TWO CASES OF NEURINOMA.

CLINICAL AND LABORATORY NOTES.

1074

TWO CASES OF NEURINOMA.

A CASE OF INTRACRANIAL NEOPLASM

BY J. HARGREAVES ROBINSON, L.A.H. DUB., L.M.R.C.P. IREL.,

WITH UNUSUALLY RAPID DEVELOPMENT OF SYMPTOMS.

HON.

SURGEON,

ROYAL MATERNITY

CHARITY,

BY JOHN INKSTER, M.D. ABERD., D.P.H. OXF.,

LONDON.

here described fall into that heterogeneous group of conditions known as " neurinomata." The first one is a typical example of neurofibromatosis as described by von Recklinghausen ; the second had a neoplasm, suggesting the condition called by Mott

THE two

"

cases

pachydermatocele."

CASE l.-A man, aged 31, noticed at the age of 17 two small nodular swellings in the course of the intermediate cutaneous nerve sheath. Three years later he noticed more swellings. He now has many neurinomata scattered profusely in the sheaths of the cutaneous branches of the posterior divisions of the spinal nerves, which are most numerous and largest in size in the lumbar region. He has 4 6 5 moles scattered about the body, most prevalent on the arms, in the axillae, and on the scrotum and chest. He states that he has always been healthy, and is a non-smoker and total abstainer. His medical history records benign tertian

malaria, phlebotomus fever, and

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amu Gbli an JL.//Ejm,//ttUt

Inguinal swelling

in Case 2.

histolytica infection. The patellar reflex

is

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slightly more marked on one side than on the other. His fingers are tremulous, and muscular fibrillation of a facial muscle was noticed during examination. There are slight alterations in epicritic and protopathic sensibility Mentally he could not be classed as a typical normal individual, but would be described by the alienist as of the mattoid type. He admits, after an obvious mental effort, that what he describes as " night starts" occur just at the moment when he is falling to sleep. He gives the impression of making a special effort to answer a

simple question. CASE 2.-A healthy young man first noticed during the year 1919 a swelling on the lower part of the abdomen. He states that it has very slowly progressed in size, and has never been painful, even on pressure, but that friction against the clothing has been rather uncomfortable. He has never noticed any other swellings anywhere on his body, and does not know of any other member of his family having had anything of the kind. During conversation he gives the impression that attention and conation are rather more marked than usual, and he speaks rather slowly. On the lower surface of the abdomen there is a large, sessile, cutaneous neoplasm, slightly pendulous, and freely movable on the aponeurosis, excepting at a small area towards the inner border where movement is not so free. The neoplasm is firm in texture, insensitive to pressure, and clinically suggestive of the pachydermatocele of Mott. The inguinal lymphatic glands are normal in size. There is a wide band of discoloration of the skin in the middle line extending from the ensiform cartilage to the pubes. No other swellings can be discovered ; 53 small moles are scattered about the body. Nerve sheaths are not palpable. I removed the growth, and it has been described as follows : " The large mass of tissue is composed of very cellular fibrous tissue, with little tendency to a whorled arrangement in most places. There are, however, areas in which the cells form small concentrically laminated masses. No epithelium is seen covering this mass. The structure appears to be that of neurofibromatosis."

M.R.C.P. LOND.,

HON. CONSULTING PHYSICIAN, STOCKTON AND THORNABY HON. PHYSICIAN, NORTH ORMESBY HOSPITAL, MIDDLESBROUGH.

HOSPITAL ;

THE following case is of interest in that rapidity of development of symptoms led to an error of diagnosis. The patient, a man aged 38, had nasal polypi removed on July 7th, and during the operation pus was found in both antra. The antra were drained, and he was discharged from hospital apparently well. He should have reported to hospital on the 16th, but was unable to do so, as he was suffering from septic tonsillitis. A week later, having recovered, he presented himself for examination, and the condition of the nose and throat was satisfactory. He remained well until August 13th, when he noticed that he had difficulty in playing the piano with the right hand. The difficulty increased, and power was completely lost in the right upper extremity. Two days later he noticed that he was dragging the right foot, and power was lost in the right lower extremity. On the following day he had involuntary movements of the upper and lower extremities, although he had no power of voluntary movement. These movements were so severe that they interfered with sleep, and his friends were unable to hold the arm and leg still.

He had had no headache, but had vomited once. When seen on August 20th he had complete paralysis of the right upper extremity, almost complete paralysis of the right lower extremity, and slight weakness of the right side of the face. The pupils were equal, and reacted normally to light and accommodation. There was no nystagmus, and the eye movements were normal. Examination of the fundi showed neither optic neuritis nor any other abnormality. He was unable to close the right eye completely. On the right side the triceps, supinator, and knee- and anklejerks were all exaggerated, and there was ankle-clonus. The plantar reflex was extensor on the right side and flexor on the left. Sensation was slightly blunted in the right arm and leg. Speech and mental condition were unaffected. There was no incontinence and no retention of urine. The heart, lungs, and abdomen, except for an old appendicectomy scar, showed no abnormality. The urine was normal. Pulse-rate was 72, and temperature was 984° F. A diagnosis of intracranial abscess was made, and he was admitted to North Ormesby Hospital. A blood count showed 10,000 white cells per c.mm., and a normal differential count. It was thought that the position of the abscess was just subcortical under the middle of the motor area, and a skiagram showed an indefinite shadow in this region. There was no development of symptoms nor of physical signs except that he vomited twice. On August 31st Mr. J. B. T. Keswick trephined over the middle of the motor area on the left side, and explored the underlying brain, but no pus was found. The opening in the skull was enlarged and the surrounding brain explored, but without success. There was practically no change until Sept. 4th, when respiration began to fail. Patient became cyanosed, and there were rales over the whole of both lungs. The temperature rose to 100-6° F. and the pulse-rate to 148, and he died on Sept. 6th. Post-mortena examination showed no meningitis, and the brain appeared normal on external examination, except that there was haemorrhagic discoloration of its surface under the opening in the skull. Section revealed a large, soft, chocolate-coloured tumour in the left cerebral hemisphere. It was irregularly fusiform in shape, with its long axis parallel to that of the cerebral hemisphere, and its widest part in the region of the Rolandic area. It extended for about an inch and a half forward, and for about 2 inches backward from this area. It lay mostly in the white matter, but extended into the grey matter of the cortex in the middle of the motor area. It was not encapsulated. Unfortunately, as a report was required for a coroner’s inquest, the brain had to be cut without being hardened, and so accurate observation of the relationships of the tumour could not be made. Histological examination showed the tumour to be a gliosarcoma. There was fine fibrous adhesion of the visceral to the parietal pleura over the greater part of the right lung, and there was congestion of the bases of both lungs. The csecum was adherent to the anterior abdominal wall by fine fibrous adhesions where the appendix had been removed 20 years previously. There was no pus in the ethmoidal cells. All other organs were healthy.

Both cases are doubtless lesions of the endoneural connective tissue, and the association of multiple Commentary. In this case the onset was not sudden enough for moles, pigmentation of the skin, and neurofibromatosis as described by Soldau are evident. the lesion to be embolism or haemorrhage, and there