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Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
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Progressive encephalomyelitis with rigidity: a case report.

Ranka Baraba1, Anica Jusić, Ana Sruk

  • 1Department of Neurology, General Hospital "Sveti Duh", Zagreb, Croatia. rankabaraba@net.hr

The Journal of Spinal Cord Medicine
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Summary

Corticosteroid therapy offers a life-saving treatment for progressive encephalomyelitis with rigidity (PER). This case report highlights its dramatic effectiveness in reversing severe neurological symptoms and improving MRI findings.

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Area of Science:

  • Neurology
  • Immunology

Background:

  • Progressive encephalomyelitis with rigidity (PER) is a rare neurological disorder characterized by painful spasms and rigidity.
  • Historically, PER has had a high mortality rate, with limited treatment options.
  • Previous case studies suggested potential benefits of corticosteroid therapy.

Observation:

  • A 50-year-old male presented with a progressive syndrome of tonic extensor spasms.
  • MRI revealed signal abnormalities in the cervical spinal cord and brainstem.
  • Cerebrospinal fluid analysis confirmed subacute encephalomyelitis.

Findings:

  • The patient experienced dramatic improvement following oral corticosteroid administration.
  • Clinical recovery was correlated with the normalization of MRI findings.
  • Corticosteroid therapy proved to be a highly effective, life-saving intervention despite delayed initiation.

Implications:

  • This case underscores the significant clinical efficacy of corticosteroids in managing PER.
  • Corticosteroid therapy should be strongly considered for patients diagnosed with PER.
  • Early and aggressive treatment may lead to better outcomes and prevent irreversible neurological damage.