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Transcript

1st clinical conference of the batch of 2020

1st clinical conference of the batch of 2020

a rare diagnosis

guide: ms hannah thomas

presenters: rabiyath hamna abdul & nihala e

wernicke's encephalopathy

a rare diagnosis

index

thanks

conclusion & take home message

audiological management

wE and hearing loss

medical management

causes & symptoms

Incidence & prevelance

wernicke's encephalopathy

thiamine

Thiamine deficiency contributes to a number of conditions; from mild neurological & psychiatric symptoms (confusion, reduced memory and sleep disturbances) to severe encephalopathy, ataxia, congestive heart failure, muscle atrophy & even death.

Thiamine deficiency contributes to a number of conditions; from mild neurological & psychiatric symptoms (confusion, reduced memory and sleep disturbances) to severe encephalopathy, ataxia, congestive heart failure, muscle atrophy & even death.

FACTS

FUNCTION

SOURCES

Thiamine deficiency contributes to a number of conditions; from mild neurological & psychiatric symptoms (confusion, reduced memory and sleep disturbances) to severe encephalopathy, ataxia, congestive heart failure, muscle atrophy & even death.

thiamine

Picture Please!

AARON B. PAUL, 2019

Question time!

first identified by the german neurologist carl wernicke in 1881, 'WE' is an acute neurological condition caused by thiamine deficiency, which primarily affects the peripheral and central nervous systems. brain regions affected by vit b1 deficiency include;

  • cerebellum
  • mamillary bodies
  • thalamus
  • hypothalamus
  • brainstem

wernicke's encephalopathy (we)

"encephalopathy" means damage or disease that affects the brain. it happens when there's been a change in the way your brain works, or a change in your body that affects your brain.

wHAt is encephalopathy?

VASAN S & KUMAR A., 2022

Incidence & prevalence

- prevalence data on 'we' comes mainly from autopsy studies with rates ranging between 1% and 3%- the estimated mortality rate is 17%- incidence of 'we' is beleived to be higher in developing countries due to vit defeciencies & malnutrition- female to male ratio; 1:1.7- there are no studies that show a particular race predisposed to 'we'

the classic triad of wernicke's encephalopathy include:

  • altered mental status
  • opthalmoplegia
  • ataxic gait

Allan D. Thomson, Christopher C.H. et al, 2002

  • Chronic alcohol abuse and malnutrition
  • Gastrointestinal surgical procedures
  • Staple diet of polished rice
  • Forced/self imposed inadequate diet
  • Patients with protracted vomiting
  • Carbohydrate loading i.v/ oral when thiamine stores are minimal
  • Cancer and chemotherpeutic drugs
  • Systemic diseases & drug misuse
  • Genetic abnormality of transketolase enzyme

CAUSES

HyperthermiaIncreased muscle tone & spastic paresisChoreic dyskinesiasComa

StuporHypotension & TachycardiaHypothermiaBilateral visual disturbancesEpileptic seizuresHEARING LOSSPapilloedemaHallucinations and behavioural disturbances

Ocular abnormalitiesMental status changesIncoordination of gait and trunk ataxia

GIANPIETRO SECHI & ALESSANDRO SERRA, 2007

LATE STAGE SYMPTOMS

UNCOMMON SIGNS

COMMON SIGNS

CLINICAL FEATURES

MICHAEL W, DONNINO et al, 2007

Teor Labore

Tempor Labore

  • Possibility of incorrect diagnosis is high in alcohol dependent patients, because common signs of WE are difficult to differentiate from drunkenness
  • Any two of the following 4 conditions should be sufficient for a presumptive diagnosis; nutritional defeciency, ocular findings, ataxia & mental status changes
  • This presumptive diagnosis of WE can be confirmed by determining blood thiamine concentrations
  • MRI is considered as the most valuable method to confirm a diagnosis

Overdiagnosis than underdiagnosis is preferred because consequences of misdiagnosis are grave

diagnosis

GIANPIETRO SECHI & ALESSANDRO SERRA, 2007

Vit B1 is the most important and urgently required of the B vitamins

Question time!

As deficiency develops, enzymes and systems dependent upon thiamine begin to function less, leading eventually to cell death.

  • Without thiamine, glucose is metabolized through less efficient anaerobic pathways, which produces lactic acid.
  • The maintainence of of myelin sheaths in NS, lipid and branches chain amino acid production is affected.
  • Affects the efficacy of citric acid cycle and result in cellular energy deficit.
  • If left untreated, can lead to permanent brain damage followed by KORSAKOFF SYNDROME, a chronic memory disorder.

'We' is a medical emergency!

Question time!

