GENERAL PARALYSIS (paralysis progressiva, synonym: general paralysis of mads, paralytic weak-mindedness, Beyl's disease) — the late form of neurosyphilis which is characterized by disturbance of mental activity — the progressing total weak-mindedness.
One of the first cases of undoubted Pct were described by Zh. Eskirol. In 1822 A. Beyl allocated Pct as an independent disease, having emphasized that at it arise as mental disturbances, and nevrol, the frustration connected with hron. inflammation of an arachnoid membrane of a brain. Beyl's statement met objections from some researchers. And only in the 50th 19 century the general paralysis was recognized as an independent disease.
The pct suffers apprx. 5% of all patients syphilis (see), and women are ill considerably less than men. The disease usually arises 10 — 15 and more years later after infection. Thus, if to consider that the period of the greatest sexual activity falls for 20 — 35 years, age of 35 — 50 years which ached most often. At infection with syphilis at late age (45 — 60 years) the specified interval usually is shortened.
A clinical picture
Pct — a serious illness of all organism, and its brightest manifestations — disturbances of mental activity. The main syndrome is progreduated total weak-mindedness (see): roughly the intelligence suffers, disorders of judgments are early designated, the criticism and especially self-criticism disappears. There is no consciousness of a disease, memory sharply decreases, there are confabulations (see. Konfabulez ). Displays of weak-mindedness amplify in connection with often observed euphoria (see. Psychoorganic syndrome ). Nevrol. the symptomatology consists in alalias, first of all in disturbance of an articulation — dysarthtias (see). The speech becomes not clear, greased, especially at a pronunciation of long words, the patient passes or rearranges syllables, does not say the terminations of words. Handwriting becomes uneven, separate letters and syllables drop out of words. The timbre of a voice changes, he becomes a deaf. The face of the patient is inexpressive, maskoobrazno, since the innervation of face muscles is broken, there is a blepharoptosis (see. Ptosis ). Tendon jerks are often raised and uneven, except for cases taboparalysis (see). Due to the frustration of sphincters patients do not hold urine and kcal. As a result of vasculomotor and trophic disturbances hypostases, fragility of bones, an atrophy of muscles, decubituses develop.
On a basis psikhopatol. manifestations allocate four stages of a disease: latent (from infection with syphilis before manifestations of Pct), a stage of initial manifestations, a stage of a full course of a disease and a stage marasmus (see). In a latent stage headaches, dizzinesses, unconscious states can be noted, in some cases characteristic changes are observed in cerebrospinal liquid (see). The stage of initial manifestations is characterized by increased fatigue, irritability, faintheartedness. Patients complain of breakdown and decrease in working capacity though they still can perform usual work to some extent. Earlier such states because of external looking alike neurotic symptomatology were incorrectly called a preparalytic neurasthenia. In certain cases in a stage of initial manifestations depressive and crazy frustration — an alarming depression with hypochiondrial statements, the alarming agitated depression, the crazy ideas of jealousy, prosecution, poisoning are observed; in process of increase of symptoms of weak-mindedness these endoformny frustration disappear. Very much early easing of memory is found. Separate acts demonstrate disturbance of criticism. The sphere of inclinations falls apart, patients become gluttonous, erotic. Increase of these frustration speaks about transition of a disease to a stage of full development, edge the wedge is shown in various. forms. Expansive, or classical, the form (earlier eurysynusic) meets at men more often. It is characterized by existence of maniacal excitement with manifestation of irascibility, grandiose nonsense of greatness (see. Nonsense ). The dementive form differs in the accruing weak-mindedness against the background of inactive euphoria. At a depressive form the suppressed mood develops, it is frequent with alarm and aspiration to suicide (see. Depressive syndromes ), the ridiculous hypochiondrial nonsense of nigilistic contents is often observed. The circular form for the first time described by S. S. Korsakov proceeds with change of conditions of excitement and a depression. The hallucinatory paranoid form differs in development paranoid syndrome (see) with preferential auditory hallucinations and a persecution complex. At a catatonic form arises struporous state (see) with the phenomena of a mutism and negativism (see. Catatonic syndrome ). In a stage of marasmus conscious activity stops, the speech disappears, patients make inarticulate sounds, cannot stand and move. In this stage they perish from an intercurrent disease.
