mise à jour du
25 août 2005
The Canadian Medical
Association Journal
1933; 412-415
A case of pachymeningitis haemorrhagica interna
Guy H. Fisk


This case of pachymeningitis haemorrhagica interna is considered worth reporting on account of the rarity o the condition, the difficulties of its diagnosis, and because it was so thoroughly worked up both clinically and pathologically.
Mr. L., an adult white male, 68 years of age, a travelling salesman, was admilted to the service of Drs. AH Gordon and CA Peters at the Montreal General Hospital, on July 13. 1932.
Complaints: Paralysis of the right side, aphasia, incontinence of faeces and urine.
Family history : Irrelevant.
Personal history: His habits were good; he had had the ordinary childhood diseases. In1901 a fistula in ano was cured by two operations. For many years there had been occasional attacks of diarrhoea. In 1931, a suprapubic proatatectomy was performed for an adenoma of the prostate, with uneventful recovery. At this time there were no cerebral signs nor symptoms and the cardio-vascular system was normal. Until the onset o the present illness the patient was quite well and carried on his business.
History of the present illness: About June 15, 1932, he began to have very frequent headaches across his forehead. They were often present on waking up in the morning and would last nearly all day. Aspirin gave him temporary relief. The headaches continued and on June 30, 1932, while returning home by train a numbness developed in his right arm and leg. On reaching home he felt very tired and next morning he still had the same numb feeling in his right extremities. Although able to walk he could not hold anything in his right hand, which hung loosely at his side. This condition lasted until July 10th, when he fell unconscious. The loss of consciousness was only temporary and he was immediately put to bed. Since that time, although he is stated to have recovered consciousness, he had said nothing but "yes" and "no." Associated with this aphasia there had been frequent yawning, snapping of the fingers and incontinence of the urine and faeces. On July 12th the paralyzed arm, which had been cold, became warm and tender and the yawning became less frequent On July 13th he was admitted to the hospital.
Physical examination : When admitted the patient was speechless and unable to move in bed, although able to move the left leg and left arm. The temperature was 98.4° F, the pulse 84, and the respirations 24. The face was reddened and the venules of the cheeks prominent. He was non-cooperative and would not even protrude his tongue when requested to do so.
The heart was enlarged. The blood vessels were not especially thickened although the temporal arteries were tortuous and slightly thickened. The blood pressure was 158/108. There were no other cardiovascular lesions.
The pupils reacted to light and accommodation and were equal and regular. The vessels of the fundus showed considerable atherosclerosis, but otherwise appeared normal. The pupils dilated under atrophie and the disc margins of the fundi appeared clear. Tests made of the cranial nerves were unsatisfactory on account of the cloudy mental state. There was slight weakness of the left facial muscles, and the tongue deviated to the right side. The uvula however, was in the midline. There was complete right-sided hemiplegia, with marked sensory loss, though the responses were very unreliable. Subjectively the right side was acutely painful.
Searching physical examination revealed no other abnormalities.
Laboratory findings: Spinal fluid - initial pressure = 165 mm. h2o; pressure after jugular compression = 265 mm. h2o. The rise on jugular compression was rapid and the fall after removing the compression was also rapid. After withdrawal of 18 cc of spinal fluid the pressure was reduced to 85 mm. h2o. The fluid withdrawn was clear, contained O-5 cells per cm3, and both the Pandy and Boss Jones tests for albumin were negative. The total protein was found to be 0.042 per cent.
The urine was turbid, acid, had a specific gravity of 1021, contained a trace of albumin, 20 red blood corpuscles and 80 "pus" cells to the high power field, but no casts and no glucose. The benzidine test for blood was positive.
Bed blood corpuscles, 6 050 000 per cm3; white blood cells, 8 650; hemoglobin, 93 (Sahli), 110 (Hellige). Differential blood count: polymorphonuclears, 64 per cent; lymphocytes, 35 per cent; eosinophiles, 1 per cent; monocytes, 1 per cent.
Blood chemistry: urea-nitrogen, 24 mgrm. per 100 cc; creatinine, 1.87 mgrm. per 100 cc; uric acid, 5.00 mgrm. per 100 cc; sugar, 0.151 per cent The Wassermann reaction was repeatedly negative on the blood and on the spinal fluid; also the colloidal gold reaction was negative on the spinal fluid.
