1. A rare neuorological complication of hyperemesis gravidarum: a unique case Shahd Hamid, Sara Misbah, A E M Ahmed and Osheik Seidi Presented by: Shahd Hassan M.Hamid MB BS 2009 Department of Neurosciences Soba University Hospital, SUDAN

2. Story 31 years old pregnant Sudanese lady Previously well Para III GII uneventful previous pregnancies

3. In 9th week of pregnancy :Severe vomiting Continued for 6 weeks, admitted to hospital

4. Had been admitted to hospital for her complicated pregnancy – Fluid depletion was replaced by dextrose 5% mainly – Received antiemetics and vitamin B6 She received parenteral vit B complex 100mg once

5. She started to feel perioral tingling and peripheral limbs numbness Her condition deteriorated and she lost her baby (15 th week GA) Dilatation & curettage was done and she was discharged

6. Continued to deteriorate neurologically and presented: Dizziness Unsteadiness Limb weakness For 15 days

7. Examination findings Hypotonia & hyporeflexia in all limbs Irritable confused Power G2 in upper limbs G3 in lower limbs Sensation ?? Gait ?? Nystagmus Lateral rectus palsy Dysarthria Both plantars were extensor

8. Laboratory investigations investigationresult HB9.5 g/dl MCV99.1 fl MCH27.6 pg TWBCs4200/ ul

9. investigationresult Serum Glucose99 mg/dl CSF Glucose58 g/dl CSF Protein30 mg/dl CSF Cells ( RBCS,WBC)0

10. investigationresult Total protein8.2 g/dl Serum Albumin3.8 g/dl Bilirubin0.6 mg/dl ALP66 mg/dl ALT33 I.U/L AST16 I.U/L

11. INVESTIGATIONRESULT Serum Urea25 mg/dl Serum Creatinine0.8 mg/dl Serum Na136 mmol/L Serum K3.6 mmol/L Serum Ca8.4 mg/dl Phosphate2.5 mg/dl

12. B12 level was normal. Transketolase activity ??

16. Report Bilateral symmetrical abnormal signal intensity in both thalami and mammillary bodies at T2-WIs and FLAIR images

17. WERNICK`S ENCEPHALOPATHY

18. Management She received iv Vit B complex (B1+B2+B12) 200 mg B.D for 3 days and continued on same dose orally and she experienced improvement since the 3 rd day of treatment in form of mental function, power and dizziness

19. Follow up She was continued on oral vitamin supplements and physiotherapy with noticed improvement from one visit to another being two weeks apart The numbness and distal weakness took three weeks to improve Mild ataxia

20. Neurophysiological tests revealed elements of sensory axonopathy with motor axonal degeneration with demyelination

21. Diagnosis Wernicks encephalopathy with parapontine myelinolysis & associated peripheral neuropathy complicating hyperemesis gravidarum

22. Discussion

23. Thiamine deficiency is characteristically associated with chronic alcoholism, because it affects thiamine uptake and utilization. However, Wernicke encephalopathy may develop in nonalcoholic conditions, such as prolonged starvation, bariatric surgery, and (HIV)/ (AIDS), rarely hyperemesis gravidarum

24. Carle Wernicke Wernicke`s encephalopathy

25. disscussion

26. Case reports review 49Total number 26.7 +/- 4.6 yearsMean age 7.7+/-2.8 weeksMean duration of vomiting 14.3+/-3.4 weeksMean gestational age 46.9%Classic triad 63.3%confusion 95.9%Ocular signs 81.6%Ataxia 6 casesPontine myelinolysis 18%Peripheral neuropathy 47.9%Overall pregnancy loss 28.4%Complete remission Chiossi, Giuseppe MD*; Neri, Isabella MD†; Cavazzuti, Milena MD‡; Basso, Gianpaolo MD§; Facchinetti, Fabio MD (April 2006) Obstetrical & Gynecological Survey: April 2006 - Volume 61 - Issue 4 - pp 255-268 doi: 10.1097/01.ogx.0000206336.08794.65

27. What happens? In the setting of thiamine deficiency, thiamine-dependent cellular systems begin to fail, resulting eventually in cell death. Because thiamine-dependent enzymes play an essential role in cerebral energy utilization, thiamine deficiency may propagate brain tissue injury by inhibiting metabolism in brain regions with higher metabolic demands and high thiamine turnover

28. Neuropathology necrosis of both nerve cells and myelinated structures. Macroscopic pathologic reports describe discoloration, shrinkage, congestion, and hemorrhage, intra- and extracellular fluid accumulation later, prominence of small blood vessels resulting from dilation of arterioles,capillaries, and venules associated with endothelial swelling and thickening of the adventitia. Hemorrhage is sometimes present and reactive astrocytes are seen on the third to fourth day, Lesions involving the mamillary bodies, the hypothalamic nuclei, the periaqueductal gray matter, the superior cerebellar vermis, and other diencephalic structures.

29. It is preventable It could be iatrogenic

30. The administration of dextrose(prolonged glucose or carbohydrate loading) in the setting of thiamine deficiency can be harmful because glucose oxidation is a thiamine-intensive process that may drive the insufficient circulating vitamin B-1 intracellularly, thereby precipitating neurologic injury

31. If she simply received : Thiamine She wouldn’t need : LP MRI NCS Neurorehabilititation LP MRI NCS Neurorehabilititation

32. Home message Thiamine supplementation is urgently required in anybody suffering prolonged vomiting; otherwise carbohydrate administration (particularly dextrose) may precipitate Wernicke's encephalopathy