Case 13

Delusions in acute radiation syndrome.

The patient was a 26-year-old male. He was discovered unconscious at the back
of a city bus. The bus driver was unable to wake him, and notified the police, who
called for an ambulance.
On admission, the patient was unresponsive (GCS 3). There was dried vomit on
his clothing. Severe first-degree burns were noted on the patient's face, chest,
groin, and the frontal surfaces of all four extremities. Less severe first-degree
burns were noted on his back and buttocks. The borders of these burns were
extremely diffuse.
Erythema and inflammation were noted in both sclera, the
nasal mucosa, the tongue, and the oral mucosa.
A complete blood panel showed
mild leukopenia (3,200 cells per microliter).
A CT scan showed diffuse edema in the abdominal tissues, especially those
surrounding the GI tract. There was edema in both optic nerves, both frontal
lobes, and the frontal portions of the basal ganglia,
but this did not appear severe
enough to be the cause of his coma.
Shortly after the scan, the patient suffered an attack of profuse watery diarrhea
which soon became bloody. He showed signs of increasing awareness, and
regained consciousness approximately two hours after admission, at which point
he began vomiting heavily, requiring fluid resuscitation. Four hours after
admission, a repeat blood panel showed a profound leukopenia of 210 cells per
microliter. He was transferred to an aseptic clean room in the ICU. In view of the
burns, edema, and leukopenia, acute radiation exposure was suspected. Serum
levels of Sodium-24, a good proxy for absorbed neutron dose, were only slightly
elevated. However, chromosomal analysis of peripheral blood lymphocytes
(using
the method described by Hyata et al. (2001), following a criticality accident at a
uranium-processing facility) suggested an absorbed dose of at least 10 Gray.
The patient's mental status improved rapidly over the course of Day 1. By the
morning of Day 2, he was well enough to be interviewed. His speech was halting,
confused, and tangential.
There was evidence of perseveration, anomia, and
severely impaired attention.
He demonstrated severe retrograde amnesia, and
could give no account of how he had been exposed to radiation. There was

evidence of delusional thinking. A partial transcript of his interview on Day 2

follows:
[Doctor 1 (the author, henceforth D1)] Okay. I've got it running now. This is
patient [name removed for privacy]. Today is [date removed]. So, can you
describe what happened to you.
[Patient (henceforth P)] (1.2 s pause) They took me up to see her.
[D1] To see who?
[P] You can't imagine.
[D1] Who did they take you to see?
[P] I told you. You can't imagine it. You can't fit it in your head. (1.0 s pause) My
stomach hurts.
[D1] Do you need the bucket?
[P] No. I think I'm okay. (3 s pause)
[D1] Who did they take you to see? Can you describe her?
[P] When you were a kid, did you ever look at the sun?
[D1] Yeah, I probably did.
[P] It was like that. It was that bright, but bigger. But it was blue. All blue and
purple. The color of lightning. Like frozen lightning. And she looked like a fish
skeleton. You know what a fish skeleton looks like? (0.5 s pause) All
those...needle bones?
[D1] Sure.
[P] It was like that. Or flowers. Or vines. Or trees. But made of lightning. And
there were black crystals. Really sharp and hard. (0.5 s pause) They took me up
to see her.
[D1] Who took you up?
[P] Those people. The people I met. They didn't tell me their names. They
thought I was ready, though.
[D1] Ready for what?
[P] (2.0 s pause) To see her, I guess.
[D1] Where did they take you?

[P] They made me take this pill, and I passed out in the van. When they took me
up, there was a big metal staircase, and there was this big lead room, like the
size of a movie theater. She was in there. There was all this ash. It was really
hot. Like an oven.
[D1] Where do you work?
[P] I haven't had a job since .
[D1] Where did you work in .
[P] (5.0 s pause) At...a leatherworks. I lost my job.
[D1] Have you ever had radiation therapy?
[P] She took me apart. I'm just a bunch of threads inside. She just sat there and
picked them apart. One at a time. She pulled them out and looked at them. Like
those counting-strings Indians used. Remember that?
[D1] Remember what?
[P] The counting strings. The Indians used.
[D1] I know what counting-strings
are
.
[P] She unraveled all my threads and read them off. She knew what was in my
brain. She said I wasn't good enough. And she threw me out. Those people
wanted to kill me, but she made them let me go.
[D1] Who wanted to kill you?
[P] (agitated) The people who took me to see her!
[At this point, the interview was interrupted by an attack of vomiting.]
By the end of Day 2, the patient's leukocyte count had fallen to zero.
He was
subjected to digestive decontamination with parenteral and enteral antibiotics,
which has been shown to improve survival in critically ill patients. His nausea and
vomiting continued,
and he developed severe edema of the face, trunk, and
limbs. He developed hypotension of 70/40 mmHg, requiring further fluid
resuscitation.
The patient was managed aggressively in spite of the high mortality rates
associated with radiation doses above 8 Gy. The patient had no identification and
could not provide contact information for any family members,
but rapid PCR
found a near-perfect HLA match in the bone marrow registry. The donor was

