FUNICULAR MYELOSIS (Latin funiculus cord + Greek myelos marrow; a synonym the subacute combined myelosclerosis) — the disease of a spinal cord which is usually combined with pernicious anemia and shown paresthesias, a sensitive ataxy and the central paresis of legs.
For the first time F. the m is described in 1887 by L. Lichtheim.
Etiology and pathogeny. Reason of development F. the m is deficit in an organism of B12 vitamin (see Cyanocobalamine), deficit of B12 vitamin and folic acid (see) or only folic to - you is more rare. The pathogeny is completely not found out. T. the m is almost constantly combined with pernicious anemia (see), however the role it as proximate cause of defeat of a nervous system is disproved by existence of typical cases F. m, not followed by pernicious anemia and changes of red marrow. There is no correlation between severity of anemia also and with a frequency of development F. m. At the same time deficit of B12 vitamin is decisive a link both in a pathogeny of pernicious anemia, and in a pathogeny F. m. B12 vitamin is utilized by an organism by means of an internal factor (Castle's intrinsic) developed by a stomach (see Kasl factors). Lack of an internal factor leads to disturbance of absorption of B12 vitamin in intestines therefore there is no transformation folic to - you in its active form — folinovy acid. At the same time process a shelter of creation changes (see), synthesis of DNA in erythroblasts is broken. T. the m develops in cases of a lack of B12 vitamin of food (e.g., at the vegetarians excluding dairy products from food); at disturbance of secretion of an internal factor — at an akhiliya of a stomach (see), after a gastrectomy (see), at a syndrome malyabsorbtsip (see Maljabsorbtion a syndrome), caused to a spr (see), a terminal ileitis (see Krone a disease), a Gee's disease (see), tubercular enteritis (see), a pellagra (see); in cases of a so-called competitive consumption of B12 vitamin when the last is absorbed by an excess indestinal flora — at a divertuculosis of intestines (see), a syndrome of «a blind loop» at enteroenteroanastomoza (see) — or helminths (see Diphyllobotriases). A part in a pathogeny is played by immune disturbances: more than diagnose autoimmune atrophic gastritis for a half of patients with pernicious anemia, and in a blood plasma find antibodies to an internal factor (see Autoantibodies).
In experiments on monkeys it was shown that the exception of food of B12 vitamin in 3 — 4 causes the changes in a nervous system identical F. m though anemia at the same time does not develop.
Pathological anatomy. Macroscopic changes consist in a moderate atrophy of a spinal cord (see). Microscopic examination finds the centers of a necrosis and a degeneration in gentle and wedge-shaped bunches of back cords, in pyramidal and partially back and cerebellar conductors of side cords. On the cuts painted on a myelin the demyelination of afferent and efferent conductors which is most expressed in back and side cords of a spinal cord (a posterolateral sclerosis) comes to light. Changes arise in nizhnesheyny and verkhnegrudny departments in the beginning, then extend on a spinal cord up and down (the ascending and descending degeneration). Along with disintegration of a myelin death of axial cylinders with formation of emptiness (vacuoles) divided by thin glial tyazha is observed. The progressing destruction of white matter of a spinal cord is not compensated by growth of a glia since glial reaction at F. m it is insignificant. In late stages of a disease on cross sections of a spinal cord only the narrow strip of not struck white matter around not changed gray matter is visible. Similar morfol. changes, but considerably less expressed find sometimes in white matter of cerebral hemispheres (see). Also the thick myelinized sensitive fibers in distal departments of peripheral nerves are surprised. In most cases the changes characteristic of pernicious anemia come to light: a glossitis, a mucosal atrophy of a stomach, a hyperplasia of red marrow, increase selezenkig inclusions of iron in cells of system of mononuclear phagocytes.
