Diagnostic Criteria and Classification of Alcohol-Related Dementia

Dementia

Dementia is defined as a significant deterioration of cognitive function sufficient to interfere in social or occupational functioning.

As defined by the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, this requires a deterioration in memory and at least one other area of intellectual functioning. Moreover, the cognitive changes are not attributable to the presence of delirium or substance-induced intoxication or withdrawal.

Definite Alcohol-Related Dementia

At the current time, there are no acceptable criteria to define definitively alcohol-related dementia.

 

Probable Alcohol-Related Dementia

I. The criteria for the clinical diagnosis of probable alcohol-related dementia include the following:

a. A clinical diagnosis of dementia at least 60 days after the last exposure to alcohol.

b. Significant alcohol use as defined by a minimum average of 35 standard drinks per week for men and 28 for women for a period greater than 5 years. The period of significant alcohol use must occur within 3 years of the initial onset of dementia.

II. The diagnosis of alcohol-related dementia is supported by the presence of any of the following:

a. Alcohol-related hepatic, pancreatic, gastrointestinal, cardiovascular, or renal disease, i.e., other end-organ damage.

b. Ataxia or peripheral sensory polyneuropathy (not attributable to other specific causes).

c. Beyond 60 days of abstinence, the cognitive impairment stabilizes or improves.

d. After 60 days of abstinence, any neuroimaging evidence of ventricular or sulcal dilatation improves.

e. Neuroimaging evidence of cerebellar atrophy, especially of the vermis.

III. The following clinical features cast doubt on the diagnosis of alcohol-related dementia:

a. The presence of language impairment, especially dysnomia or anomia.

b. The presence of focal neurological signs or symptoms (except ataxia or peripheral sensory polyneuropathy).

c. Neuroimaging evidence for cortical or subcortical infarction, subdural hematoma, or other focal brain pathology.

d. Elevated Hachinski Ischemia Scale score.

IV. Clinical features that are neither supportive nor cast doubt on the diagnosis of alcohol-related dementia include the following:

a. Neuroimaging evidence of cortical atrophy.

b. The presence of periventricular or deep white matter lesions on neuroimaging in the absence of focal infarct(s).

c. The presence of the ApolipoproteinE ε4 allele.

V. The diagnosis of possible alcohol-related dementia may be made when there are

a. A clinical diagnosis of dementia at least 60 days after the last exposure to alcohol; and

b. Either:

1. Significant alcohol use, as defined by a minimum average of 35 standard drinks per week for men and 28 for women for 5 or more years; however, the period of significant alcohol use occurred more than 3 years but less than 10 years before the initial onset of cognitive deficits; or

2. Possibly significant alcohol use, as defined by a minimum average of 21 standard drinks per week for men and 14 for women but no more than 34 drinks per week for men and 27 for women for 5 years. The period of significant alcohol use must have occurred within 3 years of the onset of cognitive deficits.

NINDS-AIREN Criteria for the Diagnosis of Vascular Dementia

I. The criteria for the clinical diagnosis of probable vascular dementia include all of the following:

A. Dementia, defined by cognitive decline from a previously higher level of functioning and manifested by impairment of memory and of two or more cognitive domains (orientation, attention, language, visuospatial functions, executive functions, motor control, and praxis), preferably established by clinical examination and documented by neuropsychological testing; deficits should be severe enough to interfere with activities of daily living not because of physical effects of stroke alone.

Exclusion criteria: cases with disturbance of consciousness, delirium, psychosis, severe aphasia, or major sensorimotor impairment precluding neuropsychological testing. Also excluded are systemic disorders or other brain diseases (such as Alzheimer’s disease [AD]) that in and of themselves could account for deficits in memory and cognition.

B. Cerebrovascular disease, defined by the presence of focal signs on neurological examination, such as hemiparesis, lower facial weakness, Babinski sign, sensory deficit, hemianopia, and dysarthria consistent with stroke (with or without history of stroke), and evidence of relevant cerebrovascular disease (CVD) by brain imaging (computed tomography or magnetic resonance imaging [MRI]) including multiple large-vessel infarcts or a single strategically placed infarct (angular gyrus, thalamus, basal forebrain, or posterior cerebral artery or anterior cerebral artery territories), as well as multiple basal ganglia and white matter lacunes, or extensive periventricular white matter lesions, or combinations thereof.

