Lower spastic paraparesis (paraplegia): causes, symptoms, diagnosis, treatment

Lower spastic paraparesis (paraplegia) develops with bilateral damage to the upper motor neurons (in the region of the paracentral lobules of the cerebral hemispheres) or in the lesion of the corticospinal tract (pyramidal) at the level of the subcortical areas, the brain stem or (more often) the spinal cord. In acute processes in the initial period of acute lesions, paraparesis may be flaccid, followed by a typical spasticity and other manifestations of the pyramidal syndrome.

[1], [2]

The main causes of lower spastic paraparesis (paraplegia):

A. Compression affections.

1. Extramedullary and intramedullary tumors of the spinal cord.
2. Late traumatic compression of the spinal cord.
3. Epidural abscess and other near-shell processes in the region of the spinal cord.
4. Herniated disc of the thoracic spine.
5. Other diseases of the spine.
6. Malformatsiya Arnold-Chiari.

B. Hereditary diseases.

1. Family spastic paraplegia of Strumpel.
2. Spinocerebellar degeneration.

C. Infections.

1. Spirochete infections (neurosyphilis, Lyme disease).
2. Vacuolar myelopathy (AIDS).
3. Tropical spastic paraparesis.
4. Transverse myelitis (including acute demyelinating, postvaccinal, necrotizing).

D. Vascular diseases.

1. Occlusion of anterior spinal artery.
2. Epidural and subdural hemorrhage.
3. Lacunar state.
4. Cervical myelopathy.

E. Other reasons.

1. Parasagital tumor or (rarely) cortical atrophic process.
2. Multiple sclerosis.
3. Syringomyelia.
4. Primary lateral sclerosis.
5. Radiation myelopathy.
6. Shaya-Dryger syndrome.
7. Insufficiency of vitamin B12.
8. Lathirism.
9. Adrenoleukodystrophy.
10. Paraneoplastic myelopathy.
11. Autoimmune diseases (systemic lupus erythematosus, Sjogren's syndrome).
12. Heroin (or other toxic) myelopathy.
13. Myelopathy of unknown etiology.

Syndromically, the lower spastic paraparesis sometimes has to be differentiated with the dystonia of the lower extremities. With Segawa's disease ("dystonia sensitive to levodopa"), for example, dystonia in the legs may manifest as a dystonic hypertonus in the leading leg muscles, hyperreflexia in them, and even the dystonic pseudosymptoms of Babinsky; while the dysbasia may resemble spastic paraparesis. The diagnosis helps to analyze the dynamics of dystonia. Another name for Segawa's disease is "dystonia with pronounced diurnal fluctuations."

A. Compression affections.

Extramedullary and intramedullary tumors of the spinal cord. Spinal cord lesions above the lumbar and lower cervical spine, especially intramedullary volumetric processes, lead to spastic lower paraparesis. Symptoms include pain, bilateral radicular disorders, spastic paraparesis with pyramidal signs, disorders of urination. Disturbances of sensitivity in extramedullary processes are sometimes limited only to the first of the above symptoms; the level of sensitive disorders appears later. Such lesions are refined with lumbar puncture and myelography. The first of the reasons are tumors that can progress for months or years (in the case of meningioma or neurinoma) or (in the case of metastases) can cause paraplegia within a few days or weeks. When radiographing the spine, special attention should be paid to expanding the interpeduncular distances or deformation of the posterior contour of the vertebral bodies, destruction of the arches, or expansion of the spinal canal.

Late traumatic compression of the spinal cord is manifested by neurological syndromes, depending on severity of trauma and features of surgical aggression, among which the lower spastic paraparesis with sensitive and pelvic disorders predominates most often. The trauma in the anamnesis leaves no doubt for the diagnosis.

Another reason is epidural hematoma, which can occur even without previous trauma, for example, during anticoagulant therapy, leads to a rapidly progressing painful paraparesis. Chronic, sometimes cystic arachnoiditis (adhesive processes) can cause a slow increase in weakness in the legs. The epidural abscess, which sometimes develops after minimal trauma, or furunculosis of the skin (or other infection), initially manifests itself only with fever and back pain, which in a few days are followed by radicular pain followed by a rapidly progressive paraparase or paraplegia with conductive sensory and pelvic disorders.

