Encephalopathy is a general term describing a disease that affects the function or structure of your brain. There are many types of encephalopathy and brain disease.
Some types are permanent and some are temporary. Some types are present from birth and never change, while others are acquired after birth and may get progressively worse. This type of encephalopathy occurs when there are multiple traumas or injuries to the brain. These blows to the head lead to nerve damage in the brain.
Glycine encephalopathy is a genetic, or inherited, condition in which there are abnormally high levels of glycine an amino acid in the brain. Symptoms of glycine encephalopathy usually appear in infants soon after birth.
Hepatic encephalopathy is a result of liver disease. Hypertensive encephalopathy is a result of severely high blood pressure that goes untreated for too long. This can cause your brain to swell, leading to brain damage and hypertensive encephalopathy. This can result in permanent brain damage or dysfunction. It can be caused by a lack of oxygen to the brain, such as when a developing baby is exposed to alcohol in the womb.
Toxic-metabolic encephalopathy is a result of infections, toxins, or organ failure. This can also include the presence of an infection in the body or presence of toxic chemicals. Transmissible spongiform encephalopathies are also known as prion diseases. Prions are proteins that occur naturally in the body, but they can mutate and cause diseases that gradually damage and deteriorate your brain neurodegenerative diseases.
Prion diseases include:. Uremic encephalopathy is a result of kidney failure. It is believed to be caused by the buildup of uremic toxins in the blood.
This condition can cause mild confusion to deep coma. Long-term alcoholism , poor nutritional intake, and poor food absorption can cause a vitamin B-1 deficiency.
You may have difficulty with memory or focusing. You may also have trouble with problem-solving skills. Other people may notice symptoms in you before you do.
A changing personality is one such symptom. For example, you may be more outgoing than you were before the encephalopathy. You may be more or less calm than you were before the disease. You could also be lethargic and drowsy. You should see a doctor right away if you experience symptoms of encephalopathy. The most common antiepileptics that have been used during hospitalization include benzodiazepines, levetiracetam and phenytoin and upon discharge levetiracetam and phenytoin, with majority of them on a single agent.
Since seizures are uncommon out of the acute phase, antiepileptic agents may be quickly tapered. In a single center study, the median duration of antiepileptic agents was 3 months IQR 2—7 months.
The overall prognosis of both generalized and focal seizures in PRES is benign. Besides, not all patients with seizures have been treated with antiepileptic agents and none of these patients developed recurrent seizures 31 , It is unclear if antiepileptic agents play a role on the risk of subsequent seizures and epilepsy in these patients.
If antiepileptic agents are started, discontinuation following resolution of PRES should be considered, once there is adequate control of risk factors, and absence of factors that might substantially lower the seizure threshold.
It is not uncommon for patients to have recurrent episodes of PRES from recurrence of risk factors like sickle cell crisis, autoimmune conditions, hypertensive crisis, renal failure, and multiorgan failure. Management of malignant PRES requires aggressive supportive care. Various interventions that have been undertaken in patients with raised ICP include osmotherapy, draining of cerebrospinal fluid by external ventricular drain, craniectomy and evacuation of hematoma.
All patients achieved favorable functional outcomes based on the mRS modified Rankin Score of 1—2 on long term follow up In patients with acute obstructive hydrocephalus, an external ventricular drain placement may be required for management Certain deficits that require long-term care include epilepsy and motor deficits. PRES is an acute neurotoxic syndrome and the prognosis is highly dependent on the etiological factor. Studies have reported that patients with preeclampsia-eclampsia have less severe cerebral edema, hemorrhage, contrast enhancement with a tendency for complete resolution on imaging and good functional outcome 10 , Other factors that have been associated with poor outcome include severe encephalopathy, hypertensive etiology, hyperglycemia, neoplastic etiology, longer time to control the causative factor, the presence of multiple comorbidities, elevated CRP, low CSF glucose, and coagulopathy 9 , 10 , 47 , Various imaging features that are associated with poor outcome include corpus callosum involvement, extensive cerebral edema or worsening imaging severity, hemorrhage, subarachnoid hemorrhage, and restrictive diffusion on imaging 47 , 60 , 76 — The type, location and severity of hemorrhage that is associated with poor outcome are inconsistent across various studies 47 , 76 , While small hemorrhages do not have an impact on outcome, multiple or massive hemorrhages might be associated with poor outcome.
Several studies have demonstrated correlation between the degree of hypertension with clinical outcome and severity of edema on imaging. Interestingly, while the severity of edema on MRI correlated with clinical outcomes, the presence or patterns of gadolinium based contrast enhancement did not correlate with functional outcomes To summarize, although there are several associations, identifying a single predictor of outcome has been challenging in these patients.
Recent data from animal studies have demonstrated blood brain barrier disruption as a possible mechanism for development of vasogenic cerebral edema from acute hypertension and thus may be a target of future intervention Currently, there is paucity of data on its clinical implications in PRES. Currently, the available data on outcomes are from single institutions with paucity of data from long-term epidemiological studies.
Its heterogeneous nature limits its ability to generalize. PRES has a favorable prognosis in general, but fatalities can occur. A standardized algorithm that incorporates the clinical, etiological, serological markers, imaging features with various comorbidities and will assist in future studies.
Various pathophysiological mechanisms need to be explored at bench side to determine reliable laboratory and imaging markers and therapeutic interventions in order to improve functional outcomes are warranted. The author confirms being the sole contributor of this work and has approved it for publication. The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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Blood may be easily seen by the naked eye overt , or blood may be present in amounts Taking certain drugs, especially alcohol, some sedatives, pain relievers analgesics , or diuretics. Symptoms are those of impaired brain function, especially reduced alertness and confusion. In the earliest stages, subtle changes appear in logical thinking, personality, and behavior. The person's mood may change, and judgment may be impaired.
Normal sleep patterns may be disturbed. People may become depressed, anxious, or irritable. They may have trouble concentrating. As the disorder progresses, people cannot hold their hands steady when they stretch out their arms, resulting in a crude flapping motion of the hands asterixis.
Their muscles may jerk involuntarily or after people are exposed to a sudden noise, light, a movement, or another stimulus. This jerking is called myoclonus Myoclonus Myoclonus refers to quick, lightning-like jerks contractions of a muscle or a group of muscles. Myoclonus may occur normally for example, jerking of a leg when a person is falling asleep Also, people usually become drowsy and confused, and movements and speech become sluggish.
Disorientation is common. Less often, people with encephalopathy become agitated and excited. Eventually, as liver function continues to deteriorate, they may lose consciousness and lapse into a coma.
Coma often leads to death, despite treatment. Diagnosis is based mainly on symptoms, results of the examination, and response to treatment. Doctors ask about possible triggers for the encephalopathy such as an infection or a drug to identify possible causes. They do blood tests Liver Blood Tests Liver tests are blood tests that represent a noninvasive way to screen for the presence of liver disease for example, hepatitis in donated blood and to measure the severity and progress of The ammonia level is also measured.
The level is usually abnormally high indicating liver malfunction , but measuring the level is not always a reliable way to diagnose encephalopathy. Doctors may do mental status tests Mental Status When a neurologic disorder is suspected, doctors usually evaluate all of the body systems during the physical examination, but they focus on the nervous system. Examination of the nervous system—the Electroencephalography Electroencephalography Diagnostic procedures may be needed to confirm a diagnosis suggested by the medical history and neurologic examination.
Electroencephalography EEG is a simple, painless procedure in which
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