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and/or proton-density-weighted images.

Adapted from World Health Organization. Human transmissable spongiform encephalopathies. Wkly Epidemiol Rec 1998;73:361-365.

Adapted from World Health Organization. Human transmissable spongiform encephalopathies. Wkly Epidemiol Rec 1998;73:361-365.

2. Probable

Table 13

Revised World Health Organization Definition of Creutzfeldt-Jakob Disease Subtypes Sporadic CJD

1. Definite Diagnosed by standard neuropathological techniques and/or immunocytochemically and/or Western blot-confirmed protease-resistant PrP and/or presence of scrapie-associated fibrils.

a. Progressive dementia and at least two out of the following four clinical features:

• Visual or cerebellar disturbance.

• Pyramidal/extrapyramidal dysfunction.

• Akinetic mutism. and b. A typical EEG during an illness of any duration and/or c. A positive 14-3-3 CSF assay and a clinical duration to death less than 2 years.

d. Routine investigations should not suggest an alternative diagnosis. Same clinical criteria as definite but no, or atypical, EEG and duration less than 2 years Progressive cerebellar syndrome in a recipient of human cadaveric-derived pituitary hormone; or

Sporadic CJD with a recognized exposure risk, e.g., antecedent neurosurgery with dura mater graft

Definite or probable CJD plus definite or probable CJD in a first-degree relative and/or

Neuropsychiatry disorder plus disease-specific PrP gene mutation CJD, Creutzfeldt-Jakob Disease; PrP, prion protein; EEG, electroencephalogram; CSF, cerebrospinal fluid.

3. Possible Iatrogenic CJD

Familial CJD

Table 14

Neuropathological Criteria for Creutzfeldt-Jakob Disease and Other Human Transmissible

Spongiform Encephalopathies

1. Creutzfeldt-Jakob disease (CJD)—sporadic, iatrogenic (recognized risk) or familial (same disease in first-degree relative or disease-associated PrP gene mutation): Spongiform encephalopathy in cerebral and/or cerebellar cortex and/or subcortical gray matter; and/or encephalopathy with prion protein (PrP) immunoreactivity (plaque and/or diffuse synaptic and/or patchy/perivacuolar types).

2. New-variant CJD: Spongiform encephalopathy with abundant PrP deposition; in particular, multiple fibrillary PrP plaques surrounded by a halo of spongiform vacuoles ("florid" plaques, "daisy-like" plaques) and other PrP plaques, and amorphous pericellular and perivascular PrP deposits; especially prominent in the cerebellar molecular layer.

3. Gerstmann-Straussler-Scheinker disease (in family with dominantly inherited progressive ataxia and/or dementia and one of a variety of PrP gene mutations): Encephalo(myelo)pathy with multicentric PrP plaques.

4. Familial fatal insomnia (in member of a family with a PrP gene mutation at codon 178 in frame with methionine at codon 129): Thalamic degeneration, variable spongiform change in cerebrum.

5. Kuru: Spongiform encephalopathy in the Fore population of Papua New Guinea.

Additional information:

1. Genetic analysis

Screening cases of CJD for the mutations associated with the hereditary forms of disease raises ethical and logistic concerns. Written consent for genetic testing is considered mandatory in many countries, but may be culturally unacceptable in others. The World Health Organization Consultation Diagnostic Procedures for Transmissible Spongiform Encephalopathies recommends that genetic counseling of patients and/or their families should be performed before any PrP gene analysis and that ideally written consent, but if not documented, oral consent should be obtained. The genetic counselor should be provided with information on the genetics of human transmissible spongiform encephalopathy to be used when seeking consent.

