Rare Endocrinology News

Disease Profile

Neuroleptic malignant syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

All ages

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ICD-10

G21.0

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Categories

Nervous System Diseases

Summary

Neuroleptic malignant syndrome is a rare neurological condition that is caused by an adverse reaction to neuroleptic (tranquilizer) or antipsychotic drugs. These drugs are commonly prescribed for the treatment of schizophrenia and other neurological, mental, or emotional disorders. Affected people may experience high fever, muscle stiffness, sweating, unstable blood pressure, altered mental status, and autonomic dysfunction. In most cases, the condition develops within the first 2 weeks of treatment with the drug; however, it may develop any time during the therapy period. The exact underlying cause of neuroleptic malignant syndrome is unknown. In some cases, more than one family member can be affected which suggests there may be a genetic component. Upon diagnosis of the condition, the neuroleptic or antipsychotic drug is generally discontinued under a physician's supervision. Medications and/or other interventions may also be recommended to manage symptoms.[1][2][3]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abnormal autonomic nervous system physiology
0012332
Extrapyramidal muscular rigidity
0007076
Fever
0001945
Hyperhidrosis
Excessive sweating
Increased sweating
Profuse sweating
Sweating
Sweating profusely
Sweating, increased

[ more ]

0000975
Mutism
Inability to speak
Muteness

[ more ]

0002300
30%-79% of people have these symptoms
Drooling
Dribbling
0002307
Dysphagia
Poor swallowing
Swallowing difficulties
Swallowing difficulty

[ more ]

0002015
Elevated serum creatine kinase
Elevated blood creatine phosphokinase
Elevated circulating creatine phosphokinase
Elevated creatine kinase
Elevated serum CPK
Elevated serum creatine phosphokinase
High serum creatine kinase
Increased CPK
Increased creatine kinase
Increased creatine phosphokinase
Increased serum CK
Increased serum creatine kinase
Increased serum creatine phosphokinase

[ more ]

0003236
Fatigue
Tired
Tiredness

[ more ]

0012378
Leukocytosis
Elevated white blood count
High white blood count
Increased blood leukocyte number

[ more ]

0001974
Metabolic acidosis
0001942
Muscle spasm
0003394
Tachycardia
Fast heart rate
Heart racing
Racing heart

[ more ]

0001649
Tremor
0001337
5%-29% of people have these symptoms
Acute kidney injury
0001919
Agitation
0000713
Anxiety
Excessive, persistent worry and fear
0000739
Aspiration pneumonia
0011951
Chorea
0002072
Coma
0001259
Dehydration
0001944
Delirium
0031258
Elevated alkaline phosphatase
Greatly elevated alkaline phosphatase
High serum alkaline phosphatase
Increased alkaline phosphatase
Increased serum alkaline phosphatase

[ more ]

0003155
Elevated hepatic transaminase
High liver enzymes
0002910
Encephalopathy
0001298
Hyperkalemia
Elevated serum potassium levels
0002153
Hypernatremia
High blood sodium levels
0003228
Hyperphosphatemia
High blood phosphate levels
0002905
Hypertensive crisis
0100735
Hyperuricemia
High blood uric acid level
0002149
Hypocalcemia
Low blood calcium levels
0002901
Hypomagnesemia
Low blood magnesium levels
0002917
Hyponatremia
Low blood sodium levels
0002902
Hypotension
Low blood pressure
0002615
Increased lactate dehydrogenase level
0025435
Myoglobinuria
0002913
Nasogastric tube feeding
0040288
Nausea
0002018
Oculogyric crisis
0010553
Proteinuria
High urine protein levels
Protein in urine

[ more ]

0000093
Pulmonary embolism
Blood clot in artery of lung
0002204
Rhabdomyolysis
Breakdown of skeletal muscle
0003201
Rigors
0025145
Thrombocytosis
Increased number of platelets in blood
0001894
Urinary incontinence
Loss of bladder control
0000020
Vomiting
Throwing up
0002013
1%-4% of people have these symptoms
Bradycardia
Slow heartbeats
0001662
Hypothermia
Abnormally low body temperature
0002045
Sepsis
Infection in blood stream
0100806
Thrombocytopenia
Low platelet count
0001873

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manual for health care professionals provides information on Neuroleptic malignant syndrome.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Neuroleptic malignant syndrome. Click on the link to view a sample search on this topic.

References

  1. Neuroleptic Malignant Syndrome Information Page. National Institute for Neurological Disorders and Stroke. April 2014; https://www.ninds.nih.gov/disorders/neuroleptic_syndrome/neuroleptic_syndrome.htm.
  2. Theodore I Benzer, MD, PhD. Neuroleptic Malignant Syndrome. Medscape Reference. February 2015; https://emedicine.medscape.com/article/816018-overview#a4.
  3. Eelco FM Wijdicks, MD. Neuroleptic malignant syndrome. UpToDate. May 2014; Accessed 7/14/2015.