Without treatment, these can be disabling and life threatening

  • is a memory disorder that results from Vit B1 deficiency &
is ass. with alcoholism
  • Symptoms: Amnesia, tremor, coma, disorientation & vision problems
  • damages nerve cells and supporting cells in brain and spinal cord, as well as part of brain involved with memory
  • is a degenerative brain disorder caused by lack of
Vit B1
  • Symptoms: Mental confusion, vision problems, low BP
hypothermia, ataxia
  • Causes acute damage to brain's thalamus and hypothalamus

WERNICKE'S ENCEPHALOPATHY

KORSAKOFF SYNDROME

vs

The Diagnosis and Management of WE is complex and requires a Team Approach. Other specialists may be required according to organ involvement. The role of a nurse, dietitian and social worker is also important. Audiologist comes into picture when auditory signs manifests as symptoms in the patient

The Diagnosis and Management of WE is complex and requires a Team Approach. Other specialists may be required according to organ involvement. The role of a nurse, dietitian and social worker is also important. Audiologist comes into picture when auditory signs manifests as symptoms in the patient

psychiatrist

NEUROLOGIST

EMERGENCY MEDICINe/physician

team MEMBERS

Kumaran Arivoli et al, 2023

WE and Hearing Loss

*An uncommon symptom, with a review from Ton et al. highlighting only 10 known cases as of 2021*Some of the nutrients suggested to play a role in human hearing are vits A and B *A lack of vit B has been reported to increase the risk of HL*It has been reported that risk of HL may be increased in adults with higher glycemic index and load, as well as higher total carbohydrate intake

WE and Hearing Loss

...detailed case history is the key,

  • drinking habits if present
  • recent medical history
  • recent surgeries if any
  • if patient is on any diet
  • nutritional deficiency
  • consciousness, eye problems etc.

if doubts arise...

  • reduced hearing (both ears)
  • tinnitus
  • SPEECH DiSCRIMINATION MAY BE POOR
  • vertigo
  • headache and nausea
  • nystagmus

audiological complaints

Question time!

BSERA

OAE

SPEECH AUDIOMETRY

PTA

OTOSCOPY

DON'T SEEK, DON'T FIND

Test battery

dBnHL

dBnHL

a 54 year old male came to the department ON 21/11/2022 WITH THE COMPLAINT OF REDUCED HEARING IN LEFT EAR SINCE 1 MONTH. the patient is under medication for diabetes and hypertension.pta results:right ear: minimal sloping hearing lossleft ear: severe mixed hearing lossbsera result (sol TESTING):stimulus used: clicks (90.1/s & 11.1/s polarity)right ear: aep's are not elicited even at 90 LEFT EAR: AEP'S ARE NOT ELICITED EVEN AT 90(MORPHOLOGY OF WAVE V- POOR: WAVE CONSISTENCY AND REPRODUCIBILITY- POOR)IMPRESSION: B/L (?) INDICATION OF RCP

PTA & BSERA REPORTS

case presentation

NEUROLOGICAL FINDINGS (23/11/2022)

COMPLAINTS: H/O VERTIGO ASSOCIATED WITH LEFT SIDED HEADACHE, DIFFICULTY TO TALK.co-morbidities: gr 1 fatty liver. THE PATIENT IS A CHRONIC ALCOHOLICMRI BRAIN (23/11/2022):CHRONIC INFARCT IN RIGHT MEDULLARY PART, small flair hyperintense focus in left posterolateral aspect of medulla- chronic lacunar infarct, no cp angle lesions, few small nonspecific subcortical hyperintensities in both cerebral hemispheresPTA DONE ON 19.01.2023:RIGHT EAR: MINIMAL HEARING LOSSLEFT EAR: SEVERE MIXED HEARING LOSS

PTA 2

NEUROLOGICAL FINDINGS (23/11/2022)

Other cases from literature...

Question time!

GERALD F. O' MALLEY & RIKA O' MALLEY, 2022

Tempor Labore

Tempor Labore

  1. Immediate adminstration of thiamin 100 mg IV or IM, continued daily for atleast 3 to 5 days
  2. Magnesium is a necessary cofactor in thiamin-dependent metabolism
  3. Supportive treatment include;
- Rehydration - Correction of electrolyte abnormalities - General nutritional therapy - Alcohol cessation is mandatory

Thiamine should be initiated immediately, either intravenously or intramuscularly to ensure adequate absorption

MEDICAL MANAGEMENT

  • MOST OF THE CASES IN LITERATURE IN WHICH MEDICAL LINE OF TREATMENT HAD BEGUN DURING THE WE STAGE, ALL THE SYMPTOMS HAVE ALLEVIATED WITHIN 2-3 WEEKS, INCLUDING HEARING LOSS.
  • HEARING LOSS AND OTHER AUDIOLOGICAL SYMPTOMS IMPROVED AFTER THIAMINE REPLETION
  • IF PRIMARY LINE OF TREATMENT FAILS TO ALLEVIATE AUDITORY SYMPTOMS OR IS INCONSISTENT, WE CAN SUGGEST THE PATIENT FOR AMPLIFICATION DEVICES SUCH AS HEARING AIDS AND COCHLEAR IMPLANTATION (IF CONDITIONS SATISFY)
  • IF NO BETTERMENT WITH ABOVE OPTIONS, WE CAN TURN TO COMPENSATORY STRATEGIES OR TOTAL COMMUNICATION

Question time!

