A particular variety of headache. By Sir Charles Symonds (From Guy's Hospital and the National Hospital, Queen Square, London). Brain 1956: 79; 217–232
Cambridge
Studies in Neurology, published in 1970, is a collection of 21 papers and two memorial addresses, reprinted from the writings of Sir Charles Symonds between 1923 and 1967, and prefaced by an informative autobiographical essay. The choice of material included was made with advice from Dr W.I. McDonald (Fig. 1). Each paper has a short annotation by Sir Charles. On item 14 he writes: ‘That this is a malady sui generis no one would deny. Its essential features were briefly described by Wilfrid Harris in 1926, under the heading "Periodic Migrainous Neuralgia". Harris had an immense store of knowledge of pain in the head and face, and this syndrome might well have been called after him "Harris's Neuralgia". The American caption "Cluster Headaches" has however gained wide acceptance and has some descriptive value. The paper reprinted here gave what I think was the most complete account of the condition up to that date, with details of a new and effective method of treatment’.
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Leaving aside examples in which his observations were incomplete, or the duration of the malady too short from which to draw useful conclusions, Sir Charles describes 17 cases observed in consulting practice. He wants to define a clinical syndrome of comparative rarity, although one that is well recognized by many neurologists. He will have little to say except by way of analogy or speculation on its causation. But on treatment, he makes observations that ‘because the pain is often of excruciating severity’ may be of practical value. Simply stated, the features are agonizing pain of sudden onset and transient duration in the supra-orbital region or in and behind the eye: in bouts lasting several weeks followed by periods of complete freedom usually lasting 6 or more months; with individual paroxysms of <2 h duration occurring at least once per day; and with no associated disease of neighbouring structures.
Now, Sir Charles describes the syndrome in more detail. The locus of the pain is above or in the eye, albeit with some radiation to the face, jaw and neck. Although strictly unilateral in a given bout, it may alternate. Most of his patients are young adult males. Some have a family history of migraine, although their own symptoms rarely include typical migrainous features. The duration of a bout varies between 2 and 10 weeks with a tendency for cases of longer duration to experience more prolonged clusters but also with incremental intervals between bouts. Occasionally, patients describe a single paroxysm, one bout only of short duration, or events occurring at >24 h intervals within an otherwise typical cluster. Case 1, a man of 35, first developed unilateral episodes of pain in the eye, like a tooth exposed to cold, evolving rapidly and lasting a further 20 min, with redness and watering of the eye, a blocked nose and occasional nausea but infrequent vomiting, for 2 or 3 days every 6–8 weeks; 10 years on, the bouts are lasting 4 weeks but with 18 month intervals between each. More generally, the attacks—around four per 24 h—tend to occur at night, and at the same times, but spill into the daytime in those with many paroxysms in each bout and with longer clusters. Case 2 describes six bouts in 3 years, varying in duration from 3 to 5 weeks, during which his single nocturnal attack always occurred at 2 a.m. on four occasions, and at 2 p.m. and 9 p.m. in the other two clusters. Each paroxysm lasts around 20 minutes and almost never longer than 2 h, although the timing may increase with duration of the illness. Bouts are not obviously provoked. The view that they may be associated with local sinus disease or facial trauma seems misplaced. The intervals may be up to a decade but usually are <1 year; and even the few patients with frequent clusters from onset may later enjoy an unexpectedly long reprieve. A male, aged 46 years, had attacks every autumn for 16 years and then represented after a symptom free interval of 8 years. But, against this background, the main burden of Sir Charles's paper is to describe an effective treatment.
For some years, Case 5 had experienced single nocturnal paroxysms of ocular pain with a blocked nose lasting 45 min in bouts of around 6 weeks. Dr (George) Riddoch had advised an injection of ergotamine tartrate (0.25 mg) at the onset of pain, but this had no effect. The patient had himself decided to inject before going to bed. The attacks stopped immediately. Thereafter, a bout was judged to be over if omitting one injection per week resulted in undisturbed sleep. But, as the frequency and severity of attacks increased during each sequential bout, he increased the dose to 0.5 mg or gave himself another early morning shot to prevent daily episodes. Worried at the increasing frequency of symptoms, despite their responsiveness to the incremental doses of ergotamine, and knowing something of the toxicity of that drug, Case 5 sought advise from Sir Charles: ‘as this present bout of attacks has now lasted a year and two months I sincerely hope it will shortly end, but I honestly believe that without the injections I should have been either a mental case or a physical wreck long before now’. Sir Charles reassures him that the twice daily injections can continue. Case 6 was considered to have trigeminal neuralgia and an alcohol injection of the Gasserian ganglion had been advised. Rather, Sir Charles suggests—in the event, successfully—prophylactic injections of ergotamine tartrate at the onset of any further bout. Case 6 also finds that 15–20 drops of dihydroergotamine by mouth also works. Case 7, a medical man, developed typical paroxysms as a student during lectures—dubbed ‘functional’ both by a physician and an ophthalmologist he consulted—that continued in bouts but did not impede his sporting activities or education. A neurosurgeon considered him to have ‘anterior migraine’ and proposed to operate on the trigeminal ganglion. One neurologist was ‘baffled’ and another diagnosed migraine prescribing sedation and ergoemetrine, all to no effect. Self administration of ergotamine tartrate at onset and the use of a trilene inhaler were equally unhelpful. When prophylactic administration also proves unsuccessful, he is admitted to hospital, observed during an attack and given pethidine 100 mg for (partial) pain relief. After responding to 8 hourly injections of ergotamine tartrate for a few days, saline is surreptitiously substituted and the pain recurs. Established thereafter on prophylactic intramuscular injections, Case 7 uses 210 ampoules of ergotamine tartrate in one bout (of unspecified duration). In the next, with attacks precipitated by a vigorous game of squash, he becomes used to 2 mg daily in divided doses supplemented with d-amphetamine sulphate 10 mg daily to offset drowsiness. Once this bout is over, Case 7 is aware of rebound phenomena: continuous throbbing headache and tremulousness, and cold in the extremities. In all, 8 of 17 patients have found that prophylactic use of ergotamine tartrate usefully modifies their malady.
