Wernicke encephalopathy: Difference between revisions
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'''Wernicke encephalopathy''' is "an acute neurological disorder characterized by the triad of ophthalmoplegia, ataxia, and disturbances of mental activity or consciousness. Eye movement abnormalities include nystagmus, external rectus palsies, and reduced conjugate gaze. [[Thiamine]] deficiency and chronic [[alcoholism]] are associated conditions. Pathologic features include periventricular petechial hemorrhages and neuropil breakdown in the diencephalon and brainstem. Chronic thiamine deficiency may lead to [[Korsakoff's syndrome|Korsakoff syndrome]].<ref name="title">{{cite web |url=http://www.nlm.nih.gov/cgi/mesh/2008/MB_cgi?mode=&index=14334 |title=Wernicke Encephalopathy|author=National Library of Medicine |accessdate=2008-01-01 |format= |work=}}</ref> | '''Wernicke encephalopathy''' is "an acute neurological disorder characterized by the triad of ophthalmoplegia, ataxia, and disturbances of mental activity or consciousness. Eye movement abnormalities include nystagmus, external rectus palsies, and reduced conjugate gaze. [[Thiamine]] deficiency and chronic [[alcoholism]] are associated conditions. Pathologic features include periventricular petechial hemorrhages and neuropil breakdown in the diencephalon and brainstem. Chronic thiamine deficiency may lead to [[Korsakoff's syndrome|Korsakoff syndrome]].<ref name="title">{{cite web |url=http://www.nlm.nih.gov/cgi/mesh/2008/MB_cgi?mode=&index=14334 |title=Wernicke Encephalopathy|author=National Library of Medicine |accessdate=2008-01-01 |format= |work=}}</ref> | ||
This disorder was first described in 1881 by Carl Wernicke based on three untreated cases.<ref name="pmid18056751">{{cite journal |author=Thomson AD, Cook CC, Guerrini I, Sheedy D, Harper C, Marshall EJ |title=Wernicke's encephalopathy revisited * Translation of the case history section of the original manuscript by Carl Wernicke 'Lehrbuch der Gehirnkrankheiten fur Aerzte and Studirende' (1881) with a commentary |journal=Alcohol Alcohol |volume= |issue= |pages= |year=2007 |pmid=18056751 |doi=10.1093/alcalc/agm144}}</ref> | |||
==Cause/etiology== | ==Cause/etiology== | ||
Wernicke encephalopathy | |||
The most common causes of Wernicke encephalopathy are [[alcoholism]] and a thiamine deficiency. Many conditions can cause a thiamine deficiency, such as hyperemesis gravidarum (vomiting during pregnancy),<ref name="pmid16551377">{{cite journal |author=Chiossi G, Neri I, Cavazzuti M, Basso G, Facchinetti F |title=Hyperemesis gravidarum complicated by Wernicke encephalopathy: background, case report, and review of the literature |journal=Obstet Gynecol Surv |volume=61 |issue=4 |pages=255–68 |year=2006 |pmid=16551377 |doi=10.1097/01.ogx.0000206336.08794.65}}</ref> dialysis,<ref name="pmid11684545">{{cite journal |author=Hung SC, Hung SH, Tarng DC, Yang WC, Chen TW, Huang TP |title=Thiamine deficiency and unexplained encephalopathy in hemodialysis and peritoneal dialysis patients |journal=Am. J. Kidney Dis. |volume=38 |issue=5 |pages=941–7 |year=2001 |pmid=11684545 |doi=}}</ref> and malnourished states in end-stage cancer, intractable vomiting, and extended courses of intravenous fluids with no other source of nutrition.<ref name="pmid11863078">{{cite journal |author=Ogershok PR, Rahman A, Nestor S, Brick J |title=Wernicke encephalopathy in nonalcoholic patients |journal=Am. J. Med. Sci. |volume=323 |issue=2 |pages=107–11 |year=2002 |pmid=11863078 |doi=}}</ref> | |||
==Biochemistry== | |||
Thiamine is converted by the enzyme thiamine pyrophosphokinase to thiamine pyrophosphate. Thiamine pyrophosphate is a coenzyme for dehydrogenase enzymes in aerobic respiration, and the enzyme transketolase in the pentose phosphate pathway.<ref name="isbn0-7167-3051-0">{{cite book |author=Stryer, Lubert; Berg, Jeremy Mark; Tymoczko, John L. |title=Biochemistry |publisher=W.H. Freeman |location=San Francisco |year=2002 |pages= |isbn=0-7167-3051-0 |oclc= |doi=}} http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=stryer [Full text]</ref> | |||