C. A relationship between the above two disorders, manifested or inferred by the presence of one or more of the following:

a. Onset of dementia within 3 months following a recognized stroke.

b. Abrupt deterioration in cognitive functions.

c. Fluctuating, stepwise progression of cognitive deficits.

II. Clinical features consistent with the diagnosis of probable vascular dementia include the following:

A. Early presence of gait disturbance (small-step gait or marche a petits pas, or magnetic, apraxic-ataxic or parkinsonian gait).

B. History of unsteadiness and frequent, unprovoked falls.

C. Early urinary frequency, urgency, and other urinary symptoms not explained by urological disease.

D. Pseudobulbar palsy.

E. Personality and mood changes, abulia, depression, emotional incontinence, or other subcortical deficits including psychomotor retardation and abnormal executive function.

III. Features that make the diagnosis of vascular dementia uncertain or unlikely include the following:

A. Early onset of memory deficit and progressive worsening of memory deficit and progressive worsening of memory and other cognitive functions, such as language (transcortical sensory aphasia), motor skills (apraxia), and perception (agnosia), in the absence of corresponding focal lesions on brain imaging.

B. Absence of focal neurological signs, other than cognitive disturbance.

C. Absence of cerebrovascular lesions on brain CT or MRI.

IV. Clinical diagnosis of possible vascular dementia may be made in the presence of dementia (section I-A) with focal neurological signs in patients in whom brain imaging studies to confirm definite CVD are missing; or in the absence of clear temporal relationship between dementia and stroke; or in patients with subtle onset and variable course (plateau or improvement) of cognitive deficits and evidence of relevant CVD.

V. Criteria for diagnosis of definite vascular dementia are:

A. Clinical criteria for probable vascular dementia.

B. Histopathological evidence of CVD obtained from biopsy or autopsy.

C. Absence of neurofibrillary tangles and neuritic plaques exceeding those expected for age.

D. Absence of other clinical or pathological disorder capable of producing dementia.

VI. Classification of vascular dementia for research purposes may be made based on clinical, radiological, and neuropathological features, for subcategories or defined conditions, such as cortical vascular dementia, subcortical vascular dementia, Binswanger’s disease, and thalamic dementia.

DSM-IV Criteria for the Diagnosis of Vascular Dementia

1. The development of multiple cognitive deficits manifested by both memory impairment (impaired ability to learn new information or to recall previously learned information) and one or more of the following cognitive disturbances: 

 a. Aphasia (language disturbance). 

 b. Apraxia (impaired ability to carry out motor activities despite intact motor function). 

 c. Agnosia (failure to recognize or identify objects despite intact sensory function). 

 d. Disturbance in executive functioning (i.e., planning, organizing, sequencing, abstracting). 

2. The cognitive deficits in criteria 1a and 1b each cause significant impairment in social or occupational functioning and represent a significant decline from a previous level of functioning. 

3. Focal neurological signs and symptoms (e.g., exaggeration of deep tendon reflexes, extensor plantar response, pseudobulbar palsy, gait abnormalities, weakness of an extremity), or laboratory evidence indicative of cerebrovascular disease (e.g., multiple infarctions involving cortex and underlying white matter) that are judged to be etiologically related to the disturbance. 

4. The deficits do not occur exclusively during the course of a delirium. 

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

Revised Criteria for the Diagnosis of Amyatrophic Lateral Sclerosis

 In order to standardize the diagnosis of ALS the  El Escorial Criteria (EEC) have been developed in 1994 following the older Airlie-House-Criteria.

The EEC have been revised in 1998 (Brooks et al. 2000). The EEC are mainly clinical and form the gold standard of ALS diagnosis. They are, however, relatively rigid and are not suitable for early diagnosis.

They are important for scientific classification and so far for recruitment into clinical trials, but not for clinical praxis. According to the EEC, the diagnosis of ALS requires the presence of A-criteria and the absence of B-criteria:

A-criteria:

A1: Degeneration of the lower motor neuron approved by clinical, electrophysiological or neuropathological examination.

