By Michael Rubin, MD
Professor of Clinical Neurology, Weill Cornell Medical College
Dr. Rubin reports no financial relationships relevant to this field of study.
SYNOPSIS: Guillain-Barré syndrome in the very old (> 80 years of age) results in more severe disease with poorer recovery.
SOURCE: Peric S, Berisavac I, Tamas OS, et al. Guillain-Barré syndrome in the elderly. J Peripher Nerv Syst 2016;21;105-110.
Guillain-Barré syndrome (GBS) affects all ages, men more than women, with a lifetime risk of one in 1,000. Overall incidence of GBS increases by 20% for every decade of life after 10 years of age, although some studies have shown an incidence drop after age 80 years. Is the disease more severe in the elderly?
In this retrospective analysis of hospital records between 2009-2013, with 2014 data entered prospectively, the authors reviewed all GBS cases evaluated in seven tertiary healthcare centers in three countries: Serbia, Republic of Srpska, and Montenegro. Based on World Health Organization guidelines, 60 years of age was the cutoff between young and old, with 61-80 years of age designated young-old, and older than 80 years designated old-old. Standard criteria were used to diagnose GBS, and the GBS disability scale was used to assess disability. Statistical analysis comprised the Kolmogorov-Smirnov, chi-square, Mann-Whitney U, and Student’s t tests, with statistical significance set at 0.05.
Over the six-year study period, 403 GBS patients were included for analysis, including 250 who were younger than 60 years of age and 153 who were older than 60 years of age. Respiratory infections or a diarrheal illness preceded GBS onset in one-third and one-fifth of cases, respectively, within a mean of 12 days prior to onset, with no significant difference appreciated between the age groups with respect to antecedent events. Frequency of prior medical illness was similar in both groups, though a history of malignancy was thrice as common in older patients. Acute inflammatory demyelinating polyneuropathy was the most common GBS variant in both age groups, but acute motor and sensory axonal neuropathy (AMSAN) was twice as common in older patients, 12% vs. 6% of all GBS cases. Elevated cerebrospinal fluid protein was less common in the older group, but hyponatremia was more common, as was severe disability, seen in 72% of the older group at nadir, compared to 42% of the younger patients. Both groups tolerated intravenous immunoglobulin or plasma exchange, or both, with similar frequencies of side effects. Non-responders comprised 5% of both groups. Comparing the old-old to the young-old, bulbar symptoms and comorbidities were more common in the old-old, 50% vs. 19%, and 100% vs. 66%, respectively. On discharge, 67% of the old-old had severe disability, compared to 37% of the young-old. No significant difference between the older groups was seen with respect to the frequency of AMSAN. Elderly patients, especially those older than 80 years of age, developed more severe GBS with slower recovery compared to those younger than 60 years.
At the other end of the age spectrum, children with GBS do well, but GBS may be a difficult diagnosis to make in this age group. Comparing preschool children, younger than six years of age, to those between the ages of 6-18 years, 68% of preschoolers are misdiagnosed, compared to 21% of older children, with misdiagnoses including tonsillitis, meningitis, myopathy, discitis, rheumatic disorders, and coxitis. Although both groups delayed seeing their pediatrician by a median of five days, delay in correct diagnosis was significantly longer in younger than in older children, three days compared to zero days. Older children presented with classical symptoms, facilitating diagnosis, where preschoolers refused to walk and complained of leg pain, delaying diagnosis. Diagnosis of GBS in preschoolers is a challenge.1
- Roodbol J, de Wit MC, Walgaard C, et al. Recognizing Guillain-Barre syndrome in preschool children. Neurology 2011;76;807-810.