Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

immunocompetent host: suspected viral aetiology

1st line – aciclovir

Back
INITIAL
|
immunocompetent host: suspected viral aetiology
1st line – 

aciclovir

All cases of suspected community-acquired viral encephalitis are started empirically on aciclovir until the cause is determined.[13] As most cases of sporadic viral encephalitis are secondary to herpes simplex virus, this is good clinical practice supported by biopsy-proven randomised controlled trials, and it reduces mortality.​[48][93]

Primary options

aciclovir: 10 mg/kg intravenously every 8 hours for 10-21 days

Plus – supportive care

Back
INITIAL
|
immunocompetent host: suspected viral aetiology
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All suspected cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]​​​

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management has failed to control elevated ICP, and for impending uncal herniation.[100] This can be considered no matter the aetiology of encephalitis; however, most outcome data have been published for viral encephalitis.

immunocompromised host: suspected viral aetiology

1st line – combination antiviral therapy

Back
INITIAL
|
immunocompromised host: suspected viral aetiology
1st line – 

combination antiviral therapy

If cytomegalovirus (CMV) encephalitis is suspected in an immunocompromised patient, ganciclovir and foscarnet are given with aciclovir until herpes simplex virus (HSV) polymerase chain reaction (PCR) is available.

Ganciclovir and foscarnet are given for 14 to 21 days unless nephrotoxicity or myelotoxicity occurs in which case one of the agents should be stopped.[114]

Aciclovir is given until HSV infection can be excluded (HSV PCR). In some cases, magnetic resonance imaging findings and clinical features strongly suggest a diagnosis of CMV encephalitis, so aciclovir may not be necessary. If a diagnosis of CMV infection is established, then aciclovir should be discontinued as it is not effective against this virus.

Primary options

ganciclovir: 5 mg/kg intravenously every 12 hours for 14-21 days

and

foscarnet: 60 mg/kg intravenously every 8 hours; or 90 mg/kg intravenously every 12 hours for 14-21 days

and

aciclovir: 10 mg/kg intravenously every 8 hours for 21 days

Plus – supportive care

Back
INITIAL
|
immunocompromised host: suspected viral aetiology
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All suspected cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management has failed to control elevated ICP, and for impending uncal herniation.[100] This can be considered no matter the aetiology of encephalitis; however, most outcome data have been published for viral encephalitis.

ACUTE

confirmed herpes simplex virus (HSV) encephalitis

1st line – aciclovir

Back
ACUTE
|
confirmed herpes simplex virus (HSV) encephalitis
1st line – 

aciclovir

Confirmed HSV encephalitis should be treated with aciclovir.[13] This is supported by biopsy-proven randomised controlled trials, showing reduced mortality.​[48][93]

Immunosuppressed patients should receive a full 21 days of treatment.

The clinician should consider repeating the lumbar puncture at day 12 to 13 with repeat polymerase chain reaction to guide the decision of whether to stop the treatment or to continue up to 21 days.

Primary options

aciclovir: 10 mg/kg intravenously every 8 hours for 14-21 days

Plus – supportive care

Back
ACUTE
|
confirmed herpes simplex virus (HSV) encephalitis
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100] In some cases of HSV encephalitis, surgical decompression has been shown to improve outcome.[101]

To date, there is limited data regarding the benefit of adjuvant corticosteroids for the treatment of viral encephalitis (in the absence of elevated ICP) and guidelines do not support their routine use.​[13][48][97]

confirmed varicella zoster virus (VZV) encephalitis

1st line – aciclovir

Back
ACUTE
|
confirmed varicella zoster virus (VZV) encephalitis
1st line – 

aciclovir

Confirmed VZV encephalitis should be treated with aciclovir.[115]

Primary options

aciclovir: 10 mg/kg intravenously every 8 hours for 14 days

Plus – supportive care

Back
ACUTE
|
confirmed varicella zoster virus (VZV) encephalitis
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100]

To date, there is limited data regarding the benefit of adjuvant corticosteroids for the treatment of viral encephalitis (in the absence of elevated ICP) and guidelines do not support their routine use.​[13][48][97]

confirmed cytomegalovirus (CMV) encephalitis

1st line – ganciclovir plus foscarnet

Back
ACUTE
|
confirmed cytomegalovirus (CMV) encephalitis
1st line – 

ganciclovir plus foscarnet

Confirmed CMV encephalitis should be treated with ganciclovir plus foscarnet.[115]

