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Differential diagnosis between HPPD and persistent aura without infarction

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Author: Klaus Podoll, Markus Dahlem, Sofia Greene	21. February 2007
Edited by: Klaus Podoll, Markus Dahlem, Sofia Greene

Differential diagnosis between HPPD and persistent aura without infarction

Hildegard of Bingen, The cosmos in the form of an egg, from Liber Scivias, 12th c.

Johanna A. Hoffman's (1984) case report

In her Letter to the Editor dated June 1984, Hoffman (1984) commented on Abraham's (1984) paper on Visual phenomenology of the LSD flashback which called to mind "the case of a woman I treated whose major difference from the patients in Abraham's group was the absence of a history of LSD ingestion" (p. 623). "This patient had experienced the onset of visual symptoms at the age of 17 years during a near-psychotic upheaval, without any known exposure to hallucinogens. Of the 16 visual symptoms described by Abraham, my patient reported 14... These visual distortions had persisted during the 20 years since their onset, although with decreased frequency" (p. 632). Considering various hypotheses as to the cause of the patient's persistent perception disorder, Hoffman suggested that "The patient's visual distortions might also represent a migraine prodrome as she occasionally suffered migraine headaches with preheadache fortifications and, on one occasion, homonymous hemianopia followed by unilateral headache and nausea. Color flashes occurred during stressful situations..." (p. 632). Discussing the differential diagnosis of this case, neither Hoffman nor Abraham, in his reply, referred to the concept of prolonged migraine aura status that had been described 2 years earlier by Haas (1982).

In a thread entitled "Visual snow and static/HPPD – are they the same thing?" at the Ezboard forum for those with Visual snow or static, the user James posted the following comment and question:

visual snow & static/HPPD

"It would seem that if you have unexplained visual disturbances with hallucinogenic drug use, it's called HPPD.

If no drug use, then it's migraine related (?).

I have had migraines in the past and used LSD 4 times. So what do I have? Maybe they are 'caused' by the same thing e.g. something over active in the brain? Does this concern anyone else or are you happy that we all have the same thing?"

(james, Ezboard forum Visual snow or static – Archive 2 - visual snow & Static / HPPD are they the same thing?, June 30, 2003)

Not only the phenomenal similarity between HPPD and persistent aura symptoms, but especially the comorbidity of migraine and previous use of hallucinogens may render it difficult to make the differential diagnosis between HPPD and persistent aura without infarction. The diagnostic algorithms to achieve this differential diagnosis are not so simple as suggested in the above-quoted post from the Ezboard forum Visual snow or static, as can be seen from the guidelines proposed to define the differential diagnosis process and the illustrative case histories of Sofia Greene, nnis, prismvision, jimjones235, ChodaKingandSprocket, AH, fockyoumang, Rachop1, Dan, Morgan, Andrew and JA.

When persisting perception disorder (PPD) occurs in subjects with a comorbidity of migraine and illegal drug abuse (el-Mallakh, 1989), one has to consider and evaluate the differential diagnosis between hallucinogen persisting perception disorder (HPPD) and persistent aura without infarction.

Comorbidity of HPPD and migraine

"I don't know, maybe it's the weather or something, but lately I have been having headaches that have gone on non-stop for days and days at a time. I normally never really had headaches in the past, but lately I have been getting these mainly in the front part of my head. I can almost press on my head and feel where it hurts. Maybe it's sinus or something (don't know how those feel and how long they last, but I don't have a sinus infection). Anyways, they are not excruciating but they bother me enough to affect my performance at work and at home. Could this be a symptom of HPPD? Does anyone else get these?"

(Cologero, HPPDonline – Message Boards – HPPD General Support Section – Constant headaches, April 30, 2003)

"Hey Cologero, how severe have your headaches been? And does your family have any history of migraine headaches?

A migraine is 'A severe recurring headache, usually affecting only one side of the head, characterised by sharp pain and often accompanied by nausea, vomiting, and visual disturbances'.

I get migraines, but mainly only the visual disturbances (a bright flashing light in my right eye that spreads to the whole eye & sometimes a little bit on my left eye), which is a bit unusual. I've only had following headaches twice, and I think mine felt like a stabbing pulsing pain just above my eyes. But you're probably best off reading a website on migraines for symptoms as I think they can vary.

Not all people get the visual disturbances, though the headaches are generally really bad & last a long time. You can get really good medication from a chemist, that if taken upon first noticing the symptoms makes the headache stop shortly after.

It's probably worth checking yourself out at a doctor's; you don't have to mention any of your HPPD conditions as it's very likely a separate condition to HPPD.

In the meantime, try not to have many dairy products or too much caffeine. If your headache does still persist over more days, definitely see a doc.

Hope you feel better soon!"

