CPC COMPETITION Chicago, IL

1. CPC COMPETITIONChicago, IL October 12, 2014 Nicole Maguire, DO, FACEP Newark Beth Israel Medical Center Emergency Medical Associates

2. INFORMATIONFROM CASE PRESENTATION • 11 year old female who is brought in by her mother for acting “strange and erratically.” • Reports “bugs are crawling all over her”. • Sees and feels spiders crawling on her.

3. INFORMATIONFROM CASE PRESENTATION • Patient and mother also report the following: • Feeling “HOT” • Lower Abdominal Pain • Headache • Waxing and Waning episodes of AMS • Symptoms worse at night • Insomnia • Heightened Energy

4. INFORMATIONFROM CASE PRESENTATION • Patient reports taking Motrin and “pink pills” • Patient had a sore throat one month ago. • Has a rash on axilla that is red and swollen. • House was recently sprayed for bugs. • No sick contacts. • Not sexually active. • No change in diet.

5. Medical History • Night Terrors age 2-4 • Menarche 1 year ago • Sore throat one months ago – Strep Negative • Two surgeries for lazy eye.

6. Physical Exam • VS – BP-121/81 P-100, RR 18, Sao2-100% T-99.5 • General: Alert, no acute distress, not ill or toxic appearing • Skin: Warm, dry, cheeks flushed, red raised rash in right axilla, one larger spot approximately 2.5 cm x 2.5 cm with satellite lesions • Head: Normocephalic, atraumaticNeck: Supple, trachea midline, nontender, no JVD • Eye: Pupils are equal, round, and reactive to light, extraocular movements are intact, normal conjunctiva, vision unchanged • Ears, Nose, Mouth and Throat: Tympanic membranes clear, nasal membranes moist without lesions, oral mucosa moist, no pharyngeal erythema or exudate

7. Physical Exam • Cardiovascular: Regular rate and rhythm, no murmur, normal peripheral perfusion, no edema. • Respiratory: Lungs clear to auscultation, respirations non-labored, breath sounds equal, symmetrical chest wall expansion • Chest Wall: no tenderness, no deformity • Back: Nontender, normal range of motion, normal alignment Musculoskeletal: Normal strength, no tenderness, no swelling • Gastrointestinal: Soft, nontender, no masses palpated, normal bowel sounds, no organomegaly • Neurological: Alert and oriented to person, place, and time; no focal neurological deficit observed, cranial nerves II – XII intact, normal sensory observed, normal motor observed, normal speech observed

8. Physical Exam • Lymphatics: No lymphadenopathy noted in cervical or axillary lymph notes. • Psychiatric: Behavior is relaxed, judgment is impaired by abnormal thoughts, and patient demonstrates delusions, tangential thoughts, and flight of ideas.

9. What are our findings? • Altered 11 year old female:

10. Differential Diagnosis for AMS • A – Alcohols • E – Epilepsy, Encephalitis, Electrolytes • I – Insulin • O- Opiates, Oxygen • U – Uremia • T – Trauma, Temperature, Thyroid • I – Infection • P – Poisons, Psychogenic • S – Shock, Stroke, Space –Occupying Lesion, SAH

11. Differential Diagnosis for AMS • A – Alcohol • E – Epilepsy, Encephalitis, Electrolytes • I – Insulin • O- Opiates, Oxygen • U – Uremia • T – Trauma, Temperature • I – Infection • P – Poisons, Psychogenic • S – Shock, Stroke, Space –Occupying Lesion, SAH

12. What is the rash? • Red raised lesion in right axilla larger one at 2.5X2.5cm with satellite lesions. • This could be about 1,000 things • How does it relate to AMS (is it related at all)?

13. Differential for Rash and AMS • TTP • Lyme Disease • Syphilis • Meningitis • Steven Johnson Disease • Parasitic Infection • Strep Infection • Varicella • Yeast Infection

14. Lab Values • BMP:Sodium 137 mMol/L Potassium 3.7 mMol/L Chloride 101 mMol/L CO2 27 mMol/L Anion Gap 9 Glucose 109 mg/dL BUN 6 mg/dL Creatinine 0.44 mg/dL • CBC with Differential: WBC 6.1 thou/mcL RBC 4.2 million/mcL Hemoglobin 12.9 g/dL Hematocrit 38.2% MCV 90.9 FL MCH 30.6 Picograms MCHC 33.8 gm/dL RDW 13.0% Platelet Count 211 thou/mcL 62% Lymphocyte % 27% Monocyte % 11% Eosinophil % 0% Basophil % 0% • Liver Enzymes and Lipase:Alkaline Phosphatase 219 IU/L ALT 33 IU/L AST 20 IU/L Bilirubin Total 0.4 mg/dL Bilirubin Direct 0.1 mg/dL Albumin 4.1 gm/dL Protein 7.2 gm/dL Lipase 106 IU/L

