Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis (ALS)

The diagnosis of Amyotrophic Lateral Sclerosis (ALS) requires:

A. the presence of:

(A:1) evidence of lower motor neuron (LMN) degeneration by clinical, electrophysiological or neuropathologic examination,

(A:2) evidence of upper motor neuron (UMN) degeneration by clinical examination, and

(A:3) progressive spread of symptoms or signs within a region or to other regions, as determined by history or examination, together with

B. the absence of:

(B:1) electrophysiological and pathological evidence of other disease processes that might explain the signs of LMN and/or UMN degeneration, and

(B:2) neuroimaging evidence of other disease processes that might explain the observed clinical and electrophysiological signs.

Diagnostic Categories

Clinically Definite ALS: is defined on clinical evidence alone by the presence of UMN, as well as LMN signs, in three regions.

Clinically Probable ALS: is defined on clinical evidence alone by UMN and LMN signs in at least two regions with some UMN signs necessarily rostral to (above) the LMN signs.

The terms Clinically Probable ALS - Laboratory-supported and Clinically Possible ALS are used to describe these categories of clinical certainty on clinical and criteria or only clinical criteria:

Clinically Probable - Laboratory-supported ALS: is defined when clinical signs of UMN and LMN dysfunction are in only one region, or when UMN signs alone are present in one region, and LMN signs defined by EMG criteria are present in at least two limbs, with proper application of neuroimaging and clinical laboratory protocols to exclude other causes.

Clinically Possible ALS: is defined when clinical signs of UMN and LMN dysfunction are found together in only one region or UMN signs are found alone in two or more regions; or LMN signs are found rostral to UMN signs and the diagnosis of Clinically Probable - Laboratory-supported ALS cannot be proven by evidence on clinical grounds in conjunction with electrodiagnostic, neurophysiologic, neuroimaging or clinical laboratory studies. Other diagnoses must have been excluded to accept a diagnosis of Clinically possible ALS.

Clinically Suspected ALS: it is a pure LMN syndrome, wherein the diagnosis of ALS could not be regarded as sufficiently certain to include the patient in a research study. Hence, this category is deleted from the revised El Escorial Criteria for the Diagnosis of ALS.

References:

1. Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial "Clinical limits of amyotrophic lateral sclerosis" workshop contributors. J Neurol Sci. 1994 Jul;124 Suppl:96-107. [Medline]
2. Mitsumoto H. Diagnosis and progression of ALS. Neurology 1997; 48(S4):2S-8S.
3. Ross MA, Miller RG, Berchert L, Parry G, Barohn RJ, Armon C, Bryan WW, Petajan J, Stromatt S, Goodpasture J, McGuire D. Toward earlier diagnosis of amyotrophic lateral sclerosis: revised criteria. rhCNTF ALS Study Group. Neurology. 1998 Mar;50(3):768-72. [Medline]

Diagnostic Criteria for Alcohol Dependence

A maladaptive pattern of alcohol use, leading to clinically significant impairment or distress, as manifested by three or more of the following seven criteria, occurring at any time in the same 12-month period:

1.       Tolerance, as defined by either of the following:

a)      A need for markedly increased amounts of alcohol to achieve intoxication or desired effect.

b)      Markedly diminished effect with continued use of the same amount of alcohol.

2.       Withdrawal, as defined by either of the following:

a)      The characteristic withdrawal syndrome for alcohol (refer to DSM-IV for further details).

b)      Alcohol is taken to relieve or avoid withdrawal symptoms.

3.       Alcohol is often taken in larger amounts or over a longer period than was intended.

4.       There is a persistent desire or there are unsuccessful efforts to cut down or control alcohol use.

5.       A great deal of time is spent in activities necessary to obtain alcohol, use alcohol or recover from its effects.

6.       Important social, occupational, or recreational activities are given up or reduced because of alcohol use.

7.       Alcohol use is continued despite knowledge of having a persistent or recurrent physical or psychological problem that is likely to have been caused or exacerbated by the alcohol (e.g., continued drinking despite recognition that an ulcer was made worse by alcohol consumption).

References:

1. DSM-IV. American Psychiatric Association. (1994). Diagnostic and Statistical Manual of Mental Disorders (4th ed.). Washington, DC.

Diagnostic Criteria for Alcohol Abuse

1. A maladaptive pattern of alcohol abuse leading to clinically significant impairment or distress, as manifested by one or more of the following, occurring within a 12-month period:

a)      Recurrent alcohol use resulting in failure to fulfil major role obligations at work, school, or home (e.g., repeated absences or poor work performance related to substance use; substance-related absences, suspensions or expulsions from school; or neglect of children or household).

b)      Recurrent alcohol use in situations in which it is physically hazardous (e.g., driving an automobile or operating a machine).

c)      Recurrent alcohol-related legal problems (e.g., arrests for alcohol-related disorderly conduct).

d)      Continued alcohol use despite persistent or recurrent social or interpersonal problems caused or exacerbated by the effects of the alcohol (e.g., arguments with spouse about consequences of intoxication or physical fights).

2. These symptoms must never have met the criteria for alcohol dependence.

References:

1. DSM-IV. American Psychiatric Association. (1994). Diagnostic and Statistical Manual of Mental Disorders (4th ed.). Washington, DC.

Diagnostic Criteria for Agoraphobia

A) anxiety about being in places or situations from which escape might be difficult (or embarrassing) or in which help may not be available in the event of having an unexpected or situationally predisposed Panic Attack or panic-like symptoms. Agoraphobic fears typically involve characteristic clusters of situations that include being outside the home alone; being in a crowd, or standing in a line; being on a bridge; and traveling in a bus, train, or automobile.

B) The situations are avoided (e.g., travel is restricted) or else are endured with marked distress or with anxiety about having a Panic Attack or panic-like symptoms, or require the presence of a companion.

C) The anxiety or phobic avoidance is not better accounted for by another mental disorder, such as Social Phobia (e.g., avoidance limited to social situations because of fear of embarrassment), Specific Phobia (e.g., avoidance limited to a single situation like elevators), Obsessive-Compulsive Disorder (e.g., avoidance of dirt in someone with an obsession about contamination), Posttraumatic Stress Disorder (e.g., avoidance of stimuli associated with a severe stressor), or Separation Anxiety Disorder (e.g., avoidance of leaving home or relatives).

References:

1. DSM-IV. American Psychiatric Association. (1994). Diagnostic and Statistical Manual of Mental Disorders (4th ed.). Washington, DC.