RANDOM Notes Neurology part2: Lakshya J Basumatary, MD

hpzoo766@gmail.com

SERUM CREATINE KINASE (CK)

Increased In
Necrosis or acute atrophy of cardiac muscle
AMI
Severe myocarditis
Necrosis or acute atrophy of striated muscle
Progressive muscular dystrophy
Amyotrophic lateral sclerosis (>40% of cases)
Polymyositis (70% of cases; average 20 times upper limit of normal [ULN])
Thermal and electrical burns (values usually higher than in AMI)
Rhabdomyolysis (especially with trauma and severe exertion; also after alcohol or
other drug abuse); associated with myoglobinemia and myoglobinuria; marked
increase may be 1000 times ULN
Severe or prolonged exercise (begins 3 hours after start of exercise; peaks after 8-
16 hours; usually normal by 48 hours)
Status epilepticus
Postoperative state. Increase may last up to 5 days. Greater increase with use of
electrocautery in surgery
Chemical toxicity (benzene ring compounds [e.g., xylene] depolarize surface
membrane and leach out low-molecular-weight enzymes, producing very high
levels of total CK (100% MM) with increased LDH of 3-5 times normal.
Half of patients with extensive brain infarction. Maximum levels in 3 days;
increase may not appear before 2 days; levels usually less than in AMI and remain
increased for longer time; return to normal within 14 days; high mortality
associated with levels >300 IU. Elevated serum CK in brain infarction may obscure
diagnosis of concomitant AMI.
Parturition and frequently the last few weeks of pregnancy
Malignant hyperthermia
Endocrine myopathy
Hypothyroidism--increased 4-8 times ULN in 60-80% of cases; becomes normal
within 6 weeks of replacement therapy.
Acromegaly--increased 2 times ULN.

Slight Increase Occasionally In

IM injections. Variable increase after IM injection to 2-6 times normal level.
Returns to normal 48 hours after cessation of injections.
Muscle spasms or convulsions in children
Electrical cardiac defibrillation or countershock in 50% of patients; returns to
normal in 48-72 hours.

Normal In
Angina pectoris
Pericarditis
Pulmonary infarction
Renal infarction
Liver disease
Biliary obstruction
Some muscle disorders
Neurogenic muscle atrophy
Thyrotoxicosis
Steroid myopathy
Pernicious anemia
Most malignancies
Following cardiac catheterization and coronary arteriography unless myocardium
has been injured by catheter
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MUSCULAR DYSTROPHY(genetic primary myopathies)

Serum enzymes (CK is most useful) are increased, especially in

Young patients. Highest levels (<=50 times normal) are found at onset of infancy
and childhood, with gradual return to normal.

The more rapidly progressive dystrophies (such as the Duchenne type) and may
be slightly or inconsistently increased in the limb-girdle and facioscapulohumeral
types.

The active early phase. Increased levels are not constant and are affected by
patient's age and duration of disease. Enzymes may be increased before disease
is clinically evident. Elevated serum enzyme levels are not affected by steroid
therapy.

Serum CK is useful for

Preclinical diagnosis of Duchenne's and Becker's dystrophy in families with history
of disease or for screening. Always increased in affected children (5-100 times
upper limit of normal [ULN] of adults) to peak by age 2 years; then begin to fall as
disease becomes manifest. Persistent normal CK virtually rules out this diagnosis.
Begin testing at 2-3 months of age. (Normal children have CK level that is very
high during first few days, falls to 3 times ULN by fourth day, falls to 2-3 times
adult level during first month of life, and levels remain > adults during first 2
years.) Neonatal screening that is positive with whole blood should be confirmed
with serum. CK >3 times ULN for age in all boys with Duchenne's dystrophy and
>2 times in Becker's dystrophy. Sex-linked dystrophy is virtually only cause of
high values in normal neonates. High values persist in dystrophy but not with
false-positives. Neonatal screening of girls has been discontinued. Prenatal
screening at 18-20 weeks' gestation by placental aspiration of fetal blood has
been abandoned due to false negative and false positive results.

Clinical diagnosis. CK is increased in almost all cases of Duchenne's (average 30

times ULN) and Becker's (average 10 times ULN) dystrophies. Diagnosis is in
doubt if CK is normal. Highest in young patients and falls with age by
approximately 50% at age 7 years; usually remains >5 times ULN but in terminal
cases may decline further. Except for polymyositis, other myopathies and
neurogenic atrophy show normal CK or <5 times ULN. Serum aldolase is increased
in approximately 20% of patients. Serum LDH is increased in approximately 10%
of patients. SGOT is increased in approximately 15% of patients.

Identifying female carriers. CK is increased in carriers with two affected sons or

one son and one affected male relative in approximately 70% of Duchenne's and
50% of Becker's dystrophy. Highest levels and greatest frequency in younger
carriers; may only be present during childhood and not in later life. Levels may be
up to 10 times ULN but usually <3 times and average = 1.5 times ULN; values
overlap with normal females. Therefore special precautions are needed: Draw
blood after normal activity in afternoon or evening but not after severe or long
exercise or IM injections or during pregnancy; recheck at weekly intervals 3 times;
values are higher in blacks than whites.

