What Happened To Steven Jackson? By: James Wafful

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

Primary Care Physician The Start To Our Case

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

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

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.

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

1 week after initial PCP visit Psychiatrist 1 week after initial PCP visit

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

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

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

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

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

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

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

CT Scan Results

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

CSF Results

CSF Results Results came back positive for Bacterial Meninigoencephalitis

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

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

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

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

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)

Normal Brain vs PAM Brain

Normal Tissue vs PAM Tissue (Brain)

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.

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

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

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

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

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.

Sources: http://emedicine.medscape.com/article/996227-overview#a0104 http://www.cdc.gov/meningitis/parasitic.html http://www.ajnr.org/content/27/6/1217/F5.expansion.html http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5240a4.htm http://www.globalrph.com/cerebrospinal_fluid.htm http://www.globalrph.com/antibiotic/meningitis.htm http://www.cjem-online.ca/v8/n5/p365 http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000339/ http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001006/ http://www.bing.com/health/article/mayo-MADS00196/Schizophrenia?q=schizophrenia&qpvt=schizophrenia http://www.localhealth.com/article/bacterial-meningitis/treatments http://www.cdc.gov/parasites/naegleria/biology.html http://www.sciencedirect.com/science/article/pii/S0344033808001349 http://alzheimers.about.com/library/blbrain.htm http://www.cdc.gov/parasites/naegleria/ http://www.google.com/imgres?q=normal+brain+tissue&um=1&hl=en&safe=off&sa=X&biw=1600&bih=799&tbm=isch&tbnid=Bai0dhZY9TxTuM:&imgrefurl=http://path.upmc.edu/divisions/neuropath/bpath/cases/case4/micro.html&docid=Js17tNhkuZ49gM&imgurl=http://path.upmc.edu/divisions/neuropath/bpath/cases/case4/images/micro2.jpg&w=632&h=473&ei=0-HLT-566rbpAczvuCk&zoom=1&iact=hc&vpx=532&vpy=182&dur=1034&hovh=194&hovw=260&tx=196&ty=103&sig=113467880028204058129&page=1&tbnh=128&tbnw=171&start=0&ndsp=32&ved=1t:429,r:2,s:0,i:89 http://www.thedailybeast.com/articles/2011/12/23/brain-eating-amoeba-cases-puzzle-and-worry-scientists.html http://www.pbs.org/newshour/rundown/2011/08/brain-eating-amoeba-strike---but-should-you-be-scared.html