Ptosis And Miosis

Symptoms of Hemicrania Continua

Since my occipital stimulator was installed in June 2009, when The Headache gets worse I can't always tell by the pain. The ONS does a pretty good job on most days in minimizing the pain. It does not totally remove the pain, but it is much less disabling than it was before I had my electronic nerve zapper.

Even with reduction of pain, The Headache continues to exist. One of the ways I can tell is that the other physicial symptoms of The Headache continue to march onward regardless of occipital stimulating wonderment.

In addition to the almost ever present nausea, I have  a runny nose on the left side of my face where The Headache resides. I have swelling under my left eye. I have nerve pain at a diagonal across the left side of my face (although the ONS has helped this a bit). I have ptosis of my left eye (as seen in this picture) which is drooping of the eyelid(s).  I have miosis of my left pupil (as seen in this picture but because of the color of my eyes and the ptosis may be difficult to discern) which is an abnormal contraction of the pupil not related to light. Sometimes I have tearing from my left eye only.  My left eye will turn in towards my nose. I have brain fog and confusion.  I continue to wake up every night at 3 am if I am lucky enough to be asleep by then.

Drs. Elisabetta Cittadini and Peter Goadsby recently published an analysis of hemicrania continua (The Headache) in the journal Brain A Journal of Neurology "Hemicrania continua: a clinical study of 39 patients with diagnostic implications" (Brain 2010: 133; 1973–1986).  In the following quote they state what has been clear to me from the beginning - hemicrania continua hurts - really really really badly hurts!!!

Hemicrania continua has been considered a syndrome with moderate intensity but with exacerbations of severe pain according to the current criteria (Headache Classification Committee of The International Headache Society, 2004). We found, in the majority of our patients, the range of the continuous pain was anything between 1 and 10 out of 10 on a verbal rating scale. In addition, 70% of patients described their painful exacerbations as excruciating and almost half of the patients considered them as the worst pain they had ever experienced with a severity of anything between 8 and 10 out of 10 on a verbal rating scale. These findings suggest that the pain in this condition, although typically fluctuating, is indeed more severe than previously considered. The data reinforce the need for rapid diagnosis and effective treatment to minimize unnecessary suffering. (Cittadini & Goadsby, Brain, 2010, p 1981)

The current IHS criteria for Hemicrania Continua is:

Persistent strictly unilateral headache responsive to indomethacin.
Diagnostic criteria:
   A. Headache for >3 months fulfilling criteria B-D
   B. All of the following characteristics:
       1.unilateral pain without side-shift
       2.daily and continuous, without pain-free periods
       3.moderate intensity, but with exacerbations of severe pain
   C. At least one of the following autonomic features occurs during exacerbations and ipsilateral to the side
      of pain:
      1.conjunctival injection and/or lacrimation
      2.nasal congestion and/or rhinorrhoea
      3.ptosis and/or miosis
   D. Complete response to therapeutic doses of indomethacin
   E. Not attributed to another

In the article Cittadini and Goadsby urge placibo controlled testing with Indocin to rule out HC for unilateral unremitting headaches.  I know one of my first steps to getting a correct diagnosis was an immediate reaction of pain reduction from a bolus of indocin given intraveniously.  I cried and panicked when the incompetent local neurologist I had refused to prescribe it.  My headache specialist I ended up with in Kansas City (Doc Optimist) said only people who actually have indomethacin responsive headache conditions felt like I did if we were refused indomethacin.  After all, for most people it is like taking naprosyn or ibuprofen or acetaminophen - not hardly an effective pain reliever for severe pain UNLESS you have a condition that it is almost the ONLY pain reliever that works.

They also state "the majority of our cohort showed signs of agitation, or restlessness, or both, and nearly one-third were aggressive during severe pain, consistent with the neuroimaging findings of hemicrania continua, which suggest some important part of the pathophysiology occurs in the posterior region of the hypothalamus, as with the other trigeminal autonomic cephalalgias." (p. 1983)  This also corresponds to my experience: I become very agitated and pace with The Headache when it gets going, where with migraines my instinct was to go to a quiet dark place and lie still.

Eight of the 39 patients followed in the article received occipital nerve stimulators, and so far six of the eight have shown great results.  I hope that this will mean that insurance companies will loosen up their purse strings for this rare condition and pay for this medical device and its installation/upkeep, because almost all of the medication remedies outside of indomethicin were ineffective.

I'm going to go and ice my head and see if I can get The Headache to quit playing games this evening.  Ready to go at it again tomorrow!