DIAGNOSIS OF AYMEN ELDESOKY HEPATIC ENCEPHALOPATHY MANSOURA FACULTY OF MEDICINE ASSISTANT PROFESSOR OF INTERNAL MEDICINE

Transcription

1

2 DIAGNOSIS OF HEPATIC ENCEPHALOPATHY BY AYMEN ELDESOKY ASSISTANT PROFESSOR OF INTERNAL MEDICINE MANSOURA FACULTY OF MEDICINE

3 DIAGNOSIS OF CHRONIC HEPATIC ENCEPHALOPATHY DR AYMEN ELDESOKY MANSOURA UNIVERSITY 24/8/006

4 When patients, with and without known liver disease, present with neuropsychiatric symptoms or neurological signs, it is necessary to ask one of the following questions: (1) Does this patient have HE? or (2) Could this patient have HE? There are two components to making a diagnosis of HE: - one is to determine that minimal or overt encephalopathy is present, and - the other is to obtain information consistent with hepatocellular insufficiency and increased portal- systemic shunting.

5 LABORATORY TESTS: A patient with significant liver injury may have normal results. Elevated serum aminotransferases (AST) Hyperbilirubinemia, hypoalbuminemia,, and hyperglobulinemia Elevated alkaline phosphatase Prolonged prothrombin time Hypomagnesemia, hypophosphatemia,, and hypokalemia DR AYMEN ELDESOKY MANSOURA UNIVERSITY 24/8/006

6 LABORATORY TESTS: Primary respiratory alkalosis, due to centrally-mediated hyperventillation is the most common acid-base disturbance in patients with severe liver disease, especially with superimposed encephalopathy. The exact etiology is unclear but may be related to the hormonal imbalance associated with liver failure. Estrogen and progesterone have been implicated, a situation somewhat similar to that seen in pregnancy. DR AYMEN ELDESOKY MANSOURA UNIVERSITY 24/8/006

7 LABORATORY TESTS: Anemia (from folic acid and vitamin B12 deficiency), gastrointestinal blood loss, or toxic effects of alcohol on bone marrow. Plasma ammonia levels are not consistently raised in patients with HE; they correlate poorly with the stage of HE and they do not provide a reliable index of the efficacy of treatments for HE. DR AYMEN ELDESOKY MANSOURA UNIVERSITY 24/8/006

8 LABORATORY TESTS: Lumbar puncture is not done unless indicated by atypical clinical or laboratory findings. It carries increased risk because of the presence of coagulopathy and, if ICP is increased in FHF, the possibility of precipitating cerebral herniation. DR AYMEN ELDESOKY MANSOURA UNIVERSITY 24/8/006

9 PSYCHOMETRIC TESTS: - Simple psychometric tests include: - orientation to time, person, and place, - recall of current events, - subtraction of serial sevens, - handwriting, and - figure drawing. The inability to draw a five-pointed star (constructional or ideational dyspraxia) has received special attention. DR AYMEN ELDESOKY MANSOURA UNIVERSITY 24/8/006

10 UROPHYSIOLOGICAL TESTS: e EEG abnormalities that occur in are non-specific, being found in er metabolic encephalopathies. e main EEG abnormalities in HE a progressive bilaterally chronous decrease in wave quency and an increase in wave plitude. Preterminally there is a

11 cipital phase shift. common with other cases of tabolic encephalopathies, roxysmal triphasic waves may cur, even in the early stages of, and are characteristically sociated with a frontal to

12 G WAVES AS DEFINED BY FREQUENCY: HA A TA LTA

13 RI: gnetic resonance imaging of the in proved an abnormally high nal on T1-weighted imaging in the al ganglia, particularly the globus lidus.. This high signal is now ieved to be due to manganese osition, and post-mortem studies e shown levels up to seven times

14

15 S, PET e MRI findings, in cirrhotic ients magnetic resonance ctroscopy and 18-fluoro fluoro-deoxy- cose positron emission ography have also disclosed ormal findings in the basal glia. The relationship of these

