raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:94093

Neuroleptic malignant syndrome

Type
Disease
Group
Disorder
Inheritance
Unknown
Age of onset
All ages
Status
active

A rare neuropsychiatric syndrome associated with administration of antipsychotic or other central dopamine (D2) receptor antagonists, and characterized by hyperthermia, muscular rigidity, autonomic dysfunction and altered consciousness.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNeuroleptic malignant syndrome
frSyndrome malin des neuroleptiques
deMalignes neuroleptisches Syndrom
esSíndrome neuroléptico maligno
itSindrome neurolettica maligna
nlNeuroleptisch maligne syndroomMaligne neurolepticasyndroom
ptSíndrome de neuroleptico maligno
plZłośliwy zespół neuroleptyczny
csMaligní neuroleptický syndrom
ukНейролептичний злоякісний синдром
zh神经阻滞剂恶性综合征
trNöroleptik habis sendrom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10G21.0Especificvalidated
ICD-118A0YNTBTindexvalidated
MONDO0019790Evalidated
MeSHD009459Evalidated
UMLSC0027849Evalidated
GARD7195Evalidated
MedDRA10029282Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

54 annotations · validated 2019-02-25
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0012332Abnormal autonomic nervous system physiology
HP:0007076Extrapyramidal muscular rigidity
HP:0001945Fever
HP:0000975Hyperhidrosis
HP:0002300Mutism
HP:0004372Reduced consciousness
Frequent (79-30%)
HP:0002307Drooling
HP:0002015Dysphagia
HP:0003236Elevated circulating creatine kinase concentration
HP:0003781Excessive salivation
HP:0012378Fatigue
HP:0000822Hypertension
HP:0001974Increased total leukocyte count
HP:0001942Metabolic acidosis
HP:0003394Muscle spasm
HP:0001649Tachycardia
HP:0001337Tremor
Occasional (29-5%)
HP:0001919Acute kidney injury
HP:0000713Agitation
HP:0000739Anxiety
HP:0011675Arrhythmia
HP:0011951Aspiration pneumonia
HP:0002072Chorea
HP:0001259Coma
HP:0001944Dehydration
HP:0031258Delirium
HP:0003155Elevated circulating alkaline phosphatase concentration
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0001298Encephalopathy
HP:0002153Hyperkalemia
HP:0003228Hypernatremia
HP:0002905Hyperphosphatemia
HP:0100735Hypertensive crisis
HP:0002149Hyperuricemia
HP:0002901Hypocalcemia
HP:0002917Hypomagnesemia
HP:0002902Hyponatremia
HP:0002615Hypotension
HP:0025435Increased circulating lactate dehydrogenase concentration
HP:0002913Myoglobinuria
HP:0040288Nasogastric tube feeding
HP:0002018Nausea
HP:0010553Oculogyric crisis
HP:0000093Proteinuria
HP:0002204Pulmonary embolism
HP:0003201Rhabdomyolysis
HP:0025145Rigors
HP:0001894Thrombocytosis
HP:0000020Urinary incontinence
HP:0002013Vomiting
Very rare (<4-1%)
HP:0001662Bradycardia
HP:0002045Hypothermia
HP:0100806Sepsis
HP:0001873Thrombocytopenia

Run the differential on the 6 obligate / very frequent terms

Annotation source: 28144147[PMID]_29464217[PMID]_30765906[PMID]

7

Sources for this card