Herniated disc of the thoracic spine with compression of the spinal cord (especially with stenosis of the spinal canal) leads to lower spastic paraplegia. CT or MRI confirms the diagnosis. Usually develops sharply during exercise. Differential diagnosis is most often performed with a tumor of the spinal cord.

Other diseases of the thoracic spine (spondylitis of various etiologies, spondylosis, osteomyelitis, deformities, spinal canal stenosis, arachnoid cyst, Paget's disease, complications of osteoporosis) lead to lower spastic paraparesis due to the process's transition to the spinal cord or its mechanical compression.

Arnold-Chiari malformation is divided into four types: type I designates a hernial protrusion into the large occipital foramen only of the cerebellar tonsils; Type II - cerebellum and lower parts of the brainstem; type III - a rare variant of herniation of the cerebral trunk in combination with cervical or occipital encephalocele; Type IV - reflects the expressed cerebellar hypoplasia and caudal displacement of the contents of the posterior cranial fossa. Malformation can manifest itself in children and adults in the form of symptoms of cerebellar dysfunction, symptoms of involvement of the cervical spinal cord, bulbar paralysis, paroxysmal intracranial hypertension, spasticity, nystagmus and other manifestations. Often a syringomyelic cavity in the cervical spinal cord, sleep apnea in adults (central type), dysphagia, progressive myelopathy, syncope, head and neck cervical pain (and trigeminal neuralgia), symptoms of hydrocephalus are often detected.

The picture of the Arnold-Chiari syndrome may include the lower spastic paraparesis.

Differential diagnosis is carried out with a brain tumor and craniocervical junction, chronic meningitis, multiple sclerosis, cervical myelopathy, traumatic syringomyelia.

B. Hereditary diseases.

Family spastic paraplegia Stryumpel can begin at any age from childhood to the elderly. The clinical picture consists of slowly progressing weakness in the legs and spasticity with increasing dysbasia. Tendon reflexes are increased, Babinsky's symptom is revealed. When the debut of the disease in childhood, pseudocontractures of gastrocnemius muscles with walking "on the thumbs" can be observed. The knees are often slightly bent (sometimes completely straightened - genu recurvarum), the legs are brought. Hands are involved in varying degrees. Possible such "plus symptoms" like dysarthria, nystagmus, atrophy of the optic nerves, pigmentary degeneration of the retina, paralysis of the oculomotor nerves, ataxia (both cerebellar and sensitized), sensorimotor polyneuropathy, epilepsy and dementia (in separate families). With the late debut (40-60 years), there are more sensory and urinary disorders, as well as kinetic tremor.

In the differential diagnosis, diseases such as a spinal cord or a large occipital opening, cervical spondylosis with myelopathy, multiple sclerosis, Arnold-Chiari malformation, primary lateral sclerosis and other diseases involving the spinal cord are considered.

Spinocerebellar degenerations are a large group of hereditary and sporadic diseases, which are combined by participation in the degenerative process of neurons and conductors of the cerebellum and spinal cord. The cardinal manifestation is progressive incoordination of movements. Initial signs in infants usually consist of hypotension and motor development delay. At an older age, children develop lower paraparesis, nystagmus, ataxia, spasticity, Babinsky's symptom, and often a delay in mental development. Tendon reflexes are variable from areflexia to hyperreflexia. In adolescents and adults, variational combinations of ataxia, dementia, ophthalmoplegia, retinitis, dysarthria, deafness, symptoms of lesions of the lateral or posterior columns of the spinal cord, extrapyramidal symptoms and peripheral neuropathy are observed.

Spinocerebellar degenerations include: Friedreich's ataxia; hereditary ataxia due to vitamin E deficiency; autosomal dominant spinocerebellar ataxia, which is based on the so-called phenomenon of expansion of CAG repeats on various mutant chromosomes); ataxia-telangiectasia, abaletiproteinemia, some forms of family spastic paraplegia, olive-ponto-cerebellar atrophies of several types, Machado-Joseph disease, dentato-rubro-pallid-Lewis atrophy, progressive myoclonic ataxia, adrenoleukodystrophy. Some researchers include spinal-cerebellar degenerations and a number of other diseases (episodic ataxia, congenital cerebellar hypoplasia, sporadic forms of OPCA).