Primary options

ganciclovir: 5 mg/kg intravenously every 12 hours for 14-21 days initially, followed by a maintenance dose of 5 mg/kg/day given once daily for 7 days/week or 6 mg/kg/day given once daily for 5 days/week

and

foscarnet: 60 mg/kg intravenously every 8 hours; or 90 mg/kg intravenously every 12 hours for 14-21 days

Plus – supportive care

Back
ACUTE
|
confirmed cytomegalovirus (CMV) encephalitis
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][90]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100]

confirmed Epstein-Barr virus (EBV) encephalitis

1st line – aciclovir, ganciclovir, or cidofovir

Back
ACUTE
|
confirmed Epstein-Barr virus (EBV) encephalitis
1st line – 

aciclovir, ganciclovir, or cidofovir

Aciclovir is first line in suspected viral encephalitis, but once the diagnosis of EBV is confirmed, ganciclovir or cidofovir are possible alternatives.[95] There are limited data to guide therapy of EBV central nervous system infections. No controlled studies have been conducted. There are case reports that suggest ganciclovir improves outcomes.

Primary options

aciclovir: 10 mg/kg intravenously every 8 hours for 14 days

OR

ganciclovir: consult specialist for guidance on dose

Secondary options

cidofovir: consult specialist for guidance on dose

Plus – supportive care

Back
ACUTE
|
confirmed Epstein-Barr virus (EBV) encephalitis
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Immunoglobulin can be used for agammaglobulinaemic patients and neonates with sepsis syndrome.[75] Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86] For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100] In some cases of herpes simplex virus encephalitis, surgical decompression has been shown to improve outcome.

To date, there is limited data regarding the benefit of adjuvant corticosteroids for the treatment of viral encephalitis (in the absence of elevated ICP) and guidelines do not support their routine use.​[13][48][97]

confirmed herpes B encephalitis

1st line – ganciclovir, aciclovir, or valaciclovir

Back
ACUTE
|
confirmed herpes B encephalitis
1st line – 

ganciclovir, aciclovir, or valaciclovir

Intravenous therapy may be preferable in acute central nervous system (CNS) disease. However, the efficacy of the intravenous approach has not been studied. Ganciclovir may be preferable as a first option in CNS disease.[95] Duration of treatment should be decided in conjunction with an infectious disease specialist.

There is also expert opinion that life-long suppression of latent infection with valaciclovir may be considered.[95]

Primary options

ganciclovir: 5 mg/kg intravenously every 12 hours for 14-21 days

Secondary options

aciclovir: 10 mg/kg intravenously every 8 hours for 14-21 days

OR

valaciclovir: 1 g orally every 8 hours for 14 days

Plus – supportive care

Back
ACUTE
|
confirmed herpes B encephalitis
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on World Health Organization guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100]

confirmed human herpes 6 encephalitis

1st line – ganciclovir or foscarnet

Back
ACUTE
|
confirmed human herpes 6 encephalitis
1st line – 

ganciclovir or foscarnet

Ganciclovir or foscarnet should be used in immunocompromised patients.[115]

Use of these agents in immunocompetent patients can also be considered, but there are no good data on their effectiveness.[115]

Primary options

ganciclovir: 5 mg/kg intravenously every 12 hours for 14-21 days

OR

foscarnet: 60 mg/kg intravenously every 8 hours; or 90 mg/kg intravenously every 12 hours for 14-21 days

Plus – supportive care

Back
ACUTE
|
confirmed human herpes 6 encephalitis
Plus – 

supportive care

Treatment recommended for ALL patients in selected patient group

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]​ For certain opportunistic infections, such as cryptococcus, antiretroviral therapy should be delayed based on WHO guidelines.[87][88][89]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90] ​Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100]

confirmed non-herpes virus aetiology

1st line – supportive care ± antiviral therapy

Back
ACUTE
|
confirmed non-herpes virus aetiology
1st line – 

supportive care ± antiviral therapy

For cases where a specific virus has been isolated and specific antiviral treatment is available, treatment is directed towards the underlying isolated virus.

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis. Antiretroviral therapy is an important treatment in all cases of HIV-associated encephalitis (whether due to HIV itself or to an opportunistic infection); in CD8 encephalitis, patients respond well to corticosteroids.[85][86]

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management (corticosteroids, mannitol) has failed to control elevated ICP, and for impending uncal herniation.[100]

non-viral aetiology

1st line – supportive care + treatment of underlying aetiology

Back
ACUTE
|
non-viral aetiology
1st line – 

supportive care + treatment of underlying aetiology

Aetiology is often unknown, and therefore no specific treatment options exist for the majority of cases. However, for cases where a diagnosis is reasonably certain, treatment is directed towards the underlying offending agent, with appropriate anti-infective measures in bacterial, fungal, or parasitic infections. If cerebrospinal fluid studies do not show a clear infectious aetiology, immunotherapy should be considered.[103][104] The decision to fight infection or suppress the immune system needs to be balanced in each case.

