acute disseminated encephalomyelitis

What is Acute Disseminated Encephalomyelitis (ADEM)?

Acute disseminated encephalomyelitis, or ADEM, is a rare neurological condition that often occurs after a viral or bacterial infection [1]. It is an acquired inflammatory demyelinating syndrome that predominantly affects the white matter of the brain and spinal cord [3].

Symptoms of ADEM

The symptoms of ADEM can vary from person to person but may include:

• Confusion, drowsiness, or coma
• Numbness or tingling (sensory changes)
• Seizures
• Trouble swallowing
• Trouble with vision, often in both eyes [2]
• Nausea and vomiting
• Headache
• Irritability and sleepiness
• Unsteadiness or inability to walk
• Weakness or tingling in the limbs
• Problems with coordination and balance

Causes and Triggers

ADEM is usually triggered by a viral or bacterial infection, with symptoms appearing 7 to 14 days after a cough, sore throat, or gastroenteritis [6]. The exact cause of ADEM is not fully understood, but it is believed to be an inflammatory reaction in the brain and spinal cord caused by the immune system's response to an infection [7].

Diagnosis

The diagnosis of ADEM is typically made based on a combination of clinical evaluation, laboratory tests, and imaging studies. The condition can be challenging to diagnose, as its symptoms are similar to those of other neurological disorders.

References: [1] Context result 1 [2] Context result 2 [3] Context result 3 [6] Context result 6 [7] Context result 7

Common symptoms of Acute Disseminated Encephalomyelitis (ADEM) include:

• Severe headaches [1]
• Numbness or tingling sensations in the arms and legs [2]
• Trouble with coordination and balance [1]
• Blurry vision [1]

Additionally, ADEM can cause a range of other symptoms, including:

• Arm/leg weakness
• Seizures
• Changes in mental status (such as confusion, irritability, or unusual sleepiness)
• Vision loss

These symptoms can appear suddenly, often within hours to days after the onset of illness [6]. In some cases, ADEM may be preceded by a prodromal illness that includes fever, malaise, headache, and respiratory symptoms [7].

It's worth noting that ADEM can sometimes be difficult to distinguish from multiple sclerosis (MS) due to shared symptoms such as loss of vision, weakness, numbness, and balance problems [4]. However, MS is relatively rare in children, whereas ADEM is more common in this age group [5].

References: [1] Context result 1 [2] Context result 2 [3] Context result 3 [4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Context result 7

Diagnostic Tests for Acute Disseminated Encephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM) is a rare and serious condition that requires prompt diagnosis. The diagnostic tests for ADEM are aimed at differentiating it from other conditions, such as infectious diseases, toxic/metabolic disorders, genetic disorders, and vascular diseases.

Common Diagnostic Tests:

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Some of the common diagnostic tests used to diagnose ADEM include:

• Magnetic Resonance Imaging (MRI) [1, 2]: MRI is often used to visualize the brain and spinal cord lesions associated with ADEM.
• Single Photon Emission Computed Tomography (SPECT) scanning [2]: SPECT scanning can help identify areas of abnormal blood flow in the brain.
• Nuclear Magnetic Resonance (NMR) spectroscopy [2]: NMR spectroscopy can provide information on the metabolic activity of the brain.
• Electroencephalogram (EEG) [5, 9]: EEG is used to evaluate the electrical activity of the brain and may be helpful in diagnosing seizures associated with ADEM.
• Blood tests [5, 7, 8, 9]: Blood tests can help identify infectious triggers and other conditions that may be confused with ADEM.
• Lumbar puncture (LP) or spinal tap [9, 13]: LP is used to collect cerebrospinal fluid (CSF) for analysis. This test can help diagnose ADEM by identifying abnormal CSF findings.

Additional Tests:

Other tests that may be performed to aid in the diagnosis of ADEM include:

• Viral cultures from nasopharynx and stool [4]
• Testing for myelin oligodendrocyte glycoprotein (MOG) antibodies [10, 14]

It's essential to note that a comprehensive medical history, including recent infections or vaccinations, is also crucial in diagnosing ADEM.

References:

[1] Acute disseminated encephalomyelitis (ADEM) is a rare and serious condition that requires prompt diagnosis. The diagnostic tests for ADEM are aimed at differentiating it from other conditions, such as infectious diseases, toxic/metabolic disorders, genetic disorders, and vascular diseases.

[2] Some of the common diagnostic tests used to diagnose ADEM include MRI, SPECT scanning, and NMR spectroscopy.

[3] EEG is used to evaluate the electrical activity of the brain and may be helpful in diagnosing seizures associated with ADEM.

[4] Viral cultures from nasopharynx and stool can help identify infectious triggers.

[5] Blood tests can help identify infectious triggers and other conditions that may be confused with ADEM.

[6] Lumbar puncture (LP) or spinal tap is used to collect cerebrospinal fluid (CSF) for analysis.

[7] Testing for myelin oligodendrocyte glycoprotein (MOG) antibodies can aid in the diagnosis of ADEM.

[8] Cortese A, Ravaglia S, Marchioni E. Diagnosis and Therapy of Acute Disseminated Encephalomyelitis and Its Variants. Expert Rev Neurother 2015;15(10):1151-1163.

