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.

ILAR Classification Criteria for Juvenile Idiopathic Arthritis (JIA)

Juvenile idiopathic arthritis is a heterogeneous group of diseases characterised by arthritis of unknown origin with onset before age of 16 years.

International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA)

JIA can be diagnosed if age at onset is under 16 years, disease duration is 6 weeks or greater, and other known conditions are excluded.

• Systemic-onset JIA: Systemic arthritis is diagnosed if there is arthritis in 1 or more joints with, or preceded by, fever of at least 2 weeks' duration. Signs or symptoms must have been documented daily for at least 3 days and accompanied by 1 or more of the following: evanescent rash, generalised lymphadenopathy, hepato/splenomegaly, serositis. (Exclusions are A, B, C, and D from the exclusion list below.)
• Persistent or extended oligoarthritis: Oligoarthritis is diagnosed if there is arthritis affecting 1 to 4 joints during the first 6 months. Persistent oligoarthritis affects up to 4 joints throughout the course of the disease, and extended oligoarthritis affects more than 4 joints after the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)
• RF-negative polyarthritis: Polyarthritis (RF-negative) is diagnosed if there is rheumatoid factor (RF)-negative arthritis affecting 5 or more joints during the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)
• RF–positive polyarthritis: Polyarthritis (RF-positive) is diagnosed if there is RF-positive arthritis affecting 5 or more joints during the first 6 months of disease. Two or more RF tests (taken at least 3 months apart) are positive during the first 6 months of disease. (Exclusions are A, B, C, and E from the exclusion list below.)
• Psoriatic JIA: Psoriatic arthritis is diagnosed if there is arthritis and psoriasis, or arthritis and at least 2 of the following: dactylitis, nail pitting, onycholysis, and/or family history of psoriasis (in a first-degree relative). (Exclusions are B, C, D, and E from the exclusion list below.)
• Enthesitis-related arthritis: Enthesitis-related arthritis is diagnosed if there is arthritis and/or enthesitis with at least 2 of the following: presence or history of sacroiliac joint tenderness with or without inflammatory lumbosacral pain; presence of HLA B27 antigen; onset of arthritis in a male over 6 years of age; acute (symptomatic) anterior uveitis; history of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, or acute anterior uveitis in a first-degree relative. (Exclusions are A, D, and E from the exclusion list below.)
• Undifferentiated: Undifferentiated arthritis is diagnosed if there is arthritis that does not fulfil criteria in any of the above categories or that fulfils criteria for 2 or more of the above categories.

Exclusions:

A. Psoriasis or history of psoriasis in patients or first-degree relatives.
B. Arthritis in HLA B27 positive males beginning after the age of 6 years.
C. Ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, acute anterior uveitis, or history of  1 of these disorders in first-degree relatives.
D. Presence of IgM rheumatoid factor on at least 2 occasions at least 3 months apart.
E. Presence of systemic JIA in patients.

References:

1. Merino R, de Inocencio J, Garcia-Consuegra J. Evaluation of Revised International League of Associations for Rheumatology Classification Criteria for Juvenile Idiopathic Arthritis in Spanish Children (Edmonton 2001). J Rheumatol 2005;32:559-61. [Medline]
2. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31:390-392. [Medline]
3. Prakken B, Albani S, Martini A. Juvenile idiopathic arthritis. Lancet. 2011;377(9783):2138-2149. [Medline]

Clinical Criteria for the Diagnosis of Acute Bacterial Sinusitis

Acute bacterial sinusitis in children is diagnosed on the basis of the history, with the use of the criteria. Imaging studies (plain-film radiography, computed tomography [CT], magnetic resonance imaging [MRI], and ultrasonography) show signs of sinus inflammation but are not recommended in patients with uncomplicated infection, given the low specificity of these studies.

Clinical Criteria for the Diagnosis of Acute Bacterial Sinusitis

Persistent symptoms

• Nasal congestion, rhinorrhea, or cough
• >/=10 Days’ duration without improvement

Severe symptoms

• Temperature >/=38.5°C for 3–4 days
• Purulent rhinorrhea for 3–4 days

Worsening symptoms

• Return of symptoms after initial resolution
• New or recurrent fever, increase in rhinorrhea, or increase in cough 

References:

1. DeMuri GP, Wald ER. Clinical practice. Acute bacterial sinusitis in children. N Engl J Med. 2012 Sep 20;367(12):1128-34. [Medline]
2. DeMuri GP, Wald ER. Acute sinusitis: clinical manifestations and treatment approaches. Pediatr Ann. 2010 Jan;39(1):34-40. [Medline]

Diagnostic Criteria for Difficult-to-Control Asthma

The difficult-to-control asthma can be defined as that which is inadequately or poorly controlled despite an appropriate therapeutic strategy that is adjusted to clinical severity.

