Wernicke’s Encephalopathy

Shyala ChandYear 4

Introduction Acute neurological disorder induced by

Thiamine deficiency(Vitamin B1) More prevalent in males (1.7: 1) Average onset age is 50 years (range: 30-70 years) First described in 1881 by Dr. Carl

Wernicke

Cont. Rate has been found to be significantly

higher in specific populations, i.e., homeless people, older people (especially those living alone or in isolation), and psychiatric inpatients, where alcohol use and poor nutritional states predominate.

Prevalence at autopsy exceeds clinical detection.

Thiamine (Vitamin B1) Water soluble vitamin absorbed from the

gut. Serves as a cofactor to several enzymes

that are responsible for lipid and carbohydrate metabolism, production of amino acids and production of glucose derived neurotransmitters.

Also have a role in axonal conduction esp. of acetylcholinergic and serotoninergic neurons.

Cont. Cellular impairment and injury occur

within 2-3 weeks of decreased intake and thiamine depletion.

Acute thiamine deficiency leads to mitochondrial dysfunction resulting in oxidative toxicity in areas of brain.

Causes of Thiamine Deficiency

Chronic alcoholism Malnutrition or prolonged starvation Hyperemesis Gravidarum Bariatric surgery Gastric malignancy (inflammatory

bowel disease) Intestinal obstruction (abscess) Thyrotoxicosis

Cont. Iatrogenic (IV glucose without thiamine

supplement or chronic hemodialysis) Systemic diseases (AIDS, disseminated

TB) Thiamine deficient formula/

breastfeeding by mothers with inadequate thiamine intake

Infection( precipitating factor)- pneumonia, meningitis

Clinical Presentation Clinical Triad - ocular abnormalities

(29%) - encephalopathy (82%) - ataxia (23%) Occurs in 1/3 of cases.

Ocular Abnormalities Hallmark of WE Nystagmus, bilateral rectal

palsies and conjugate gaze palsies (involvement of oculomotor, abducens and vestibular nuclei)

Less common manifestations are pupillary abnormalities, ptosis, sctomata and anisocoria

Encephalopathy Global confusion state, disinterest,

inattentiveness or agitation. Most common presentation is mental

state changes. Stupor and coma observed in severe

cases

Ataxia Due to polyneuropathy, cerebellar

damage and vestibular paresis. Wide based stance Slow and uncertain short stepped gait. Inability to walk without support in

severe cases.

Other symptoms Peripheral neuropathy (weakness, foot

drop & decreased proprioception) GI symptoms (nausea, vomiting, lactic

acidosis) Hypotension Hypothermia Memory disturbances

Anatomical Changes Acute symmetrical lesions in thalamus,

mamillary bodies, tectal plate, periaqueductal area, floor of 4th ventricle (includes oculomotor and vestibular nuclei and cerebellar vermis)

Lesions are in form of vascular congestion, microglial proliferation and petechial hemorrhages.

Wernicke- Korsakoff Syndrome (WKS)

Chronic complication of WE Occurs in 2/3 of patients with untreated

WE Only 25% of patients fully recover. Lesions are similar to WE expect they

are not hemorrhagic. Results in cerebellar atrophy

(irreversible change).

Characteristics: Anterograde amnesia (inability to form

new memories) Retrograde amnesia ( inability to recall

past events) Confabulations

Diagnosis Detailed patient history Physical and neurological examination Laboratory evaluation CBC (rule out infections, severe

anemia) Serum thiamine levels Erythrocyte transketolase levels Serum glucose levels Toxic drug screening

Cont. Lumbar puncture (rule out CNS

infections) Imaging MRI (fluid attenuated inversion

recovery {FLAIR} images) CT ( not specific) EEG ( rule out non- convulsive status

epilepticus)

MRI scan of before and after Thiamine administration

Differential Diagnosis Hepatic encephalopathy Hypoglycemia Anorexia nervosa Alcohol related psychosis Withdrawal syndromes Delirium tremens

Treatment Considered a medical emergency Emergency care : Parenteral Thiamine

(multiple daily doses – 500mg/dose) Alcohol withdrawal In case of WKS, use of oral Thiamine to

prevent further complications. Parenteral magnesium sulfate in case of

hypomagnesaemia Balanced diet with high thiamine containing

foods.

THANK YOU..