15. Lab Values • Urine: HCG Qual Negative Color Yellow Appearance Clear Specific Gravity 1.105 pH 6.0 Glucose Negative mg/dL Ketones >80 mg/dLBilirubin Negative Urobilinogen 0.2 unit/dL Leukocyte Negative Nitrite Negative Protein Negative • Urine Drug of Abuse Screen: Negative • Ethanol: Negative • Salicylate: 1.0 mg/dL • Acetaminophen: 0.7 mcg/mL

16. Lab Results • Mildly Elevated Alk Phos • Mildly Elevated Lipase • Significant Amount of Ketones • Negative Drug Screen

17. Differential Diagnosis for AMS • A – Alcohol • E – Epilepsy, Encephalitis, Electrolytes • I – Insulin • O- Opiates, Oxygen • U – Uremia • T – Trauma, Temperature • I – Infection • P – Poisons, Psychogenic • S – Shock, Stroke, Space –Occupying Lesion, SAH

18. Differential Diagnosis for AMS • Epilepsy • Encephalitis • Poisons • Psychogenic • Stroke • Space –Occupying Lesion • SAH

19. MRI without Contrast

20. MRI with Contrast

21. Differential Diagnosis for AMS • Epilepsy • Encephalitis • Poisons • Psychogenic • Stroke • Space –Occupying Lesion • SAH

22. So Where Are We? • We have an 11 year old female with waxing and waning AMS, abdominal pain, headache, dizziness, history of sore throat, rash, slightly dehydrated with a mildly elevated alk phos and lipase, negative tox screen, negative etoh, no laboratory or clinical signs of infection, and a normal MRI.

23. Differential Diagnosis • Encephalitis • Epilepsy • Poisons • Psychogenic

24. Epilepsy • Patient has no evidence that she is experiencing tonic clonic seizure. • Does not appear to be having focal seizures. • Could she be having absent seizures?

25. Absent Seizures

26. Encephalitis

27. LP findings in Encephalitis PCR can be done to detect viral causes.

28. Poisons • What was that pink pill she was referring to? • Anti-cholinergic • Hot as a hare (hyperthermia) • Dry as a bone (dry skin) • Red as a beet (flushed) • Blind as a bat (mydriasis) • Mad as a hatter (delirium)

29. Poisons • Sympathomimetic • T – tachycardia • H – hyperthermia • I – increased motor activity • R – restlessness • D – dilated pupils • S - sweating

30. Poisons • Was it the Pesticide? – Usually carbamates or organic phosphates. • D – diarrhea, diaphoresis • U – urination • M – miosis • B – bronchorrhea, bradycardia, bronchospasm • E – emesis • L – lacrimation • S - salivation

31. Psychogenic • Diagnosis of elimination. • Other physical complaints occurring.

32. Differential Diagnosis • Encephalitis • Epilepsy • Poisons • Psychogenic

33. Autonomic Instability • Dizziness or fainting Yes • Inability to alter HR Not Sure • Sweating Yes • Digestions Difficulties Not reported • Urinary Problems Not reported • Blurry Vision Yes • Headache Yes

34. Abdominal Pain • Suggested that the abdominal pain be worked up with US as below

35. Differential Diagnosis • Encephalitis + Ovarian Mass = Anti-NMDA Encephalitis • Epilepsy • Poisons • Psychogenic

36. Anti-NMDA Encephalitis • New diagnosis first case report 2005. • Typically young woman • Early findings include behavioral changes, memory loss, hallucinations, irritability, sleep disturbances and seizures • Often mis-diagnosed as a psychiatric illness or drug abuse. • Frequently associated with an ovarian tumor. • LP will be positive for anti-NMDAR subunit antibodies in the CSF

37. Clinical Findings of the disease • Lancet Neurol. 2008 Dec;7(12):1091-8. doi: 10.1016/S1474-4422(08)70224-2. Epub 2008 Oct 11. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. • 100 patients, 91 were woman • All presented with memory loss or psychiatric symptoms • 77 had seizures • 88 had decreased level of consciousness • 86 dyskinesias • 69 Autonomic Instability • 58 had tumors mostly ovarian teratomas • Interpertation - A well-defined set of clinical characteristics are associated with anti-NMDA-receptor encephalitis. The pathogenesis of the disorder seems to be mediated by antibodies

38. Can an Ovarian Tumor Cause these Symptoms?

39. Information that would be useful: • What was the pesticide used and onset of symptoms in relation to it. • Rectal Temperature • Skin exam – states her cheeks were flushed and she was dry later reports diaphoresis and axillary exam reporting if she was sweating would have been helpful • Picture of the Rash • More images of US and MRI • Specific tests that were performed on CSF

40. FINAL DIAGNOSIS Anti-NMDA Encephalitis Secondary to ovarian tumor likely teratoma