- 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.
-
- SUMMARY
-
- 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.
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