All cases of encephalitis should be admitted and fully evaluated. Some patients with milder symptoms and signs can be managed in a regular nursing unit, with access to an intensive care unit (ICU) bed if needed. All other patients, and in particular those with complications (e.g., significant electrolyte abnormalities, strokes, raised intracranial pressure [ICP], cerebral oedema, coma, seizures activity, or status epilepticus) should be managed in an ICU, preferably a neuro-intensive care unit.[33][84]

Supportive care is the cornerstone of treatment in most cases. This may include endotracheal intubation and mechanical ventilation, circulatory and electrolyte support, prevention and management of secondary bacterial infections, deep venous thrombosis prophylaxis, and gastrointestinal (ulcer) prophylaxis.

In patients with elevated ICP, initial measures are elevation of head of bed to 30° to 45°, avoiding compression of the jugular veins, and brief episodes of hyperventilation.[90]​ Subsequently, hyperosmolar therapy with mannitol boluses or hypertonic saline can be used to decrease ICP.[91]

Shunting or surgical decompression (by craniectomy) is indicated in some cases where medical management has failed to control elevated ICP, and for impending uncal herniation.[100] This can be considered no matter the aetiology of encephalitis; however, most outcome data have been published for viral encephalitis.

Plus – immune-modulating therapy

Back
ACUTE
|
non-viral aetiology
|
autoimmune/paraneoplastic encephalitis
Plus – 

immune-modulating therapy

Treatment recommended for ALL patients in selected patient group

When the initial evaluation does not support an infectious cause and an autoimmune cause is suspected, aggressive immunotherapy with intravenous corticosteroids, immunoglobulin, or plasma exchange should be considered.[1]​[102]​​​​ Cases with persistent altered mental status not responsive to first-line therapy should be treated with rituximab and/or cyclophosphamide.​​[31][51][52][103][104]​​

In most newly diagnosed cases, it is difficult to determine clinically whether autoimmune encephalitis is antibody or cell-mediated before the antibody results are available.[105] Some clinical clues may help the clinician come to a preliminary hypothesis regarding aetiology (e.g., leucine-rich glioma-inactivated 1 antibodies are associated with faciobrachial dystonic seizures, such as rapid jerks of the face and/or ipsilateral arm and shoulder, while patients with known or increased cancer risk are more likely to have cell-mediated autoimmune encephalitis).[39] Based on these clues, clinicians may decide to use rituximab or cyclophosphamide as a second-line agent if antibody results are delayed, or if there is no access to antibody testing.[39]

Rituximab is now generally preferred over cyclophosphamide if monotherapy is used in highly suspected antibody-mediated autoimmunity (e.g., N-methyl-D-aspartate receptor-antibody encephalitis).[106] Rituximab is less toxic than cyclophosphamide.[39][107] Cyclophosphamide may be considered if rituximab is contraindicated or not available in these cases.[106] Some patients may be treated with a combination of rituximab and cyclophosphamide.[105]

Cyclophosphamide can be considered in known, or highly suspected, cell-mediated autoimmunity (e.g., classical paraneoplastic syndrome), since rituximab may not be as effective for cell-mediated inflammation.[39] Some patients may be treated with a combination of cyclophosphamide and rituximab.[105]

Primary options

methylprednisolone sodium succinate: 1000 mg intravenously once daily for 3-5 days

OR

normal immunoglobulin human: 2 g/kg intravenously given in divided doses over 4-5 days

Secondary options

rituximab: consult specialist for guidance on dose

and/or

cyclophosphamide: consult specialist for guidance on dose

Consider – treatment of underlying malignancy

Back
ACUTE
|
non-viral aetiology
|
autoimmune/paraneoplastic encephalitis
Consider – 

treatment of underlying malignancy

Additional treatment recommended for SOME patients in selected patient group

Management of autoimmune encephalitis associated with malignancy (paraneoplastic encephalitis) involves diagnostic testing and treatment of the underlying tumour. However, treatment directed towards the paraneoplastic syndrome should not be delayed by failure to identify the underlying tumour, as there is a risk for development of permanent sequelae.