2. Electroencephalogram interpretation a. Preliminary notes:

i. The finding of a characteristic periodic EEG pattern is very helpful in the diagnosis of sporadic CJD.

ii. Some cases of sporadic CJD never show this pattern. A "negative" result cannot exclude the diagnosis.

iii. A periodic EEG, such as that seen in CJD, may rarely be found in a number of other conditions, and these must be considered in the clinical context. A list of these conditions is given in Table 15.

iv. The EEG changes in CJD undergo evolution. A periodic pattern may not be seen in the early phases of disease. The EEG may progress from showing nonspecific abnormalities to the characteristic appearance within days. Therefore, frequent serial EEG recordings should be undertaken whenever possible.

v. If a typical periodic EEG is obtained, then it is not absolutely necessary to repeat it, although this should be considered if there is any clinical doubt about other possible causes of the EEG pattern (such as metabolic factors).

vi. A repeatedly normal EEG is not consistent with a diagnosis of sporadic CJD.

b. Technical notes:

i. Bipolar montages including the vertex should be used.

ii. Referential montages including vertex and CZ reference electrodes should be used.

iii. The ECG should be coregistered.

iv. External alerting stimuli should be used.

v. The whole record should be viewed whenever possible, and a 2-minute continuous sequence used as a minimum.

Adapted from Budka H, Agguzi A, Brown P, et al. Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease and other human spongiform encephalopathies [prion diseases]. Brain Pathol 1995;5:459-466.

Table 15

Conditions That May Cause a Creutzfeldt-Jakob Disease-Like Electroencephalogram

Alzheimer's disease Hyperammonemia Lewy body disease Binswanger's disease AIDS dementia Hyperparathyroidism Hypo- and hypernatremia Hypoglycemia Multiple cerebral abscesses MELAS syndrome Hepatic encephalopathy

Baclofen, mianserin, metrizamide, and lithium toxicity Postanoxic encephalopathy

MELAS, mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes.

(Adapted from Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: Report of a WHO consultation. Geneva, Switzerland, 9-11 February 1998.)

Table 16

Conditions Other Than Creutzfeldt-Jakob Disease That Can Have a Positive 14-3-3 Result

Herpes simplex and other encephalitides Stroke (especially recent) Subarachnoid hemorrhage Hypoxic/Ischemic encephalopathy Barbiturate intoxication Glioblastoma

Carcinomatous meningitis (especially small-cell lung carcinoma) Paraneoplastic encephalopathy Corticobasal degeneration

Adapted from Global surveillance, diagnosis and therapy of human transmissible spongiform encephalopathies: Report of a WHO consultation. Geneva, Switzerland, 9-11 February 1998.

Table 17

DSM-IV Diagnostic Criteria for Delirium Caused by a General Medical Condition

A. Disturbance of consciousness (i.e., reduced clarity of awareness of the environment) with reduced ability to focus, sustain, or shift attention.

B. A change in cognition (such as memory disturbance, disorientation, language disturbance) or the development of a perceptual disturbance that is not better accounted for by a preexisting, established, or evolving dementia.

C. The disturbance develops over a short time (usually hours to days) and tends to fluctuate during the course of the day.

D. There is evidence from the history, physical examination, or laboratory findings that the disturbance is caused by the direct physiological consequences of a general medical condition.

Adapted from American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th rev. ed. Washington, DC: American Psychiatric Association, 1994.

The International Statistical Classification of Diseases and Related Health Problems, 10th Edition, Classification of Mental and Behavioral Disorders: Diagnostic Criteria for Research (Tables 18-45)

Table 18 Intoxication

1. There must be clear evidence of recent use of a psychoactive substance (or substances) at sufficiently highdose levels to be consistent with intoxication.

2. There must be symptoms or signs of intoxication compatible with the known actions of the particular substance (or substances), such as specified in Table 19, and of sufficient severity to produce disturbances in the level of consciousness, cognition, perception, affect, or behavior that are of clinical importance.

3. The symptoms or signs present cannot be accounted for by a medical disorder unrelated to substance use, and not better accounted for by another mental or behavioral disorder.

Acute intoxication frequently occurs in persons who have more persistent alcohol- or drug-related problems as well. Where there are such problems, e.g., harmful use, dependence syndrome, or psychotic disorder, they should also be recorded.

Table 19

Acute Intoxication Owing to Alcohol Use

A. The general criteria for acute intoxication must be met.

B. There must be dysfunctional behavior, as evidenced by at least one of the following:

Table 19

Acute Intoxication Owing to Alcohol Use

A. The general criteria for acute intoxication must be met.

B. There must be dysfunctional behavior, as evidenced by at least one of the following:

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