Clinical picture. T. the m occurs equally often at men of t of women. Approximately in 90% of observations the first symptoms of a disease appear aged 40 years are more senior., however can be observed in children's: and senile age. There are paresthesias (see) in the form of feeling of numbness, heat, a pricking, half of zanya goosebumps, arising usually in toes, is more rare in fingers, hands, to-rye gradually extend up to proximal departments of extremities, and then to a stomach and a breast. The aching onychalgias are sometimes noted. Gradually the progressing disturbances of muscular and joint feeling join (see Proprioceptors) and vibration sensitivity (see), also more expressed in nogakhg the sensitive ataxy develops, (see). Sometimes there steps decrease in superficial sensitivity in extremities with distribution on a trunk, usually without the clear upper bound. Morbidity at a prelum of gastrocnemius muscles is quite often noted. Along with disturbances of sensitivity also motive frustration appear, to-rye are shown by weakness of legs less often than hands. At moderately expressed damage of a spinal cord paresis has' spastic character, at more rough damage of back cords there is a hypomyotonia and paresis becomes sluggish (see Paralyses, paresis). Incremental weight of defeat of back and side cords of a spinal cord defines the nature of change of tendon jerks (see): in initial stages F. m tendon jerks are increased, but more than at a half of patients decrease, and then and loss of Achilles reflexes is noted; later also knee jerks disappear. In the developed stage of a disease it is constant are found-foot patol. reflexes (see Reflexes pathological) — Babinsky's symptom (see Babansky a reflex), etc. The combination of pyramidal symptoms to sluggish paresis is feature a wedge, pictures F. m. Also dysfunctions of pelvic bodies are noted (impotence, imperative desires to an urination, a delay or an incontience of urine and a calla). Typiforms F. m are characterized by a combination of the sensitive ataxy and a paraplegia (see) expressed preferential in yogas (an atactic paraplegia). However nevrol. disturbances can be and dissociated: are possible isolated a sensitive ataxy either spastic couple - or a tetra-lot. Quite often F. the m is combined with an iolnnevropatiya (see the Neuropathy) when peripheral nerves also are involved in process along with a spinal cord. Approximately in 5% of cases F. the m is observed decrease in sight because of development of an atrophy of optic nerves (see. Optic nerve) and emergence of the central scotomas (see Scotoma), occasionally there is a miosis (see) at the kept pupillary tests. In some cases drowsiness develops. apathy, there are disturbances of mentality which are shown a depression, emotional lability, sometimes acute psychotic reactions. In cerebrospinal liquid (see) small increase in protein content is sometimes noted. On the electroencephalogram (see Elektroentsefalografiya) the diffusion slow activity disappearing against the background of reception of B12 vitamin can come to light.
Current F. m various. Along with usual gradual development of a disease acute development nevrol is in some cases noted. disturbances, at Krom during
2 — 3 weeks there is a picture of cross damage of a spinal cord with the lower paraplegia, loss of all types of sensitivity, pelvic frustration.
Now F. the m is characterized polymorphic a wedge, honor with a picture with an atypical current in 50% of observations. Due to the use of B12 vitamin the classical developed forms F. m develop seldom.
The diagnosis at typical a wedge, a picture F. the m, developed against the background of pernicious anemia, comes easy. It is necessary to differentiate with a B12-scarce polyneuropathy, it is noted edges approximately at 40% of patients with pernicious anemia and also it is characterized by distal paresthesias, a sensitive ataxy and areflexia. The differential diagnostic character testifying in favor of F. the m, is defeat of pyramidal system, and first of all existence patol. Babinski's reflex or others-foot patol. reflexes.
The main difficulties of differential diagnosis arise when nevrol. symptoms F. m develop before emergence of symptoms of pernicious anemia. Diagnosis F. the m is confirmed at detection of akhilichesky gastritis and mega a forehead of last in the material received at a sternal puncture.
