Cancer Diagnosis and Prognosis After Guillain–Barré Syndrome: A Population-Based Cohort Study

Abstract

Introduction It is unclear whether Guillain–Barré syndrome (GBS) can be a marker of a paraneoplastic syndrome. We examined whether GBS is associated with cancer and whether the prognosis of GBS patients with cancer differs from that of other cancer patients. Materials and Methods We conducted a population-based cohort study of patients diagnosed with GBS between 1978 and 2017 using Danish registry-data. Main outcome measures were cancer incidence and mortality after cancer diagnosis. We calculated absolute risks of a cancer diagnosis, treating death as competing risk, and standardized incidence ratios (SIRs) as measures of relative risk. We matched each GBS cancer patient with up to 10 cancer patients without a GBS diagnosis and examined the six-month survival after cancer diagnosis using Cox regression analysis. Results We identified 7897 patients (58% male, median age 57 years) with GBS. During a median follow-up of 9.5 years, the one-year risk of cancer was 2.7% (95% confidence interval (CI), 2.4–3.1). The SIR was increased throughout follow-up, but most noticeably during the first year after diagnosis (SIR: 3.35, 2.92–3.83). SIRs were particularly elevated for hematologic cancers (SIR: 8.67, 6.49–11.34), smoking-related cancers (SIR: 3.57, 2.81–4.47), and cancers of neurological origin (SIR: 8.60, 5.01–13.77). Lung cancer was the main contributor to the overall excess risk, which persisted after 36 months of follow-up (SIR: 1.17, 1.09–1.25). The mortality rate ratio comparing patients diagnosed with any cancer within one year of their GBS diagnosis and matched GBS-free cancer cohort members was 1.56 (95% CI, 1.27–1.90). Conclusion GBS patients had a three-fold increased risk of cancer diagnosis in the first year of follow-up. The absolute cancer risk was almost 3.0%. A GBS diagnosis was an adverse prognostic marker for survival following cancer diagnosis. Clinicians should consider occult cancer in patients hospitalized with GBS.

Acute sensorimotor paraneoplastic neuropathy in a patient with small cell prostate cancer

“…In contrast, the association between GBS and malignancy is still debated as Graus et al do not define it as a paraneoplastic syndrome in the absence of high-risk antibodies 9. A number of case reports described GBS in patients with an existing or later diagnosed malignancy12–25 and a small number of population-based studies have identified an increased risk of cancer in patients who developed GBS 8 26 27. Nevertheless, it is not clear whether these findings demonstrate an actual causal link or simply two separate concurrent diagnoses.…”

“…Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy which, although not classically recognised as a paraneoplastic syndrome, has been reported to occur in association with malignancy 8. To our knowledge, there have been no reported cases of a GBS-like PNS in a patient with a diagnosis of small cell prostate cancer.…”

SOX‐1 antibodies positive Lambert–Eaton myasthenic syndrome with occult small cell lung cancer: A case report

“…In SCLC‐associated paraneoplastic syndrome, Guillain–Barre syndrome (GBS) should be account for differential diagnosis of LEMS. 16 Both diseases have clinical manifestations of limb weakness and decreased reflexes. Weakness of LEMS normally spreads proximally to distally.…”

Intravenous immunoglobulin is safe and effective in controlling pre-existing paraneoplastic neuromuscular diseases in cancer patients treated with immune checkpoint inhibitors: two case reports and literature review

“…For involvement of the peripheral nerves or muscles, the clinical presentations may be similar to GBS (an acute inflammatory polyneuropathy), CIDP, autoimmune autonomic ganglionopathy, and inflammatory myopathy ( 11 – 14 ). A population-based cohort study in Denmark suggested that GBS patients had a three fold increased risk of cancer diagnosis in the first year of follow-up with an absolute cancer risk of 2.7% ( 15 ). Some cases with paraneoplastic neuromuscular disorders respond to immunosuppressants or immune modulators including IVIG ( 12 ).…”