Encephalopathy

Clinic

• Encephalopathy means any disorder or disease of the brain esp chronic degenerative conditions.
• Encephalopathy does not refer to a single disease, but rather to a syndrome of overall brain dysfunction

Signs / Symptoms

• The hallmark of encephalopathy is an altered mental state or delirium. Characteristic of the altered mental state is impairment of the cognition, attention, orientation, sleep–wake cycle and consciousness. An altered state of consciousness may range from failure of selective attention to drowsiness. Hypervigilance may be present; with or without: cognitive deficits, headache, epileptic seizures, myoclonus (involuntary twitching of a muscle or group of muscles) or asterixis ("flapping tremor" of the hand when wrist is extended).

Depending on the type and severity of encephalopathy, common neurological symptoms are loss of cognitive function, subtle personality changes, and an inability to concentrate. Other neurological signs may include dysarthria, hypomimia, problems with movements (they can be clumsy or slow), ataxia, tremor. Other neurological signs may include involuntary grasping and sucking motions, nystagmus (rapid, involuntary eye movement), jactitation (restlessness while in bed),^{[citation needed]} and respiratory abnormalities such as Cheyne-Stokes respiration (cyclic waxing and waning of tidal volume), apneustic respirations and post-hypercapnic apnea. Focal neurological deficits are less common.

Wernicke encephalopathy can co-occur with Korsakoff alcoholic syndrome, characterized by amnestic-confabulatory syndrome: retrograde amnesia, anterograde amnesia, confabulations (invented memories), poor recall and disorientation.

Anti-NMDA receptor encephalitis is the most common autoimmune encephalitis. It can cause paranoid and grandiose delusions, agitation, hallucinations (visual and auditory), bizarre behavior, fear, short-term memory loss, and confusion.

HIV encephalopathy can lead to dementia.

Types

Metabolic

• Mitochondrial encephalopathy: Metabolic disorder caused by dysfunction of mitochondrial DNA. Can affect many body systems, particularly the brain and nervous system.
• Glycine encephalopathy: A genetic metabolic disorder involving excess production of glycine.
• Hepatic encephalopathy: Arising from advanced cirrhosis of the liver.
• Hypoxic / Ischemic encephalopathy: Permanent or transitory encephalopathy arising from severely reduced oxygen delivery to the brain.
• Static encephalopathy: Unchanging, or permanent, brain damage, usually caused by prenatal exposure to ethanol.
• Uremic encephalopathy: Arising from high levels of toxins normally cleared by the kidneys—rare where dialysis is readily available.
• Wernicke's encephalopathy: Arising from thiamine (B₁) deficiency, usually in the setting of alcoholism.
• Hyperammonemia: a condition caused by high levels of ammonia, which is due to inborn errors of metabolism (including urea cycle disorder or multiple carboxylase deficiency), a diet with excessive levels of protein, deficiencies of specific nutrients such as arginine or biotin, or organ failure.
• Toxic-metabolic encephalopathy: A catch-all for brain dysfunction caused by infection, organ failure, or intoxication.
• Gluten encephalopathy: Focal abnormalities of the white matter (generally area of low perfusion) are appreciated through magnetic resonance. Migraine is the most common symptom reported.

Ischemic/ Traumatic

• Hypertensive encephalopathy: Arising from acutely increased blood pressure.
• Chronic traumatic encephalopathy: a progressive degenerative disease associated with multiple concussions and other forms of brain injury
• Toxic encephalopathy: A form of encephalopathy caused by chemicals and prescription drugs, often resulting in permanent brain damage.
• Neonatal encephalopathy (hypoxic-ischemic encephalopathy): An obstetric form, often occurring due to lack of oxygen in bloodflow to brain-tissue of the fetus during labour or delivery.

Autoimmune

• Hashimoto's encephalopathy: Arising from an auto-immune disorder.
• Anti-NMDA receptor encephalitis: An auto-immune encephalitis.

Bacterial/ Viral / Prion

• Transmissible spongiform encephalopathy: A collection of diseases all caused by prions, and characterized by "spongy" brain tissue, impaired locomotion or coordination, and a 100% mortality rate. Includes
  • KRU
  • CJD
  • NVCJD (mad ocw disease)
• HIV encephalopathy (Characterized by atrophy and ill-defined white matter hyperintensity).

• Sepsis-associated encephalopathy

• Salmonella encephalopathy caused by food poisoning
• LBB encephalopathy

Accompanied by

• Encephalomyopathy: A combination of encephalopathy and myopathy. Causes may include mitochondrial disease (particularly MELAS) or chronic hypophosphatemia, as may occur in cystinosis.
• Early myoclonic epileptic encephalopathy (possibly due to metabolic disorders).

• Epileptic encephalopathies: Early infantile epileptic encephalopathy (acquired or congenital abnormal cortical development)