C. Infections.

1. Neurosyphilis spinal (in addition to the form tabes dorsalis) is manifested by two more forms. It's about syphilitic meningomyelitis (spastic paraplegia of Erba) and spinal meningovascular syphilis. The latter is sometimes manifested by the syndrome of the anterior spinal artery. Gunma shells of the spinal cord is also found, but even more rarely. Syphilitic hypertrophic pachymeningitis with root pains, amyotrophies of the hands and pyramidal syndrome in the legs (syphilitic amyotrophy with spastico-atactic paraparesis) is described.

Another spirochete infection that can affect the spinal cord and lead to the development of lower paraparesis is Lyme disease.

Vacuolar myelopathy (AIDS) is characterized by the defeat of the posterior and lateral columns of the spinal cord at the level of the upper-thoracic part of the spinal cord and manifests itself by lower spastic paraparesis (paraplegia) and sensitized ataxia. To diagnose this form, serological tests for HIV infection are important.

Tropical spastic paraparesis is caused by the human T-lymphotropic virus (HTLV-I) and manifests itself in slow progressive paraparesis with hyperreflexia, pathological stop signs and disorders of pelvic organs. Some patients simultaneously have symptoms of polyneuropathy. In the cerebrospinal fluid there is a small lymphocytic pleocytosis (from 10 to 50 cells), a normal protein and glucose content and an increased amount of IgG with antibodies to HTLV-I. The diagnosis is confirmed by the detection of antibodies to the virus in the serum.

Transverse myelitis, caused by viruses, bacteria, fungi, parasites, as well as non-infectious inflammatory processes (postinfection and postvaccinal, subacute necrotizing, idiopathic). The onset of these diseases is usually acute with fever and signs of meningomyelitis. Characteristic paresthesia or back pain at the level of the myelitic process, weakness in the legs and sphincter disorders. At first, paresis often becomes sluggish, spasticity develops later. Upon reaching the peak of the disease, the typical subsequent recovery. The improvement is most pronounced in the first 3-6 months.

Differential diagnosis of transverse myelitis is carried out with a spinal cord abscess, acute poliomyelitis, acute disseminated encephalomyelitis, acute necrotizing hemorrhagic leukoencephalitis, adrenoleukodystrophy, Behcet's disease, cervical spondylosis, heroin myelopathy, Lyme disease, multiple sclerosis, radiation myelopathy and other diseases.

D. Vascular diseases.

Occlusion of the anterior spinal artery is observed rarely and manifests itself in different ways depending on the magnitude of the infarction. Typical pain in the neck and back, weakness in the legs, sensitive and pelvic disorders. Symptoms develop instantly or within 1-2 hours. Sometimes there are radicular pains on the upper level of the lesion. Paralysis is usually bilateral, sometimes unilateral and rarely complete.

Epidural or subdural hemorrhage at the level of the spinal cord is much less common ischemic heart attacks and manifests itself suddenly developed compression myelopathy.

The lacunar state that developed as a result of cerebral multiple lacunar infarctions in hypertensive disease can be manifested by pseudobulbar syndrome, pyramidal symptoms on both sides of the body, general weakness (mainly in the legs), dysbasia, and sometimes dementia. Disturbance of walking due to lower spastic paraparesis and falls sometimes becomes the main disadaptive factor of this form of dyscirculatory encephalopathy.

Cervical myelopathy is a serious complication of cervical spondylosis or, more rarely, calcification of the posterior longitudinal ligament at the cervical level, especially if they are combined with a congenital narrowing of the spinal canal. Myelopathy develops in about 5-10% of patients with cervical spondylosis. Since the lateral and posterior columns of the spinal cord are affected, typical complaints of these patients are reduced to numbness and awkwardness of the hands, deterioration of fine motor functions and gradual deterioration of the gait.

In the future, several variants of clinical manifestations may develop:

1. a syndrome of transverse lesion involving corytic-spinal, spinal-thalamic tracts and conductors of the posterior columns of the spinal cord with severe spasticity, sphincter disturbances and a symptom of Lermitt;
2. syndrome of involvement of anterior horns and pyramidal tracts with paresis, marked spasticity, but without sensitive disorders (amyotrophic lateral sclerosis syndrome);
3. syndrome of spinal cord injury with severe motor and sensory impairment, in which there is predominantly weakness in the arms and spasticity in the legs;
4. Brown-Sekar syndrome with a typical contralateral sensory deficit and ipsilateral-motor syndrome;
5. Brachialgia with symptoms of involvement of the lower motor neuron (anterior horns) on the arms.

Many patients also note pain in the neck. Pelvic disorders generally are not common. The first and further steadily progressing symptom is often dysbasia.