(koolaid, HPPDonline – Message Boards – HPPD General Support Section – Constant headaches, May 1, 2003)

"Hello guys, my name is Cameron, I enjoy playing computer games and hanging out with my girlfriend and am very happy that after two years I am not doing any drugs. I used to be a huge pothead smoking an average of a quarter of an ounce a day for about 2 years, before I got very very bad respiratory problems and decided to stop, I am only 16 btw. But anyway, before I stopped I had done Ecstasy about 3 months before I quit smoking pot. I felt VERY strange a few days after doing Ecstasy but this is known as the 'hangover' of the drug. Anyways after a few days I felt alright and felt alright for the next month of two, then after that one day, I had a flash of insight and started to have CHRONIC headaches, I had a huge migraine for a period of about 26 days accompanied by visual things as well, I had eye floaters and a constant feeling of disorientation, since I stopped having the headaches this has not stopped, I have OCD (obsessive compulsive disorder) and I constantly obsess and become depressed about how I feel, I cannot do anything in life anymore without feeling like complete crap about how I feel, nothing is fun anymore, everything reminds me of how I feel, it looks like there is a constant layer of Gauze over my vision, halos around lights, and floaters, is this because of doing Ecstasy once? I live with constant regret and fear should I have this in my years to come, I would give one of my legs if just this would go away and I could be normal again, I feel that I am on God's bad side or something. Will this ever go away? I am still very young and still have alot to enjoy in life, I don't want to smoke pot anymore but I still want to be able to have some drinks, but I can't do anything without seeing something that reminds me of my symptoms, looking around the dark room just seeing a filmy layer over anything I should see normally, I know in my head that I should see it without that, but its not like that, it has the floaters and the layers of fuzziness, I hate it, please help me."

(Cameron, HPPDonline – Message Boards – HPPD General Support Section – hello people, let me introduce myself, May 12, 2003)

"1) I have had HPPD-like symptoms since I was a child. When I would look up at the sky I would see these little translucent balls that would fly up and then descend. Since I have used psychedelics these symptoms have only gotten more complex, and more frequent. They also appear on more surfaces than the sky. I have talked to others who are non-psychedelic users and they have told me that they have experienced similar things.
2) After a migraine headache, my pre-psychedelic HPPD-like symptoms would act up. One time I saw a block of static on a friend of mine's face.
3) HPPD symptoms seem to peak for me when I have low blood sugar, or I'm fatigued.
4) If I tell myself that it doesn't exist it goes away periodically.
5) If I cover one eye it seems to go away periodically.
6) If I try to focus on it, it usually goes away periodically.
7) HPPD symptoms seem to peak for me when my eyes are unfocused. I also never seem to have any symptoms at night."

(HuckleBones, Shroomery - Mushrooms, Mycology and Psychedelics - The Psychedelic Experience - Observations regarding HPPD, April 7, 2006)

"just catching up with the board here today, and yet more strangeness, this morning, of all mornings, i woke up with an 'occular migraine' - if anyone else has experienced these, it's a whole different weirdness in the visual realm (for me there is no headache or anything, but visually, it's like you have a folded up piece of cellophane on your eye, angles and whatnot, usually only one eye or the other) - these are totally unrelated to my hppd visuals and always go away fairly quickly. it's just kind of freaky that i revisited this post today of all days, especially since i only get occular migraines once or twice a year at most."

"ymmit, HPPDonline – Message Boards – HPPD General Support Section – After Images Yellow Street Paint, April 12, 2007)

According to Sofia Greene, "There are a mixture of sufferers that have used, and not used, LSD and have had, and haven't had, classic migraines... Separation of criteria is great, but where for example would I fit in? I have a history of extensive drug abuse including acid (quit 8 years ago), I also have classic migraine, and my father has had a similar problem (recently discovered, fill you in more later)? How could people like me fit into these categories?"

(Sofia Greene [subject #1], Email to Klaus Podoll, February 24, 2006)

In case histories like Sofia Greene's (or nnis's, prismvision's, jimjones235's or ChodaKingandSprocket's), where one can make a definite diagnosis of persistent aura without infarction according to the diagnostic criteria used in the present study, a concurring diagnosis of HPPD can be excluded on the grounds of a diagnostic hierarchy explicitly expressed in its DSM-IV criteria. These diagnostic criteria state that a diagnosis of HPPD cannot be made if it is "due to a general medical condition (e.g., anatomical lesions and infections of the brain, visual epilepsies)", a list of neurological disorders to which persistent aura without infarction should be added in forthcoming revisions of the DSM-IV. Likewise, Abraham (1983) had "excluded ... all subjects ... whose conditions were diagnosed as organic brain syndrome, seizure disorder, migraine, cerebral trauma, or a history of CNS tumor or infection" from his classic study of the visual phenomenology of the LSD flashback (p. 885).

In cases of probable or possible persistent aura without infarction according to the diagnostic criteria used in this study, as operationalised by the MAS score, the degree of certainty of this diagnosis is considered not to suffice to act as an exclusion criterion for the diagnosis of HPPD as stated by the DSM-IV diagnostic criteria. Hence, in cases raising this differential diagnosis, the evidence for a diagnosis of HPPD is taken to outweigh the evidence for a diagnosis of probable/possible persistent aura enough to make a diagnosis of HPPD according to DSM-IV criteria. The diagnostic algorithm proposed for the differential diagnosis of HPPD and persistent aura without infarction is summarized in the following table.

