Professional Documents
Culture Documents
HY
Dr.Muhammad
Yusuf,SpS
Definisi
Encephalopathy adalah istilah yang
berarti penyakit, kerusakan, atau
malfungsi otak.
Penyebab
infeksi-infeksi (bakteri, virus,parasit, atau prions),
anoxia (kekurangan oksigen pada otak),
alkohol,
gagal hati,
gagal ginjal,
penyakit metabolik,
tumor otak,
kimia
Perubahan pada tekanan dalam otak
nutrisi yang buruk.
Gejala-Gejala
Keadaan mental yang berubah
kelesuan,
dementia,
seizures,
tremor-tremor,
kejang otot, dan
koma.
Diagnosa Encephalopathy
Status mental, tes memori, tes koordinasi
Complete blood count (CBC ) , infeksi,
Tekanan darah
Pemeriksaan metabolik (elektrolit, glucose,
lactate, ammonia, oksigen, dan enzim hati)
Obat-obatan atau racun (alkohol, cocaine,
amphetamines, DLL)
Kultur darah dan cairan tubuh (infeksi)
Creatinine (fungsi ginjal)
CT dan MRI scans
Doppler ultrasound
Encephalogram atau EEG
Auto-antibody
Penanganan
Bervariasi tergantung penyebab primer yang
mendasarinya
Anoxia : terapi oksigen
Keracunan alkohol jangka pendek: cairan-cairan IV
(intravena)
Penyalahgunaan alkohol jangka panjang (sirosis atau
gagal hati kronis: oral lactulose, diet rendah protein,
antibiotik
Uremic encephalopathy (gagal ginjal): mengkoreksi
penyebab fisiologi yang mendasarinya, dialysis,
transplantasi ginjal
Diabetic encephalopathy: glucose hypoglycemia
Hypo- atau hypertensive encephalopathy
Hashimoto's encephalopathy
kebingungan, dementia
Wernicke's encephalopathy
kebingungan mental, memori, kemampuan
yang berkurang untuk menggerakan mata
Bovine spongiform encephalopathy atau "Mad
Cow disease"
ataxia, dementia dan myoclonus atau kejang
Shigella encephalopathy
sakit kepala, leher yang kaku, delirium,
seizures, koma
infeksius dari pediatric encephalopathy
lekas marah, anoreksia, hypotonia atau floppy
baby syndrome, seizures, kematian
Hepatic Encephalopathy
Definition (1)
Hepatic encephalopathy
(HE)
It represents a reversible
decrease in neurologic
function, based upon the
disorder of metabolism which
are caused by severe
Incidence/prevalence
Universal feature of
acute liver failure
50%~70% in chronic
hepatic failure
Difficult to estimate
Etiology
Fulminant hepatic failure
acute severe viral hepatitis,
drug/toxin
acute fatty liver of pregnancy
Due to acute hepatocellular
necrosis
Chronic liver disease
cirrhosis of all types (70%),
primary liver cancer surgically
induced portal-cava shunts
Due to one or more potentially
HE---common
precipitating factors
Nitrogenous
Nitrogenous
Non-Nitrogenous
Non-Nitrogenous
Encephalopathy
Encephalopathy
Encephalopathy
Encephalopathy
Uremia/azotemia
Gastrointestinal
bleeding
Dehydration
Metabolic alkalosis
Hypokalemia
Constipation
Excessive dietary
protein
Infection
Sedative
Benzodiazepines
Hypoxia
Hypoglycemia
Hypothyroidism
Anemia
Pathogenesis (1)
Toxic materials derived from
nitrogeneous substrate in the
gut and bypass the liver
Caused by several factors
act synergistically
Several putative gut-derived
toxins identified
Pathogenesis (2)
Postulated factors/mechanisms:
Ammonnia neurotoxicity
Synergistic neurotoxins
Excitatory inhibitory
neurotransmitters and plasma
amino acid imbalance
hypothesis
-Aminobutyric acid
Ammonia neurotoxicity
Ammonia production
resulting from the degradation of urea
or protein
primary site:
gut
other site:
kidney and skeletal
muscles
Gut-generating ammonia: 4g/day
Equilibrium of ammonia and
ammonium:
Ammonia neurotoxicity
Ammonia elimination
Transfer to the liver
Metabolized by series of urea
cycle enzymes
Comsumpted by brain, liver,
kidney: to
synthesize glutamic acid and
glutamine
Excreted into the urine
Ammonia neurotoxicity
Over production and/or hypoeccrisis
Poor hepato-cellular function:
incomplete metabolism
Portal-systemic encephalopathy:
bypass
Ammonia intoxication
Interfere with cerebral metabolism:
Depletion of glutamic acid, aspartic
acid and ATP
Depression cerebral blood flow and
Ammonia neurotoxicity
Elevation of ammonia:
detected in 60%~80%
Absolute concentration of
ammonia, ammonia
metabolites in blood or
cerebrospinal fluids, correlates
only roughly with the presence
or severity of HE
Few cases: within normal
Synergistic neurotoxins
Ammonia
Mercaptans
Short-chain fatty
acids
Phenols
Excitatory inhibitory
neurotransmitter
Neurotransmission:
Excitatory inhibitory
neurotransmitter
Imbalance of plasma amino acid:
More AAAs enter into blood-brain barrier and
CNS
Decreased synthesis of normal
neurotransmitters
L-Dopa
Dopamine
Noradrenoline
Enhanced synthesis of false
neurotransmitters
Octopamine
Tryptophan
Serotonin
- Aminobutyric acid
hypothesis
