Encephalopathy

Encephalopathy
Classification and external resources
ICD-9	348.30
MeSH	D001927

Encephalopathy /ɛnˌsɛfəˈlɒpəθi/ means disorder or disease of the brain.^[1] In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of global brain dysfunction; this syndrome can be caused by many different illnesses.

Contents 1 Terminology 2 Types 3 Symptoms 4 Diagnosis 5 Therapy 6 Prognosis 7 See also 8 References 9 Further reading

Terminology

In some contexts it refers to permanent (or degenerative)^[2] brain injury, and in others it is reversible. It can be due to direct injury to the brain, or illness remote from the brain. In medical terms it can refer to a wide variety of brain disorders with very different etiologies, prognoses and implications. For example, prion diseases, all of which cause transmissible spongiform encephalopathies, are invariably fatal, but other encephalopathies are reversible and can be caused by nutritional deficiencies, toxins, and several other causes.

Types

There are many types of encephalopathy. Some examples include:

• 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
• 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 deficiency, usually in the setting of alcoholism
• Hashimoto's encephalopathy—Arising from an auto-immune disorder
• Hypertensive encephalopathy—Arising from acutely increased blood pressure
• Lyme encephalopathy—Arising from the Borrelia burgdorferi bacteria.
• Toxic encephalopathy—A form of encephalopathy caused by chemicals, often resulting in permanent brain damage
• Toxic-Metabolic encephalopathy—A catch-all for brain dysfunction caused by infection, organ failure, or intoxication
• Transmissible spongiform encephalopathy—A collection of diseases all caused by prions, and characterized by "spongy" brain tissue (riddled with holes), impaired locomotion or coordination, and a 40 out of 40 fatality rate. Includes bovine spongiform encephalopathy (mad cow disease), scrapie, and kuru among others.
• Neonatal encephalopathy—an obstetric form, often occurring due to lack of oxygen in bloodflow to brain-tissue of the fetus during labour or delivery
• Encephalomyopathy, a combination of encephalopathy and myopathy. Causes may include mitochondrial disease (particularly MELAS) or chronic hypophosphatemia, as may occur in cystinosis.^[3]

Symptoms

The hallmark of encephalopathy is an altered mental state. Depending on the type and severity of encephalopathy, common neurological symptoms are loss of cognitive function, subtle personality changes, inability to concentrate, lethargy, and depressed consciousness. Other neurological signs may include myoclonus (involuntary twitching of a muscle or group of muscles), asterixis (abrupt loss of muscle tone, quickly restored), nystagmus (rapid, involuntary eye movement), tremor, seizures, jactitation (restless picking at things characteristic of severe infection), and respiratory abnormalities such as Cheyne-Stokes respiration (cyclic waxing and waning of tidal volume), apneustic respirations, and post-hypercapnic apnea.

Diagnosis

Blood tests, spinal fluid examination by lumbar puncture, imaging studies, electroencephalograms and similar diagnostic studies may be used to differentiate the various causes of encephalopathy.

Diagnosis is frequently clinical. That is, no set of tests give the diagnosis, but the entire presentation of the illness with nonspecific test results informs the experienced clinician of the diagnosis.

Therapy

Treatment varies according to the type and severity of the encephalopathy. Anticonvulsants may be prescribed to reduce or halt any seizures. Changes to diet and nutritional supplements may help some patients. In severe cases, dialysis or organ replacement surgery may be needed.

Prognosis

Treating the underlying cause of the disorder may improve or reverse symptoms. However, in some cases, the encephalopathy may cause permanent structural changes and irreversible damage to the brain. Some encephalopathies can be fatal.

See also

• Brain damage
• Neurobiology
• Neurobiological brain disorder

References

• Adapted from: Office of Communications and Public Liaison (9 November 2010). "NINDS Encephalopathy Information Page". National Institute of Neurological Disorders and Stroke, National Institutes of Health. http://www.ninds.nih.gov/disorders/encephalopathy/encephalopathy.htm. 

Further reading

• The Diagnosis of Stupor and Coma by Plum and Posner, ISBN 0195138988, remains one the of best detailed observational references to the condition.

Pathology of the nervous system, primarily CNS (G04–G47, 323–349) Inflammation Brain Encephalitis (Viral encephalitis, Herpesviral encephalitis) · Cavernous sinus thrombosis · Brain abscess (Amoebic) Spinal cord Myelitis: Poliomyelitis · Demyelinating disease (Transverse myelitis) · Tropical spastic paraparesis · Epidural abscess Both/either Encephalomyelitis (Acute disseminated) Meningoencephalitis Brain/ encephalopathy Degenerative Extrapyramidal and movement disorders Basal ganglia disease: Parkinsonism (PD, Postencephalitic, NMS) · PKAN · Tauopathy (PSP) · Striatonigral degeneration · Hemiballismus · HD · OA Dyskinesia: Dystonia (Status dystonicus, Spasmodic torticollis, Meige's, Blepharospasm) · Chorea (Choreoathetosis) · Myoclonus (Myoclonic epilepsy) · Akathesia Tremor (Essential tremor, Intention tremor) · Restless legs · Stiff person Dementia Tauopathy: Alzheimer's (Early-onset) · Frontotemporal dementia/Frontotemporal lobar degeneration (Pick's, Dementia with Lewy bodies) Multi-infarct dementia Mitochondrial disease Leigh's Demyelinating autoimmune (Multiple sclerosis, Neuromyelitis optica, Schilder's disease) · hereditary (Adrenoleukodystrophy, Alexander, Canavan, Krabbe, ML, PMD, VWM, MFC, CAMFAK syndrome) · Central pontine myelinolysis · Marchiafava-Bignami disease · Alpers' disease Episodic/ paroxysmal Seizure/epilepsy Focal · Generalised · Status epilepticus · Myoclonic epilepsy Headache Migraine (Familial hemiplegic) · Cluster · Tension Cerebrovascular TIA (Amaurosis fugax, Transient global amnesia) Stroke (MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke) Sleep disorders Insomnia · Hypersomnia · Sleep apnea (Obstructive, Ondine's curse) · Narcolepsy · Cataplexy · Kleine-Levin · Circadian rhythm sleep disorder (Advanced sleep phase syndrome, Delayed sleep phase syndrome, Non-24-hour sleep-wake syndrome, Jet lag) CSF Intracranial hypertension (Hydrocephalus/NPH, Idiopathic intracranial hypertension) · Cerebral edema · Intracranial hypotension Other Brain herniation · Reye's · Hepatic encephalopathy · Toxic encephalopathy Spinal cord/ myelopathy Syringomyelia · Syringobulbia · Morvan's syndrome · Vascular myelopathy (Foix-Alajouanine syndrome) · Spinal cord compression Both/either Degenerative SA Friedreich's ataxia · Ataxia telangiectasia MND UMN only: PLS · PP · HSP LMN only: PMA · PBP (Fazio-Londe, Infantile progressive bulbar palsy) · Spinal muscular atrophies (SMA, SMARD1, SBMA, SMAX2, SMA-PCH) both: ALS M: CNS anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr proc, drug(N1A/2AB/C/3/4/7A/B/C/D)