Encephalopathy

Encephalopathy (/ɛnˌsɛfəˈlɒpəθi/; from Ancient Greek: ἐνκέφαλος "brain" + πάθος "suffering") means any disorder or disease of the brain, especially chronic degenerative conditions.[1] In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of overall brain dysfunction; this syndrome has many possible organic and inorganic causes.

Not to be confused with Anencephaly.
Encephalopathy
SpecialtyNeurology

Signs and 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.[2] An altered state of consciousness may range from failure of selective attention to drowsiness.[3] 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).[3]

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.[2] Other neurological signs may include involuntary grasping and sucking motions, nystagmus (rapid, involuntary eye movement), jactitation (restlessness while in bed), 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.[3]

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.[4]

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.[5]

HIV encephalopathy can lead to dementia.

Types

There are many types of encephalopathy. Some examples include:

Toxicity from chemotherapy

Chemotherapy medication, for example, fludarabine can cause a permanent severe global encephalopathy.[9] Ifosfamide can cause a severe encephalopathy (but it can be reversible with stopping use of the drug and starting the use of methylene blue).[9] Bevacizumab and other anti–vascular endothelial growth factor medication can cause posterior reversible encephalopathy syndrome.[9]

Toxicity from psychotropic medications

All therapeutic interventions are double-edged swords with benefits and adverse effects, and pharmacotherapy is not an exception. Shortly after the introduction of conventional antipsychotic drugs into clinical practice, relatively rare but serious complications with hyperthermia, muscle rigidity, autonomic instability, and disturbed mental status were recognized to develop in some patients treated with antipsychotics. This type of encephalopathy induced by the use of antipsychotics was referred to as neuroleptic malignant syndrome (NMS), and almost all physicians prescribing antipsychotics are nowadays aware of this adverse phenomenon. Another well-known type of encephalopathy associated with psychotropic drug therapy is serotonin toxicity (ST) or serotonin syndrome (SS), which is characterized by autonomic and neuromuscular symptoms and altered mental status. In contrast with the idiosyncratic nature of NMS, ST is a spectrum pathophysiological state assumed to derive from excess serotonergic neural transmission caused by serotonin-related psychotropic agents. In these two decades, pharmacotherapy with psychotropic drugs for mentally ill patients has been dramatically changed, and classical prototypal antipsychotics and antidepressants have been replaced with atypical antipsychotics and selective serotonin reuptake inhibitors (SSRIs), respectively. These newly developed psychotropic drugs are generally safer and more tolerable than older drugs. However, atypical antipsychotics are not free of the risk of development of NMS, and the explosive prevalence of SSRIs prescribed not only for depression but also for a number of psychiatric diagnoses such as anxiety, eating, impulse-control, and personality disorders may increase the incidence of ST. Therefore, these two pathological states still remain as major adverse effects of psychotropic drugs involving altered functioning of the central nervous system (CNS), to which all clinicians prescribing psychoactive drugs should pay attention. The popularity of SSRIs also increased the case reports of patients experiencing discontinuation syndrome, which sometimes includes CNS symptoms like anxiety and irritability. In this chapter, the author provides a comprehensive overview of the above- mentioned adverse effects affecting the CNS function associated with psychotropic pharmacotherapy. In addition, several other pathological conditions potentially causing encephalopathic symptoms in psychiatric patients treated with psychotropic drugs, e.g., hyponatremia, valproate-induced hyperammonemia, transient splenial lesion of the corpus callosum, and so on, are also described.

Diagnosis

Blood tests, cerebrospinal fluid examination by lumbar puncture (also known as spinal tap), brain imaging studies, electroencephalography (EEG), neuropsychological testing 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.

Treatment

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 people. In severe cases, dialysis or organ replacement surgery may be needed.

Sympathomimetic drugs can increase motivation, cognition, motor performance and alertness in persons with encephalopathy caused by brain injury, chronic infections, strokes, brain tumors.[10]

When the encephalopathy is caused by untreated celiac disease or non-celiac gluten sensitivity, the gluten-free diet stops the progression of brain damage and improves the headaches.[7]

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. These permanent deficits can be considered a form of stable dementia. Some encephalopathies can be fatal.

