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Acute disseminated encephalomyelit (ADEM)

Definition 

Acute disseminated encephalomyelit (ADEM) is an autoimmune inflammatory disease of the central nervous system

  • The disease is characterized by demyelination in the brain and spinal cord
  • The disease is monophasic ally, as opposed to multiple sclerosis (MS)
  • Symptoms are headache, consciousness effects, confusion, visual disturbances and sometimes convulsions. In addition focal neurological symptoms varying depending on the affected areas of the brain and spinal cord, for example hyperkinesia, palsy, loss of sensibility, bladder is exposed to more
  • The disease occurs mainly in children and young adults

Occurrence 

Epidemiology

  • Acute disseminated encephalomyelit hits mainly children, but adults can also get the disease
  • The incidence in Denmark is 20-40 cases annually
  • 65-85% of cases occur in the winter months when the incidence of viral infections is highest

Etiology and pathogenesis

  • Acute disseminated encephalomyelit is characterized by widespread demyelination in the brain and spinal cord secondary to autoimmune inflammation 
  • Demarcations with relapsing-remitting multiple sclerosis, relapse remitting MS and neuromyelitis optica is difficult
  • Approximately 50-85% of cases occurring after infection or vaccination
  • Suspicion of vaccination as a possible cause has diminished considerably after the production of vaccines is changed  
  • Corona virus, Coxsackie B, dengue, hepatitis A and C virus, herpes simplex virus, measles virus, paramyxovirus, varicella, rubella, Epstein-Barr virus can all cause acute disseminated encephalomyelitis
  • Bacteria such as streptococcus, Mycoplasma, Legionella, Chlamydia, Borrelia, Richettsie and parasite plasmodium vivax may rarely cause acute disseminated encephalomyelit

Acute disseminated encephalomyelit

Pathophysiology

  • MRI of the brain and spinal cord often show widespread demyelination  
  • The existence of a damaged blood-brain barrier
  • Cerebrospinal fluid often show lymphocytosis and increased protein content, while the intrathecal synthesis of oligoclonal bands are less frequently 
  • Follow-up MRI scan of the brain and spinal cord show complete or partial remission of plaques in the white matter in 90% and no new plaque

Read More About: Creutzfeldt-Jakob Disease?

Predisposing factors

  • No one certainly known
  • For measles vaccination has reduced the incidence of acute disseminated encephalomyelit

ICPC-2

  • N71 meningitis / encephalitis
  • N99 Neurological disease IKA

ICD-10

  • G04 Cerebral and spinal cord
  • G040 Acute disseminated encephalitis
  • G048 Other encephalitis, myelitis el encephalomyelitis
  • G049 Encephalitis, myelitis el encephalomyelitis UNS

Diagnosis

  • Internationally accepted diagnostic criteria are not currently
  • Acute disseminated encephalomyelit ally is a monophasic autoimmune inflammatory disease with secondary demyelininisering in the brain and spinal cord
  • Symptoms are headache, consciousness effects, confusion, weakness, personality disorders, depression, psychosis and delirium
  • Focal neurological symptoms depend on the affected areas of the brain and spinal cord, such as visual disturbances secondary to optius neuritis, hyperkinesia, palsy, sensory loss, convulsions, and bladder impact
  • The disease occurs mainly in children and young adults
  • Typically clinic and affirmative findings on MRI of the brain and spinal cord

Differential

  • Multiple sclerosisis typically a relapsing disease in young adults, while the acute disseminated encephalomyelit typically a monophasic disease
    • Cramps and awareness impact is rarely at first relapse of multiple sclerosis
    • Follow-up MRI scan of the brain and spinal cord showing full or partial recurrences plaques in the white matter of 90% and no new plaques in patients with acute disseminated encephalomyelit, while all MS patients have new plaques in the white matter  
    • The disease is monophasic ally, as opposed to multiple sclerosis
  • Neuromyelitis optica characterized by bilateral optic neuritis, where 76% have antibodies against aquaporin-4 water channels4
  • Guillain Barré syndrome  
  • viral meningoencephalitis
  • Other tious neurological syndromes with degeneration of peripheral nervous system and / or relapsing or chronic progression of symptoms

Clinical findings

  • Prodrome
    • Child / young adult with prior infection disease
    • The usual cause of acute disseminated encephalomyelitis 1-20 days after infection, as in the vast majority of cases has been a febrile illness
    • In 10% of cases a lack of a clear prodrome
  • Symptoms 
    • Headaches, irritability, weakness, meningism, consciousness influence, personality disorders, depression, psychosis, delirium, coma, convulsions and fever new episode is seen in 65-85% 
    • Focal neurological deficits depends on the affected areas and are seen in 35-60%.Frequently it’s ataxia, loss of vision due to opticusneurit, hemiplegia, paraplegia, sensory loss and impact of urination
    • The condition progresses over days up to 4 weeks

Investigations

Acute disseminated encephalomyelit

  • Cerebrospinal Fluid
    • Examined for cells, protein, sugar and oligoclonal bands
    • Protein is often elevated 0.5-1.5 g / L
    • Lymphocytosis is frequent 
    • Oligoclonal bands seen in up to 30%
  • MRI of the brain and spinal cord
    • Detection of plaques in the white matter
    • Follow-up MRI shows complete or partial remission of plaques in the white matter in 90% and no new plaque

When the patient should refer?

  • On suspicion of acute disseminated encephalomyelit patient should be admitted to the neurology department for further investigation

Treatment

Medical treatment

  • The treatment is not proven in randomized controlled trials. It is believed by treatment inhibits inflammation and accelerates remission
  • Intravenous methylprednisolone- children 20-30 mg / kg / day and adult 1g / day for 3-5 days, followed by 1-2 mg / kg / day for 1-2 weeks and then gradually weaning of 2-6 weeks 
  • plasmapheresis
  • intravenous immunoglobulin

Physical therapy in acute phase

What can the patient do?

  • No restrictions in physical activity
  • Once the acute phase is over, it is important to retrain motor functions etc.

Course, complications and prognosis

Progress

  • The disease is usually self-limiting in days for 2-4 weeks, but 3-5% of recurrent acute elapsed
  • subsequent stabilization
  • Remission extend over months, and it is often good even after severe illness
  • The mortality rate is less than 5% of children and 8-25% of young adults; 5 
  • Approximately 25-30% of adults develop since multiple sclerosis

Complications

  • Neurological sequelae in the form of visual impairment, motor and autonomic symptoms, intellectual defects and epilepsy seen in 35% of patients after the acute phase. At most, these sequelae, however, disappeared within the first year after disease onset

Forecast

  • The prognosis is generally good. Most achieve complete remission or less sequelae such as easier visual impairment
  • Remission seem independent of the severity of the disease
    • Full remission seen in children who have been blind, comatose and quadriplegic
    • Children under 2 years, the lowest remission rates, but also among those being most healthy
    • 10-20% incurs term sequelae
    • Relapse occurs in about 5%
    • Approximately 25-30% of adults develop since multiple sclerosis
    • The mortality rate is less than 5% of children and 8-25% of young adults; 

 

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Updated: February 17, 2017 — 11:40 am

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