Encephalitis Summary

Definition

  • Inflammation of brain parenchyma
  • Associated with neurological dysfunction
  • Distinct from meningitis

Common Causes

  • Viral infections (HSV most common)
  • Bacterial/fungal/parasitic infections
  • Autoimmune/paraneoplastic
  • Unknown etiology (50% of cases)

Clinical Features

  • Altered mental status
  • Seizures
  • Focal neurological deficits
  • Behavioral/cognitive changes
  • Fever, headache, meningism

Diagnosis

  • Lumbar puncture (CSF analysis)
  • MRI (imaging of choice)
  • EEG for seizures/mental status changes
  • PCR for viral pathogens

Management

  • Empirical acyclovir for viral encephalitis
  • Supportive care (ABCs)
  • Seizure control
  • ICP management if elevated
  • Immunotherapy for autoimmune cases

Encephalitis

Inflammation of brain parenchyma with neurological dysfunction

Overview

Encephalitis is defined as inflammation of the brain parenchyma associated with neurological dysfunction such as altered state of consciousness, seizures, personality changes, cranial nerve palsies, speech problems, and motor and sensory deficits.

It is the result of direct inflammation of the brain tissue, as opposed to the inflammation of the meninges (meningitis), and can be the result of infectious or non-infectious causes. An aetiological agent is only identified in around 50% of cases.

Causes

Infectious Causes

  • Viral infections - most common (herpes virus most frequently identified)
  • Bacterial infections
  • Fungal infections
  • Parasitic infections
  • Prion diseases (Creutzfeldt-Jakob disease)

Non-Infectious Causes

  • Para-infectious
    • Acute disseminated encephalomyelitis (ADEM)
    • Acute haemorrhagic leukoencephalitis
    • Bickerstaff's encephalitis
    • Rasmussen's encephalitis
  • Paraneoplastic (autoimmune) syndromes
    • Anti-NMDA receptor encephalitis
    • Anti-GABA receptor encephalitis
    • Anti-VGKC antibody-associated encephalitis

Clinical Features

Encephalitis is caused by a heterogeneous group of pathogens, but presents with a near-uniform neurological dysfunction.

Mental Status Changes

  • Altered mental status ranging from mild lethargy to profound coma
  • Altered personality, withdrawal
  • Inability to make decisions
  • Akinetic mutism, bizarre behavior
  • Memory problems, amnesic state

Neurological Symptoms

  • Seizures of all varieties (complex partial most common)
  • Focal neurological signs
  • Aphasia, visual disturbances
  • Cranial nerve deficits
  • Lateralized motor weakness, ataxia
  • Tremors, myoclonus, paraplegia

Systemic Symptoms

  • Signs of meningoencephalitis:
    • Headache
    • Photophobia
    • Neck stiffness
  • Fever
  • Upper respiratory or gastrointestinal symptoms

Differential Diagnosis

Investigations

Lumbar Puncture Indication

A lumbar puncture is always recommended as long as there is no contraindication, and 2 out of the following 4 symptoms are present: fever, headaches, altered mental status of unknown aetiology, meningism.

The main differential to distinguish is encephalopathy secondary to metabolic or toxic disturbances. Once a primary central nervous system disease is diagnosed, the diagnostic approach is geared towards determining the aetiology and appropriate therapy.

Routine Investigations

Cerebrospinal Fluid (CSF)

  • Opening pressure
  • Cell count
  • Protein, Glucose
  • Gram stain
  • Bacterial culture
  • Herpes simplex virus-1/2 PCR
  • Enterovirus PCR
  • Hold for further testing

Serum Tests

  • FBC
  • Serum electrolytes/LFT
  • Blood cultures
  • Hold for further testing

Imaging & Other Tests

  • Chest x-ray
  • Neuroimaging (MRI is study of choice)
  • Electroencephalogram (for persistent altered mental status or seizures)

Management

Medical Emergency

Management consists of basic resuscitation measures ensuring adequacy of the airway, breathing, and circulation, and empirical antiviral therapy in cases of suspected viral encephalitis concurrently with diagnostic steps.

General Measures

Specific Treatments

Antiviral Treatment

  • All cases of suspected community-acquired viral encephalitis are started empirically on acyclovir
  • Reduces mortality in HSV encephalitis

Corticosteroids

  • Use is controversial but may have role in certain cases
  • Duration should be short (3-5 days)
  • Used for complications of encephalitis

Other Therapies

  • Appropriate antimicrobials for non-viral infections
  • Aggressive immunotherapy for autoimmune encephalitis:
    • IV corticosteroids
    • Immunoglobulin
    • Plasma exchange

ICP Management

Complications

Acute Complications

  • Death
  • Status epilepticus
  • Cerebral haemorrhage
  • Cerebral vein thrombosis
  • Cerebral vasculitis

Chronic Complications

  • Neurological sequelae
  • Persistent seizures
  • Hydrocephalus
  • Hypothalamic and autonomic dysfunction
  • Encephalitis lethargica
  • Post-viral chronic fatigue syndrome