Progressive encephalomyelitis rigidity and myoclonus (PERM)
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Progressive encephalomyelitis rigidity and myoclonus (PERM). Muscle rigidity, Stimulus sensitive spasms. Fully referenced neurology checklist.
Clinical features
- Muscle rigidity
- Stimulus sensitive spasms
- Prominent brainstem signs
- Generalized myoclonus
- Hyperekplexia
- Cerebellar ataxia
- Pyramidal signs
- Autonomic dysfunction
- Stiff tongue
- Rapid progression
Electromyogram (EMG)
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Antibodies
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References
- Mas N, Saiz A, Leite MI, et al. Antiglycine-receptor encephalomyelitis with rigidity. JNNP 2011; 82:1399-1401.
- Dalakas MC. Stiff person syndrome: advances in pathogenesis and therapeutic interventions. Curr Treat Options Neurol 2009; 11:102-110.
- Niedernhöfer P, Schwabe J, Seidel G. Progressive encephalomyelitis with rigidity and myoclonus : Stiff tongue as initial symptom. Nervenarzt 2017; doi: 10.1007/s00115-017-0429-5 (Epub ahead of print).
- Balint B, Jarius S, Nagel S, et al. Progressive encephalomyelitis with rigidity and myoclonus: a new variant with DPPX antibodies. Neurology 2014; 82:1521-1528.
