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 816 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
---------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 -----------------------------------------------#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