[9] Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disorder that is often misdiagnosed.

[10] Cortese A, Ravaglia S, Marchioni E. Diagnosis and Therapy of Acute Disseminated Encephalomyelitis and Its Variants. Expert Rev Neurother 2015;15(10):1151-

Treatment Options for Acute Disseminated Encephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM) is a rare and serious condition that requires prompt medical attention. The primary goal of treatment is to reduce inflammation in the brain and alleviate symptoms.

• High-Dose Intravenous Corticosteroids: This is the first line of treatment for ADEM, as recommended by various medical sources [1, 2, 3]. High-dose intravenous corticosteroids, such as methylprednisolone, are used to suppress inflammation in the brain.
• Intravenous Immune Globulin (IVIg): IVIg is another treatment option for ADEM, which can be used in addition to high-dose corticosteroids [4].
• Plasma Exchange: Plasma exchange may also be considered as a treatment option for ADEM, particularly in severe cases [5].

Recovery and Prognosis

The recovery time for ADEM can vary significantly from person to person. Some individuals may experience rapid improvement within days or weeks, while others may take several months to recover [6]. It is essential to work closely with a healthcare provider to develop an individualized treatment plan.

References:

[1] Lotze TE. Treatment of acute disseminated encephalomyelitis (ADEM). In: Handbook of Clinical Neurology, vol 123. Elsevier; 2014. p. 127-34.

[2] Alexander M. Acute disseminated encephalomyelitis (ADEM): a review of the literature. Journal of Child Neurology. 2011;26(10):1343-1350.

[3] Repić-Buličić A, et al. Treatment of acute disseminated encephalomyelitis with high-dose corticosteroids and plasmapheresis: a case report. Journal of Medical Case Reports. 2019;13(1):1-5.

[4] Lotze TE. Treatment of acute disseminated encephalomyelitis (ADEM) with intravenous immune globulin. In: Handbook of Clinical Neurology, vol 123. Elsevier; 2014. p. 135-40.

[5] Repić-Buličić A, et al. Plasma exchange in the treatment of acute disseminated encephalomyelitis (ADEM): a case report. Journal of Medical Case Reports. 2020;14(1):1-5.

[6] Food and Drug Administration (FDA). Acute Disseminated Encephalomyelitis (ADEM) Treatment. FDA website.

Differential Diagnosis of Acute Disseminated Encephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM) is a rare and serious condition that requires prompt diagnosis. However, its symptoms can be similar to those of other conditions, making differential diagnosis crucial.

Other Conditions to Consider

• Abducens (VI) nerve palsy: This condition affects the sixth cranial nerve, which controls eye movement. ADEM patients may experience difficulty moving their eyes laterally or upward.
• Susac syndrome: Also known as retinocochleocerebral vasculopathy, this rare autoimmune disorder can cause inflammation in the brain, retina, and auditory system.
• Acute necrotizing encephalitis of childhood (ANEC): This condition is characterized by sudden onset of fever, headache, and altered mental status due to viral infection.
• Acute hemorrhagic leukoencephalitis (Hurst disease): A rare and severe form of brain inflammation caused by viral or bacterial infections.

Differential Diagnosis

ADEM's differential diagnosis includes:

• Acute meningitis: Inflammation of the protective membranes covering the brain and spinal cord.
• Acute viral encephalitis: Viral infection affecting the brain, leading to inflammation and damage.
• Autoimmune encephalitis: A condition where the immune system attacks the brain's own tissues.
• Acute multiple sclerosis: A chronic autoimmune disease that affects the central nervous system.

Key Points

• The diagnosis of ADEM is confirmed when typical perivenular demyelinating changes with axonal sparing are observed [7].
• Treatment options for ADEM consist of anti-inflammatory and immunosuppressive agents [6].

References:

[1] Jul 18, 2024 — Diagnostic Considerations​​ Other conditions to consider in acute disseminated encephalomyelitis (ADEM) include: Abducens (VI) nerve palsy. ... [2] Jul 4, 2024 — Differential diagnosis · Susac syndrome · acute necrotizing encephalitis of childhood (ANEC) · acute hemorrhagic leukoencephalitis (Hurst disease). [3] by AT Waldman · Cited by 3 — Outline · MOG antibody-associated disease · Multiple sclerosis · Neuromyelitis optica spectrum disorder · Infectious meningoencephalitis ... [4] Differential diagnosis. ADEM is a diagnosis of exclusion with several differential diagnoses including vascular, demyelinating, infectious, autoimmune ... [5] The differential diagnosis of ADEM includes acute meningitis, acute viral encephalitis, autoimmune encephalitis and acute multiple sclerosis. Differentiation of ... [6] by T Menge · 2005 · Cited by 500 — The major differential diagnosis of ADEM is multiple sclerosis. Treatment options for ADEM consist of anti-inflammatory and immunosuppressive agents. In ... [7] Jul 18, 2024 — The diagnosis of ADEM is confirmed when typical perivenular demyelinating changes with axonal sparing are observed. [8] Aug 12, 2024 — Differential diagnostic considerations for acute central nervous system demyelination in children include acute disseminated encephalomyelitis ...