1976
DOI: 10.1093/brain/99.1.27
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Progressive Encephalomyelitis With Rigidity

Abstract: Two cases of encephalomyelitis are described in which the major clinical manifestation was muscular rigidity and stimulus-sensitive muscular spasms. It is suggested, from pathological evidence, that this rigidity was of spinal origin, and that this disorder is a rare but recognizable entity. Comparison is made with previously reported cases of rigidity of spinal origin, including encephalitis lethargica, and with 'subacute myoclonic spinal neuronitis' and the 'stiff man syndrome.'

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Cited by 166 publications
(60 citation statements)
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“…Since the earliest descriptions of this disorder, 3 it has been variably termed progressive encephalomyelitis with rigidity and myoclonus (PERM) 4 or stiff-person syndrome plus (SPS-Plus). 5 This syndrome has been previously associated with GlyR but not NMDAR antibodies.…”
Section: Discussionmentioning
confidence: 99%
“…Since the earliest descriptions of this disorder, 3 it has been variably termed progressive encephalomyelitis with rigidity and myoclonus (PERM) 4 or stiff-person syndrome plus (SPS-Plus). 5 This syndrome has been previously associated with GlyR but not NMDAR antibodies.…”
Section: Discussionmentioning
confidence: 99%
“…Although the cause remains unknown for all of them, the gene responsible for idiopathic torsion dystonia (1TD)Q in some families was mapped to chromosome band 9q34, [16][17][18][19] and the gene causing X-linked dystonia-parkinsonism14J5 was mapped to chromosome band Xq13. [20][21][22] We recently evaluated a large Indiana family with a new autosomal dominant syndrome affect- …”
mentioning
confidence: 99%
“…Disease relapses may occur in 15-25% of cases, especially in non-paraneoplastic cases without adequate immunotherapy during their previous encephalitic episode 21,24,25 . As far as investigations are concerned, CSF typically reveals an early lymphocytic pleocytosis followed by intrathecal synthesis of antibodies 21,26 .…”
Section: Glycinermentioning
confidence: 99%
“…Despite being membrane antibodies, they have been reported to commonly associate with SCLC 31 . GlycineR -patients may present with progressive encephalomyelitis with rigidity and myoclonus (PERM) 24 , a rare condition showing limb and axial rigidity, muscle spasms, brainstem signs and hyperekplexia [32][33][34] . Investigations may show CSF with inflammatory changes, and MRI has been normal in all of the published clinical reports 33,34 .…”
mentioning
confidence: 99%