remain negative prognostic indicators. Much work remains to be done to improve the lot of GBS sufferers.1
Reference
1. Rajabally YA, Uncini A. Outcome and its predictors in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 2012;83: 711-718.
Normal or hyperactive reflexes do not necessarily rule out a diagnosis of Guillain-Barré' syndrome.
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