contacted and hematopoietic stem cells transplanted. The patient was started on
aggressive therapy with erythropoietin, colony-stimulating factors, vitamins,
electrolytes, and hydration.
By Day 6, large oozing lesions were present on the patient's trunk. His diarrhea
was extremely bloody, and in spite of aggressive fluid resuscitation and
developing oliguria, his blood pressure could not be brought above 100/60. He
required constant inotropic support, whole-blood transfusions, and fluids in
excess of 8 liters per day. His mental status remained unchanged, and he
remained delusional. Multiple interviews revealed the severity of his retrograde
amnesia, as well as short-term memory impairment, deficits in executive
function, and bizarre thinking. A neuropsychiatric interview was performed on
Day 7. An excerpt from this interview follows.
[Doctor 2 (the neuropsychiatrist, henceforth D2)] Do you want to talk about what
you saw?
[Patient (henceforth P)] Why?
[D2] I'm just asking if you want to talk about it. It sounds like it was a pretty big
deal.
[P] She took me apart. Like...like...like...like...like (5.0 s pause) Like when a kid
takes apart a toy. Just for fun. Just to see what's inside. I don't know what she
wanted. She just kept me there, and I was screaming, and she wouldn't tell me
what she wanted. I was burning to death, but she wouldn't let me go. I just had
to stare at her. I could see her when my eyes were closed.
[At this point, D2 asked D1 to leave. D1 was reprimanded for interrupting the
interview.]
A bone marrow biopsy on Day 8 showed some proliferation of the transplanted
cells, but in the afternoon, the patient developed a high fever (104 F),
jaundice,
and a papular rash, all suggestive of graft-versus-host disease.
His mental status
began to deteriorate, and he became lethargic and frequently unresponsive. He
suffered from periodic bouts of screaming lasting a few seconds. He required
increasing fluid support (up to 12 L/day) and further transfusions.
On Day 9, he
developed hypoxemia and respiratory distress requiring mechanical ventilation. A
chest X-ray revealed severe pneumonitis with pulmonary edema and
consolidation. In spite of a maximal dose of tacrolimus, the patient's condition

continued to worsen. Severe graft-versus-host disease can sometimes be treated

by bone marrow ablation with ionizing radiation. Obviously, in this patient's case,
that was impossible.
On Day 9, the patient developed anuria, fulminant hepatic failure, pulmonary
edema, cardiomyopathy, and hypotensive shock. In spite of aggressive
resuscitation with 15 L of crystalloid, two units whole blood, one unit plasma, and
one unit packed red cells, the patient went into asystole on the evening of Day 9.
He could not be resuscitated and was pronounced dead.
Autopsy revealed severe edema and inflammation in all tissues, much worse on
the frontal surface of the body.
There was extensive necrosis, hemorrhage, and
infection throughout the GI tract. There were several intestinal perforations, with
severe peritonitis. There were hundreds of small bacterial abscesses in the liver.
The kidneys were grossly ischemic and necrotic. The heart was enlarged and the
myocardium thickened throughout. Histology demonstrated interstitial
myocarditis. The lungs were congested and edemataneous with atelectasis and
hemorrhage in both lower lobes and fibrosis, pneumonitis, and edema in both
upper lobes. Blood samples showed anemia, leukopenia, and thrombocytopenia. A
bone marrow biopsy showed inflammation suggestive of graft-verus-host disease.
On inspection, the brain was grossly intact, with only mild edema in the frontal
lobes and a few pinprick hemorrhages. On microscopic examination, however,
there was evidence of Wallerian degeneration in approximately 25% of axons,
neuronal loss, and reactive astrocytosis. Of note, a pattern of microscopic
(approx. 5 um) holes was observed in the hippocampus and temporal lobes.
These did not appear to be associated with amyloid plaques or inflammation, but
may have been a result of connective-tissue loss.
An attempt was made to fingerprint the patient post-mortem, but because of
extensive swelling in all fingers and radiation-induced desquamation, no usable
fingerprints could be taken. A pre-mortem photograph was sent to the police, but
because of the swelling in the face, they were unable to match him to any known
person. Public appeals for his identity went unanswered.
This case was also of concern to the Department of Homeland Security, the FBI,
and Department of Energy, as such acute radiation exposures have only been
documented following nuclear explosions, acute poisoning with radioisotopes (as

in the Litvenenko case), and criticality accidents during the processing of nuclear
fuel and nuclear weapons. It is unlawful for an unlicensed individual to own a
quantity of radioactive material sufficient to cause this patient's degree of
poisoning. However, there were no records of criticality accidents, thefts, or
exposures at any nuclear facility. Members of the staff were questioned
extensively about the patient, as there were concerns he may have been
manufacturing, storing, or transporting nuclear materials illegally, possibly with
malicious intent.
DNA testing on blood samples taken at admission identified the patient as a local
man with no criminal record who had been unemployed for several years. He had
no connections to terrorist groups, had never worked with radioisotopes, and his
apartment contained no nuclear material or paraphernalia. The investigation is
ongoing.