On a current allocate especially malignant agitated form (the galloping paralysis) with sharp motive arousing and disturbance of consciousness on amental type and so-called stationary paralysis, at Krom drift with gradual decrease in intelligence and slackness is noted.
Atypical forms of Pct are the youthful and senile Pct, and also Lissauer's paralysis and taboparalysis (see). The youthful Pct develops on the basis of inborn syphilis; usually begins at the age of 10 — 15 years. Sometimes it is preceded by symptoms of inborn syphilis, in other cases it arises at children who were considered so healthy. Most often proceeds in a dementive form; local symptoms, napr, an atrophy of optic nerves are quite often observed. The senile Pct arises aged 60 years are more senior and it is characterized, first of all, by a long latent stage (up to 40 years). Klien, a picture reminds senile dementia (see) with the expressed dysmnesias; sometimes the disease proceeds as a korsakovsky syndrome (see).
Lissauer's paralysis and a taboparalysis are characterized by rather slow increase of weak-mindedness. At Lissauer's paralysis the tendency to local damage of a brain, preferential parietal shares is observed, at the same time develop aphasia (see), agnosia (see), apraxia (see), apoplektiformny and epileptiform attacks. The taboparalysis represents a combination of symptoms of Pct and back tabes (see), and spinal frustration precede development of symptoms of Pct, usually its dementive form.
An etiology and a pathogeny
In 1913 X. Noguchi proved a syphilitic etiology of Pct, having found a pale treponema in tissues of a brain.
The pathogeny is studied insufficiently. K. Levaditi on the basis of observations when Pct got sick both spouses or several persons who caught syphilis from the same source made the assumption of existence of a special neurotropic treponema. what, however, is not proved. Perhaps, in a pathogeny disturbances of reactivity of an organism play a role (see) with a sensitization of tissue of brain therefore in some cases treponemas get into tissue of a brain.
The pathological anatomy
Macroscopically at Pct is noted an atrophy of crinkles of a brain and expansion of furrows. The soft cover of a brain is dimmed, spliced with substance of a brain; it is observed ependimatit (see. Horioependimatit ). Microscopically inflammatory process in a soft cover of a brain and in vessels, especially small comes to light. About vessels infiltration by plasmocytes is characteristic. In tissue of a brain desolation of bark in connection with destruction of nervous cells and fibers is observed. In a neuroglia gliozny fibers expand, rhabdoid and amebiform cells appear. About nervous cells find pale treponemas.
Diagnosis in a stage of a full course of a disease usually does not cause difficulties. It is established on the basis of psychopathological, neurologic manifestations and data a lab. researches. Increase of the phenomena of total weak-mindedness with lack of criticism, a dysarthtia, disturbances of a pupillary test (see. Argayll Robertson syndrome ), constancy serol. indicators — all this in total does the diagnosis of Pct reliable. Wassermann reaction in blood in most cases positive (see. Wasserman reaction ); in cerebrospinal liquid it, as well as proteinaceous reactions (Nonna — Apelta, Pandi, Veykhbrodta), is sharply positive (see. Coagulative tests , Cerebrospinal liquid). The quantity of cellular elements in cerebrospinal liquid is increased, sometimes considerably. The general protein content is increased. At statement of reaction of Lange (see. Cerebrospinal liquid ) decolouration of liquid in the first 4 — 6 test tubes and increase in intensity of coloring in the following (a so-called paralytic curve) is noted. Reaction of an immobilization of treponemas (see. Nelsona-Meyer reaction ) and reaction of an immunofluorescence (see) are sharply positive in blood and in cerebrospinal liquid.
Differential diagnosis carry out with syphilis of a brain (see. Syphilis ), at Krom weak-mindedness has lacunary character with bigger or smaller safety of criticism, hallucinations are more often observed; the curve of reaction of Lange has a so-called syphilitic tooth. Pct differentiate with an alcoholic pseudoparalysis (see. Alcoholic encephalopathies) and senile dementia (see) on the basis serol. indicators; with brain injuries (see), especially frontal lobe, on nevrol, symptomatology and serol. to indicators. The pseudoparalytic syndrome at tumors of a brain is followed by the increased intracranial pressure (see. Hypertensive syndrome ). Psychoses of vascular genesis differ from Pct in development of lacunary weak-mindedness (see. Atherosclerosis ), not inherent Pct. Existence of paralytic weak-mindedness allows to delimit a circular form of Pct from maniac-depressive psychosis (see), and a hallucinatory paranoid form — from schizophrenia (see).