Limb-Girdle Dystrophy
(heterogeneous group of disorders in both sexes; autosomal recessive that begins
in second decade and progresses to disability by age 30 and death by age 50)
Serum CK is increased in 70% of patients to average 10 times ULN. Not useful to
detect carriers. Not useful to distinguish from other autosomal recessive forms of
dystrophy, myopathy, or neurologic disorders (e.g., hereditary proximal spinal
muscular atrophy).

Fascioscapulohumeral Dystrophy
(begins in late adolescence; normal life span)
Serum CK is increased in 75% of patients to average 3 times ULN. Frequently
normal by age 50.

MYOTONIC DYSTROPHY
(autosomal dominant disorder presents in adolescence)
Serum CK is increased in 50% of cases to average 3 times ULN.
Increased creatine in urine may occur irregularly.
Findings due to atrophy of testicle and androgenic deficiency are noted.
Urine 17-ketosteroids are decreased.
Thyroid function may be decreased.

METABOLIC DISEASES OF MUSCLE

Hypothyroidism (rarely associated with myotonia)
Increased serum CK in 60-80% of cases to average 4-8 times ULN; becomes
normal 4-6 weeks after treatment. CK-MB is rarely increased up to 6% of total.
Decreased urine creatine
Increased creatine tolerance
Other serum enzyme levels normal
Hyperthyroidism
Normal serum enzyme levels
Increased urine creatine
Decreased creatine tolerance
Normal muscle biopsy
Causes some cases of hypokalemic periodic paralysis.
Acromegaly
Serum CK may be increased to average of 2 times ULN.
Associated with administration of adrenal corticosteroids and with Cushing's
syndrome
Increased serum enzymes--uncommon, and may be due to the primary disease
Muscle biopsy--degenerative and regenerative changes in scattered muscle fibers;
no inflammatory cell infiltration
Increased urine creatine

MYOTUBULAR MYOPATHY, MITOCHONDRIAL MYOPATHY, NEMALINE (ROD)

MYOPATHY
Routine laboratory studies including measurement of serum enzymes are normal.
Biopsy of muscle with histochemical staining reaction establishes the diagnosis.

MYOPATHY ASSOCIATED WITH ALCOHOLISM

Acute
Increased serum CK, SGOT, and other enzymes. Serum CK increased in 80% of
patients; rises in 1-2 days; reaches peak in 4-5 days; lasts approximately 2 weeks.
CK in CSF is normal, even when serum level is elevated.
Gross myoglobinuria
Acute renal failure (some patients)
Chronic--may show some or all of the following changes
Increased serum CK in 60% of patients to average of 2 times ULN
SGOT and other enzymes may also be increased due to liver as well as muscle.
Increased urine creatine
Diminished ability to increase blood lactic acid with ischemic exercise
Abnormalities on muscle biopsy (support the diagnosis)
Myoglobinuria
OSTEOMYELITIS
WBC may be increased, especially in acute cases.
ESR is increased in <50% of patients but may be important clue in occult cases
(e.g., intervertebral disk space infection).
Bacteriology
Staphylococcus aureus causes almost all infections of hip and two-thirds of
infections of skull, vertebrae, and long bones. Other bacteria may simultaneously
be present and contribute to infection.
Gram-negative bacteria cause most infections of mandible, pelvis, and small
bones.
Salmonella is more commonly found in patients with sickle hemoglobinopathy.
Laboratory findings due to underlying conditions, e.g., postoperative status,
irradiation therapy, foreign body, tissue gangrene, contiguous infection.
---------------------

Rh. Factor is seen in :

1.SLE

2.Sjogren;s syndrome

3. Chronic liver disease

4. Sarcoidosis

5.Interstitial pulmonary fibrosis

6.Infectious mononucliosis

7.Hepatitis-B

8.TB

9.Leprosy

10.Syphilis

11.Malaria

12.Schistosomiasis

13.Subacute bacterial endocarditis

14. Visceral Leishmaniasis

Also note that RH. Factor is of PROGNOSTIC imp NOT DIAGNOSTIC of Rheumatoid
arthritis

RH. Factor mayalso appear transiently in normal persons after Vaccination &
Transfusion

CAUSES OF SPASTIC PARAPLEGIA

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#hereditary spastic paraplegia

#familial spastic paraplegia
#tropical spatic paraparesis
#lathyrism ( toxin frm Lathyrus sativus )
#konzo ( toxin frm cassava )
#primary lateral sclerosis
#multiple sclerosis
#spinal cord injury or tmrs
#cerebral palsy
#amyotropic lateral sclerosis
#vitamin def
#herniated thoracic discs