Symonds acknowledges that Wilfrid Harris first described this condition (Neuritis and Neuralgia, 1926, 301–305): for example, ‘in 1907 I saw a man, aet 44, who every year since he was 17 years old had suffered for some weeks in the autumn from neuralgia ... he was awakened at 3am, the pain appearing to start quite suddenly as a sharp stabbing ... behind the outer canthus of the eye ... so severe that he was almost crazy with it ... he had two or three attacks in one day’; and another, from the age of 19 years, ‘began to suffer from daily attacks, for three or four weeks every year, of severe pain in the back of the right eyeball and forehead, with lacrimation and reddening of the eye ... for an hour or two’. Dr Harris advises alcohol injection of the Gasserian ganglion. Section of the greater superficial petrosal nerve, tried some years later on the basis that the lacrimation and nasal congestion implicate neuralgia primarily affecting this structure, was also later abandoned by its protagonists. A proportion of cases described by Horton and colleagues from the Mayo Clinic in 1939, seemed to have attacks precipitated by histamine leading to the hypothesis of treatment by histamine desensitisation. This has brought relief to the majority of recipients for up to 18 months. And the same claim for therapeutic success from manipulating histamine is apparent from Horton's follow-up series from 1941 and 1944. But Symonds is not persuaded. The descriptions are incomplete, follow-up too short and the natural history of the paroxysms interpreted without taking account of observations in controls. Although, for Horton, bedtime attacks can be relieved promptly by an intravenous injection of dihydroergotamine or a rectal suppository of ergotamine tartrate (2 mg) with caffeine (100 mg) ... ‘the writer in describing Cases 5 and 6 of the present series [an earlier paper of CP Symonds from 1951 first reporting two of the present series] appears to have given the first account of the prevention of attacks by the injection of ergotamine tartrate’. And this is Sir Charles's main message: whilst he does not necessarily disagree with the analysis that features of migrainous neuralgia invade the diagnostic boundaries of typical migraine, making it unhelpful to distinguish cluster headaches from other variants, it is the feature of bouts that alerts the neurologist to the preferred prophylactic treatment ‘for there is relatively little risk of toxic symptoms resulting from injections of ergotamine tartrate when given for limited periods with long intervals’.
On that, the hazards from indiscriminate use of ergotamine are already known. It is recommended that exposure should be limited to 0.5 mg given no more than twice per week and not to individuals with sepsis or peripheral vascular disease. Although perhaps over-cautious in this advice, angina pectoris, gangrene and other circulatory symptoms have each developed in a number of patients receiving large (such as 4 mg daily for 12 days) or even standard doses for migrainous neuralgia, whereas another had used 15 mg per month continuously for 3 years without complication. Symonds himself has only run into trouble on one occasion. After a refractory period, Case 8 is eventually stabilised on injections of ergotamine tartrate (0.5 mg thrice daily) and discharged home but his doctor fails to follow the advice to ‘watch the peripheral pulses’ and omit the dose on one day each week: 3 months later, free from headache, Case 8 has pulseless arms and symptoms of ischaemia during exertion (when playing golf or batting in the nets) but all is well after a further interval without ergotamine; and ‘if precautions are observed, together with a watch for the symptoms and signs of peripheral vasoconstriction, daily injections of ergotamine tartrate of the order described in this paper may be given over long periods without any serious toxic effects’.
At the time, migrainous neuralgia, separated from the generality of migraine by its periodicity and response to the prophylactic use of ergotamine, provided a welcome exception to the dogma that effective therapies in general, and those applicable to disease of the nervous system in particular, were few and far between. Also basing their formulation on the role of prophylactic treatment—here with oral indomethacin—Peter Goadsby and colleagues now further refine the classification of periodic unilateral headache with autonomic features on the basis of the response to oral indometacin (page 1142).
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