Research Diagnostic Criteria for Neuroleptic Malignant Syndrome

1. Altered sensorium (any one of the following):

a. Confusion.

b. Clouding of consciousness.

c. Mutism.

d. Stupor.

Rating of severity should be done by at least two independent observers on Glasgow Coma Scale on at least 2 consecutive days. Nonspecific changes in mental state, e.g., restlessness or agitation, should not be equated with altered sensorium.

2. Extrapyramidal motor symptoms (any one of the following):

a. Muscular rigidity.

b. Dysphagia.

c. Dystonia.

d. Motor symptoms should be rated on the Simpson-Angus (extrapyramidal symptoms) Rating Scale.

3. Hyperpyrexia of unknown origin:

a. Should be greater than 38.5°C orally.

b. Should be sustained for at least 48 hours in duration.

c. No concurrent physical/medical cause for hyperpyrexia.

4. Autonomic dysfunction (at least two of the following):

a. Tachycardia (pulse more than 100 beats/min).

b. Tachypnea (respiration more than 25 breaths/min).

c. Blood pressure fluctuations (at least a change of 30 mmHg in systolic pressure or 15 mmHg in diastolic pressure).

d. Excessive sweating (diaphoresis).

e. New-onset incontinence.

5. Relationship of onset of symptoms with exposure event defined by any one of the following:

a. Oral ingestion or parenteral administration (dose increase, dose decrease, discontinuation) of an antipsychotic drug (typical or atypical), a dopamine depleter (e.g., tetrabenazine) dopamine blocker (e.g., metoclopramide) or a psychostimulant drug (e.g., cocaine) during the previous

2 weeks.

b. Withdrawal of antiparkinsonian (e.g., amantidine) or anticholinergic drug during previous week.

c. Intramuscular administration of a long-acting depot antipsychotic medication during the previous 8 weeks.

6. Exclusion criteria:

Symptoms not caused by any other existing or new general medical (secondary to substance abuse, infectious illnesses, metabolic, delirium, etc.), neurological (encephalitis, epilepsy, brain tumors, etc.) or psychiatric disorder (e.g., catatonic schizophrenia, mood disorder with catatonic features).

7. Supportive features (any two of the following):

a. Elevations in serum creatine phosphokinase levels.

b. Leukocytosis.

c. Low serum iron levels.

d. Elevation of liver enzymes.

e. Myoglobinuria. Type I NMS

Criteria 1-6 must be present for making a research diagnosis. Type II NMS

Criteria 1 and 3 and 4-6, and any one item from criterion 7 must be present for the diagnosis. Criterion 2 is not necessary for making diagnosis. Standardized assessment

All the patients with a suspected diagnosis should be rated on the following: Glasgow Coma Scale, Simpson-Angus (extrapyramidal symptoms) Rating Scale, and Bush-Francis Catatonia Rating Scale.

Adapted with permission from Adityanjee, Mathews T, Aderibighe YA. Proposed research diagnostic criteria for neuroleptic malignant syndrome. Int J Neuropsychopharmacol 1999;2:129-144.

Diagnostic Criteria for Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections Syndrome

1. Presence of obsessive-compulsive disorder and/or a tic disorder.

2. Pediatric onset of symptoms (age 3 years to puberty).

3. Episodic course of symptom severity.

4. Association with group A p-hemolytic streptococcal infection (a positive throat culture for strep throat or history of scarlet fever).

5. Association with neurological abnormalities (motoric hyperactivity or adventitious movements, such as choreiform movements).

Adapted from Frequently asked questions about PANDAS. Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections. Available via

Secondary cases may be seen in association with multiple sclerosis, metabolic derangements (hypo- or hyperglycemia), cerebrovascular disease, or after trauma.

The exact gene has not been identified, but PKD may be seen in conjunction with the syndrome of infantile convulsions and choreoathetosis, which is linked to the pericentric region of chromosome 16. However, cases linked to loci elsewhere on chromosome 16 or other chromosomes have been reported. PKD has also been seen in episodic ataxia type 1, which suggests that it may be a channelopathy.

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