Department of Neurology, National Neuroscience Institute, Singapore, Singapore
Copyright © 2017 The Korean Movement Disorder Society
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NMS [6] | PERM [1] | |
---|---|---|
Diagnostic criteria/features | DSM-V criteria: | |
- Hyperthermia | ||
- Rigidity | - Rigidity, painful spasms | |
- CPK at least 4 times the upper limit | ||
- Changes in mental status | - Changes in mental status | |
- Autonomic disturbances | - Autonomic disturbances | |
- Stimulus sensitive spasms, myoclonus, hyperekplexia, brainstem signs | ||
Predisposing factors | NMS | Autoimmune disorders, malignancy |
Prodromal symptoms | Alteration in mental status | Alteration in mental status |
Onset | Acute onset (16%), subacute presentation (66%), within 30 days (18%) | Acute onset (20%), subacute presentation (44%), subacute with acute exacerbation (7%), chronic (11%), chronic with acute exacerbation (18%) |
Other reported features | Gait difficulties, excessive startle, limb paresis | |
Blepharospasm, oculogyric crisis, nystagmus or trismus | Diplopia, nystagmus, ptosis, trismus | |
Dysphagia, dysarthria, aphonia | Dysphagia and speech difficulties | |
Sensory symptoms and painful spasms | ||
Urinary incontinence (uncommon) | Urinary incontinence (common) | |
Disseminated intravascular coagulation, multiorgan failure | Respiratory failure | |
CK elevation | 600–10,000 UI/L | Not described previously |
CSF | Normal (≥ 95%) | Pleocytosis (60%) |
EEG | Generalized slowing | Generalized slowing |
Focal epileptic activity | ||
Prognosis | 63% resolve within the first week and nearly all within a month of stopping treatment | Generally good |
Relapses common |
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