Home / Neurological disease of the spinal cord - the description, diagnostics, treatment / Myodystrophy the suffer from landouzy-Dejerine and paralysis of Klumpke – similarities and differences

Myodystrophy the suffer from landouzy-Dejerine and paralysis of Klumpke – similarities and differences

Myodystrophy suffer from landouzy (L. Landouzy) Dejerine (J. Dejerine) was identified in 1984. The disease is inherited in an autosomal dominant type. The pathology is benign, as it progresses slowly, and the man long retains the ability to self-service.

The disease Appears at the age of 20-25 years, when first symptoms of muscle weakness in the hands (muscle-facial plexitis). From poor muscle tone of the masticatory muscles in humans is formed by specific facial expressions.

syndrome suffer from landouzy-Dejerine

What is the syndrome of Dejerine and how different it is from paralysis of Klumpke

The Syndrome Dejerine-suffer from landouzy develops due to paralysis of the lower branches of the brachial plexus. It is manifested by paresis of the skeletal muscles of the hand and face with pupillary disorders.

The Disease is inherited in an autosomal dominant.

The Main symptoms of maxillofacial plexitis:

  • Weakness of facial muscles;
  • "Transverse" smile;
  • "Polished" the forehead;
  • Thick lips ("tapir").

The atrophy of the triceps and biceps muscles of the shoulder, scalloped, trapezoidal, the pectoralis major contributes to the appearance of the following signs:

  • Wing blade
  • the Symptoms of shoulder free;
  • Flattening of the chest;
  • Lateral curvature of the spine;
  • Broad interscapular period.

In some forms of pathology is the weakness of skeletal muscles of the legs. Neurologists thus distinguish the following symptoms of tension:

  1. Precipitously-lichenometry;
  2. Placemaking-shoulder;
  3. Femoral-placeagain-lichenometry;
  4. Placeagain-peroneal.

False hypertrophy are expressed at the deltoid and gastrocnemius muscles. In the early stages of the pathology are also observed reduced reflexes from the biceps and triceps muscles. The disease progresses slowly, so patients keep working.

What is lower Plex

The Symptom of Dejerine Klumpke (lower Plex) is observed due to the internal trauma of the brachial, ulnar and median nerves. Against this background affects the skeletal muscles of a brush except for muscle-ligamentous apparatus, innervated by the radial nerve.

Clinical symptoms of Dejerine Klumpke:

  • Weakness of the muscles of the forearm, fingers, hypothenar, worm-like muscles;
  • is Broken corporately reflex
  • there is a decrease in the sensitivity of the muscles of the forearm, upper arm, dorsal carpal surface, and palms;
  • Appears Horner's syndrome-Bernard (narrowing of the palpebral fissure, pupil, eyeball).

top Symptoms of plexitis (ERB Duchenne)

Top plexitis (ERB Duchenne) should not be confused with an analogue of Klumpke. When it is disturbed innervation of the following muscle groups:

  • the Shoulder
  • Biceps
  • Infraspinatus and supraspinatus;
  • Deltoid
  • Shoulder-beam.

Gradually the above-described muscle groups will atrophy, and the clinical symptoms of the pathology progresses.

Initially the patient cannot lift the shoulder and upper arm to the horizontal level. Over time, it becomes impossible flexion in the elbow joint, abduction and adduction. The reflex of the biceps muscle disappears last.

At the same time occur the symptoms of metabolic disorders:

  1. Pain upper third of the arm;
  2. Pain the ERB's point (in the area of attachment of the sternocleidomastoid muscle);
  3. Weakening of pain sensitivity or absent on the outer edge of the shoulder.

The Symptoms of upper plexitis not lead to paralysis of Dejerine Klumpke not appear with the syndrome suffer from landouzy.

How to manifest pathology

symptom of Dejerine the young manBottom of the Plex Klumpke (distal) occurs when there is an injury of segmental nerve trunks at the level of C7-Th1. The defeat of the lower and middle beams generates the following symptoms:

  • muscle Weakness of the wrists and forearms
  • Brush in the "seal legs";
  • Cyanotic skin – a symptom of "ischemic gloves";
  • Lower grasping reflex (Babkin, Bernard Horner).

Muscular hypotonia and symptoms of tension ERB-Duchenne, Dejerine-suffer from landouzy, Klumpke in the initial stages, unilateral, what distinguishes pathology from meningeal signs when inflammation of the cerebral membranes.

When damage to the nerve fibers at the levels Th4-Th8 appears bilateral hypotonia and symptoms of tension. The clinic is complicated by the syndrome Unterested – with a sharp turn of the head appears spasmodic contraction of the vertebral artery. Against this background, the cerebral symptoms are traced:

  • Cold extremities
  • dysesthesia of the hands;
  • Spinal shock with failure of the lower limbs
  • Defeat at the level of Th3-Th6 leads to respiratory disorders;
  • trauma to the lower thoracic segmental nerves accompanied by weakness of the muscles of the abdominal wall.

Paralysis of the branchesbrachial plexus Klumpke unlike Dejerine syndrome suffer from landouzy leads to a forced position of the patient:

  • Upper limb clutched to her chest;
  • 4 and 5 finger hands are in flexion position;
  • reinforced beam Relative to reflex against the weakness of the other (designated the thumb).

In children, this pathology leads to the curvature of the thoracic spine with the formation of kifisou and scoliokyphosis. A distinctly local symptoms of tension.

Treatment of tension symptoms in lesions of the brachial nerves

the disease Dejerine - rear viewTreatment of brachial nerve lesions depends on the cause of the pathology. Myodystrophy suffer from landouzy, of Dejerine is inherited, therefore, etiological therapy is not carried out. Facilitates the human condition symptomatic treatment.

Paralysis of Klumpke on the background of inflammatory diseases requires antibiotics and anti-inflammatories. If you are experiencing muscle tension, the neurologist prescribed a means for the normalization of blood supply in the spinal cord and the brain.

In traumatic injuries of the spine surgical treatment.

Loss of skin sensation, changes of sweating, vegetative-vascular disorders require rehabilitation procedures.

The Symptoms of tension of Dejerine suffer from landouzy and Klumpke is the first token of subsequent neurologic disorders causing disability.