What Happened To Steven Jackson?By: James Wafful

Steven Jackson

Age: 18

Height: 6’ 2”

Weight: 164

Born: Caribou, Maine

Primary Care PhysicianThe 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

Psychiatrist1 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

Neurologist1 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 ResultsNormal Patient Bacterial Meningitis

Pressure (cmH20) 5 to 20 40 >30Appearance Normal Turbid TurbidProtein (g/L) 0.18-0.45 2 >1

Glucose (mmol/L) 2.5-3.5 2 <2.2Gram Stain Normal 90% Positive 60-90% Positive

Glucose- CSF:Serum Ratio 0.6 0.2 <0.4WCC < 3 550 >500Other 90% PMN 90% PMN

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

Medical Examiner18 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/mmwr/preview/mmwrhtml/mm5240a4.htm• 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