T. the m proceeding with domination a wedge, symptoms of defeat of back cords reminds back tabes (see), however at F. the m
is absent Argayll Robertson's syndrome (see Argayll Robertson a syndrome) and serological tests for syphilis (see) in blood and cerebrospinal liquid are negative. Wedge, form F. the m with preferential defeat of side cords can remind multiple sclerosis (see). For multiple sclerosis unlike F. m much is characteristic ochagovost defeats (including and symptoms of damage of a brain), increase in most cases in Achilles reflexes, and also, as a rule, a remittiruyushchy current. The combined defeat of back and side cords is observed at a compression of a spinal cord by a tumor or at a vertebrogenny cervical myelipathy (see). Diagnosis of a compression of a spinal cord is based on detection by means of liquorodynamic tests (see) blockade of subarachnoid space and identification of a characteristic picture at a miyelografiya, a spinal radio isotope stsintigra-fiya, a computer tomography. In many cases of a compression of a spinal cord of a different origin unlike F. the m is defined the clear upper bound of disorders of sensitivity. The assumption about F. the m quite often arises at inspection of the patients having a so-called paraplegia of middle or advanced age, at a cut develops progressing for many years spastic couple - or tetraparesis at normal composition of cerebrospinal liquid and negative data of a miye-lografichesky research. Diagnosis F. m in these cases put on the basis of detection of deficit of B12 vitamin. Important criterion in diagnosis F. the m serves symmetry arising nevrol. defects. The expressed asymmetry of paresis or disorders of sensitivity, it is a little long existing, testifies against F. m.
Treatment is directed to elimination of deficit in an organism
Treatment begin with intramuscular introduction 500 — 1000 mkg of a vitakhmin of B12 (cyanocobalamine) daily or in 1 — 2 day. After
5 injections of drug in this dose B12 vitamin is entered on 100 mkg within half a year once a week. The maintenance therapy in the next years consists in injections of 100 mkg of B12 vitamin once a month. In case of developing of an infectious disease or a renal failure the dose of B12 vitamin shall be increased. At an opportunity elimination of causes of infringement of absorption of B12 vitamin is necessary in went. - kish. path. The diet with high content of vitamins of group B, reasonablly parenteral administration of vitamins of Vkh and B6, and also fosfaden on 60 mg twice a day for 4 — 8 weeks is shown (recycles). Appointment folic to - you in a dose of 5 — 15 mg a day is admissible only at seldom found folic and scarce form F. m. Otherwise the aggravation nevrol is possible. process. Appoint massage, LFK. At substantial increase of a tone of muscles in the affected extremities reception of Seduxenum, Baclofenum, Mydocalmum is shown. At a paraplegia are necessary careful care of skin for the prevention of development of decubituses and prevention of an urigenous infection.
The forecast is defined by hl. obr. duration of existence nevrol. symptoms prior to treatment; the age of patients and weight of anemia do not play an essential predictive role. At an initiation of treatment in several weeks after emergence of symptoms of spinal defeat it is possible to expect recovery of patients; if started treatment in several months — considerable improvement. In late stages F. m only minor improvement or stabilization of process is possible. The main regress of symptoms is noted to the first 3 — 6 months of treatment, further recovery of the broken functions goes more slowly.
Prevention assumes early detection and adequate treatment of pernicious anemia, and also careful overseeing nevrol. the status of such patients for the purpose of perhaps earlier detection of symptoms of damage of a spinal cord at them.
Bibliography: Alimov I. Yu. and Mand-richenko V. M. A funicular myelosis at the Addisonovy disease, Zdravookhr. Tajikistan, No. 1, page 90, 1983; D and in a reptile of N to about in S. N. Clinical lectures on nervous diseases, century 4, page 172, JI., 1961; Martynov Yu. S., Malko-va E. V. and Chekneva N. S. Changes of a nervous system at diseases of internals, M., 1980; The Multivolume guide to neurology, under the editorship of
S. N. Davidenkov, t. 3, book 2, page 902, M., 1962, bibliogr.; The guide to hematology, under the editorship of. And. II. Vorobyova and Yu. I. Loriye, page 376, M., 1979;
Evans D. L., E del sohn G. And. Golden R. N. Organic psychosis without anemia or spinal cord symptoms in patients with vitamin B12 deficiency, Amer. J. Psychiat., v. 140, p. 218, 1983; Folic acid in neurology, psychiatry, and internal medicine, ed. by M. I. Botez a. E. H. Reynolds, p. 427, N. Y., 1979; Reynolds E. H. Pathogenesis of subacute combined degeneration, Lancet, v. 2, p. 1109, 1981; Shorvon S. D. a. o. The neuropsychiatry of megaloblastic anemia, Brit. med. J., v. 281, p. 1036, 1980., D.R. Shtulman.