On this page
Referral and warning signs
When to refer
Refer ALL individuals with potential rabies exposure IMMEDIATELY for assessment and initiation of Post-Exposure Prophylaxis (PEP). Refer confirmed/suspected clinical rabies cases to hospital (H/RR) for palliative care and infection control.
Red flags
- Hydrophobia.
- Aerophobia.
- Unexplained progressive encephalitis or ascending paralysis following animal bite/exposure.
- Paraesthesia or pain at site of previous bite.
Signs and symptoms
- Incubation period: Highly variable, typically 1-3 months, but can range from <1 week to >1 year.
- Prodromal Phase (2-10 days):
- - Non-specific symptoms: Fever, malaise, headache, fatigue, anorexia, nausea, vomiting.
- - Pain, tingling, itching, or paraesthesia at the site of the bite/exposure (highly suggestive if present).
- Acute Neurologic Phase (2-7 days): Two main forms:
- - Furious Rabies (~80%):
- - Episodic psychomotor agitation, hyperactivity, restlessness, anxiety, confusion, hallucinations.
- - Hydrophobia: Painful, violent spasms of pharyngeal/laryngeal/inspiratory muscles triggered by attempting to drink or sight/sound of water.
- - Aerophobia: Similar spasms triggered by draft of air.
- - Fluctuating consciousness.
- - Autonomic instability (hypersalivation, sweating, priapism, unstable BP/HR).
- - Paralytic ('Dumb') Rabies (~20%):
- - Progressive ascending flaccid paralysis, often starting near bite site.
- - Mimics Guillain-Barré Syndrome.
- - Fever and headache common.
- - Consciousness often preserved longer than in furious form.
- - Hydrophobia/aerophobia usually absent.
- Coma Phase: Both forms progress to coma, autonomic failure, respiratory arrest, and death, usually within 7-10 days of symptom onset.
Risk factors
- Bite, scratch, or lick from a potentially rabid animal (dogs are main reservoir in many developing countries; also cats, bats, monkeys, wild carnivores).
- Exposure of broken skin or mucous membranes to saliva or nervous tissue of infected animal.
- Failure to receive or complete timely Post-Exposure Prophylaxis (PEP) after a potential exposure.
- Living in or travelling to rabies-endemic areas.
- Occupational risk (veterinarians, animal handlers, lab workers).
Diagnosis
Clinical assessment
Diagnosis based on characteristic clinical syndrome (encephalitis with hydrophobia/aerophobia OR ascending paralysis) following a history of potential animal exposure. Often diagnosis is made clinically when typical symptoms appear.
Differential diagnosis
- Tetanus (trismus, spasms, but usually clear sensorium, no hydrophobia).
- Other viral encephalitis (Herpes Simplex, Arboviruses).
- Guillain-Barré Syndrome (ascending paralysis, but usually symmetrical, sensory changes, CSF high protein).
- Psychiatric disorders / Hysteria.
- Poisoning (e.g., strychnine).
Investigations
- Ante-mortem diagnosis difficult, often clinical. Tests usually confirm posthumously.
- - Saliva: RT-PCR for rabies virus RNA.
- - Skin Biopsy: (Nape of neck at hairline) - DFA staining or RT-PCR for viral antigen/RNA.
- - CSF: RT-PCR or antibody detection (usually positive later in illness). CSF often normal initially.
- - Serum: Antibody detection (appears late, after symptoms start, may be absent if patient dies quickly).
- Post-mortem: Brain tissue examination (DFA, histology for Negri bodies, RT-PCR, culture) is definitive.
Diagnostic criteria
Clinical syndrome + History of exposure. Laboratory confirmation difficult ante-mortem.
Management
Non-pharmacological management
- Once symptoms develop, rabies is essentially untreatable and fatal. Focus is on Palliative Care.
- Hospital Admission: Isolate patient in quiet room to minimize stimuli triggering spasms.
- Strict Infection Control Precautions: Standard + Contact + Droplet precautions. Use PPE (gloves, gown, mask, eye protection) when handling patient or potentially contaminated fluids/secretions. Saliva is infectious.
- Comfort measures.
- Maintain hydration/nutrition as possible.
- Avoid futile intensive interventions once diagnosis established.
Pharmacological treatment
- Palliative Care:
- - Sedation (e.g., Diazepam, Midazolam, Morphine) to manage agitation, spasms, pain, anxiety.
- - Antipsychotics (e.g., Haloperidol) for agitation/hallucinations.
- - Medications to reduce saliva production (e.g., Glycopyrrolate) may provide comfort.
- - There is NO curative antiviral treatment.
Monitoring
Focus on patient comfort, symptom control (pain, agitation, spasms), and family support. Monitor airway minimally to avoid triggering spasms.
Complications
- Progression to coma.
- Respiratory failure.
- Autonomic collapse.
- Cardiac arrest.
- Death (almost universal).
Prevention
- Post-Exposure Prophylaxis (PEP): CRITICAL intervention AFTER exposure but BEFORE symptom onset.
- - Immediate and thorough wound washing with soap and water for at least 15 minutes.
- - Rabies Vaccine: Administration of effective rabies vaccine according to recommended schedule (e.g., Essen regimen - days 0, 3, 7, 14, 28; or Zagreb regimen).
- - Rabies Immunoglobulin (RIG): Administration of Human RIG (HRIG) or Equine RIG (ERIG) infiltrated into and around the wound(s) on day 0 for severe exposures (Category III, some Category II), along with first vaccine dose. Provides passive immunity.
- *(See UCG Section 1.2.1.3 for details on wound categories and PEP).*
- Pre-Exposure Prophylaxis (PrEP): Recommended for high-risk individuals (vets, animal handlers, lab workers, travellers to high-risk areas).
- Animal Control: Mass vaccination of domestic dogs, control of stray animal populations, wildlife rabies control (where applicable).
Patient education
- For Exposure: Extreme importance of immediate wound washing and seeking prompt medical care for PEP. Need to complete full vaccine schedule. Information about RIG if given.
- For Clinical Disease: Explanation of fatal nature once symptoms start. Focus on palliative care, comfort, dignity. Need for strict infection control by caregivers/staff. Counseling for family.
- General: Avoid contact with unknown/stray animals, especially if behaving abnormally. Importance of vaccinating domestic animals (dogs).
Uganda-specific notes
Rabies is endemic, dogs are the main reservoir. Access to timely and complete PEP (vaccine + RIG) can be challenging and costly, especially outside major centres. Clinical rabies cases require careful management to ensure staff/caregiver safety and provide compassionate palliative care.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 2, Section 2.3.5 (Clinical Disease)
- Uganda Clinical Guidelines (UCG) 2023
Chapter 1, Section 1.2.1.3 (Animal Bites - PEP)