An Unusual Case of Sensory Polyneuritis

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Why should they not occasionally seeat the other end of the scale a case that was exclusively sensory ? He remembered the case of. (liphtheritic polyneuritis ...
874

Proceedings of the Royal Society of Medicine

56

On examination.-Dysphasia which is executive as regards spoken speech and both receptive and executive as regards written speech. He can carry out complicated verbal requests. He cannot spell out his name correctly on a typewriter. Calculation grossly impaired. Moderate degree of retinal arteriosclerosis. Left pupil a little bigger than right. Slight but definite wasting of right intrinsic hand muscles. Power everywhere normal save in right hand where there is some reduction. Apraxia is present in the right upper limb. Thus when combing his hair he slowly and jerkily directs the comb to his face and then, with clumsy sweeping movements, digs the teeth into his nose or cheek. He uses excessive force. The disorder is not due to spatial disorientation as he can place objects (though with mechanical difficulty) in various spatial relationships with other objects. The disorder is more than can be accounted for by incoordination, for simple tests for ataxia are fairly well executed. Sensation is normal save in the right upper limb. Pin-prick, temperature, and cotton-wool, are not so clearly felt over the dorsum and palm. Vibration sense is diminished below the elbow. Passive movement and joint position sense diminished at right wrist and more so at fingers. Tactile localization is impaired in the hand as is 2-point discrimination. There is astereognosis. Tendon-jerks exaggerated on right side. Plantar responses flexor. Heart enlarged to left. Brachial vessels thickened and tortuous. Blood-pressure 175/100. Cerebrospinal fluid: Normal pressure and constituents. Blood Wassermann reaction negative.

An Unusual Case of Sensory Po1yneurit s.-DAVID KENDALL, M.B.

20.1.39: T. L., aged 59, admitted to National Hospital under the care of Dr. Gordon Holmes. Fourteen weeks before admission he noticed pain and numbness on the right side of the lower jaw. Within a few days the whole of the right side of the face was numb and this rapidly involved the tongue and the left side of the face. Fourteen days later his feet felt numb. The numbness spread from the toes upwards and involved the whole of both limbs in one week from the onset. A few days after appearing in the limbs, the numbness started in the fingers and passed up the arms. Within three weeks of the onset the whole body felt numb. For three weeks before admission he had tingling sensations in his limbs which led to incessant rubbing of his arms with his hands. Throughout his illness he has experienced difficulty in swallowing solids. Food gets lost in his mouth. He cannot keep his false teeth in as he does not know where they are. 2 st. in weight lost in the last five months. 20.11.38: Admitted to St. Bartholomew's Hospital. While there he received 4 mgm. of vitamin B daily, without any change in his condition. 21.1.39: Developed retention of urine. 23.1.39: Became incontinent of urine. 29.1.39: Bladder function returned to normal. Past history and family history negative. Patient has worked as a boiler cleaner for the last thirty-seven years. Uses no special preparations. No other employees have had simiar illnesses;. Diet not deficient. Moderate alcohol consumption. No history of febrile illness in past year. On examination.-A very emaciated patient. Speech nasal and slurred. Constant rubbing of hands and arms, even during sleep. Both corneal reflexes absent. Complete failure to appreciate pin-prick over distribution of trigeminal nerves, including the tongue. Gross impairment to perception of light touch, and temperature. Apparent loss of position sense in the tongue. Salivary secretion much diminished. Ta,ste bilaterally impaired. No facial weakness. Slight middle-ear deafness on the right side (of many years' standing). Palate normally elevated on phonation. Palate reflex absent. Pharyngeal reflex juist present. Arms: Wasting corresponding to general emaciation. Marked hypotonia. Power good in all muscle groups. Co-ordination grossly impaired. No fibrillation. Trunk: Intercostal muscles and abdominal muscles contract well.

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Section of Neurology

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Legs: Wasting corresponfds to general emaciation. Marked hypotonia. Power good in all muscle groups. Co-ordination grossly impaired. Reflexes (20.1.39) Biceps present; supinators and triceps equiivocal; abdominals absent. Knee and ankle jerks absent. Plantars: Both weak flexor. (23.1.39) All ten?don reflexes absent. Plantars not obtained. (1.2.39) Biceps and supinator jerks just obtainable. S5ensation.-Gross impairment of perception of all forms of sensation throughout the body; maximal on peripheral parts of limbs and least marked on neck. Vibration sense just present on clavicles only. No muscle tenderness; superficial nerves not thickened. Gait grossly ataxic. Patient is only able to walk or stand with support. Cardiovascular and respiratory systems normal. Abdomen: No abnormality detected. Rectal examination negative. IJvstigation.s at St. Bartholomew's Hospital22.11.38: Blood-count normal. 23.11.38 Cerebrospinal fluid clear: four lymphos. per c.mm. Total protein 0*100 grm. %. Lange 0001100000. At the National Hospital24.1.39 : Blood-count normal. 26. 1A.39 Cerebrospinal fluid clear : Cells 1 per c.mm. Total protein 0 100 grm.00'. Nonne-Apelt weakly positive. Pandy positive. Lange 0001221000. Wassermann reaction negative. 3.2.39: Electrical reactions of muscles of face and limbs normal. A recent finding, since the note was compiled, is that this patient has a considerable quantity of lead in the urine. It is not possible to estimate this accurately because he has become incontinent. His trade has been that of a boiler cleaner, and it has been part of his job to scrape the paint outside boilers. Discussion. Dr. GORDON HOLAIES said that he coul(l ad(l nothing to the history of this case or its present state, but he was very (loubtfuil with regard to the (liagnosis set otut by Dr. Kendall. The history was a cuirious one, beginning with pain and numbness on one side of the face and loss of sensation developing first on that side and within a day or two appearing on the other side of the face as well. The loss of sensation was very profound, but even more striking was the almost total escape of the motor system. The wasting of the arms and legs was only part of the general emaciation, and the motor movements were extraor(linarily good in relation to the loss of sensation. As to the question of lead in the turine, when lea(l affected the nerv-ouis system it seemed to fall on the motor rather than on the sensory system. Dr. D. DENNY-BROWN recalled an unusual case of diphtheritic polyneuritis commencing wx,ith ntumbness in the tip of the tonguie and the middle of the lips. The loss of sensation spread, and a few days later affected the tips of the fingers and spread proximally in the limbs. The tendlon-jerks were late in disappearing. WN'hile he didI not think that the present case was one of diphtheritic polyneuritis, he thouight it not impossible for the sensory element to be unusually predominant in other forms of polyneuritis. Dr. C. P. SYNIONDS said that he had been extremely interested in this patient. It seemed to him that this kind of case was one which theoretically ought to occur. In other words, if one lived long enough and were lucky one ought to see something of this sort, because it was the corresponding opposite to what after all was not so very rare ; the polyneuritis that was exclusively motor was not an extremely rare occurrence. Why should they not occasionally see at the other end of the scale a case that was exclusively sensory ? He remembered the case of (liphtheritic polyneuritis, certainly not so extensiv-e as this, thouigh it began in the same way; also another case of polyneuiritis which was under observation for some time and which was pturely sensory andI spread all oxer the body very like Dr. Kendall's case, though not with the same degree of sensory loss. This latter case was apparently duie to gold treatment for rheumatoid arthritis. It seemed to him that the increased protein in the cerebrospinal fllid with a normal cell count was in favour of polyneturitis. Dr. HUGH GARLAND said that it would be interesting to know something about the gastric secretion in this case. He thouight that the finding of lead in the urine was of no significance. Lead was found in the urine of many workmen, and had nothing to do with a neuirological syndrome.

