CLINICAL ASPECTS AND HISTOPATHOLOGY OF A DISEASE OF HEMORRHAGIC FEVER TYPE IN BUKOVINA
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Publication Date:
May 23, 1952
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REPORT
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CLASSIFICATION S-E-C-R-E-T
CENTRAL INTELL GENCE AGENCY
INFORMATION FROM
FOREIGN DOCUMENTS OR RADIO BROADCASTS
COUNTRY USSR
SUBJECT Scientific - Medicine, virus diseases
LANGUAGE
HOW
PUBLISHED Bimonthly periodical
WHERE
PUBLISHED Moscow
DATE
PUBLISHED May 1951 (delayed)
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DATE DIST. 1$ May 1952
SUPPLEMENT TO
REPORT NO.
Nevropatologiya i Peikhiatriya, Vol XX, No 2, 1951, PP 56.60.
CLINICAL ASPECTS AND HISTOPATHOLOGY OF A DISEASE
OF HEMORRHAGIC FEVER TYPE IN BUKOVIKA
Prof S. N. Savenko, Yu. G. Ruzinova
Clinic of Nerve Diseases, Chernovtsy Med Inst
In the summers of 1947 and 1948, a considerable number _ asee oo in-
fections of a general fever type were observed in the forest regions o Bukovina.
These cases exhibited a general hemorrhagic syndrome, as well as considerable
involvement o: the nervous system. In the majority of cues, sojourn in the
woods and tick bites preceded the infection, so that a virus et, ')gy of the
disease could be suspected. This suspicion was confirmed by neurological and
virusological institutes.
While the clinical picture, pathomorphological data, and results of viruso-
logical investigations show close resemblances to the so-called hemorrhagic fevers
(Crimea cr Omsk hemorrhagic fevers), the disease still has clinical and patho-
morphological peculiarities which permit it to be classified as a separate
entity, i. e., the Bukovina hemorrhagic fever.
Clinically, the disease is characterized by uninterrupted fever for 7 days,
acute general weakness, pains in the vii ,t and muscled of the calf, headache,
loss of consciousness; there were also hyperemia of the face, congestion of
scleral vessels, slight swelling of the face and brows, subicteric rtatu of the
sclera, hyperemia of the throat, hemorrhagic rashes of various localization and
types, weakly defined tendency towards bleeding of the gums and of the mucous
membranes of the mouth, and, in some cases, bleeding from the nose and'bruises
on various parts of the skin. In some cases, the pulse lagged behind the temp.-.
ature and there were brac'iycardia and lowering of the blood pressure.
The blood composition showed characteristic changes. During the first days,
there was leukocytosis which often changed into leukopenia; furthermore, a pro-.
pounced shut to the left at the expense of the rod-shaped forms and monocytes
C. r~a observed. This was accompanied by'an increase in the number of lymphocytes.
h.eame cas,s, young forms, myelocytes, and Tuerk's irritation cells were present.
In K number of cases, thrombopenia was noted.
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. In or;ier to carry out a complete clinical investigation of hemorrhagic
fever on our patients, we studied the clinical aspects of the afflictions of
the nervous system in this disease and, in the course of this study, came to
the conclusion that the neurological syndrome is often in the forefront in this
disease, in many cases becoming predominant.
Symptoms of affliction of the somatic and vegetative nervous system vhidh
were more or less pronounced were detected in all patients examined in the acute
fever period, as well as during convalescence.
We investigated 26 hemorrhagic fever patents in the summer of 1947 and
ten patients in 1948.
According to the degree of affliction of the nervous system, the cases
may be classified as follovst
Group 1, Acute disease with a lethal outcome, accompanied by diffuse
meningoencephalytia with ex'ensive participation of the cortex, subcortical
nodes, and the stem (eight cased.
Group 2. Disease ending in recovery, but patients exhibiting appreciable
cerebral symptoms expressed in the presence of an ataxia syndrome, a rmpyramidal
pathology, and changes of the psyche (three cases),
Group 3. Disease of a medium degree of severity. Patients in the fever
stage shoved meningeal symptoms, were in a stunned state, exhibited slight stem
and pyramidal symptoms: during the period of convalescence, these symptoms dis-
appeared almost completely, leaving only slight residual traces (13 cases),
Group 4, A light form of the disease without meningeal symptoms. However,
neurological examination of the patients still disclosed symptoms of affliction
of the nervous system, radiculo-;:ui ic symptoms, and phenomena of vegetative
dystonia,
['Case histories and clinical symptoms of three typical cases, belonging to
groups 1, 2, and 3 respectively, are described by the authors in detail, in order
to illustrate the following text?]
In cases of Group 1, the disease set in very rapidly, without preliminary
symptoms, and reached its culmination on the second and third day. Primary
symptoms comprised fever, headache, pains in th. waist, and muscle pains in the
legs. Vomiting was often observed on the first or second day. The temperature
immediately reached a high level and remained on that level. In all cases, there
were more or less pronounced symptoms of a hemorrhagic syndrome. Extensive nasal,
gastro-intestinal, pulmonary, and other hemorrhages of the type observed in Crim-
ean hemorrhagic fever and infectious nephroso.nephritis were absent. The following
changes ;,f internal organs were observed- the tongue was usually dry, with a
white or brown film; s +enuous pulse, occasionally accelerated, often lagging
behind the temperature, bradycardia, lcvered blood pressure, urination unaffected,
blood showing the characteristic changes mentioned above.