Progress notes: The pain and tenderness on movement of the right arm and leg remained, but there was no associated swelling nor local redness. On the second day after admission the patient was able to talk a little but relapsed into aphasia. The heart became irregular in rate and volume towards the end. On July 25, 1932, he became weak and there was stertorous slow breathing. The face became ashypale and cold, the eyes fixed and staring, and then, with slight twitching of the fingers of the right hand, he suddenly died.
Clinical diagnosis: Cerebral thrombosis in the left internal capsule involving the thalamic fibres, with sudden death due to a left-sided cerebral hemorrhage involving the precentral gyrus.
Autopsy : The findings at post-mortem were of interest chiefly in connection with the meninges and brain.
Meninges: These showed some thickening, grossly. The under-surface of the dura mater was lined by a thick granulation membrane, rich in new capillaries, young fibroblasta, and diffusely infiltrated with lymphocytes, eosinophiles and a few polymorphonuclear cells. There was a fresh haemorrhage into its deeper layer and there was evidence of old blood pigment in the phagocytic cells. Particularly noticeable was a marked dilatation of the thinwalled capillaries in the deeper layer. There were numerous loculi, most of which were dear, scattered through the granulation tissue lining the dura. These were devoid of any lining endothelium and a few contained an albuminous substance. These loculi were interpreted as spaces remaining after the absorption of extravasated blood. No bleeding point could be detected anywhere in or on the dura. In the outer layer of the dura mater the arteries showed a marked medial hyperpiasia, degeneration and calcification.
On the left side, just beneath the dura, there was a thin haemorrhagie membrane containing a blood clot which fell out as soon as it was incised. This membrane with its enclosed blood dot extended over the posterior part of the frontal, the whole parietal, the upper part of the temporal, and the anterior part of the occipital lobes. The membrane peeled away from the arachnoid easily. The outer layer of the membrane was composed to a large extent of a thin layer of fibrous tissue with organized granulation tissue and pigment cells similar to, but not so gross as, those in the dura mater. The inner surface was covered by a thick layer of red blood corpuscles.
The arachnoid showed a wide separation from the brain over the left parietal lobe with an area of great thickening and diffuse inifitration with lymphoeytes polymorphonuclears, plasma cells, and red blood corpuscles. The inflammatory reaction was most marked on the upper surface, but many of the cells passed down to the deeper structures, and the subarachnoid space at this point contained quite a number of free red blood corpuscles. Over the left occipital lobe the arachnoid was thickened at one point and contained considerable pigment The pis mater over the right frontal lobe showed lymphocytic and plasma-cell infiltration with small haemorrhages into its substance. Over the left parietal lobe the vessels of the pia mater were all engorged with blood. The remainder of the meninges was normaL
Brain: The area of the brain under the blood clot was depressed one centimetre. Gross sections of the brain showed no hemorrhage, no thrombosis, no softening, nor any sclerotic changes in the cerebral vessels. Microscopically, there were small perivascular hemorrhages in the brain tissue in the right and left temporal lobes, right and left basal ganglia and the upper, middle and lower pens. The left basal ganglia showed in the subependymal tissue enormous numbers of amyloid bodies and a marked subependymal gliosis. No other lesion of the brain. was found in the numerous sections examined. None of the vessels showed thrombosis or sclerosis microscopically.
Pathological diagnosis: Pachymeningitis hamorrhagica interna over the left parietal lobe of the brain. (There is an old organized haemorrhagie membrane beneath the dura and a fresh heamorrhage beneath it.)
An interesting feature is the atherosclerosis of the vessels of the dura. The condition here was pachymeningitis haemorrhagica interna, localized over the parietal lobe and extending from the frontal area to the occipital area and down to the temporal lobe on the left side. Nothing was found in the brain to account for the signs and symptoms of pontine and basal ganglia lesions except the minute perivascular hemorrhages localized to these regions on both sides. It is suggested, however, that the displacement of the brain and pressure against the tentorium would account for the clinical features.
A case is reported which presents both clinically and pathologically the typical features of pachymeningitis haemorrhagica interna.
Clinically, there were obscure etiology, probably a combination of old age and alcoholism; symptoms of headache, hemiplegia, and aphasia, none of which are diagnostic.
Pathologically, there.were a recent large subdural hemorrhage enclosed in a laminated membrane; a laminated membrane consisting of layers of granulation tissue, the oldest on its outer surface and the youngest on its inner; phagocytic monocytes in the membrane containing blood pigment; depression of the brain beneath the hemorrhage; dilatation of the thin walled capillaries in the deeper layer of the membrane; absence of other lesions in the brain.