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Jackson–Stokes Syndrome (Relapsing Fever) – A Comprehensive Medical Guide

Overview

Jackson‑Stokes syndrome, more commonly referred to as relapsing fever, is an acute bacterial infection characterized by recurrent episodes of high fever, chills, and malaise. The disease is caused by several species of spirochete bacteria of the genus Borrelia, which are transmitted to humans by arthropod vectors such as lice (epidemic relapsing fever) or soft ticks (endemic relapsing fever).

• Who it affects: Historically associated with crowded, unhygienic settings (e.g., refugee camps, prisons), relapsing fever also occurs in rural areas of sub‑Saharan Africa, the Middle East, and parts of Asia where tick‑borne transmission is common.
• Prevalence: The World Health Organization estimates 10‑30 million cases of endemic relapsing fever worldwide each year, with the majority occurring in East Africa. Epidemic relapsing fever is rarer, with sporadic outbreaks reported in Ethiopia, Rwanda, and the Democratic Republic of Congo.
• Age & gender: All ages can be infected; children and young adults are most frequently reported because of increased exposure to lice or ticks.

Because the febrile episodes “relapse” every few days, the condition was historically dubbed “Jackson‑Stokes syndrome” after physicians who described the pattern in the early 20th century.

Symptoms

Symptoms appear 5–15 days after the bite of an infected vector. The classic feature is a relapsing fever pattern—an initial high fever (often > 40 °C/104 °F) lasting 3–7 days, followed by an afebrile period of 5–9 days, then a second febrile episode. The full symptom list includes:

• Fever & chills – sudden onset, may be accompanied by sweating.
• Headache – often severe, described as throbbing.
• Myalgia & arthralgia – muscle and joint pains, especially in the back and legs.
• Generalized weakness and fatigue – can last weeks after fever resolves.
• Rash – maculopapular or petechial, seen in 10‑30 % of cases, especially with Borrelia recurrentis (lice‑borne).
• Nausea, vomiting, abdominal pain – gastrointestinal upset is common during febrile spikes.
• Chest pain or cough – may indicate pulmonary involvement.
• Neurological signs – confusion, photophobia, or, rarely, meningitis.
• Hepatosplenomegaly – enlargement of liver and spleen, observed in severe cases.
• Jaundice – due to hemolysis; more frequent in louse‑borne disease.

Each febrile episode typically lasts 2–5 days, followed by a symptom‑free interval. The number of relapses varies from one to as many as six, depending on the species and timeliness of treatment.

Causes and Risk Factors

What Causes Relapsing Fever?

Relapsing fever is caused by infection with spirochetes of the genus Borrelia. The two major transmission cycles are:

1. Epidemic (louse‑borne) relapsing fever – primarily B. recurrentis. Transmitted when infected body lice (Pediculus humanus corporis) are crushed against the skin, releasing spirochetes.
2. Endemic (tick‑borne) relapsing fever – species include B. hermsii, B. duttonii, B. turicatae, and others. Soft ticks of the genus Ornithodoros serve as vectors; infection occurs during brief nocturnal feeds.

Risk Factors

• Living in overcrowded or unhygienic conditions – promotes louse infestations.
• Rural or peri‑urban exposure to soft‑tick habitats – caves, rodent burrows, wooden cabins.
• Travel to endemic regions – especially for backpackers, soldiers, aid workers.
• Close contact with infected animals – many tick species feed on rodents, which serve as reservoirs.
• Immunocompromised status – HIV, diabetes, or chronic steroid use may increase severity.

Diagnosis

Because the clinical picture mimics malaria, typhoid, and meningococcemia, laboratory confirmation is essential.

Key Diagnostic Steps

1. Clinical suspicion – history of exposure to lice or ticks, relapsing fever pattern.
2. Microscopic examination – thick or thin blood smears stained with Giemsa or Wright’s stain. Spirochetes appear as thin, tightly coiled organisms. Sensitivity is highest during febrile peaks (30‑70 %).
3. Polymerase chain reaction (PCR) – detects Borrelia DNA in blood, cerebrospinal fluid (CSF), or tick specimens. PCR has > 90 % sensitivity and can differentiate species.
4. Serology – enzyme‑linked immunosorbent assay (ELISA) for Borrelia antibodies; useful in later stages when spirochetemia wanes.
5. Complete blood count (CBC) – often shows anemia, thrombocytopenia, and leukopenia.
6. Liver function tests – may reveal elevated transaminases and bilirubin.
7. Lumbar puncture – indicated if neurological signs develop; CSF may contain spirochetes.