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Proceedings of the Royal Soctety of Medtcine

58

He had seen fouir cases of gold polyneuritis, they were all like each other an(d the motor and sensory disturbances were almost entirely confined to the feet. They wsere not at all like the case now shown. Dr. PARKES WN-EBER asked Nhether it was worth while givinr intensive treatment by intramuscular injections of liver extract. In spite of the blood-count, there remained a bare chance of the symptoms being of the nature of combined degeneration. Dr. KENDALL replied that as for gastric analysis the patient 'was too ill to have it (lone. As to liver extracts the patient had been having large doses of liver for the last four weeks without showing any change. In reply to another question, he said that the electrical reactions of all the muscles were perfectly normal. l)r. GORDON HOLAIES said that the condition was progressive. It w%as four months since the onset of the case. Since the patient had been transferred from St. 'Bartholomew's to the LNational Hospital he hadl developed sphincter disturbance, with incontinence and retention of turine.

POSTCRIPT (D.K., 30.5.39). The patient died four weeks later. Post-mortem findings, described at the meeting on 18.5.39: Oat-celled carcinoma of the right lung with metastases in the wall of the stomach. The dorsal nerve-roots were grey and, on microscopical examination of the dorsal root ganglia, almost all the ganglion cells had disappeared. The ventral roots were normal. The motor nerve-endings in the muscles showed some early degenerative changes. In addition, chemical analysis of the bones showed the presence of 1 04 mgm. of lead per 100 grm. of bones.

Myopathic Wasting associated with Ptosis and External Ophthalmoplegia.-F. A. ELLIOTT, M.B.

Dorothy K., aged 38. Admitted under the care of IDr. Denis Brinton. For ten years slhe has had progressive drooping of the lids without relationship to ocular fatigue. For four years, difficuilty in turninog the eyes in any direction, reading being unaffected. No diplopia admitted. For three years, weakness and wasting of neck, shoulders, and entire upper extremities. No difficulties in relaxing the grasp. Weakness not improved by rest. Family hi8tory.-Negative for myopathv. No cataracts or other stigmata of family decadence. On examination.-Pupils sluggish to light; contract on attempted convergence. E.O.M. limited to very slight downward and lateral movement. Bilateral ptosis. Extreme weakness of orbicularis palpebrarum and oris, zygomaticus, and frontalis. Tongue weak but does not deviate. Severe wasting and moderate weakness of sternomastoids, shoulder girdle, and entire uipper extremity, except for the relative sparing of upper trapezius, clavicular portion of pectoralis major, rhomboids, and serratus anterior. Supinator longus not conspicuously affected. The trunk, pelvic girdle, and lower extremities appear large, buit are normal for age and stature. Collar of tender fat obliterates the tapering from calf to ankle. Sensation normal. Tendon reflexes brisk. Plantars flexor. No myotonia. No cataract. No reaction of degeneration. Cerebrospinal fluid normal. Blood Wassermann reaction negative. Dr. PURDON MARTIN said that he had been interested in this case because recently he had had two cases of progressive external ophthalmoplegia with what he regarded as myopathic features. The first, a girl aged about 12, whom he saw at Moorfields with a similar condition in the eyes, had pronounced weakness of nearly all the muscles of the face, but especially the orbicularis oculi. He was rather inclined to think that the case was associated with myasthenia, but the injection of prostigmin did not produce any effect on her, and apart from a weakness of the sternomastoids and the facial musculature, no other somatic muscles were affectedl. About six months ago he saw a little boy with progressiv e external ophthalmoplegia, who had a general hypotonia of his somatic musculature. He did not recall having seen any patient in which there was such advanced muscular wasting as in the one shown by Dr. Elliott. Although the condition was usually called progressive nuclear ophthalmoplegia, he thought that quite a good case could be made out for some of these patients being considered as myopathic, and not nerv%wous.

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