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encephalopathy
syndrome of global brain dysfunction
Etiology:
- infection
- metabolic brain disease (mitochondrial dysfunction)
- increased intracranial pressure
- toxins
- chronic traumatic brain injury
- malnutrition
- cerebral anoxia [2]
- autoimmune encephalopathy (anti-LGI1 > anti-IgLON5) [3]
Clinical manifestations:
- altered mental status
- common
- progressive memory loss & cognitive impairment
- subtle personality changes
- inability to concentrate
- fatigue, lethargy
- headache [4]
- progressive loss of consciousness
- other
- myoclonus
- nystagmus
- tremor
- muscle atrophy & muscle weakness
- delirium
- seizures
- dysphagia
- aphasia
Laboratory:
- complete blood count
- serum electrolytes
- serum glucose
- serum calcium
- renal function tests
- liver function tests, serum albumin
- plasma ammonia
- serum TSH
- serum C-reactive protein or erythrocyte sedimentation rate
- urinalysis & urine toxicology screen
- arterial blood gas
- lumbar puncture with CSF analysis
- IgLON5 Ab in CSF
- LGI1 Ab in CSF
- HIV testing
Special laboratory:
- electroencephalogram
Radiology:
- neuroimaging (MRI)
- autoimmune encephalopathy in the elderly may not be associated with signs of inflammation [3]
Management:
- symptomatic [2]
Related
cognitive impairment
Specific
chronic traumatic encephalopathy (CTE)
EMPF encephalopathy; encephalopathy, lethal, due to defective mitochondrial & peroxisomal fission (EMPF)
encephalitis
encephalomalacia
ethylmalonic encephalopathy
familial encephalopathy with neuroserpin inclusion bodies
Hashimoto encephalopathy
hyperglycemic-hyperosmolar syndrome; hyperglycemic-hyperosmolar nonketotic coma (HHNC)
infantile epileptic encephalopathy
isolated (primary) central nervous system vasculitis (angiitis) (PACNS)
leukoencephalopathy
limbic-predominant age-related TDP-43 encephalopathy (LATE); limbic-predominant amnestic neurodegenerative syndrome (LANS)
metabolic encephalopathy; toxic encephalopathy
mitochondrial encephalomyopathy
myoclonic encephalopathy
portal systemic encephalopathy (PSE)
posterior reversible encephalopathy syndrome (PRES)
Reye's syndrome
transmissible spongiform encephalopathy (prion disease)
Wernicke's syndrome (Wernicke's encephalopathy)
General
disease/disorder primarily affecting brain
neurologic disease
syndrome
References
- Wikipedia: Encephalopathy
http://en.wikipedia.org/wiki/Encephalopathy
- National Institute of Neurological Disorders and Stroke (NINDS)
NINDS Encephalopathy Information Page
http://www.ninds.nih.gov/disorders/encephalopathy/encephalopathy.htm
- Probasco JC
Antibody-Associated CNS Syndromes Without Inflammation in
Elders.
NEJM Journal Watch. Nov 7, 2017
Massachusetts Medical Society
(subscription needed) http://www.jwatch.org
- Escudero DEscudero D, Guasp M, Arino H et al.
Antibody-associated CNS syndromes without signs of
inflammation in the elderly.
Neurology 2017 Oct 3; 89:1471
PMID: 28878050
- Blum SM, Prust ML, Patel R et al
Stream of Consciousness.
N Engl J Med 2018; 378:1336-1342. April 5, 2018
PMID: 29617591
http://www.nejm.org/doi/full/10.1056/NEJMcps1714950