It is shown pyrotherapy (see) — inf. therapy and the pyrogenic substances (see) in a combination, as a rule, to an antibioticotherapia. In 1845 F. Sabler noted beneficial effect of a number of feverish diseases on the course of psychoses. A priority in inf. therapies of psychoses belongs to A.S. Rozenblyum who treated mentally sick with an inoculation of a typhinia. In 1917 the Vienna psychiatrist Yu. Wagner-Yauregg offered treatment of sick Pct inoculations of malaria. The method was widely adopted; it consists in introduction subcutaneously to sick Pct of the blood taken from the patient with a tetrian fever. The incubation interval proceeds 4 — 20 days, the first attacks proceed as a tetrian fever, further are, as a rule, observed daily. After 10 — 12 attacks they are stopped reception of quinine of a hydrochloride. Inf. therapy is carried out also, infecting sick Pct with the European and African typhinia. Results of this therapy are less expressed, but it is convenient since the inoculative material which is previously received as a result of infection of a mouse can be transported on long distances. Somatic the weakened patient impart the Japanese rat typhus — a sodoka. When infection does not work well, and also at somatic contraindications, appoint the pyrogenic substances (pyrogenal, etc.).
From 40th 20 century carry out complex treatment — a pyrotherapy in combination with introduction of antibiotics. At the same time appoint 40 000 000 PIECES of penicillin to a course of treatment; repeated courses of a penicillin therapy are conducted with intervals to 2 months iod control serol. indicators. In some cases apply Bicillinum in combination with drugs of bismuth. In a combination with an antibioticotherapia recommend inf. therapy (inoculation of malaria), edges promotes penetration of antibiotics into tissue of a brain, but also — to increase in protective forces of an organism. Inf. therapy is contraindicated at senile age, at marasmus, heart failure, an aortic aneurysm, diseases of kidneys, a diabetes mellitus. Besides, treatment is developed by one penicillin. Several schemes of treatment by penicillin are offered, but some researchers consider use of one penicillin insufficient.
Duration of a current of uncured Pct from its initial manifestations till a lethal outcome on average apprx. 2U2 years. The youthful Pct flows more slowly (5 — 6 years), stationary paralysis — up to 20 and more years. The agitated form comes to an end with the death of the patient in several months. At an expansive form long remissions are observed. Remissions after treatment have various duration. Remissions of St. 20 years are known. According to a number of researchers, remissions are more than 2 years steady.
Prevention consists in timely treatment of syphilis, a cut carry out by specific and nonspecific means according to certain schemes (see. Syphilis ).
Social and sudebnopsikhiatrichesky value
Only in isolated cases (permanent remission after treatment) it is possible to allow the patient to return to former professional activity. At forensic-psychiatric examination (see) the patient almost in all cases establish diminished responsibility (see). Only at treated Pct with steady remission lasting not less than 3 years of the patient it can be recognized as responsible.
Bibliography: Venereal diseases, under the editorship of O. K. Shaposhnikov, page 174, M., 1980; Gordova T. N. Clinic and current of a general paralysis, treated malaria, M., 1959, bibliogr.; Gurevich M. O. Pathoanatomical features of a general paralysis in connection with clinical data and treatment by malaria, in book: Psikhiat. hospital on the ways of reconstruction, under the editorship of I. N. Koganovich, page 179, M. — L., 1934; Miniovich P. A. Malarial therapy of a nevrolyues and other diseases of a nervous system, Rostov N / D., 1934; R about-zenblyum A. S. About the relation of feverish diseases to psychoses, Works of doctors Odessk. mountains. hospitals, century 2, page 73, Odessa, 1876; Bruetsch W. L. Neu-rosyphilitic conditions, Amer. handb. psy-chiat., ed. by S. Arieti, v. 2, p. 1003, N. Y., 1959, bibliogr.; Gerstmann J. Die Malariabehandlung der progressiven Paralyse, Wien, 1928; Handbuch Geisteskrankheiten, hrsg. v. O. Bumke, Bd 8, T. 4, S. 147, 315, B., 1930, Bibliogr.; W a g-ner-Jauregg J. Fieber-und Infektionstherapie, Wien u. a., 1936; Weitbrecht H. J. Psychiatrie im Grundriss, S. 216, B. — N. Y., 1973.