Bichat / [William Sydney Thayer].

  • William Sydney Thayer
Date:
[1903]
Catalogue details

Licence: In copyright

Credit: Bichat / [William Sydney Thayer]. Source: Wellcome Collection.

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    \ > A CASE OF NEURO-FIBROMATOSIS (VON RECKLINGHAUSEN’S DISEASE) WITH PARALYSIS AND MUSCULAR ATROPHY OF ARMS AND LEGS.1 By H. M. Thomas, M. D., Neurologist to the -Johns Hopkins Hospital. Dr. Osier lias asked me to demonstrate this patient to the Society, on account of the very great interest of the nervous symptoms which she shows. The patient is a woman from West Virginia, Cyrena W., aet. 51, single; admitted Oct. 17, 1902, to Dr. Osier’s clinic; Med. No. 14,942. Complains of pain and stiffness all over. The family history is unimportant. She has never heard of a case similar to hers occurring in the family. She is the eldest of nine children. Has been always rather sickly. Went very little to school and has worked hard on a farm all her life. She had scarlet fever in infancy; diph¬ theria and measles when 27; “ grippe ” six years ago. Is sub¬ ject to occasional headaches. Menstruated first at 16; was fairly regular from then up to 48, when menopause occurred. Tumors of the skin have been present since early girlhood, but she paid little attention to them and can give little definite information about them. She has, however, noticed a great increase in the last four or five years and she has consulted a physician about them. Those on the face appeared last. She has noticed dark bluish spots on the body for many years. About five years ago she began to suffer from a burning, stinging pain in her left ankle. This was not constant, but recurred from time to time and was so intense that at first she thought her skirts were on fire. The pains gradually affected more and more of the leg, extending up to the hip and back, the right leg being affected after the left. This pain was at first very severe; each attack would come on very suddenly and last only a second or two. She describes them very much as a tabetic describes lightning pains. For one or two years there has been some pain of a similar character in her arms and shoulders. There are no pains in the face except those which the patient refers to defective teeth. With the onset of the pains, the patient began to have some difficulty in walking. Her brother first noticed that she limped a little, dragging one leg. The muscular weakness in¬ creased gradually and two years ago she had a good deal of difficulty in getting about, and for the last year has been en¬ tirely unable to walk. During this year she has spent her time between her bed and her chair, and up to a few weeks ago was able to get from bed to chair unassisted. For about four months her legs have been contracted at the knees and hips. For a little less than a year she has noticed some weakness in her arms, commencing in the right. This has gradually in¬ creased, and for about five months she has been unable to knit or sew, and has had difficulty in dressing herself. She has not dressed her hair for several months, but man still feed herself. 1 Presented to The Johns Hopkins Medical Society, November 17, 1902. From the very first she has noticed muscular twitchings and from time to time she has had some singing in the ear. • She made no complaint about her bladder before admis¬ sion, but since then has often had to be catheterized, although she is able, at times, to voluntarily pass a little urine. For the last two or three months she has noticed some diffi¬ culty in swallowing—chokes easily. There has been no regur¬ gitation through the nose. For about the same length of time she has noticed that her voice has become thick and weak. She complains of no trouble with her eyes or ears except slight tinnitus. As you see, the patient is a poorly nourished woman, with a careworn expression. Scattered irregularly over the face, trunk, arms and legs there are numberless little tumors. These vary in size from the little ones, about as large as a pin-head, to those as large as a good-sized cherry, and some few even larger. (See Plate XIII.) Some of these tumors are defi¬ nitely pedunculated and others are sessile. There are num¬ erous pigmented areas scattered over the body and in several spots the skin has a bluish look, which is apparently due to the coming through of the nodular tumors. Some of the larger tumors feel lobulated and liodular, but most of the masses have a homogeneous consistency. Two of the tumors were excised for histological examination and show the ordinary structure of neurofibromata. In so far as the skin manifestations go, the patient presents a typical picture of cutaneous neuro-fibromatosis. No definite tumors on the nerve-trunks have been made out, nor have we felt any definite plexiform growths, although the subcutane¬ ous fat below the popliteal space has a curious cord-like feel. Examination of the nervous system, however, reveals a very remarkable condition, and it is in this respect that I particu¬ larly wish to call your attention to the case. The patient is apathetic and somewhat dull. She speaks in a thick, muffled voice, suggesting some weakness of the laryngeal muscles. Her answers seem quite unreliable, especially in regard to dates. I could find no definite abnormality of any of the cranial nerves. There are no changes in the retina or the optic nerve. No definite disturbances of hearing could be made out, although the patient does complain from time tc time of subjective auditory sensations. The muscles of the pharynx appear to act well; the tongue is protruded straight but is slightly tremulous. The muscles of the neck are strong but all the muscles mov ing the arms are more or less weak; there is no absolute los of power in any of these muscles. The muscles of the arm are generally atrophied, but particularly so in the intrinsi muscles of the hand. Fibrillary tremors are noticed in th muscles of both arms, and I think can be seen by those wh
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