BackgroundAdd caption Causes
- Commonest (in order): herpes simplex (HSV1 > HSV2), VZV, EBV.
- Others: CMV, HIV seroconversion, measles, mumps, arboviruses (West-Nile, Japanese, tick-borne, St Louis), rabies.
Autoimmune (20% of cases):
- Often affects the medial temporal lobes, when it is known as 'limbic encephalitis'.
- Often paraneoplastic.
- Antibodies (and associations): anti-Hu (small cell lung cancer), anti-Ma2 (germ cell tumour of testes), anti-NMDAR (50% have ovarian tumour), anti-LGI1 (formerly 'anti voltage-gated potassium channel'; only 20% have cancer).
Other causes:
- Idiopathic (30% of cases).
- Any bacterial meningitis can become a meningoencephalitis.
- Spirochetes: Listeria, Lyme, syphilis.
- TB
- Protozoa: malaria, Toxoplasma.
- Fungal: Aspergillus, Cryptococcus.
Epidemiology
- Annual incidence: 1/20,000.
- Commonest under 1 years old or over 65.
Signs and symptoms
- Initially non-specific: fever, headache, nausea, vomiting, malaise.
- Neuro symptoms: seizures, odd behaviour or confusion, ↓level of consciousness, focal signs.
- In addition to cerebrum, may also affect brainstem, dorsal roots (sensory), and autonomic nervous system, with an associated wide range of symptoms.
Investigations
- Blood culture.
- Serum viral PCR.
- If suspected: toxoplasma IgM, malaria film.
Image with MRI or contrast-enhanced CT:
- Temporal lobe inflammation: usually HSV or autoimmune.
- Meningeal irritation: meningoencephalitis.
Special tests:
- LP: ↑protein (most causes), ↑lymphocytes (viral, autoimmune), ↑PMNs (bacterial), ↓glucose (bacterial). Identify pathogen with viral PCR and Gram stain.
- EEG: optional.
Management
- Aciclovir IV stat to cover HSV. Continued for 14-21 days if HSV confirmed.
- Consider ganciclovir for CMV if immunosuppressed.
- Anticonvulsants for seizures.
- Autoimmune encephalitis: immunosuppression (steroids, IVIg, plasma exchange) and treat any underlying cancer.
- If infectious, notify public health authorities.
Complications and prognosis
- Short term: seizures, ↑ICP, SIADH or diabetes insipidus.
- Long-term: neurological complications are common, including motor and cognitive problems.
- 10% mortality overall. For HSV, treatment reduces mortality from >50% to 20%.
Brain abscess
Pathophysiology
- A focal infection of brain tissue, comprising encapsulated pus, in contrast to the generalized infection/inflammation of encephalitis.
- Source of pathogen: direct inoculation (penetrating trauma, neurosurgery), contiguous spread (mastoiditis, sinusitis, dental infection) or haematogenous (endocarditis, IVDU, right-to-left shunt).
- Often polymicrobial. Staophylococci and Streptococci are common. In the immunosuppressed, consider Aspergillus, Toxoplasma, and Nocardia.
Signs and symptoms
- Classic triad (25%): headache (70%), fever (60%), focal neurology (60%).
- Others: vomiting, confusion, lethargy, seizures.
Investigations
- CT/MRI head with contrast shows ring-enhancement.
- Avoid LP due to risk of brainstem herniation.
- Image-guided needle aspiration for culture, but do not delay antibiotics while waiting.
Management
- Ceftriaxone plus metronidazole IV for 6-8 weeks.
- Aspiration or surgical drainage needed for most, though can trial antibiotics alone if small (<2 cm).
Acute disseminated encephalomyelitis (ADEM)
Pathophysiology and epidemiology
- Autoimmune CNS disease, defined by acute-onset encephalopathy, polyfocal neurology, and imaging evidence of multifocal demyelination.
- Commoner in kids and young adults.
Presentation
- Encephalopathy: altered consciousness or behaviour.
- Polyfocal neurology e.g. weakness, ataxia, gait abnormality, cranial nerve palsies, visual problems, seizures, spinal deficits.
- Non-specific symptoms: fever, headache, nausea/vomiting.
- ⅔ have a recent (<4 weeks) history of infection, which is thought to be a trigger.
Investigations
- MRI: multiple, large white matter lesions, usually brain, but spine affected in 1/3.
- CSF: ↑WBC and ↑protein (both around 50% sensitive).
- No definitive diagnostic test.
Management
- Steroids, IV then oral taper.
- IVIg or plasma exchange 2nd line.
Prognosis
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