The use of botulinum toxin type A (BTA) is the most effective treatment for cranial muscular dystonia and facial hemispasm (PH). For the treatment of this pathology, the drug BTA dysport is most often used.
The clinical efficacy of the drug is quite high. However, there are cases of treatment of blepharospasm (BS) with dysport, when the effect of its administration is completely absent. In such cases, it is very important to take into account the condition of the skin, because changes in them can become a mechanical obstacle to the normal distribution of the drug and to the realization of its therapeutic effect. Here is a description of this clinical observation.
Patient Kh., born in 1934, has been ill since February 2001. For the first time, for no apparent reason, he noted involuntary closing of both eyes while reading, watching TV. Gradually, these symptoms were joined by a feeling of pain in the eyes, accompanied by hyperemia of the conjunctiva. Since April, he was treated on an outpatient basis for conjunctivitis without effect. In June, to clarify the diagnosis, he was sent to the regional clinical hospital. Performed computed tomography of the brain: diffuse-atrophic process.
The diagnosis was established: dyscirculatory encephalopathy II st. with BS syndrome. In the hospital, the patient received vasoactive, nootropic drugs, underwent a course of massage and acupuncture.
The symptoms gradually progressed: the severity and duration of episodes of involuntary squinting and rapid blinking increased. In December 2002, the patient was undergoing inpatient examination in the neurological department of the City Clinical Hospital.
Objectively in neurological status: reduced memory for current events. From the side of the cranial nerves: the pupils are equal in size, their reaction to light is alive. There are no oculomotor disorders, ptosis. During the examination, episodes of rapid blinking, squinting of both eyes are noted. The left nasolabial fold was smoothed out. Tongue in the midline. Positive reflexes of oral automatism. The strength and tone of the muscles of the limbs were not changed. Tendon-periosteal reflexes from the arms of medium liveliness, equal in size, equal in size from the legs, knees of medium liveliness, Achilles' reflexes are reduced. No sensory disturbances were found. In the Romberg position, he is stable, performs coordinating tests satisfactorily, slight tremor of the fingers of outstretched hands.
The diagnosis was established: focal muscular dystonia in the form of BS, 2nd tbsp. gravity. Concomitant diagnosis: arterial hypertension of II degree, risk of — 3; coronary heart disease: postinfarction (1999) and atherosclerotic cardiosclerosis, atherosclerosis of the aorta, coronary arteries; scleroderma, local form.
Treatment: 250 units of Dysport were injected subcutaneously in the region of the orbicular muscles of the eyes (CMG), there were no complications. Subsequently, during control examinations after 2 and 4 weeks, there were no objective changes in the status, subjectively: a state without dynamics. No adverse drug reactions were noted. 1 mg of clonazepam 2 r/day was prescribed.
Analyzing the possible reason for the lack of at least a minimal response to the introduction of dysport, we paid attention to the density and appearance of the skin of the patient's face, including the periorbital region — injection zone. The patient was consulted at the Department of Skin and Venereal Diseases, diagnosed with scleroderma, local form.
6 months after the first injection, i.e. in June 2003, re-introduced 250 IU of dysport, without effect. The last attempt to administer the drug at a standard dose of 250 IU to the affected area of the face was made in December 2004, but it was also unsuccessful.
DISCUSSION
In the form of a drug, botulinum toxin is a mixture of various components, the main ones being neurotoxin and non-toxic proteins. To stabilize neurotoxin molecules that are unstable to the action of mechanical, physical and chemical factors, the drug contains large peptide molecules of hemagglutinins and non-toxic non-hemagglutinin proteins. This prevents the breakdown of the toxin and its rapid diffusion into the surrounding tissues, providing localized exposure.
The total initial dose of Dysport in BS is 120 IU per eye and is injected subcutaneously at 4 points above the CMG. Improvement is noted on the 1-4th day from the moment of injection and persists for 9-10 weeks (according to some reports, with BS — up to 18 weeks, with LH — up to 22). The effectiveness of botulinum therapy ranges from 70 to 94% of cases with BS and approximately 75% — in PH
The main point of action of the drug in BS (CMH) is located under the skin covering the orbit, upper and lower eyelids in the form of a thin plate. A small amount of muscle tissue causes subcutaneous injection of botulinum toxin. The anatomical features of the mimic muscles of the face largely determine the tactics of drug administration, as well as the causes of possible adverse reactions.
Individual differences in mimic muscles — a variety of options for starting from bone formations and attachments to the skin, often occurring shapes and sizes, sometimes the fusion of adjacent muscles or the division of one muscle into separate bundles — possibly, partly determine the ineffectiveness of treatment or a different effect with the same indications and conditions for the administration of the BTA drug.
The inefficiency of BTA can also be associated with insufficient dose, wrong choice of muscle, resistance to the drug. The main reason for the loss of sensitivity to BTA is the formation of neutralizing antibodies, especially in patients receiving high doses of the toxin in relatively short periods of time. It is believed that after 1-2 years, circulating antibodies leave the blood and sensitivity to BTA is restored.
After analyzing our clinical observation, we came to the conclusion that the most likely cause of the patient's resistance to botulinum therapy was a change in the tissues of the paraorbital region due to scleroderma. Morphologically, this disease is characterized by predominantly progressive sclerosis of the connective tissue of the skin and vascular walls. There are a systemic form of scleroderma (with damage to the skin, musculoskeletal system, internal organs, in particular, the gastrointestinal tract, lungs, kidneys, heart, Raynaud's syndrome) and limited — with skin lesions only. The limited form includes plaque, ribbon-like, Pasini's atrophoderma — Pierini, white spot disease.
The disease is caused by a chronic autoimmune process, which is based on dystrophic changes in collagen and elastic fibers, mucoid and fibrinoid degeneration of connective tissue. Scleroderma skin lesions go through three stages: dense edema, induration and atrophy. Edema persists, depending on the nature of the course of the process, for several months or years and passes into an indurative phase. The skin becomes dense, is not taken into a fold, its diffuse or focal hyperpigmentation appears. Subsequently, the face acquires a characteristic appearance: a mask-like appearance with dense stretched skin, purse-string wrinkles around the mouth, thinning of the lips, nose, sclerotic eyelids ("face of the icon"). The presence of telangiectasias is characteristic. With a long course of the disease, the stage of induration can pass into the stage of atrophy,
Based on materials from mif-ua.com
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