16 EBRAL BLOOD FLOW: rebral blood flow is low in patients h liver cirrhosis and, among rhotics,, it is lower in alcoholic and al l cirrhosis than in cholestatic r disease. In patients with vious alcohol abuse, cerebral od flow is reduced in the frontal

17 TOPSY STUDIES: uctural changes in neurons In ients who die with cirrhosis and tal-systemic shunts, proved an rease in the number and size of rocytes, particularly Alzheimer e 2 astrocytes. Such changes may induced by raised concentrations ammonia, but they are not a ture of the brain in fulminant

18 IFFERENTIAL DIAGNOSIS F HEPATIC ENCEPHALOPATHY

19 ther metabolic encephalopathies: ypernatraemia ypernatraemia, the manifestations of pernatemia are those of perosmolality.. The symptoms range m lethergy to seizures, coma and ath. yponatraemia yponatraemia, the manifestations are ainly attributable to CNS edema,, which usually not seen until the serum dium falls to 120 meq/l or less. mptoms range from mild lethergy to

20 poxic-ischemic encephalopathy: lateral hippocampal damage causes sakoff's amnesia. This is a memory rder characterized by inability to retain information (anterograde amnesia) and less severe defect of recall of old mories (retrograde amnesia). ffuse cortical, thalamic, or combined ronal loss (with intact brainstem) ults in dementia or the persistent etative state (loss of cognitive functions emotion with preservation of sleep-

21 erglycaemia or hypoglycaemia, percapnia percapnia, the clinical manifestations hypoventilation syndromes include piratory acidosis, usually warsened at ht with subsequent morning headache daytime somnolence with eventual llectual impairment. emia, Uremic encephalopathy is an anic brain disorder. It develops in ients with acute or chronic renal ure, usually when creatinine clearance

22 nifestations of this syndrome vary from d symptoms (eg( eg,, lassitude, fatigue) to ere symptoms (eg( eg,, seizures, coma). erity and progression depend on the of decline in renal function; thus, ptoms are usually worse in patients h acute renal failure. Prompt ntification of uremia as the cause of ephalopathy is essential because ptoms are readily reversible following

23 ilson's disease (Hepatolenticular generation): autosomal recessive disorder, typically earing in late adolescence. Copper is reased to saturation levels in the liver owed by accumulation in the brain, nea (Kayser-Fleisher ring round corneal bus), and kidney.. Metabolic defect nown, may be inability of bile duct to

24 in: Degeneration of basal ganglia: oordination e movements), (especially involving clumsiness, wness of voluntary limb vements and speech, tremor, arthria, excessive salivation, xia, dysphagia,, and mask-like

25 ntoxication with sedative/hypnotic gs (e.g. triazolam): izziness - drawsiness eadache - nervousness ausea - vomiting oordination problems

26 Consequences of head trauma stconcusive syndrome): lirium and wishing not to be moved. vere memory loss. cal deficit. bal confusion. petitive vomiting and nystagmus. owsiness. betes insipidus. sitive findings on CT scan or EEG

27 Organic intracranial lesions: In me cases the symptoms are latively nonspecific and usually e characterized by an intermittent adache accompanied by some gree of personality change, owsiness,, or confusion.. This ndition is easily confused with ug intoxication, cerebral stroke,

28 Alcohol intoxication and with- wal syndromes: rnicke's encephalopathy: nystagmus, xia,, and confusion often accompanied ophthalmoplegia.. Cardiovascular olvement may be signaled by hycardia as an early manifestation of ipheral vasodilatation. Thiamine should administered promptly-preferably preferably ore glucose is given-to any person in om subclinical thiamine deficiency is

29 rsakoff's syndrome: many of the oholic patients who recover from acute encephalopathy will be left h profound defect in memory and rning known as Korsakoff s chosis.

