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 |