Endogenous ligands for the BZ
receptor: unknown
VEP = induced by BZ or
barbiturate
Substances in the brain: bind
to BZ receptor
GABA/BZs receptors
antagonists:
improve HE symptoms
Pathohistology
Brain may be normal or cerebral edema
Particularly in fulminant heptic failure
Cerebral edema is likely the secondly
changes
In patients with chronic liver disease
Astrocytes: increase in number and
enlargement
In a very long-standing case
Thin cortex, loss of neurons fibers,
laminar
PathoPhisIology
CT/MRI :
- Cerebral atrophy related to
the severity of the liver
dysfunction
- Marked in chronic persistent
encephalopathy
Clinical manifestation
In acute liver failure
Spontaneously appearing
Severe fatal hepatic dysfunction +
abrupt mental
deterioration + coma/death
high fever ,tachycardia ,
tachypnea
hyperventilation
Clinical manifestation
In chronic liver disease
Insidious onset
Characterized by subtle and/or intermittent
changes in consciousness personality intelligence
speech
Disturbed consciousness:
slowness,
somnolence,
disorientation, confusion deep coma
Personality changes: childishness
irritability
Intellectual deterioration: inability to produce
simple designs
with blocks or matches
Speech: slow
slurred
monotonous voice
Clinical manifestation
Criteria for clinical stages
Personality and mental
changes
Abnormal EEG patterns
Asterixis
Clinical stages of HE
Clinical stages of HE
Differential diagnosis
Hypoglycemia
Uremia
Diabetic ketoacidosis
Nonketotic hyperosmolar
syndrome
Subdural hematoma
Cerebrospinal infection
Treatment
Strategy for the management of
HE
Identify and correct the
precipitating cause(s)
Initiate ammonia-lowering
therapy
Minimize the potential medical
complications
of cirrhosis and depressed
Initiate ammonia-lowing
therapy
Initiate ammonia-lowing
therapy
Antibiotics
Neomycin: 2~4g/D (4~6g/D)
Litter is absorbed Impaired
hearing or deafness (in long term
use) Long term use (>1 month) is
not advisable
Metronidozol: 0.2g qid as
effective as neomycin
Prognosis
HE results from fulminant
hepatic failure (FHF): Poor 20%
survival rates
HE results from chronic liver
disease
Short-term: better than FHF
Long-term: guarded
Hypoxic encephalopathy
Brain perfusion
Mekanisme hipoksik
perception
Goetz:
of Clinical Neurology,
ed.,
< 25 to 20 mm
Hg :Textbook
consciousness
is rapidly2nd
lost
Copyright 2003 Elsevier
Parkinsonism
Cerebral palsy
Treatment
Initial resuscitation and stabilization,
Supportive treatment of hypoxicischemic
Perfusion and Blood Pressure
Management
fluid management,
hypoglycemia and hyperglycemia
treatment of seizures
Encephalopathy,
Hypertensive
hypertensive encephalopathy to
describe the encephalopathic
findings associated with the
accelerated malignant phase of
hypertension.
Hypertensive crisis
1. hypertensive emergency :
Acute or ongoing vital target organ
damage, such as damage to the brain,
kidney, or heart, in the setting of severe
hypertension is considered a hypertensive
emergency. It requires a prompt reduction
in blood pressure within minutes or hours.
2. hypertensive urgency.
The absence of target organ damage in
the presence of severe elevation of blood
pressure with diastolic blood pressure
frequently greater than 120 mm Hg is
considered hypertensive urgency, and it
requires reduction in blood pressure within
24-48 hours.
Manifestasi klinik
neurologic symptoms :headache,
confusion, visual disturbances, seizures,
nausea, and vomiting. Headaches are
usually anterior and constant in nature.
The onset of symptoms usually occurs
over 24-48 hours, with neurologic
progression over 24-48 hours.
end organ damage. :
Cardiovascular symptoms of aortic dissection,
congestive heart failure, angina, palpitations,
irregular heart beat, and dyspnea , Renal
Physical examination
Funduscopic examination: papilledema,
hemorrhage, exudates, and cotton-wool spots.
Neurologic examination :neurological nonfocal
deficits nystagmus to weakness ,mental status
ranging from confusion to coma.
vascular examination
Other target organ damage that may be found
includes the following:
Cardiovascular - S3, elevated neck veins, peripheral
edema, murmurs, abdominal pulsations, and
diminished pulses
Renal - Acute renal failure, pulmonary edema, and
peripheral edema
Pulmonary - Pulmonary edema, rales, and wheezes
Terapi
Medical Care
CBF ---60-90 mm Hg
Lowering the mean arterial pressure by
25% and the diastolic blood pressure to
100-110 mm Hg
Pharmacologic agents selected for use in
hypertensive encephalopathy should
have few or no CNS adverse effects.
Avoid agents such as clonidine,
reserpine, and methyldopa.