In addition to general infection symptoms, changes of consciousness appeared
on the second and never later than the fourth day. These included stupor, amential-
delirious , 'drone, sopor, and coma. There was a rapid transition from stupor to
sopor. L cases, there were symptoms of psychomotor excitement accompanied
by disconnected delirium, hallucinations, and attempts to get up and run ome-
where. These symptoms most frequently appeared at night; during the day, a
completely passive state predominated.
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Since the first day of the disease, meningeal syn'toms were observed. Among
them were rigidity of the back of the neck, symptom of Kernig and Brndzinskiy,
and a meningeal position (head thrown back, bent lower extremities, abdomen
pulled in). Affection of nerves of the large brain was not sharply expressed in
patients of this group. It mainly involved the oculomotor group, so that anise-
coria, mioeis, and mono- and bilateral ptosis were observed. In two cases, a
well-expressed Claude Bernard - Corner symptom of brain stem origin was observed.
Among other nerves, the sublingual Tingual7 and losso.-p
affected, As far as eye fu%dua was concerned, there werenoparti nerves were
symptoms, particular patho-
logical except for a slight hyperemia
Motor disturbances were expressed chiefly in changes of muscular tonus;
there was often bending contracture of the upper extremities and unbending con-
tracture of the lower extremities with a pronounced plants function of the foot
(posture of decerebral rigidity),
In other cases, both upper and lower extremities were in a flexed state.
Increased tonicity predominated in proximal regions, and this condition was in
all cases of the mixed pyramidal-extrapyramidal type. In three cases, the
"midwife's hands" position was observed, In three cases, there was change of
the tonne and position of the upper extremities, depending on the angle through
which the head was turned, a phenomenon belonging to a type which indicates a
midbrain mechanism, There were no pareses or paralyses. In some cameo, hyper-
kinesis, athetoic position of the hands, and trembling of the extremities and
tongue were observed, The tendon reflexes were increased and there was usually
anisoreflexion, in the majority of cases the knee reflexes were heightened,
wtule the Achilles tendon reflexes were absent. Abdominal reflexes were absent
in practically all cases. Plantar reflexes were frequently increased and ac-
companied by a heightened tonic flexion of the toed. In other words, there
was frequent divergence between the intensities of abdominal and plantar
reflexes. Often there were pathological reflexes of the flexion--releasing
type (Babinaki's zeflex, Oppenheim?s reflex), In the majority of cases,
pronounced defense reflexes, pain reflexes, and the Marie-Prix--Bekhterev
symptom were present, in all cases, symptoms of oral automatism (of the
snout type, nasolabial, or suction type) were observed, In 4.4 instances,
there was a grasping reflex,
Disturbances of sensibility could not be investige.ted because of the
serious con'ition of the patient,. The speech was slow, monotonous, and hollow,
In the vegetative system, the following conditions were present: regional
hyperemia,, acrocyanosis, persistent red dermographism, increased pilomotor
reflex, and a well-expressed Aechner phenomenon,
The spinal fluid was colorless and transparent in all cases. It flowed out
under raised pressure. The quantity of proteins was somewhat increased (0,66%);
the globulin reactions were 1 'sitive, as a rule, in some cases, there was a slight
cytosis (20-10 lymphocytes),
In cases of Group 2, pronounced changes of the psyche occurred. These
changes 'ere particularly noticeable during the period of ~nvnl.,r.nc., while
during the acute period they were masked by disturbances of consciousness, These
changes had the characteristics of a frontal syndrome. The pathological state of
motor functions in th's group was particularly pronounced, with especially strong
impairment of the extrapyramidal system. This was expressed in hypokinesis, absence
of synergetic movements of the arms in walking, cataplectic freezing in imparted
positions, athetoic tendencies, trembling of fingers, and myoclonic twitchings of
the face musculature. In this group, disturbances of motor coordination of the
cerebellum type were especially noticeable. The neurological symptoms were domi-
nated by these phenomena to such an extent that one might have assumed an acute
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Leydan-Westphal ataxia, As far as sensibility is concerned, there was general
hyperesthesia.
The patients of Group 3, just as those of Group 2, initially complained
about a headache and had symptoms of a meningeal condition. However, the disease
took a less severe course. As for neurological symptoms, there were disturbances
of motor coordination that were expr-esed chiefly in atactic walking and insta-
bility in Romberg`a posture, Furthermore, strengthening of radiculoneuritic
symptoms was observed during the period of convalescence.
Group L was distinguished by a light course of the disease, absence of
meningeal symptoms, and weak neurological symptoms indicating affliction of the
pyramidal tract and of the radiculoneuritic section of the nervous system.