  • MOST OF THE CASES IN LITERATURE IN WHICH MEDICAL LINE OF TREATMENT HAD BEGUN DURING THE WE STAGE, ALL THE SYMPTOMS HAVE ALLEVIATED WITHIN 2-3 WEEKS, INCLUDING HEARING LOSS.
  • HEARING LOSS AND OTHER AUDIOLOGICAL SYMPTOMS IMPROVED AFTER THIAMINE REPLETION
  • IF PRIMARY LINE OF TREATMENT FAILS TO ALLEVIATE AUDITORY SYMPTOMS OR IS INCONSISTENT, WE CAN SUGGEST THE PATIENT FOR AMPLIFICATION DEVICES SUCH AS HEARING AIDS AND COCHLEAR IMPLANTATION (IF CONDITIONS SATISFY)
  • IF NO BETTERMENT WITH ABOVE OPTIONS, WE CAN TURN TO COMPENSATORY STRATEGIES OR TOTAL COMMUNICATION

audiological management

WE SHOULD BE ABLE TO FULFIL OUR ROLES IN MANAGEMENT LIKE OTHER FACETS OF OUR PROFESSION

Provide the patient with proper counselling and information about various amplification devices to facilitate their hearingKeep tabs on the patient and call them for regular follow up/ regular communicationProvide them with personalised strategies/tips which can ease their difficulty

  • OUR FURTHER INVOLVEMENT WOULD COME INTO PICTURE WHEN THE PRIMARY LINE OF TREATMENT FAILS TO ALLEVIATE HEARING. OUR MAJOR GOAL IS TO IMPROVE THE QUALITY OF LIFE OF THE INDIVIDUAL.
  • WE SHOULD PROVIDE THE PATIENT WITH PROPER COUNSELLING, AND IF A CANDIDATE FOR AMPLIFICATION DEVICES, SHOULD RECOMMEND THEM SO
  • IF OTHERWISE, COMPENSATORY STRATEGIES SHOULD BE TOLD AND REGULAR FOLLOW UPS IS A MUST

CONCLUSION & TAKE HOME MESSAGE

Consectetur siadipiscig eli

Consectetur siadipiscing elit

the session is now open for discussion

OUR REFERENCES

Minieniam

Lorem Ipsum

  • Morgan, Z. J., Cler, L., & Hunter, L. (2020). I can’t hear you, you said I had what?: A case report and literature review. Journal of Community Hospital Internal Medicine Perspectives
  • Flabeau, O., Foubert-Samier, A., Meissner, W., & Tison, F. (2008). Hearing and seeing: Unusual early signs of Wernicke encephalopathy. Neurology.
  • Andrew Benvenuto, BS; Emily Stucken, MD; Kumaran Arivoli, BS; A Case of Sensorineural Hearing Loss in Wernicke Encephalopathy (Pratt et al., 1981b)
  • Henrietta Afari, BS, Melissa A. Walker, MD, PhD, Rodrigo Zepeda, MD, Adam B. Cohen, MD, Hearing loss in Wernicke encephalopathy (Pratt et al., 1981)
  • GianPietro Sechi and Alessandro Serra, "Wernicke's Encephalopathy: New Clinical Settings and Recent Advances in Diagnosis and Management" (Pratt et al., 1981)
  • Day, G. S., & Del Campo, C. (2014). Wernicke encephalopathy: a medical emergency. Canadian Medical Association Journal, 186(8), E295
  • O’Malley, G. F., & O’Malley, R. (2023b, March 15). Wernicke Encephalopathy. MSD Manual Professional Edition.

OUR REFERENCES

  • Thiamin: MedlinePlus Medical Encyclopedia.
  • Wernicke encephalopathy hearing loss and palinacousis Janette T. T. Nguyen1, Catherine Franconi1, Andrew Prentice2, and Victor Wycoco3, Royal Perth Hospital, Perth, Western Australia
  • Wernicke's encephalopathy Myths and Misconceptions: What Every ER Physician Should Know Michael W. Donnino, MD Jose Vega, MD Joseph Miller, MD Mark Walsh, MD (O’Malley & O’Malley, 2023)
  • A case report of bilateral hearing loss due to non-alcohol Wernicke encephalopathy was written by Luca Prosperini1, Alessandro Stasolla1, Gabriella Grieco2, Carmela Gerace1, and Carla Tortorella1 (O’Malley & O’Malley, 2023).
  • Don't look for it; it won't be there: Wernicke's encephalopathy's diagnostic conundrum Sara Kohnke1 and Claire L Meek1,2,3 (O’Malley & O’Malley, 2023)
  • Matt Lallas and Jay Desai's study on Wernicke encephalopathy in children and adolescents (O’Malley & O’Malley, 2023)
  • Sleeve gastrectomy is complicated by hearing loss brought on by Wernicke's encephalopathy, according to Elaf Abdulnabi Mohammed, Sulaiman Ali Hajji, Khaled Aljenaee, and Mohammad Ibrahim Ghanbar (O’Malley & O’Malley, 2023).

thanks