Progressive encephalomyelitis with rigidity: a case report.
OBJECTIVE
The 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.


METHODS
Case report.


RESULTS
A 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.


CONCLUSIONS
For this patient with PER, corticosteroid therapy was a dramatically effective and life-saving treatment, although initiated rather late in the course of the disease.
Baraba
Ranka
R
Department of Neurology, General Hospital "Sveti Duh", Zagreb, Croatia. rankabaraba@net.hr
Jusić
Anica
A
Sruk
Ana
A
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001933:Brain Stem / Q000473:pathology; D004679:Encephalomyelitis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009127:Muscle Rigidity / Q000188:drug therapy / Q000209:etiology*; D013116:Spinal Cord / Q000473:pathology
D000305:Adrenal Cortex Hormones
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