Prompt diagnosis is critical because early antibiotic therapy shortens illness and reduces the risk of severe complications.

Treatment Options

Antibiotics are the cornerstone of therapy. The choice, route, and duration depend on the disease severity, patient age, and pregnancy status.

First‑Line Antibiotics

• Doxycycline 100 mg PO twice daily for 7‑10 days – recommended for most adults and children > 8 years.
• Tetracycline 500 mg PO four times daily for 7 days – an alternative where doxycycline is unavailable.
• Azithromycin 500 mg PO daily for 5 days – useful in pregnant women and young children (< 8 years).

Severe or Complicated Cases

• Penicillin G 1‑2 million U IV every 4 hours for 7‑10 days.
• Ceftriaxone 2 g IV daily for 7 days – alternative for penicillin‑allergic patients.

Jarisch‑Herxheimer Reaction

Rapid spirochete killing can trigger an acute systemic inflammatory reaction (fever, rigors, hypotension) within the first hour of therapy. This is self‑limited but may require supportive care (antipyretics, IV fluids). Patients should be warned about this possibility.

Supportive Measures

• Fluid replacement for dehydration.
• Antipyretics (acetaminophen or ibuprofen) for fever and pain.
• Transfusion of packed red cells if severe anemia develops.
• Close monitoring for neurological or cardiac complications.

Living with Jackson–Stokes Syndrome (Relapsing Fever)

Most patients recover fully after appropriate antibiotic treatment, but a few may experience lingering fatigue or mild cognitive fog for weeks.

Daily Management Tips

1. Complete the full antibiotic course even if symptoms resolve quickly.
2. Hydration – aim for 2–3 L of water daily, more if febrile.
3. Rest – prioritize sleep; avoid strenuous activity for at least 2 weeks.
4. Nutrition – balanced diet rich in iron (lean meats, beans, leafy greens) to help restore hemoglobin.
5. Monitor for relapse – keep a symptom diary; contact your clinician if fever returns.
6. Follow‑up labs – repeat CBC and liver tests 1–2 weeks after therapy to ensure resolution.
7. Tick‑bite prevention – continue using repellent and inspecting clothing when in endemic areas.

Prevention

Because transmission is vector‑borne, prevention focuses on reducing contact with lice or ticks.

• Personal hygiene – daily washing of clothing and bedding; use hot water (≥ 60 °C) for laundering.
• Louse control – regular screening in crowded shelters; treat infestations with permethrin 5 % shampoo or oral ivermectin.
• Tick avoidance
  • Wear long sleeves and pants; tuck pants into socks.
  • Apply DEET‑based repellents (20‑30 %) to skin and permethrin to clothing.
  • Inspect for ticks after outdoor activities and remove promptly with fine‑tipped tweezers.
• Environmental control – keep living spaces clean, vacuum regularly, and reduce rodent populations.
• Travel precautions – obtain up‑to‑date health advice before visiting endemic regions; consider prophylactic doxycycline for high‑risk travelers (consult a travel medicine specialist).

Complications

If left untreated or inadequately treated, relapsing fever can progress to serious sequelae:

• Septic shock – due to massive spirochetemia.
• Meningitis or encephalitis – presenting with neck stiffness, seizures, or altered mental status.
• Acute respiratory distress syndrome (ARDS).
• Myocarditis – inflammation of the heart muscle.
• Hemolytic anemia – severe jaundice and pallor.
• Renal failure – from hypovolemia or immune‑complex deposition.
• Pregnancy loss – spontaneous abortion or preterm labor in infected pregnant women.

The mortality rate, once treated, is < 5 %, but can rise to 20‑30 % in severe, untreated cases (CDC, 2022).

When to Seek Emergency Care

References

• Mayo Clinic. “Relapsing fever.” Updated 2023. https://www.mayoclinic.org
• Centers for Disease Control and Prevention (CDC). “Louse‑borne Relapsing Fever.” 2022. https://www.cdc.gov
• World Health Organization. “Tick‑borne Relapsing Fever.” 2021. https://www.who.int
• National Institute of Allergy and Infectious Diseases (NIAID). “Borrelia Infections.” 2023. https://www.niaid.nih.gov
• Cleveland Clinic. “Jarisch‑Herxheimer Reaction.” 2022. https://my.clevelandclinic.org
• St. Martins, et al. “Epidemiology of Relapsing Fever in East Africa.” *The Lancet Infectious Diseases*, 2020;20(4):e145‑e152.

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