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The First Description of Idiopathic Progressive Who First Described The 1270 Landi, Motto, Celia, Musicco, Lipani, Boccardi, Guidotti J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.12.1270 on 1 December 1993. Downloaded from Neurosurg Psychiatty 1991;54:793-802. esis. Stroke 1990;21:1251-7. 20 Dennis MS, Bamford JM, Sandercock PAG, Warlow CP. 25 Fisher CM. Lacunes: small, deep cerebral infarcts. A comparison of risk factors and prognosis for transient Neurology 1965;15:774-84. ischemic attacks and minor ischemic strokes. The 26 Ostrander LD, Brandt RL, Kjelsberg MO, Epstein FH. Oxfordshire Community Stroke Project. Stroke 1989;20: Electrocardiographic findings among the adult popula- 1494-9. tion of a total natural community, Tecumseh, 21 Landi G, Candelise L, Celia E, Pinardi G. Interobserver Michigan. Circulation 1965;31:888-98. reliability of the diagnosis of lacunar transient ischemic 27 Kannel WB, Abbott RD, Savage DD, McNamara PM. attack (TIA with lacunar syndrome). Cerebrovasc Dis Epidemiologic features of chronic atrial fibrillation: the 1992;2:297-300. Framingham Study. N EnglJ Med 1982;306: 1018-22. 22 Cartlidge NEF, Whisnant JP, Elveback LR. Carotid and 28 Faris AA, Poser CM, Wilmore DW, Agnew CH. vertebro-basilar transient ischemic attacks. Mayo Clin Radiologic visualization of neck vessels in healthy men. Proc 1977;52:117-20. Neurology 1963;13:386-96. 23 Sacco SE, Whisnant JP, Broderick JP, Phillips SJ, 29 Harrison MJG, Marshall J. Angiographic appearance of O'Fallon WM. Epidemiological characteristics of lacu- carotid bifurcation in patients with completed stroke, nar infarcts in a population. Stroke 1991;22:1236-41. transient ischaemic attacks, and cerebral tumour. BMJ7 24 Millikan CH, Futrell N. The fallacy of the lacune hypoth- 1976;1:205-7. The first description of idiopathic progressive the modes of presentation of motor neuron disease bulbar palsy and which may occasionally remain confined to the bulbar region. Given the advanced disease, a brain- Furukawa has recently recounted the lesser known stem tumour seems unlikely in the absence of sensory contributions to clinical neurology of Sir Charles Bell signs and if the bulbar palsy was due to myasthenia who first described the numb chin syndrome' and the gravis, ocular and limb symptoms would be expected. phantom phenomenon2 in his book on the nervous Bell's life is well documented but little is known of system.3 We would like to report an account of a the referring physician. Robinson was born in patient with progressive bulbar palsy (PBP) who was Lancashire and graduated Doctor of Medicine at referred to Bell, is included in the same book and pre- Edinburgh on 12 September 1800. He was president dates the writings of Duchenne de Boulogne,4 who is of the Royal Medical Society, founded in 1734 by a usually credited with the first descriptions.5 group of medical students after the successful acquisi- The patient's details are contained in a letter to Bell tion of a fresh body for dissection. The founders from Robert RW Robinson, of Preston, England, initially met in taverns ".... with the intention that dated 21 July 1825. He asked for advice about "an a dissertation in English or Latin on some medical subject unmarried lady, nearly seventy years of age, who has ... should be composed and read"6 and later meetings enjoyed uninterrupted good health up to the present were attended by Charles Darwin. There is no evi- illness". He states: dence that Robinson had an earlier interest in neurol- From the first of her complaint to the present moment ogy as he obtained a doctorate based on a urological she has been free from headach (sic) and from pain, subject (Disputatio medica inauguralis de vesicea, ure- numbness, or debility of the limbs. The vision and hearing thraeque morbis). He became a Licentiate of the are natural; the appetite good; the bowels regular, and the College of Physicians (London) in 1807 and returned sleep natural .... Somefew months ago she had some dif- to his native Lancashire where he was an influential ficulty in the and in member of the board of management of the Preston using tongue expressing particular http://jnnp.bmj.com/ words. This difficulty has gradually increased, and now Dispensary, founded in 1809. Unfortunately 169 she cannot protrude the tongue, or even move it. She has years after his original account, the aetiology of PBP lost her speech altogether. The tongue itself is soft and remains obscure. but it retains its sense taste and The ANDREW M CHANCELLOR pulpy; of feeling. deg- Department ofNeurology lutition is impaired and occasionally she is distressed with a University Hospital of Wales, sense of suffocation, in attempting to swalow food, which CardiffCF4 4XW, UK she is now obliged to do with great care. She afnot hack JOHN D MITCHELL up any thingfrom the throat, nor draw any thingfrom the Department ofNeurology, posterior nares by a back draught. The features of the face Royal Preston Hospital, on September 30, 2021 by guest. Protected copyright. are quite natural, and the skin retains its feeling. The Sharoe Lane, Preston, UK saliva occasionallyflows from the mouth." ROBERT J SWINGLER Bell noticed the similarity between this patient's Department ofNeurology, condition and the syndrome observed in a dog after Dundee Royal Infirmary, section of the lower cranial nerves and concluded that Dundee, UK the patient was suffering from "a paralytic affection of the ninth nerve" and noted that the "function of thefifth nerve was entire." Unfortunately no follow up or Correspondence to: Dr Chancellor necropsy results are available to tell us if the disease became more widespread, neither is there any infor- mation about the deep tendon reflexes, as this physical 1 Furukawa T. Charles Bell's description of numb chin syn- sign was not introduced into the neurological exami- drome. Neurology 1988;38:331. nation until 1875. Bell advised nauseating medicines 2 Furukawa T. Charles Bell's description of the phantom and leeches under the other reme- phenomenon in 1830. Neurology 1990;40:1830 mastoid, amongst 3 Bell C. The nervous system of the human body: As explained dies. in a series ofpapers read before the Royal Society ofLondon. This description would rival any modem case With an appendix of cases and consultations on nervous dis- report for conveying clearly the clinical signs, the eases. London: Longman, 1836: 390-1. 4 Duchenne de Boulogne G. Paralysie musculaire progres- important negative features for the diagnosis and the sive de la langue, du voile du palais et des 1evres. Archiv disability resulting from this tragic affliction. We Gin Mid 1860;2:283-96. would argue that this patient's illness, which combines 5 Morton LT. A medical bibliography. An annotated checklist oftext illustrating the history ofmedicine. 4th edn. London: anarthria, impaired deglutition and tongue movement Butler and Tanner, 1983:603-46. with ptyalism, probably with mixed upper and lower 6 Holmes JD. Early years of the medical society of motor neuron signs, is due to idiopathic PBP, one of Edinburgh. Univ Edin J 1968;23:333-40..
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