Headache, by Robert Magginetti (Tranquility Base)
In the last post we learned about Alice in Wonderland syndrome, a rare phenomenon involving distortions of visual perception and body image, most often caused by migraines. Although a specialty practice in headache might seem dull [so to speak] at first glance to those interested in behavioral neurology, unusual and colorfully-named types of headaches can make things more interesting. In Case Studies of Uncommon Headaches (2006), Dr. Randolph Evans reviews a number of these unfortunate ailments, which include exploding head syndrome, neck-tongue syndrome, red ear syndrome, and burning mouth syndrome. So let's begin with a hot ear.
Case 6. My ear is red, hot, and burningA 54-year-old white woman was seen who had a 10-year history of episodes of a burning sensation of the left ear. The episodes are preceded by nausea and a hot feeling for approximately 15 seconds and then the left ear becomes visibly red for an average of approximately 1 hour, with a range of approximately 30 minutes to 2 hours. Approximately once every 2 years, she had a flurry of episodes occurring over approximately a 1-month period during which she averaged approximately five episodes, with a range of 1 to 6. There also was an 18-year history of migraine without aura occurring approximately once a year. ... A cerebral arteriogram revealed a proximal left internal carotid artery occlusion of uncertain cause after extensive testing. MRI scan at age 45 was normal. Neurologic examination was normal. A carotid ultrasound study demonstrated complete occlusion of the left internal carotid artery and a normal right.The diagnosis? Red Ear Syndrome, first described by Lance (1994) in the aptly-titled article, The mystery of one red ear. Following a plea to colleagues to "lend me your ear," Lance (1996) subsequently reported on 12 cases. He concluded that RES:
may be associated with irritation of the third cervical root [nerve in the neck], temporomandibular joint [jaw] dysfunction, or thalamic [pain] syndrome. It may also occur without obvious structural cause in response to touch or heat. The condition may be an example of the ABC (Angry Back-firing C-nociceptor) syndrome with the increase in ear temperature being caused by the antidromic ["backwards"] release of vasodilator peptides [calcitonin gene-related peptide and substance P].It can also occur in association with migraines, glossopharyngeal and trigeminal neuralgia, upper cervical spine pathology, and herpes zoster [shingles]. The GABA analogue and anti-seizure medication gabapentin can be helpful in preventing RES.
By the way, the girl in the picture above [who is not Case 6] says her red ear doesn't hurt, and that she doesn't get headaches.
Case 7. My mouth is burningA 49-year-old woman was referred by her primary care physician with a 1.5-year history of daily constant burning or numbness of the entirety of her tongue and the back of her throat. She also complains that the inside of her mouth is sensitive. She has had a dry mouth for the past year. ... Artificial saliva has not been helpful. She has tried a variety of pain pills without any help....Burning Mouth Syndrome1 most often afflicts middle-aged and older women. Causes include dry mouth (e.g., from medications or diabetes), nutritional deficiency, food allergies, fungal infection (candidiasis aka thrush), trigeminal small fiber neuropathy (nerve damage), and hormonal changes. Treatments range from estrogen-progesterone replacement therapy to nutritional supplements to switching prescription medications to addressing an underlying medical condition. I don't know if this syndrome can be considered a "headache" in the standard usage of the word, but then again I'm not a neurologist.
Case 1. Noises in the nightA 43-year-old woman was seen with a 5-month history of a noise in her head. On an almost nightly basis, as she was falling asleep, she would hear a loud noise like "electrical current running" lasting a second. Sometimes her whole body would shake for a second afterwards. Very occasionally, she would have an associated flash of light. Frequently, a second episode of the loud noise occurred shortly after the first. She then could fall asleep without any problem.Exploding Head Syndrome (Pearce, 1988) is a bang-up way to be aroused from your nightly slumber. A small percentage (~10%) of sufferers see a flash of light, even fewer feel as if they've stopped breathing for a short time. It's more frightening than it is painful. Interestingly, Evans (2006) suggests that EHS might be caused by delay in the reduction of activity in the brainstem reticular formation as the patient transitions from wakefulness to sleep. In 1949, Moruzzi and Magoun were the first to recognize that stimulation of the brainstem reticular formation produces low-voltage fast activity in the EEG, characteristic of an alert and attentive behavioral state. So something might be neurologically amiss with the EHS patient's sleep-wake cycle, although Evans gave no direct evidence of this. And the explosion phenomenology is largely unexplained, as noted by Pearce (1988):
The cause of the bomb-like noise remains a mystery: no known vascular or hydrodynamic changes in the brain, labyrinths, or cerebrospinal fluid would cause such a symptom, although a momentary (almost ictal) disinhibition of the cochlea or its central connections in the temporal lobes, or a sudden involuntary movement of the tympanum or tensor tympani, might be the explanation...Evans' other case studies recounted complaints of numb tongue (neck-tongue syndrome), painful scalp (nummular headache), and moderately severe bifrontal pressing headaches between 1:00 AM and 2:30 AM (hypnic headache). Cough, exertional, and sex headaches (Cutrer & Boes, 2004) will have to wait for another time...
1 On the NIH website, the photo illustrating this painful condition is that of a smiling older woman (rather comical, I thought). Synonyms for burning mouth syndrome include glossodynia, glossopyrosis, glossalgia, stomatodynia, stomatopyrosis, sore tongue and mouth, burning tongue, oral or lingual paresthesia, and oral dysesthesia.
Cutrer FM, Boes CJ. (2004). Cough, exertional, and sex headaches. Neurol Clin. 22:133-49.
EVANS, R. (2006). Case Studies of Uncommon Headaches Neurologic Clinics, 24 (2), 347-362 DOI: 10.1016/j.ncl.2006.01.006
Lance JW. (1994). The mystery of one red ear. Clin Exp Neurol. 1994;31:13-8.
Lance JW. (1996). The red ear syndrome. Neurology 47:617-20.
Moruzzi G, Magoun HW. (1949). Brain stem reticular formation and activation of the EEG. Electroencephalogr Clin Neurophysiol. 1:455-73.
Pearce JM. (1988). Exploding head syndrome. Lancet 332:270-1.
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