Diagnostic Criteria for Difficult-to-Control Asthma

A diagnosis of difficult-to-control asthma is established when, once false difficult-to-control asthma has been ruled out, 2 major criteria or 1 major and 2 minor criteria are met. Criteria are modified in part from the proposal of the American Thoracic Society Workshop on Refractory Asthma

Major criteria
– Use of oral corticosteroids continuously or for more than 6 months in the last year
– Continuous use of inhaled corticosteroids at high doses (budesonide or equivalent >1200 ug/day or fluticasone >880 ug/day) alongside another antiasthmatic drug, usually a long-acting B2-adrenergic (LABA)

Minor criteria
– Daily requirement for short-acting B2-adrenergic (SABA) rescue medication
– FEV1 less than 80% of theoretical value or greater than 20% variability of PEF
– One or more visits to the emergency department in the last year
– Three or more courses of oral corticosteroids in the last year
– Prior episode of life-threatening asthma
– Rapid deterioration of lung function

*FEV1 indicates forced expiratory volume in the first second; PEF, peak expiratory flow.




References:

1. López-Viña A, Agüero-Balbín R, Aller-Alvarez JL, Bazús-González T, Cosio BG, de Diego-Damiá A, Martínez-Moragón E, Pereira-Vega A, Plaza-Moral V, Rodríguez-Trigo G, Villa-Asensi JR; Area de Asma-SEPAR. Guidelines for the diagnosis and management of difficult-to-control asthma. Arch Bronconeumol. 2005 Sep;41(9):513-23. [Medline]
2. Le AV, Simon RA. The Difficult-to-Control Asthmatic: A Systematic Approach. Allergy Asthma Clin Immunol. 2006 Sep 15;2(3):109-16. [Medline

Typical Laboratory Abnormalities in Alcoholic Liver Disease (ALD)

Alcoholic liver disease (ALD) encompasses a spectrum of injury, ranging from simple steatosis to frank cirrhosis.

Typical Laboratory Abnormalities in ALD

Serum enzymes

• AST >>> ALT: Both usually <300 IU/L
• Alkaline phosphatase (ALP) and g-glutamyl transpeptidase (GGT): Both usually elevated to a variable degree

Metabolic alterations

• Hyperglycemia
• Hypertrygleridemia
• Hyperuricemia
• Electrolyte abnormalities: low potassium, magnesium and phosphorus

Tests of liver function

• Serum albumin, prothrombin time, and serum bilirubin usually normal until significant liver injury present

Hematological abnormalities

• Mild anemia common (usually macrocytic)
• Platelets (normal to markedly decreased)
• Elevated white blood cell count: Leukemoid reactions associated with alcoholic hepatitis

Abbreviations: AST, aspartate aminotransferase;  ALT, alanine aminotransferase

References:

1. O'Shea RS, Dasarathy S, McCullough AJ. Alcoholic liver disease. Am J Gastroenterol. 2010 Jan;105(1):14-32. [Medline]
2. McCullough AJ, O'Connor JF. Alcoholic liver disease: proposed recommendations for the American College of Gastroenterology. Am J Gastroenterol. 1998 Nov;93(11):2022-36.[Medline]

Medical Management of Kidney Stones: AUA Guideline

Purpose

The purpose of this guideline is to provide a clinical framework for the diagnosis, prevention and follow-up of adult patients with kidney stones based on the best available published literature.

Materials and Methods

The primary source of evidence for this guideline was the systematic review conducted by the Agency for Healthcare Research and Quality on recurrent nephrolithiasis in adults. To augment and broaden the body of evidence in the AHRQ report, the AUA conducted supplementary searches for articles published from 2007 through 2012 that were systematically reviewed using a methodology developed a priori. In total, these sources yielded 46 studies that were used to form evidence-based guideline statements. In the absence of sufficient evidence, additional statements were developed as Clinical Principles and Expert Opinions.

Results

Guideline statements were created to inform clinicians regarding the use of a screening evaluation for first-time and recurrent stone formers, the appropriate initiation of a metabolic evaluation in select patients and recommendations for the initiation and follow-up of medication and/or dietary measures in specific patients.

Conclusions

A variety of medications and dietary measures have been evaluated with greater or less rigor for their efficacy in reducing recurrence rates in stone formers. The guideline statements offered in this document provide a simple, evidence-based approach to identify high-risk or interested stone-forming patients for whom medical and dietary therapy based on metabolic testing and close follow-up is likely to be effective in reducing stone recurrence.