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==Diagnosis== | ==Diagnosis== | ||
===History and physical examination=== | ===History and physical examination=== | ||
Most patients will have absent nystagmus to cold caloric testing.<ref name="pmid5305749">{{cite journal |author=Ghez C |title=Vestibular paresis: a clinical feature of Wernicke's disease |journal=J. Neurol. Neurosurg. Psychiatr. |volume=32 |issue=2 |pages=134–9 |year=1969 |pmid=5305749 |doi=}}</ref> | Most patients will have absent nystagmus to cold caloric testing.<ref name="pmid5305749">{{cite journal |author=Ghez C |title=Vestibular paresis: a clinical feature of Wernicke's disease |journal=J. Neurol. Neurosurg. Psychiatr. |volume=32 |issue=2 |pages=134–9 |year=1969 |pmid=5305749 |doi=}}</ref> | ||
===Blood tests=== | |||
;Thiamine level | |||
The concentration of thiamine (or more specifically thiamine pyrophosphate) can be measured in whole blood by high performance liquid chromatography (HPLC). Thiamine pyrophosphate may also be low in elderly and cause subtle peripheral neuropathy.<ref name="pmid10791444">{{cite journal |author=Wilkinson TJ, Hanger HC, George PM, Sainsbury R |title=Is thiamine deficiency in elderly people related to age or co-morbidity? |journal=Age Ageing |volume=29 |issue=2 |pages=111–6 |year=2000 |pmid=10791444 |doi=}}</ref> | |||
;Erythrocyte thiamine transketolase activity | |||
Thiamine transketolase functional assay in whole blood may be used.<ref name="pmid7306754">{{cite journal |author=Leigh D, McBurney A, McIlwain H |title=Erythrocyte transketolase activity in the Wernicke-Korsakoff syndrome |journal=Br J Psychiatry |volume=139 |issue= |pages=153–6 |year=1981 |pmid=7306754 |doi=}}</ref>, However, it can also be falsely low in other conditions. | |||
<!-- effect of thiamine pyrophosphate (TPP) on transketolase activity (TPP effect) --> | |||
===MRI=== | ===MRI=== | ||
The MRI may show enhancement of mamillary bodies with gadolinium suggesting reversible cytotoxic | The MRI may show enhancement of mamillary bodies with gadolinium suggesting reversible cytotoxic edema in 58% of patients.<ref name="pmid17698536">{{cite journal |author=Zuccoli G, Gallucci M, Capellades J, ''et al'' |title=Wernicke encephalopathy: MR findings at clinical presentation in twenty-six alcoholic and nonalcoholic patients |journal=AJNR Am J Neuroradiol |volume=28 |issue=7 |pages=1328–31 |year=2007 |pmid=17698536 |doi=10.3174/ajnr.A0544}}</ref> | ||
==Treatment== | ==Treatment== | ||
The treatment is thiamine, thiamine tetrahydrofurfuryldisulphide (TTFD). Outcomes are worse if treatment is delayed.<ref name="pmid7306754">{{cite journal |author=Leigh D, McBurney A, McIlwain H |title=Erythrocyte transketolase activity in the Wernicke-Korsakoff syndrome |journal=Br J Psychiatry |volume=139 |issue= |pages=153–6 |year=1981 |pmid=7306754 |doi=}}</ref> | The treatment is thiamine, thiamine tetrahydrofurfuryldisulphide (TTFD). Outcomes are worse if treatment is delayed.<ref name="pmid7306754">{{cite journal |author=Leigh D, McBurney A, McIlwain H |title=Erythrocyte transketolase activity in the Wernicke-Korsakoff syndrome |journal=Br J Psychiatry |volume=139 |issue= |pages=153–6 |year=1981 |pmid=7306754 |doi=}}</ref> | ||
==References== | ==References== | ||
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Latest revision as of 00:29, 5 January 2008
Wernicke encephalopathy is "an acute neurological disorder characterized by the triad of ophthalmoplegia, ataxia, and disturbances of mental activity or consciousness. Eye movement abnormalities include nystagmus, external rectus palsies, and reduced conjugate gaze. Thiamine deficiency and chronic alcoholism are associated conditions. Pathologic features include periventricular petechial hemorrhages and neuropil breakdown in the diencephalon and brainstem. Chronic thiamine deficiency may lead to Korsakoff syndrome.[1]
