Upper Paraparesis : Causes, Symptoms, Diagnosis & Treatment

Last Updated: 15/07/2022

Upper paraparesis is a common symptom of ALS. It occurs in one of the varieties of motor polyneuropathy, some variants of the course of spinal amyotrophy, Hirayama's disease. In combination with lower paraparesis, it is found in trauma, circulatory disorders, compression myelopathy, and a number of degenerative and hereditary diseases. The cause of the formation of the violation is established on the basis of complaints, anamnesis, neurological examination data, neuroimaging, electrophysiological and laboratory studies. Treatment includes exercise therapy, massage, physiotherapy, drug therapy. Operations are sometimes shown.

general information

Upper paraparesis - weakening of the strength of the muscles of the hands. Often asymmetric, develops gradually. There are such variants of the syndrome as:

  • Sluggish. Muscle tone and reflexes are reduced, hypotrophy is determined.
  • Spastic. Muscle tone and reflexes are increased, there is no hypotrophy. The occurrence of pathological reflexes and concomitant movements that are absent in the norm is characteristic (when a motor act is performed by one limb, the second repeats the same movement in a reduced form).
  • Mixed. The symptoms of the above varieties of paraparesis are combined with each other.

The severity of manifestations varies from a mild weakening of the muscles to a complete loss of the ability to make active movements. In an isolated form, the upper paraparesis is much less common than the lower one (muscle weakness in the legs). In a number of diseases, it precedes the development of the lower or occurs some time after a decrease in the muscle strength of the legs. When the cervical thickening of the spinal cord (below the IV cervical vertebra) is affected, the upper spastic paraparesis is combined with a flaccid lower one.

Why does upper paraparesis occur?

amyotrophic lateral sclerosis

Upper paraparesis is considered a characteristic sign of ALS; when this symptom appears, this pathology is first of all assumed, since it is much less common in other diseases. In ALS patients with a cervical debut, the disorder is observed already during the manifestation period, has a flaccid asymmetric character, is combined with pyramidal signs and hyperreflexia.

In ALS with progressive bulbar palsy, the occurrence of these manifestations is preceded by dysphagia, dysarthria, fasciculations, and atrophic changes in the tongue. Patients with ALS with a lumbar onset develop flaccid inferior paresis before the onset of upper asymmetric paraparesis. The formation of pseudobulbar and bulbar syndrome begins later.

Spinal amyotrophies

This group of diseases belongs to the category of hereditary, becomes the result of degeneration of motor neurons. Spinal amyotrophies differ significantly in clinical manifestations, the predominance of upper paraparesis is characteristic of two types of the disease. In distal Duchenne-Aran SMA, the first signs often appear in youth. Hands weaken, take the form of a "clawed paw".

Then the changes spread to the proximal parts of the limb. Lower paraparesis develop much later. Sometimes the disease debuts with upper paraparesis. Vulpian's scapuloperoneal SMA occurs in young or middle age. Unlike the previous amyotrophy, it is not the hands that suffer, but the shoulder girdle, which causes weakness in the arms. Then the muscles of the legs and feet weaken.

Multifocal motor neuropathy

MMN is a type of polyneuropathy, has an autoimmune etiology. The upper paresis is asymmetric and develops gradually. Pain and sensory disturbances are absent. In 95% of cases, the hands are affected, half of the patients suffer from fasciculations and muscle spasms. There is a progressive or undulating course. Hypotrophy is absent or occurs only at a late stage.

Upper paraparesis

 

Hirayama disease

This rare cervical myelopathy develops in adolescence. The main symptom is asymmetric bilateral or unilateral upper paresis. The disease is caused by compression of the spinal cord by a displaced dural sac. It starts slowly and progresses over several years. Then the changes become stable. There are no paraparesis of the legs, proprioceptive reflexes and sensitivity in the hands are not disturbed.

Other reasons

The simultaneous occurrence of upper spastic and lower flaccid paraparesis is noted in traumatic injuries and acute disorders of the spinal circulation in the projection of the V-VII cervical vertebrae. Possible traumatic injuries with such symptoms include contusion and rupture of the spinal cord, as well as compression of nerve tissues due to edema, hematoma, or displacement of bone structures during fractures and dislocations of the vertebrae.

Simultaneous development of similar symptoms is observed in spinal stroke and apoplexy form of arteriovenous malformation. The gradual formation of paresis of the lower extremities, followed by the attachment of the upper extremities, is detected in degenerative diseases such as Strümpel's disease and multiple sclerosis. It is found in some hereditary pathologies, for example, Refsum and Machado-Joseph diseases.

A special clinical picture is determined in dissociative conversion disorder. Symptoms do not have an organic basis, unreliably or partially imitate some kind of neurological pathology. They arise suddenly against the background of stress, social or intrapsychic conflict, persist for several days, then disappear without a trace. With the next stressful situation, they can recur.