A2: Degeneration of the upper motor neuron approved by clinical examination.

A3: Progressive dissemination beyond typical nerve supply areas.

B-criteria:

B1: Electrophysiological or neuropathological findings typical for other diseases which could explain the degeneration of the upper and lower motor neuron.

B2: Findings in imaging studies which can explain the clinical symptoms.

The diagnosis ALS requires an extensive history and a thorough clinical and electrophysiological examination. The electrophysiological tests can demonstrate the involvement of the lower motor neuron in affected and frequently also in clinically not affected body regions. The EEC define four body regions to be evaluated:

• brain stem (bulbar),

• cervical (neck and upper extremities),

• thoracal (trunk, abdominal wall)

• lumbosacral (lumbar spine and lower extremities).

The classification according to the revised EEC distinguishes between:

Clinically definite ALS

Proof of both, lower and upper motor neuron signs in at least 3 body regions.

Clinically probable ALS

Proof of both, lower and upper motor neuron signs in at least 2 body regions. The upper motor neuron signs have to be demonstrated rostrally to the region of the lower motor neuron signs.

Clinically probable – laboratory supported ALS

Clinical signs of the lower and the upper motor neuron in 1 body region only. Additionally there are electromyographic signs of active and chronic degeneration in at least 2 extremities.

Possible ALS

Signs of lower and upper motor neuron in 1 body region only or upper motor neuron signs in 2 or more body regions.

The old version of the EEC additionally contained the category “clinically suspected ALS” which has been deleted in the revised version.

Neuromyelitis Optica (Devic's Disease)

Diagnosis requires all absolute criteria and one major supportive criterion or two minor supportive criteria
Absolute criteria:

1. Optic neuritis
2. Acute myelitis
3. No evidence of clinical disease outside of the optic nerve or spinal cord

Major supportive criteria:

1. Negative brain MRI at onset (does not meet criteria for multiple sclerosis)
2. Spinal cord MRI with signal abnormality extending over >/=3 vertebral segments
3. CSF pleocytosis of >50 WBC/mm3 or >5 PMNs/mm3

Minor supportive criteria:

1. Bilateral optic neuritis
2. Severe optic neuritis with fixed visual acuity worse than 20/200 in at least one eye
3. Severe, fixed, attack-related weakness (MRC </=2) in one or more limbs

Acute Disseminated Encephalomyelitis (ADEM)-Weston Hurst disease

Clinical features

1. First clinical attack of inflammatory or demyelinating disease in the CNS
2. Acute or subacute onset
3. Affects multifocal areas of the CNS
4. Polysymptomatic presentation
5. Must include encephalopathy:

Acute behavioral change such as confusion or irritability and/or
Alteration in consciousness ranging from somnolence or coma

Attack should be followed by improvement on clinical and/or neuroradiologic (MRI) measures

Sequelae may include residual deficits

No other etiologies can explain the event

ADEM relapses (with new or fluctuating symptoms, signs or MRI findings) occurring within three months of the inciting ADEM episode are considered part of the same acute event. In addition, ADEM relapses that occur during a steroid taper or within four weeks of completing a steroid taper are considered part of the initial inciting ADEM episode.

Lesion Characteristics on MRI FLAIR and T2 weighted images

1. Large (>1 to 2 cm in size) multifocal, hyperintense, bilateral, asymmetric lesions in the supratentorial or infratentorial white matter. Rarely, brain MRI shows a single large ([greater than or equal to]1 to 2 cm) lesion predominantly affecting white matter.
2. Gray matter, especially basal ganglia and thalamus, may be involved
3. Spinal cord MRI may show confluent intramedullary lesion(s) with variable enhancement, in addition to the abnormalities on brain MRI
4. No radiologic evidence of previous destructive white matter changes

Encephalopathy is a required feature for the diagnosis of ADEM, but is not a typical feature of multiple sclerosis. In addition, a cerebrospinal fluid pleocytosis [greater than or equal to] 50 white blood cells/mm can be observed in ADEM, whereas this finding is highly atypical for multiple sclerosis.

 

Nihon Rinsho. 2013 May;71(5):887-92. [Acute disseminated encephalomyelitis (ADEM): its diagnostic criteria and therapy]. Hara T.