Diagnostic algorithm used for the differential diagnosis between HPPD and persistent aura without infarction
Drug history	Migraine history	Diagnosis
Previous use of hallucinogens	Definite persistent aura without infarction (MAS score ≥ 4 and criterion B.8)	Definite persistent aura without infarction & Substance abuse or dependency
Previous use of hallucinogens	Probable persistent aura without infarction (MAS score ≥ 4 without criterion B.8)	Hallucinogen Persisting Perception Disorder & Migraine with aura or migraine without aura
Previous use of hallucinogens	Possible persistent aura without infarction (MAS score < 4)	Hallucinogen Persisting Perception Disorder & Migraine with aura or migraine without aura

It must be emphasized that the diagnostic hierarchies used in this diagnostic algorithm serve the pragmatic purpose of establishing criteria for the differential diagnosis between HPPD and persistent aura without infarction. However, empirical studies are needed to study the assumptions underlying the use of these diagnostic hierarchies.

William of Ockham (ca. 1288 - ca. 1348)

According to damianSB, another participant in Sofia Greene's internet survey, he had been acquainted with "visual snow since I was born... I did a few things (3 lsd trips...) which enhanced/exaggerated that snow..."

(damianSB [subject #231], Ezboard forum Visual snow or static – Archive - Newbie, born with snow n' stuff; amazed to find this forum!, January 2, 2004)

The essential feature of HPPD is the recurrence of one or more of the perceptual symptoms that are reminescent of those experienced during one or more earlier hallucinogen intoxications (see here). In case histories like damianSB's (or VisualSnowSufferer's) where the onset of one or more episodes of PPD precedes the onset of hallucinogen use, whereas one or more later episodes of PPD follow the onset of hallucinogen use, a diagnosis of HPPD can be excluded by definition for the subject’s pre-drug use episodes, but not for the post-drug use episodes of PDD. In this study, following the principle of Ockham's razor, we introduced a diagnostic algorithm whereby we made a diagnosis of definite, probable or possible persistent aura without infarction in these cases and abstained from making a comorbid diagnosis of HPPD. According to Ockham's razor, diagnostic parsimony advocates that when diagnosing a given set of signs and symptoms a doctor should strive to look for the fewest possible causes that will account for all the symptoms. Again, empirical studies are needed to study the assumptions underlying the use of this diagnostic algorithm. While diagnostic parsimony might often be beneficial, credence should also be given to the counter-argument modernly known as Hickam's dictum, which put succinctly states that "patients can have as many diseases as they damn well please" (Trobe, 2002).

Another problem that has to be addressed by empirical studies is the question whether the comorbidity of previous drug use and migraine may have pathoplastic effects on the visual phenomenology and other clinical features of PPD. This can only be discerned by empirical studies comparing groups of PPD sufferers distinguished by the presence or absence of previous drug history and migraine history, respectively.

Dr. Abraham on the differential diagnosis between HPPD and persistent aura without infarction

"As a psychiatrist I have not seen this form of migraine [i.e. persistent aura without infarction]. By the definition of HPPD which I wrote in DSM-IV and still support, HPPD involves prior use of hallucinogens. Migraine does not. This distinction should help differentiate the majority of cases of persistent visual disturbances. I am skeptical of labelling a patient with PPD as suffering from HPPD if there is no history of prior psychostimulant drug use. I am also skeptical of labelling PPD as migraine in the absence of headache, but you may educate me more on that point [as a matter of fact, headaches may be completely absent in the history of a patient fulfilling the ICHD-II criteria of persistent aura without infarction if the present attack in a migraineur who has hitherto suffered exclusively from attacks of typical aura without headache is typical of previous attacks except that one or more aura symptoms persists for > 1 week]. My answer to your dilemma [of mutually contradictory diagnoses of persistent aura without infarction and HPPD in cases featuring positive evidence for both diagnoses (due to the exclusion criteria required for the diagnosis of persistent aura without infarction, i.e. 'Not attributed to another disorder', and HPPD, i.e. 'The symptoms are not due to a general medical condition')], is to accept the existential reality of the patient's distress, avoid the rush to diagnosis, and carefully follow him. In my experience, time is the best diagnostician."

(Henry David Abraham, Email to Klaus Podoll, March 19, 2006; additions in square brackets by Klaus Podoll)

References

Abraham HD. Visual phenomenology of the LSD flashback. Arch Gen Psychiatry 1983; 40: 884-889. [PDF]
el-Mallakh RS. Migraine headaches and drug abuse. South Med J 1989; 82: 805.
Haas DC. Prolonged migraine aura status. Ann Neurol 1982; 11: 197-199.
Hoffman JA. LSD flashbacks. Arch Gen Psychiatry 1984; 41: 631-632.
Russo E. Cannabis for migraine treatment: the once and future prescription? An historical and scientific review. Pain 1998; 76: 3-8.
Trobe JD. Noble J. David, MD, reminisces. J Neuroophthalmol 2002; 22: 240-246. [PDF]

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