Wernicke’s encephalopathy is an acute neuropsychiatric condition which occurs
due to thiamine deficiency in various catabolic states and can often go overlooked.
A high index of suspicion for the condition in suggestive clinical settings is
important as rapid reversal of symptoms is possible with treatment and if left
untreated it may progress to permanent neurological disability.
Case report
A 30 year old Indian lady,primigravida at 16 weeks gestation, belonging to a low
socioeconomic class, presented to the casualty of our hospital on Oct 20th 2013,
with history of acute onset difficulty in walking and weakness of all four limbs for
six days, headache for two days and double vision and severe breathlessness for
last one day.
She did not have any decreased vision,eyelid drooping,facial numbness or
deviation, vertigo,decreased hearing,ringing in ears,change in speech, difficulty in
swallowing,unilateral limb weakness,tingling,burning sensation,numbness or any
bladder bowel disturbances.There were no associated seizures,loss of
consciousness,back or neck pain.
For the past one and a half month she had excessive nausea and repeated
vomitings and was not eating well.She was hospitalized elsewhere about 15 days
3
back for recurrent vomitings and intravenous fluids and antiemetics were
administerd.Since then vomitings were controlled.
During her routine pregnancy check up she was also found to have
hyperthyroidism.There were no other known associated medical illnesses.She had
lost about 5 kg of weight in the past 2-3 months.
There was no history of any prior or associated fever,cough,loose motions,joint
pains or swelling,skin rashes,body aches or abdominal pain or ingestion of any
canned foods ,shell fish,any drug or toxin,snake bite or dog bite any time prior to
the illness.
On examination her pulse rate was 130 per minute,blood pressure was 100/60
mm Hg.She was breathless and respiratory rate was 40 per minute and
temperature was 99oF.She was very thin ,weighed 36 kg and was pale but there
was no clinical evidence of jaundice,clubbing,cyanosis ,lymphadenopathy,edema or
goiter but had bulging eyes.
She was conscious but disoriented to place and time. She had difficulty in speaking
because of breathlessness but had no apparent nasal twang in speech.Pupils were
normal size reacting normally to light ,bilateral horizontal gaze paresis and
bilateral horizontal gaze evoked jerky nystagmus were present.Vertical eye
movements were normal.Fundus examination revealed no papilloedema.Other
cranial nerve examination was also normal.
There was generalized hypotonia,severe weakness of neck, trunck and all four
limbs(MRC grade 2/5). She was bedbound,unable to even sit up unsupported in
bed .All deep tendon reflexes were absent.Plantar responses were bilateral
flexor.Abdominal reflexes were present.Limb ataxia could not be assessed because
of marked weakness of all four limbs. Sensory examination was completely
normal.There were no signs of meningeal irritation.
Chest auscultation revealed basal crepitations bilaterally.Per abdominal and
cardiovascular examination were normal. Gynecological assessment revealed a 16
weeks gravid uterus.
Considering the acute onset of weakness of all four limbs, neck and truncal
weakness with headache, horizontal gaze paresis, nystagmus with respiratory
involvement,possibilities of Miller Fisher variant of Guillaine Barre Syndrome,
acute disseminated encephalomyelitis, brainstem lesions such as
demyelination,pontine myelinolysis or space occupying lesions,acute myasthenic
crisis or metabolic causes like electrolyte disturbances were initially
considered.Also in view of a hypermetabolic state as suggested by the history of
4
HG and hyperthyroidism,a possibility of WE was considered.CT Brain, plain and
contrast, was normal.
Supportive management was started in the casualty and injection thiamine in high
dose (500 mg in normal saline,three times a day)was immediately started after
sending blood samples for thiamine level.ABG revealed hypoxia.As the patient was
in respiratory distress she was immediately put on artificial ventilation in the
medical ICU. Electrocardiogram showed sinus tachycardia and chest
roentgenogram was normal.No evidence of cardiomegaly or pneumonia were
found.Blood reports came back as detailed in Table 1.She had anemia ,hypokalemia
and hypoalbuminemia.