DSM-IV-TR Diagnostic criteria for Alzheimer's Disease (AD)

A. The development of multiple cognitive deficits manifested by both memory impairment and one or more of the following

1. Aphasia
2. Apraxia
3. Agnosia
4. and disturbances in executive functioning

B. The cognitive deficits represent as decline from previous functioning and cause significant impairment in social or occupational functioning

C. The course is characterized by gradual onset and continuing decline

D. The cognitive deficits are not due to other central nervous system, systemic, or substance-induced conditions that cause progressive deficits in memory and cognition

E. The disturbance is not better accounted for by another psychiatric disorder.

NINDS-ADRDA Diagnostic criteria for Alzheimer's Disease (AD)

Probable AD: A plus one or more supportive features B, C, D, or E

Core diagnostic criteria

A. Presence of an early and significant episodic memory impairment that includes the following features:

1. Gradual and progressive change in memory function reported by patients or informants over more than 6 months
2. Objective evidence of significantly impaired episodic memory on testing: this generally consists of recall deficit that does not improve significantly or does not normalise with cueing or recognition testing and after effective encoding of information has been previously controlled
3. The episodic memory impairment can be isolated or associated with other cognitive changes at the onset of AD or as AD advances

Supportive features

B. Presence of medial temporal lobe atrophy

1. Volume loss of hippocampi, entorhinal cortex, amygdala evidenced on MRI with qualitative ratings using visual scoring (referenced to well characterised population with age norms) or quantitative volumetry of regions of interest (referenced to well characterised population with age norms)

C. Abnormal cerebrospinal fluid biomarker

1. Low amyloid ?1–42 concentrations, increased total tau concentrations, or increased phospho-tau concentrations, or combinations of the three
2. Other well validated markers to be discovered in the future

D. Specific pattern on functional neuroimaging with PET

1. Reduced glucose metabolism in bilateral temporal parietal regions
2. Other well validated ligands, including those that foreseeably will emerge such as Pittsburg compound B or FDDNP

E. Proven AD autosomal dominant mutation within the immediate family

 

Exclusion criteria

1. History
2. Sudden onset
3. Early occurrence of the following symptoms: gait disturbances, seizures, behavioural changes
4. Clinical features
5. Focal neurological features including hemiparesis, sensory loss, visual field deficits
6. Early extrapyramidal signs
7. Other medical disorders severe enough to account for memory and related symptoms

Non-AD dementia, Major depression, Cerebrovascular disease, Toxic and metabolic abnormalities, all of which may require specific investigations

MRI FLAIR or T2 signal abnormalities in the medial temporal lobe that are consistent with infectious or vascular insults

Criteria for definite AD

AD is considered definite if the following are present:

1. Both clinical and histopathological (brain biopsy or autopsy) evidence of the disease, as required by the NIA-Reagan criteria for the post-mortem diagnosis of AD; criteria must both be present
2. Both clinical and genetic evidence (mutation on chromosome 1, 14, or 21) of AD; criteria must both be present

Criteria for the Neuroimaging Diagnosis of Periventricular Leukomalacia

I. Serial ultrasonography:

A. Cyst formation in periventricular area.

B. Periventricular ultrasonographic echodensity greater than choroid plexus echogenicity.

C. Findings of B prolonged over 3 weeks with irregularity of lateral ventricular walls and/or less uniform echodensity.

Findings of B or C indicate periventricular leukomalacia, whereas a finding of C indicates possible periventricular leukomalacia.

II. Computed tomography examination:

A. At 40 weeks, corrected postlast menstrual period, a low density of the periventricular area, and/or centrum semiovale with dilatation and irregularity of lateral ventricle wall suggests periventricular leukomalacia.

III. Magnetic resonance imaging:

B. After age 11 months, periventricular hypodensities (with dilatation and/or irregularity of lateral ventricular walls) on spin echo T2-weighted image and proton density image are consistent with the diagnosis of periventricular leukomalacia.

 

Hashimoto K, Hasegawa H, Kida Y, Takeuchi Y. Correlation between neuroimaging and neurologic outcome in periventricular leukomalacia: diagnostic criteria. Pediatr Int 2001;43:244.