A story of Tic Douloureux

Sat, 24 Jul 2010 20:50:38 +0000
Num. 32

Autopathography of a Ferrara man with trigeminal neuralgia from 1803 to 1824

Keeping a diary on the symptoms of your illness (autopathography) was common practice in the first half of the XIX century, especially by the more cultured or wealthier classes. Perhaps the very act of keeping the diary was a type of catharsis or liberation in itself. This is what we gather from this "story of tic douloureux". The person in question generally described the path his illness was taking when in the throes of stabbing pain, and was usually silent during his long symptom-free periods. He involved as many people as he could, getting in touch with others affected by the same illness, swapping treatments and supporting each other in hope. If an autopathography is written by someone with a certain level of education, as in this case, it can be very important, even from the strictly medical-historical point of view. This is because it summarises developments in the diagnoses and therapies that are directly related to the medical theories of the time, through observation of the medical records of the patient, the doctors and the treatments given. This autopathography was found during a library reorganisation of the old arcispedale Sant'Anna (St. Anna's hospital) in the nineteen fifties, and tells the story of the tic douloureux of Ruggero Ragazzi from its onset in 1803 to the last diary entry in 1823. The diary comprises about thirty hand-written pages. Attached were letters signed by some of the leading doctors and university professors of the day and notes on various treatments taken from the scientific journals of the time. This is the medical history of Ruggero Ragazzi, a Ferrara man, with a degree in law and who had dealings with some of the most important families of the city such as the Sacrati, Aventi and Trotti families. He began writing his diary in the summer of 1803 at the age of 57, when for the first time he felt a ferocious pain on the left side of his face, at the corner of his nose, lasting four or five seconds. The pain came back in winter and he got the pain four or six times a day for a few days. A doctor advised him to rub it with volatile oil and the pain disappeared. In the winter of 1804 he suffered new attacks which were more frequent and intense, leaving him with a strong pain in the bone under his left eye socket. General cures such as enemas, bloodletting, and laxatives seemed to alleviate the pain. In autumn 1805, the pain came back and the doctor advised him to have the area sprayed with water at the Battaglia baths. The patient met a doctor called Tuenal here and told him of his symptoms. The diagnosis was immediate: "Ah ha, the tic douloureux of Mesier André; stop spraying the water and only bathe in natural waters, then take a grain of musk every evening for 12 evenings, but the musk has to be good and pure". Following these instructions, 1806 passed by quite uneventfully. Clearly, the doctors had not diagnosed trigeminal neuralgia up to that point, an illness that was still poorly understood at the time. There are occasional mentions of intermittent face or temple area pain by ancient medical experts (Hippocrates). A more accurate description is given by Aretaeus of Cappadocia in the first century AD. Caelius Aurelianus also recognised a facial nerve syndrome, and tried to differentiate it from the "tic non douloureux" or the "raptus caninus". It seems that the illness was more widely known in the Arab world, but trigeminal neuralgia began to be described on a more widespread basis only from the XVII century on, without however providing any medical or causal opinions: in fact trigeminal neuralgia was confused and described with other types of neurological disorders of the head and face like primary and secondary headaches. It was 1765 before R.B. Hirsch began to study the trigeminal nerve and its semilunar ganglion in detail. John Foothergill had provided an essential contribution to the definition of trigeminal neuralgia in 1773 when he published an important monograph on the "painful affection of the face". In 1800 the French doctor, François Chaussier replaced the name "facial neuralgia" with "tic douloureux" describing in detail the medical variants in the three nerve branches and the facial muscle "shocks" near the pain centre. Theories on the nature of the illness depended on the schools of thought of the individual medical groups. Patient treatments in turn depended on the specific theories held: musk (sold in granule form) opium for oral administration, mercury-based medicines in the area innervated by the nerve, bloodletting and laxatives with the idea of eliminating the negative "humours" or any toxins in the system, deadly nightshade alkaloids like the "atropa belladonna" extract, the gastric juices of crows used as a carrier for the active ingredients of common topical medicines (to increase the skin