Central line was passed and she was simultaneously initiated on intravenous
fluids, potassium correction, vitamin B12 1500micro grams daily,intravenous
antibiotics,folvite and iron supplementation and low molecular weight heparin for
prophylaxis for deep venous thrombosis.
Thyroid profile suggested hyperthyroidism.HIV ELISA,Hbs Ag,Anti HCV,ANA ,ACE
,Anti TPO antibodies were negative.Serum CPK was 60IU/L.Serum lactate was 10
mg/dL(Normal 9-16 mg/dL).Cerebrospinal fluid examination showed normal
sugar and protein and no cells.A 2D echocardiogram was also normal with a
normal left ventricular ejection fraction .USG abdomen showed a single live fetus
of 16 weeks gestational age.
A plain MRI brain done the next day revealed symmetrical areas of altered signal
intensity in the periaqueductal grey matter,tectal plate,both thalami,dorsal
brainstem and in the mamillary bodies ,highly suggestive of Wernickes’s
encephalopathy (Figure 1 &2) .MRI of the spine was normal.
On the basis of the brain MRI findings,the differential diagnosis was narrowed
down and pointed strongly in favour of Wernicke’s encephalopathy with
associated generalized weakness either due to neuropathy ,most probably
secondary to nutritional deficiency of thiamine and other B complex vitamins or
hypokalemia or a combination of both.
Her serum thiamine levels came low 0.025 µg/dL (0.2-2 µg/dL).She was also
started on propylthiouracil 50 mg three times a day and beta blockers,after
consultation with endocrinologist.
During the next few days she had significant daily fluctuation in heart rate from
50-140 bpm but maintained blood pressure without any ionotropic support.She
was tracheostomised at the end of ten days of mechanical ventilation.She
continued to be on ventilator for the next two months.During the course of
ventilation she developed ventilator associated pneumonia.
5
After about a week of thiamine administration ophthalmoparesis started
improving ,by the end of one and a half month she was moving her eyes completely
in all directions ,though nystagmus persisted for a longer time and took another
month to recover. Injectable thiamine was continued at a high dose of 500 mg
three times a day for one month and later on she was started on oral thiamine 100
mg daily.Power of all four limbs gradually improved in the ICU to MRC grade 4/5
in all four limbs at the end of three months of hospitalization. Physiotherapy of
limbs and chest was carried out regularly.
She continued to be in the ICU due to hospital acquired pneumonia and septicemia
and was being treated with multiple antibiotics according to the culture sensitivity
results.
After a month propylthiouracil was replaced with neomercazole 10 mg three times
a day on the advice of the endocrinologist.The family was explained about the risk
to the fetus because of exposure to multiple drugs and radiation due to repeated X
rays.The mother and the family wished to continue with the pregnancy despite all
these concerns.As the abdominal girth of the patient was increasing due to
advancing pregnancy, weaning her from ventilator was not possible.
She started feeling the fetal movements around the fifth month of gestation. At six
months of gestation,while still in the ICU, she started having lower abdominal pain
and vaginal bleeding.Gynecologist decided for a lower segment caesarean section
and a live male baby of 900 grams was delivered ,who was admitted in the
neonatal ICU.The baby was fed on expressed breast milk and formula feeds
through the nasogastric tube.Over the next 2 months the baby gained weight and
was discharged from the neonatal ICU.The baby weighed 1.5 kg at discharge.
After the delivery,the ventilatory requirements of the patient improved and
weaning was facilitated.Over the next fifteen days we could wean her from the
ventilator.She was shifted to the ward and was able to walk unassisted.Mild
recent memory impairment was observed on later assessment in the ward.
A nerve conduction study could not be done initially because of the critical
condition of the patient and the necessity to shift her to the ICU for mechanical
ventilation.After discharge from ICU, however, a nerve conduction study of all four
limbs was normal. Her repeat thyroid profile postpartum showed was normal.The
lady was discharged home on 10th of March 2014 with her baby who was
developing normally.
On 23 rd of September 2014 this lady came for her last follow up.She does not have
any neurological deficits except mild memory deficits but independently managing
her life activities and her child who is 6 months old is developing normally.
.