1. What Happened To Steven Jackson?
By: James Wafful

2. Steven Jackson
Age: 18 Height: 6’ 2” Weight: 164 Born: Caribou, Maine

3. Primary Care Physician
The Start To Our Case

4. Initial Visit: May 31, 2012 Patient is a 18-year old male
Healthy weight and height
States that he has been getting severe migraines for two days now and also states he has been experiencing seizures
Says he has been feeling very lethargic and forgets things easily
Ran the Complete Blood Count(CBC) test and results came back normal
A partial mental status exam was performed

5. Diagnosis Patient is diagnosed with Dementia and Epilepsy
Increasingly serious forgetfulness has been noted for about two days. Seizures have been noted as becoming more frequent and more debilitating the past two days. More symptoms include:
Severe migraines
Hallucinations
Loss of Balance
Lack of attention

6. Treatment Patient is prescribed Donepezil 10 mg once daily
Donepezil is used to treat Dementia. It’s in a class of drugs called cholinesterase inhibitors. These improve mental function by increasing the amount of a certain naturally occurring substance in the brain.
Felbamate 300 mg four times daily
Felbamate is used to treat seizures in epilepsy patients when other treatments don’t work. It’s in a class of drugs called anticonvulsants. It works by decreasing abnormal activity in the brain.
Patient is advised these medications may take up to 4 weeks for full benefits.
Patient is told to call if any severe side-effects are experienced.
Follow up appointment scheduled in a week.

7. Follow Up Visit: June 7
CBC test ran again and levels were lower than before, but still normal.
Seizures seem to have subsided but not fully treated.
Patient seems to still forget things and lose attention.
Dosage of Donepezil increased to 20mg once daily
Referred to psychiatrist for further analysis

8. 1 week after initial PCP visit
Psychiatrist
1 week after initial PCP visit

9. Psychiatric Referral Patient referred by primary care physician
No improvement, and actual decrease in memory, attention, and hallucinations
Decrease but no permanent cure for seizures
On medication for about a week and a half
Patient seems to be getting worse in mental condition

10. Psychiatric Review Meet with patient for 3- 1 hour sessions Observed
Seems to get confused easily
Cant keep focus for longer than five minutes
Experienced several bizarre hallucinations
No sign of any seizures

11. Psychiatric Diagnosis/Treatment
Schizophrenia
Treatment
Prescribed Abilify
Provided patient with mental health journal and asked to keep strict notes
Continue therapy two or three times a week

12. Continued Therapy and Treatment
One week after first visit
Missed one appointment
Said he forgot he had an appointment
Could barely keep focused on talking with doctor
Medical journal reports some missed medications, hallucinations, and increased seizing
Could barely remember own name

13. Treatment Continued
Treatment for Schizophrenia was deemed unhelpful and thus stopped
Patient’s mental health has gotten worse
Referred to Neurologist for further testing and diagnosis

14. 1 and half weeks after initial PCP visit
Neurologist
1 and half weeks after initial PCP visit

15. Neurologist Review Referred by Psychiatrist Appointment 6/11/12
Signs of Dementia
Memory loss
Seizures
Hallucinations
Lack of attention
Loss of balance
Referred for Contrast CT Brain Scan and Spinal Tap

16. CT Scan Results

17. CT Scan Results No brain tumors found
Figure A shows right basal ganglia infarction
Figure B shows enhanced exudates in the perimesencephalic cistern

18. CSF Results

19. CSF Results
Results came back positive for Bacterial Meninigoencephalitis

20. Treatment Patient given antibiotic Vancocin 500mg
Patient sent home and told to return in a week or call if any complications arise

21. Treatment Contd.
Patient’s mother called about four days later crying hysterically and telling us that her son’s condition is getting worse by the minute
Patient brought in immediately in a coma-like state
Patient died two days after 6/18/12

22. 18 days after initial PCP visit
Medical Examiner
18 days after initial PCP visit

23. Autopsy Report
An autopsy referral was received from Johns Hopkins Department of Neurology
The patient was presented with symptoms close to that of Meningoencephalitis
Immunofluorescence testing with N. Fowleri-specific antibody was ordered
Another lumbar puncture was ordered to make sure nothing was missed
Brain was also ordered to be thoroughly examined

24. Autopsy Results Brain has suffered massive degeneration
A postmortem lumbar puncture demonstrated a few motile amebae
Autopsy findings revealed acute Primary Amoebic Meningoencephalitis caused by Naegleria fowleri identified by immunofluorescence testing with an N. fowleri-specific antibody (see Stain)

27. Normal Brain vs PAM Brain

28. Normal Tissue vs PAM Tissue (Brain)

29. Why Did This Happen?
Steven Jackson, about two weeks ago snuck out of the house and went swimming at the local lake. The lake consisted of freshwater. On a normal day, the freshwater would do nothing. However, the state of Maine recently experienced a heat wave of temperatures reaching the high nineties. Thus, the water was warmed to a greater temperature than normal days. Steven went underwater and got some water to go up his nose, something deemed harmless. What he didn’t know was that the amoeba Naegleria fowleri went in with that water and went up his nasal cavity to the olfactory bulb and quickly destroyed his brain.

30. What is Primary Amoebic Meningoencephalitis
a very rare form of parasitic meningitis that causes a fatal brain infection. The parasite enters the body through the nose and is caused by the microscopic ameba (a single-celled living organism) Naegleria fowleri.
The disease progresses rapidly and usually causes death within 1 to 12 days
Commonly misdiagnosed as bacterial or viral meningitis

31. Symptoms
Initial symptoms start 1 to 7 days after infection and include:
Headaches
Fever
Nausea
Vomiting
Stiff neck
Later Symptoms:
Confusion
Lack of attention
Loss of balance
Seizures
Hallucinations

32. What is Being Done?
Some drugs are available in laboratories however these are of no use if the patient cannot be diagnosed at early stages
The effectiveness of these drugs are even unclear since almost every case of PAM is fatal
Drugs include: Amphotericin B, Rifampin, Doxycycline, Sulfisoxazole, Azithromycin, and many more
There have been 32 reported infections in the U.S. in the 10 years from 2001 to 2010, despite millions of recreational water exposures each year. By comparison, in the ten years from 1996 to 2005, there were over 36,000 drowning deaths in the U.S.
There isn’t much that can be done since the are millions of fresh bodies of water in the world that can contain these amoebas. All that you can do is hope that the amoeba doesn’t get forced up your nose

33. How Can One Get This?
You can’t get this from swallowing the water or having an open wound come in contact with the water. The amoeba doesn’t even like people. It only attacks people when it is forced up the nose. Only infectious in trophozoite phase.
It’s still unclear why some people get this and so many walk away unscathed, but scientists believe it has to do with behavior such as diving, splashing and other roughhousing.
Some other risk factors are:
Geothermal water (hot springs)
Warm water discharge from industrial plants
Soil
Swimming pools that are poorly maintained or un-chlorinated
Water heaters with temperatures less than 47°C

34. How Can This Be Prevented?
Avoid water activities in freshwater during periods of high temperature and low water levels
Hold nose close or use nose clips when participating in these water-related activities
Avoid digging in or stirring up the sediment while participating in freshwater areas.

36. Sources: http://emedicine.medscape.com/article/996227-overview#a0104