• J Spinal Cord Med · Jan 2010

    Case Reports

    Progressive encephalomyelitis with rigidity: a case report.

    • Ranka Baraba, Anica Jusić, and Ana Sruk.
    • Department of Neurology, General Hospital "Sveti Duh", Zagreb, Croatia. rankabaraba@net.hr
    • J Spinal Cord Med. 2010 Jan 1;33(1):73-6.

    Background/ObjectiveThe most prominent clinical features of progressive encephalomyelitis with rigidity (PER) are painful spasms and rigidity accompanied by clinical signs of brainstem and spinal cord involvement. In initial reports, PER had fatal outcome. Later, clinical improvement related to corticosteroid therapy has been described in some cases. The objective of this study was to signify a reputed clinical significance of corticosteroid therapy in PER.MethodsCase report.ResultsA 50-year-old man developed progressive syndrome of tonic extensor spasms. Magnetic resonance imaging (MRI) showed areas of signal changes in cervical spinal cord and lower brainstem, whereas cerebrospinal fluid analysis indicated subacute encephalomyelitis. His condition dramatically improved on oral corticosteroid therapy. Clinical improvement was accompanied by normalization of MRI findings.ConclusionFor this patient with PER, corticosteroid therapy was a dramatically effective and life-saving treatment, although initiated rather late in the course of the disease.

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