absorption), zinc oxide, lemon and orange flowers, etc. The musk was derived from the sebaceous contents of two glands taken from the stomach of certain species of male Asian roe deer, especially in Tibet, was thought to act on diseases of the nervous system and so was used in the cure of the tic douloureux after the encouraging results observed in its use in the treatment of epilepsy. It is interesting that the most effective treatment for trigeminal neuralgia nowadays includes the drugs used to treat epilepsy. Over the course of his illness, Mr. Ragazzi underwent all these treatments in accordance with whoever was treating him at the time. However he didn't obtain any significant results, and in fact even suffered negative results on occasion. In fact from 1814 on, the story written in the diary becomes sketchy. We only have occasional comments on the fact that his condition wasn't changing despite all the treatments. In July 1814 he heard about a surgical procedure entailing "cutting the diseased nerve" but after gathering more detailed information on the procedure, reluctantly decided not to go through with it. The diary then goes silent, but the letters and appointments attached allow us to reconstruct the treatments he underwent, despite his scepticism. Electric shock therapy was possibly applied, probably involving local applications. Magnets were also recommended by leading doctors. We are not given the results of these treatments, but they certainly didn't change things. Mr. Ragazzi was advised to apply oxymuriatic gas in 1816. This acid, also called anti-inflammatory hydrochloric acid or chlorine, was already believed to yield oxygen at the end of the XVIII century. But even this treatment failed miserably. We read the following in 1816: "Well, we have got to this point, and I have decided not to do anything else based on what I have observed so far, and to live as well as a I can and resign myself to God's will". Mr. Ragazzi may possibly have also been subject to other fashionable treatments for prosopalgia (facial pain) at the time: thorn-apple, the European cherry-laurel, the foxglove, mustard and arsenic, in addition to a lot more. Finally in 1823 a particularly painful attack was reported, attributed to a very windy season, which is very harmful to the illness. The diary finishes here. Ruggero Ragazzi died on 23 March 1824. The doctor Pietro Paolo Malagò, surgeon at Sant'Anna hospital, carried out the autopsy which found that the entire area considered to be affected was actually perfectly healthy. This is where the tic douloureux story of Ruggero Ragazzi ends. The diary allowed us to track his symptoms and the development of the treatment, in addition to giving us some idea of the medical knowledge of the illness. The leading names in medicine, surgery and chemistry are invoked and the therapies used at the time are mentioned: emetics, musk, opium, cockscomb, henbane, electrotherapy and chemical-gas therapies. In a period that medicine was in constant debate between theories and counter theories, and therefore the treatments related to such theories, we witness the various experiments attempted: from the prolonged use of emetics to the intensive use of deadly nightshade or stinking nightshade, which would subject the patient to periods of extreme weakness to the extent of preventing him from being able to move, even during the few, short periods of relief between bouts of pain. If a diagnosis had to be made, it would clearly have to involve what is known as essential trigeminal neuralgia. Having become manifest at an adult age, the facial pain continued to worsen, was paroxysmal, and made more acute by exposure to cold or drafts of air. While the post-mortem was specifically aimed at the morphological analysis of the trigeminal nerve, it ruled out the possibility that the pain felt for over 20 years was secondary to a local illness along the course of the nerve or at its origin, and diagnosed tic douloureux or essential neuralgia which is idiopathic. However, notwithstanding the illness, Mr. Ragazzi died at 78 for other reasons, possibly cardiovascular failure as can be inferred from the autopsy. Prosopalgia is not a serious illness in itself, its seriousness lies in the negative influence it has on the social, relational and family life of the patient, and his or her quality of life in general, i.e. the patient's perception of his or her mental and physical well being. Nowadays, although there has been a definite improvement in our knowledge of essential trigeminal neuralgia, we are still unsure as to what triggers it. However, the medical and surgical treatment available is capable of alleviating the symptoms, both as regards their intensity and the frequency of recurrences, in many cases, defeating them completely.

By Enrico Granieri, in colloboration with Vittorio Govoni, Ilaria Bissi, Riccardo De Gennaro e Patrik Fazio of the Neurogical Institute of the University of Ferrara