Data obtained by a catamnestic examination, extending over one year, of
patients who had the disease in 1947 disclosed in some cases the presence of
diffuse symptoms of an affliction of the nervous system, constant headaches,
pronounced vegetative emotional instability, light pareeea of nerves of the
large brain, strengthening or weakening of tendon reflexes, and pathological
reflexes.
Pathologo-anatomical investigations disclosed a pronounced hyperemia of
brain membranes and the brain substance, in some cases subararhnoidal hemorrhages,
extended hemorrhages into the gastroWictestinal tract fsubmuooua hematomata of
the stomach), hemorrhages into the lung tissue and the spleen capsule, and de-
generative changes of internal organs.
Microscopic examination showed in all cases oedema of the pia mater and
loosening of the connective fibers. There is an acute state of excessive filling
of vessels of this membrane with blood, and, in some cases, hemorrhages into
membranes and pronounced infiltration are observed. Blood vessels in the large
brain and the brain stem are extended and filled to excess with blood. They
also contain stases. Around capillaries and precapillaries there are diapedetic
hemorrhages. in addition to hemorrhages, plasmorrhages (accumulations of oe-
dematous liquid) are observed.. The walls of blood vessels are oedematous, with
swollen endothelium. Occasionally, there is proliferation of blood vessel endo-
thelium with subsequent desquamation.. In the intraadventitial spaces of blood
vessels, particularly those of the brain stem. there is light lymphccytic in-
filtration, Diffuse proliferation of micro.- and oligo--dendroglia is noticeable,
and the glia around vessels frequently contracts, forming loosened knots. In
astrocytes, there are degenerative changes of irregular shape, more pronounced
are degenerative changes of microgliu cells.. Nerve cells are comparatively
unaffected, However, in almost all cases we were able to detect either an
acute swelling of these cells or ischemic, sometimes perivascular, impairment
of them. A diffuse pathological process affects the whole brain. However, the
strongest changes are noticeable in the middle brain,,. the pone varioli, and after
this in the putamen, visual bulge, and, finally, in the brain cortex. In other
words, Bukovina hemorrhagic fever is essentially a diffuse hemorrhagic capil-
larotoxicosis involving elements of an exudative proliferative process.
On summarizing our data, we see that the clinical aspects of the affliction
of the nervous system in Bukovina hemorrhagic fever are characterized by the
following conditions? disturbances of consciousness; meningeal symptoms; af-
fection of the brain nerves of the oculomotor and bulbar groups; motor dis-
turbances, particularly disturbances of muscle tonus of the pyramidal as well
as extrapyramidal type, hyperkinesea, disturbed reflexes, disturbed coordination
of the cerebellum type, oral automatism reflexes, tonic reflexes affecting the
neck, grasping reflex^ vegetative pathology. This clinical multiplicity of
symptoms indicating affection of the cortex, subcortical ganglia, and the brain
axis is satisfactorily explained by pathological data, which disclose a diffuse
process.
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One must emphasize the shallow, unstable, c-id reversible character of the
clinical symptoms, as well as the absence of symptoms indicating spinal pathology.
All these data permit one to distinguish between cases of hemorrhagic fever
and those of tick encephalitis, which also occur in the wooded regions of Buko-
vino. The clinical picture of Bukovina tick encephalitis is extremely multi-
farious: stem myelitic, myeloradiculoneuritic forms, and forms with predominant
affection of the radiculoneuritic section of the nervous system were observed.
However, in all cases of Bukovina t+-k Ancephalitis, there were clear symptoms
of affection of the gray as well as white matter of both the brain and the spine,
which was clinically expressed in pareses and trophies in the cervico-clavlcat
region. No such indications were present in our clinical material, However,
upon comparing the clinical picture of affection of the nervous system in Buko-
vina hemorrhagic fever with that in Crimean hemorrhagic fever, we must stress
the much greater intensity and stability of neurological symptoms in Bukovina
hemorrhagic fever. This may be connected with a more pronounced neurotropic
quality of the v:Lius of the disease under discussion.
According to Shutova's data, the neurological symptoms ic. Crimean hemor-
rhagic fever basically indicate an affection of the vegetative nervous system;
symptoms of affection of the somatic nervous system are indistinctly expresseu
and bear a transient character (there are light disturbances of consciousness,
weakly expressed meningeal symptoms, unstable pyramidal and extrapyramidal die-
f++rhancr-~ In our acute cases, we observed a syndrome of diffuse meningo
encephalitis with pronounced symptoms of the decerebral rigidity and tonic neck
.2flex type, 1. e., symptoms connected with elimination of the functions of the
brain cortex, of pyramidal as well as extrapyramidal tracts. The neurological
syndrome in our acute cases is almost identical with that of Japanese mosquito
encephalitis: only virusological investigations permit a differentiation of
these two diseases. Furthermore, in our rases, relatively stable residual
symptoms of disturbed psyche, as well as pathological symptoms of the extrapyra-
midal and cerebellum type, were present. All this is not observed in Crimean
hemorrhagic fever,
The typical traits outlined above lead to the --onclueion that the disease
which was observed in Bukovina bears a distinct and independent character,
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