Diagnostics

An examination to establish the causes of paraparesis is carried out by a neurologist. If a conversion disorder is suspected, patients are referred for a psychiatric consultation. As part of the survey, the specialist finds out when the symptom first appeared, how it changed over time, what other manifestations it was accompanied by. To clarify the nature of the pathology, the following diagnostic measures are performed:

  • Neurological examination. The doctor evaluates the appearance of the limbs, determines the signs of hypotrophy, other specific changes (for example, "clawed paw"). Reveals the zone of muscle weakness and the nature of paraparesis (spastic or flaccid), explores reflexes and sensitivity. The data obtained during the examination make it possible to suspect a particular pathology, to compile a list of additional procedures.
  • Magnetic resonance imaging. It is the most revealing study in most diseases accompanied by paresis or paraplegia of the upper extremities. It makes it possible to clarify the localization, nature and prevalence of the pathological process. Brain MRI is recommended for suspected ALS, other degenerative and hereditary diseases. MRI of the spinal cord is informative in patients with spinal atrophy, Hirayama disease, tumors, injuries, circulatory disorders.
  • Electrophysiological methods. When conducting electroneurography and electromyography, signs of degeneration of motor neurons, spontaneous bioelectrical activity, indicating the presence of fasciculations, can be detected. Studies are carried out with motor neuropathy, spinal amyotrophy, etc. They are indicative in determining the localization of damage, detecting areas of demyelination, and other changes.
  • Radiography of the spine. It is a mandatory part of the examination for spinal cord injury. Confirms the presence of fractures, fractures, dislocations, displacement of the vertebrae or their fragments. If it is necessary to identify obstructions in CSF circulation, myelography is performed using a contrast agent.
  • Laboratory tests. Histological examination of the nerve biopsy in patients with ALS confirms the presence of signs of demyelination. During PCR, in some cases, mutations in the C9orf72 and SOD1 genes are detected, and in the familial form, in the TARDBP and FUS genes. With spinal amyotrophies, the corresponding genetic mutations are also detected, which make it possible to verify the pathology. In some cases, CSF analysis, blood tests for antibodies and other studies are required.

Physiotherapy

 

Treatment

Conservative therapy

With upper paraparesis, treatment in most cases is symptomatic. A significant role is played by non-drug methods aimed at maintaining and restoring motor functions, improving mobility and self-care ability, and, if necessary, correcting respiratory and nutritional disorders caused by damage to intracerebral structures. The program of non-drug therapy includes the following activities:

  • Physiotherapy. To prevent atrophy, increase muscle tone, patients are taught special exercise therapy complexes. If self-execution is possible, active loads are recommended. With a significant limitation of motor activity, passive exercises are shown, in which the movements of the patient's limb are performed by the exercise therapy instructor.
  • Physiotherapy. To activate metabolic processes in the muscles, electrical stimulation is performed. Depending on the stage of the disease and the violations identified, drug electrophoresis, UHF, laser therapy, mud applications, and other methods may be prescribed. Physiotherapeutic procedures are complemented by manual and hardware massage.
  • Orthopedic devices. To increase the functionality of the limbs, to prevent contractures, special orthoses are used. With concomitant lower paresis, orthopedic shoes are selected, with weakness of the muscles of the body, corsets are used. In Hirayama disease, the use of a Shants collar is recommended to limit neck mobility and prevent excessive neck flexion.
  • Providing food, breathing. With mild and moderately severe dysphagia, special techniques are effective: feeding with liquid and semi-solid dishes, eating in an upright position. In severe upset, a nasogastric tube is placed. Respiratory support is provided by oxygen inhalation or non-invasive ventilation.

Drug therapy is selected taking into account the identified symptoms. To improve metabolism, nootropics and medications that stimulate mitochondrial function are used. Patients with dysphagia and secondary disorders of the upper gastrointestinal tract may require drugs to activate motility, proton pump inhibitors. With weakness of the respiratory muscles, drugs are used to thin and stimulate expectoration of sputum.

Patients with spinal atrophy are prescribed valproic acid derivatives, which increase the survival of motor neurons and have a beneficial effect on the course of the disease. In patients with ALS, anticonvulsants and muscle relaxants are indicated to reduce spasticity and eliminate seizures. With MMN, intravenous immunoglobulin is recommended for long courses according to a special scheme.

Surgery

In severe cases of Hirayama's disease, the dural sac is resected with laminoplasty, sometimes with fixation of the vertebrae by arthrodesis. In case of compression myelopathy, various variants of discectomy are performed; in case of injuries, stabilizing operations on the spine are performed. Tumors are excised. In other cases, interventions are mainly indicated for the correction of secondary disorders: orthopedic techniques to eliminate contractures, the imposition of a gastrostomy for artificial nutrition, tracheostomy.

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