Terminology

Encephalopathy is a difficult term because it can be used to denote either a disease or finding (i.e., an observable sign in a person).

When referring to a finding, encephalopathy refers to permanent (or degenerative)[11] brain injury, or a reversible one. It can be due to direct injury to the brain, or illness remote from the brain. The individual findings that cause a clinician to refer to a person as having encephalopathy include intellectual disability, irritability, agitation, delirium, confusion, somnolence, stupor, coma and psychosis. As such, describing a person as having a clinical picture of encephalopathy is not a very specific description.

When referring to a disease, encephalopathy refers 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 have a number of causes including nutritional deficiencies and toxins.

See also

References
  1. The British Medical Association (BMA) (2002). Illustrated Medical Dictionary. A Dorling Kindersley Book. p. 199. ISBN 978-0-75-133383-1.
  2. Manfred Oehmichen; Roland N. Auer; Hans Günter König (2006). Forensic Neuropathology and Associated Neurology. Springer Science & Business Media. p. 611. ISBN 978-3-540-28995-1.
  3. A.J. Larner (2016). A Dictionary of Neurological Signs. Springer. p. 112. ISBN 978-3-319-29821-4.
  4. American Psychiatric Association (2006). American Psychiatric Association Practice Guidelines for the Treatment of Psychiatric Disorders: Compendium 2006. American Psychiatric Pub. p. 210. ISBN 978-0-89042-385-1.
  5. Bost C, Pascual O, Honnorat J (2016). "Autoimmune encephalitis in psychiatric institutions: current perspectives". Neuropsychiatr Dis Treat. 12: 2775–2787. doi:10.2147/NDT.S82380. PMC 5089825. PMID 27822050.
  6. Müller M, Baumeier A, Ringelstein E, Husstedt I (2008). "Long-term tracking of neurological complications of encephalopathy and myopathy in a patient with nephropathic cystinosis: a case report and review of the literature". J Med Case Rep. 2: 235. doi:10.1186/1752-1947-2-235. PMC 2491650. PMID 18644104.
  7. Losurdo G, Principi M, Iannone A, Amoruso A, Ierardi E, Di Leo A, et al. (2018). "Extra-intestinal manifestations of non-celiac gluten sensitivity: An expanding paradigm". World J Gastroenterol (Review). 24 (14): 1521–1530. doi:10.3748/wjg.v24.i14.1521. PMC 5897856. PMID 29662290.
  8. Carapancea, Evelina; Cornet, Marie-Coralie; Milh, Mathieu; De Cosmo, Lucrezia; Huang, Eric J.; Granata, Tiziana; Striano, Pasquale; Ceulemans, Berten; Stein, Anja; Morris-Rosendahl, Deborah; Conti, Greta; Mitra, Nipa; Raymond, F. Lucy; Rowitch, David H.; Solazzi, Roberta (2023-03-21). "Clinical and Neurophysiologic Phenotypes in Neonates With BRAT1 Encephalopathy". Neurology. 100 (12): e1234–e1247. doi:10.1212/WNL.0000000000206755. ISSN 1526-632X. PMC 10033164. PMID 36599696.
  9. Ed. Stephen L. Hauser, S. Andrew Josephson (2013). Harrison's Neurology in Clinical Medicine (3rd ed.). McGraw Hill Professional. p. 438. ISBN 978-0-07-181501-7.
  10. Benjamin J. Sadock; Virginia A. Sadock (2008). Kaplan & Sadock's Concise Textbook of Clinical Psychiatry. Lippincott Williams & Wilkins. p. 78. ISBN 978-0-7817-8746-8.
  11. "encephalopathy" at Dorland's Medical Dictionary

Further reading
  • The Diagnosis of Stupor and Coma by Plum and Posner, ISBN 0-19-513898-8, remains one of the best detailed observational references to the condition.
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