This disorder was first described in 1881 by Carl Wernicke based on three untreated cases.[2]
Cause/etiology
The most common causes of Wernicke encephalopathy are alcoholism and a thiamine deficiency. Many conditions can cause a thiamine deficiency, such as hyperemesis gravidarum (vomiting during pregnancy),[3] dialysis,[4] and malnourished states in end-stage cancer, intractable vomiting, and extended courses of intravenous fluids with no other source of nutrition.[5]
Biochemistry
Thiamine is converted by the enzyme thiamine pyrophosphokinase to thiamine pyrophosphate. Thiamine pyrophosphate is a coenzyme for dehydrogenase enzymes in aerobic respiration, and the enzyme transketolase in the pentose phosphate pathway.[6]
Diagnosis
History and physical examination
Most patients will have absent nystagmus to cold caloric testing.[7]
Blood tests
- Thiamine level
The concentration of thiamine (or more specifically thiamine pyrophosphate) can be measured in whole blood by high performance liquid chromatography (HPLC). Thiamine pyrophosphate may also be low in elderly and cause subtle peripheral neuropathy.[8]
- Erythrocyte thiamine transketolase activity
Thiamine transketolase functional assay in whole blood may be used.[9], However, it can also be falsely low in other conditions.
MRI
The MRI may show enhancement of mamillary bodies with gadolinium suggesting reversible cytotoxic edema in 58% of patients.[10]
Treatment
The treatment is thiamine, thiamine tetrahydrofurfuryldisulphide (TTFD). Outcomes are worse if treatment is delayed.[9]
References
- ↑ National Library of Medicine. Wernicke Encephalopathy. Retrieved on 2008-01-01.
- ↑ Thomson AD, Cook CC, Guerrini I, Sheedy D, Harper C, Marshall EJ (2007). "Wernicke's encephalopathy revisited * Translation of the case history section of the original manuscript by Carl Wernicke 'Lehrbuch der Gehirnkrankheiten fur Aerzte and Studirende' (1881) with a commentary". Alcohol Alcohol. DOI:10.1093/alcalc/agm144. PMID 18056751. Research Blogging.
- ↑ Chiossi G, Neri I, Cavazzuti M, Basso G, Facchinetti F (2006). "Hyperemesis gravidarum complicated by Wernicke encephalopathy: background, case report, and review of the literature". Obstet Gynecol Surv 61 (4): 255–68. DOI:10.1097/01.ogx.0000206336.08794.65. PMID 16551377. Research Blogging.
- ↑ Hung SC, Hung SH, Tarng DC, Yang WC, Chen TW, Huang TP (2001). "Thiamine deficiency and unexplained encephalopathy in hemodialysis and peritoneal dialysis patients". Am. J. Kidney Dis. 38 (5): 941–7. PMID 11684545. [e]
- ↑ Ogershok PR, Rahman A, Nestor S, Brick J (2002). "Wernicke encephalopathy in nonalcoholic patients". Am. J. Med. Sci. 323 (2): 107–11. PMID 11863078. [e]
- ↑ Stryer, Lubert; Berg, Jeremy Mark; Tymoczko, John L. (2002). Biochemistry. San Francisco: W.H. Freeman. ISBN 0-7167-3051-0. http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=stryer [Full text]
- ↑ Ghez C (1969). "Vestibular paresis: a clinical feature of Wernicke's disease". J. Neurol. Neurosurg. Psychiatr. 32 (2): 134–9. PMID 5305749. [e]
- ↑ Wilkinson TJ, Hanger HC, George PM, Sainsbury R (2000). "Is thiamine deficiency in elderly people related to age or co-morbidity?". Age Ageing 29 (2): 111–6. PMID 10791444. [e]
- ↑ 9.0 9.1 Leigh D, McBurney A, McIlwain H (1981). "Erythrocyte transketolase activity in the Wernicke-Korsakoff syndrome". Br J Psychiatry 139: 153–6. PMID 7306754. [e]
- ↑ Zuccoli G, Gallucci M, Capellades J, et al (2007). "Wernicke encephalopathy: MR findings at clinical presentation in twenty-six alcoholic and nonalcoholic patients". AJNR Am J Neuroradiol 28 (7): 1328–31. DOI:10.3174/ajnr.A0544. PMID 17698536. Research Blogging.