Oophorectomy is indicated as an acute treatment if ovarian teratomas are present.[9] Tumour resection is associated with a faster rate of recovery and reduced relapse rate.[31][108]

Plus – immune-modulating therapy

Back
ACUTE
|
non-viral aetiology
|
acute disseminated encephalomyelitis
Plus – 

immune-modulating therapy

Treatment recommended for ALL patients in selected patient group

High-dose corticosteroids are advocated by experts.[109][110]

In cases where corticosteroids fail to show benefit, plasma exchange or immunoglobulin can be considered.​[109]​​ Plasma exchange is performed in consultation with a haematologist. Immunoglobulin has been shown to reduce duration of the illness.

Primary options

methylprednisolone sodium succinate: 1000 mg intravenously once daily for 3-5 days

Secondary options

normal immunoglobulin human: 2 g/kg intravenously given in divided doses over 4-5 days

Plus – targeted antimicrobial treatment

Back
ACUTE
|
non-viral aetiology
|
confirmed syphilis encephalitis
Plus – 

targeted antimicrobial treatment

Treatment recommended for ALL patients in selected patient group

The microbiology laboratory should be alerted if unusual organisms are suspected (e.g., Treponema pallidum), for which special microbiological procedures are necessary. Neurosyphilis presenting with features of encephalitis is a rare form of central nervous system infection by Treponema pallidum.[116][117]​​ Targeted therapy is available if isolated. Consult a specialist for guidance on further management. See Syphilis infection.​

Plus – targeted antimicrobial treatment

Back
ACUTE
|
non-viral aetiology
|
confirmed listeria encephalitis
Plus – 

targeted antimicrobial treatment

Treatment recommended for ALL patients in selected patient group

The microbiology laboratory should be alerted if unusual organisms are suspected (e.g., Listeria), for which special microbiological procedures are necessary. Listeria encephalitis is rare but carries a high mortality rate.[118] ​While listeria meningitis is more common, patients with high risk factors may also develop meningoencephalitis. Targeted therapy is available if isolated. Consult a specialist for guidance on further management. See Listeriosis.

Plus – targeted antimicrobial treatment

Back
ACUTE
|
non-viral aetiology
|
confirmed Mycoplasma pneumoniae encephalitis
Plus – 

targeted antimicrobial treatment

Treatment recommended for ALL patients in selected patient group

The microbiology laboratory should be alerted if unusual organisms are suspected (e.g., Mycoplasma species), for which special microbiological procedures are necessary. M pneumoniae is commonly attributed to upper and lower respiratory tract infections in paediatric patients and central nervous system symptoms may reflect extrapulmonary infections or post-infectious encephalitis.[119]​ Targeted therapy is available if isolated. Consult a specialist for guidance on further management.

Consider – immune-modulating therapy

Back
ACUTE
|
non-viral aetiology
|
confirmed Mycoplasma pneumoniae encephalitis
Consider – 

immune-modulating therapy

Additional treatment recommended for SOME patients in selected patient group

Immunomodulatory treatments have been hypothesised to benefit these patients based on the proposed antibody response to the pathogen. Immunotherapy with intravenous corticosteroids, immunoglobulin, or plasma exchange is typically considered as a first-line option.

Case reports suggest possible benefit.

Plasma exchange is performed in consultation with a haematologist.

Primary options

methylprednisolone sodium succinate: 1000 mg intravenously once daily for 3-5 days

OR

normal immunoglobulin human: 2 g/kg intravenously given in divided doses over 4-5 days

Consider – targeted antimicrobial treatment

Back
ACUTE
|
non-viral aetiology
|
confirmed Rocky Mountain spotted fever encephalitis
Consider – 

targeted antimicrobial treatment

Additional treatment recommended for SOME patients in selected patient group

The microbiology laboratory should be alerted if unusual organisms are suspected (e.g., Rickettsia rickettsii), for which special microbiological procedures are necessary. Neurological involvement including encephalitis in patients with Rocky Mountain spotted fever is associated with higher mortality rates.[120]​ Targeted therapy is available if isolated. Consult a specialist for guidance on further management. See Rocky Mountain spotted fever.

ONGOING

convalescent phase: all aetiologies

1st line – rehabilitation

Back
ONGOING
|
convalescent phase: all aetiologies
1st line – 

rehabilitation

Starts once the acute, life-threatening phase has passed. It can begin with the initial evaluation during acute hospitalisation by the rehabilitation medicine personnel and be continued in various in- or outpatient settings.

The need for rehabilitation is varied and depends on the functional deficits present in the individual patient. It can include cognitive/behavioural rehabilitation and motor/ambulatory rehabilitation.[111]

The most frequently used non-pharmacological treatments to treat dementia and apathy following encephalitis are music therapy and cognitive rehabilitation.[113]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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