Neyromiotoniya

Description:

Neyromiotoniya (syndrome of "battleship") — the syndrome which is shown the constant tension, spasms or the slowed-down relaxation of muscles after reduction which are caused by the constant categories arising in distal departments of motive nerve fibrils.

Neyromiotoniya's symptoms:

The idiopathic neyromiotoniya is most often shown on 2 — the 3rd decades of life by gradually increasing muscular tension (rigidity), wavy reductions of muscle bundles (miokimiya), fastsikulyation, kramp. Muscular pains are noted infrequently. Spasms and rigidity can involve both distal, and proximal muscles of extremities, trunk muscles (including a stomach), chewing and mimic muscles. Strengthening of muscular tension and muscular spasms in time and after autokinesia because of what they are complicated and slowed down is characteristic.
At repetition of the movement are carried out easier, and rigidity weakens, however then there is a bystry exhaustion. When involving muscles of a throat and throat difficulties of the speech, swallowing, breath are possible. Walking becomes slowed down and demands from the patient of considerable efforts. Pathological installation of brushes and feet is observed, it is frequent with constant bending or extension of fingers. The pose of extremities reminds a carpopedal spasm at a tetany. Constant muscular tension can cause a generalized myopachynsis. At late stages contractures form. Unlike extrapyramidal hyperkinesias, muscular spasms and a miokimiya remain in a dream.

Tendon jerks are absent, usually because constant muscular activity brakes spinal monosinaptichesky reflexes. Its suppression by means of carbamazepine or dipheninum leads to recovery of reflexes. Disturbances of sensitivity and other neurologic symptoms at an idiopathic neyromiotoniya do not come to light. At a part of patients vegetative signs of a hyper metabolic state — intolerance of high temperature, a hyperhidrosis, tachycardia are noted.

The current of an idipatichesky neyromiotoniya rather high-quality is also quite often characterized by the long periods of stabilization and incomplete remission.

Neyromiotoniya's reasons:

Etiology and pathogeny of a neyromiotoniya. Allocate the idiopathic neyromiotoniya which is often designated also as Isaaks's syndrome (in honor of the English neurologist of H.Isaacs which described it in 1961) and a secondary (symptomatic) neyromiotoniya. Presumably autoimmune process is the cornerstone of an idiopathic form of a disease. At patients with Isaaks's syndrome the antibodies directed against antigens of potassium channels of a presynaptic membrane which can cause hyper excitability nervous bombways in muscles come to light and do possible their frequent spontaneous categories.

The secondary neyromiotoniya usually arises owing to a polyneuropathy (for example, a chronic inflammatory demyelinating poliradikulo-neuropathy, Giyen's syndrome — Barret, toxic polyneuropathies, hereditary sensomotor neuropathies). The paraneoplastic cases connected with lung cancer are described.

Neyromiotoniya's treatment:

Carbamazepine and Phenytoinum reduce muscular tension at a neyromiotoniya. Diazepam and other benzodiazepines, unlike a syndrome of the rigid person, are inefficient. In resistant cases apply a plasma exchange, corticosteroids, cytostatics, in/in immunoglobulins (IS-vein, octadin, etc.).