30 Delirium tremens (DTs) may occur in atient with underlying alcoholic liver ase. It is important, therefore, to inguish this syndrome from HE.. In trast to asterixis associated with HE, ients with DTs have a rapid postural action tremor.. Furthermore, the nifestations of DTs, including delirium, gest cortical excitation rather than the sumed cortical inhibition that seems to

31

32 DIAGNOSIS OF MINIMAL EPATIC ENCEPHALOPATHY

33 e technique of critical flicker quency might be effective in ntifying cases of MHE. This hnique establishes the frequency which a flashing light appears to p flashing and becomes tinuous (fusion frequency). The ion frequency dropped with

34 ropsychological tests: Can be lied to detect and quantitate ormalities of mental function in ients with liver diseases, who have E or early prestupor stages of HE. mber connection tests part A T-A); is a derivative of the trial making lities that measures the cognitive ction. Patients perform the test by

35 igit symbol test (DST); this is a set of the Wechsler Adult elligence Scale and measures tor speed and accuracy. The ient is given a list of digits ociated with symbols from 1-91 is asked to fill in blanks with bols that correspond to each

36 urophysiological assessment: e EEG was recorded by standardized hniques. Patients were graded into the erent stages of HE according to their n Dominant Frequency (MDF), and the tive powers of delta and theta activity. oked potentials testing is of greatest ity in detecting subclinical spinal cord optic nerve lesions. However, it could

37 euroimaging techniques such as gnetic resonance spectroscopy S) and positron emission ography (PET) have been used in assessment of MHE, but at the ment they are more useful in earch and in further establishing pathophysiology of the condition.

38

39 IAGNOSIS OF FULMINANT HEPATIC FAILURE

40 ute hepatic necrosis leading to atic encephalopathy and gulopathy develops secondary to virus, toxin or immune mediated ack. It is associated with failure hepatic regeneration.. The cesses leading to such profound atic damage are unknown, but multifactorial and depend on the

41 b Studies: er function studies: evels of hepatic enzymes do not orrelate well with the severity of the isease; they may be elevated, normal, r even decreased in patients with FHF. evels often are markedly elevated in atients with metabolic disorders. ith progressive necrosis of the liver,

42 um bilirubin: Both direct and indirect um bilirubin levels usually are elevated. ically, conjugated hyperbilirubinemia is sent. chemistry: Glucose level is decreased, ecially in infants. Hyponatremia, erkalemia, respiratory alkalosis, or tabolic acidosis also may be present. gulation profile: Prothrombin time (PT) rolonged. However, it does not respond

43 al studies: AV, HBV, HCV, HDV, and hepatitis E iruses account for approximately 50% f cases. Many viruses other than epatitis also are recognized causes of HF in childhood. BV is the most common cause of FHF endemic areas. Presence of IgM anti- BcAg or HBsAg in serum supports the

44 AV infection is a recognized cause of HF in individuals of all ages. Diagnosis f HAV infection is made by the resence of anti-hav IgM in the atient's serum. CV infection is diagnosed with etection of anti-hcv antibody or HCV NA in the serum. DV is diagnosed by the presence of nti-hdv RNA in the serum. ther causative viruses include Epsteinarr virus, CMV, herpesviruses, and

45 er biopsy: iver biopsy is usually an essential rocedure to consider in the anagement of FHF. It contributes to e working diagnosis and subsequent erapy. However, samples should be xamined with caution because results orrelate poorly with prognosis. Liver iopsy mostly is required to further ssist in reaching a likely diagnosis or in

46 view of the presence of gulopathy, weight the risk of liver psy against its contribution to gnosis and management. ministration of vitamin K typically not been found to result in a isfactory drop in PT in FHF. nsvenous biopsy is not ommonly used as a relatively e route in such a clinical

47 nclusion: re are no specific clinical features or terns of laboratory test results that diagnostic of HE. Accordingly, the gnosis of HE requires: linical judgment and involves establishing the presence of ato-cellular insufficiency and

48