Differential diagnosis is carried out with multiple sclerosis, vacuolar myelopathy in AIDS, lupus myelopathy, abetalipoproteinemia, subacute combined degeneration of the spinal cord, tumor, syringomyelia, Arnold-Chiari malformation, primary lateral sclerosis, chronic vertebral-basilar insufficiency, sometimes with Guillain-Barre syndrome, poliomyelitis and peripheral neuropathy. To clarify the diagnosis resort to functional radiographs of the cervical spine, as well as to computer and magnetic resonance imaging.

E. Other reasons.

A parasagital tumor or (rarely) a cortical atrophic process can cause lower spastic paraparesis. The cortical atrophic process, predominantly limited to the precentral gyrus, may manifest unilateral (in the first stages) or bilateral motor disorders of varying severity from (par) paresis to tetraparesis, which progress slowly over the years. Brain atrophy can be detected with computed tomography (Mills paralysis).

Multiple sclerosis.

The spinal form of multiple sclerosis, manifested by the lower spastic paraparesis, in the absence of clear atactic and visual disorders, can be difficult to diagnose. It is important to search for at least one more lesion, to attract MRI, the evoked potentials of different modalities, and to determine the oligoclonal IgG groups in the CSF. However, we must not forget that multiple sclerosis is primarily a clinical diagnosis. Transverse myelitis in the acute stage as a whole is manifested by a more severe clinical symptomatology than the spinal form of multiple sclerosis.

Syringomyelia is a chronic degenerative disease of the spinal cord, characterized by the formation of cavities mainly in the central part of the spinal cord, more often in the cervical region, and manifested by amyotrophy (arms) and dissociated sensitivity disorders of the segmental type. Tendon reflexes in the amyotrophic zone fall out. Quite often develops lower spastic paraparesis (not very roughly expressed) with hyperreflexia. Possible involvement of the posterior columns with ataxia. Approximately 90% of syringomyelia is accompanied by symptoms of malformations of Arnold-Chiari. Other dysraphic signs are often identified. Painful syndrome occurs in about half of the patients. Syringomyelia can be idiopathic or combined with other diseases of the spinal cord (most often tumors and trauma). CT or MRI can confirm the diagnosis.

Primary lateral sclerosis is a rare variant of motor neuron disease, which is characterized by a predominant lesion of the upper motoneuron in the absence of clinical signs of lower motoneuron failure, and manifests itself first by lower spastic paraparesis, and then tetraparesis with hyperreflexia and then involving oropharyngeal muscles. Sensory disturbances are absent. Many researchers consider it to be a form of amyotrophic lateral sclerosis.

Radiation myelopathy is known in two forms: transient and delayed progressive radiation myelopathy. Lower spastic paraparesis develops only in the second form. The disease appears after 6 months (more often in 12-15 months) after radiation therapy in the form of paresthesia in the feet and hands. In the future, one-sided or bilateral weakness in the legs develops. Often in the beginning there is a picture of the syndrome of Brown-Sekar, but later a symptom complex of transverse spinal cord injury with spastic paraplegia, conductive sensory and pelvic disorders is formed. In liquor there is a slight increase in the protein content. Diagnosis is assisted by an MRI study.

Shaya-Dryger syndrome. The pyramidal signs in this disease sometimes take the form of a fairly pronounced lower spastic paraparesis. The accompanying symptoms of parkinsonism, cerebellar ataxia and progressive autonomic insufficiency make the diagnosis of Shay-Dryger syndrome not very difficult.

Insufficiency of vitamin B12 is manifested not only by hematologic (pernicious anemia), but also by neurological symptoms in the form of subacute combined degeneration of the spinal cord (affection of the posterior and lateral columns of the spinal cord). The clinical picture consists of paresthesia in the feet and hands, which are gradually joined by weakness and stiffness in the legs, instability when standing and walking. In the absence of treatment, atactic paraplegia develops with a variable degree of spasticity and contracture. Tendon reflexes on the legs can change both downward and upward. Clones and pathological stop reflexes are possible. Sometimes neuropathy of the optic nerve is observed with a decrease in visual acuity and changes in the mental state (affective and intellectual disorders up to reversible dementia). To reverse the development of symptoms leads only to timely treatment.

Latirism develops with poisoning by a special kind of lentil (rank) and is characterized by a predominant lesion of pyramidal tracts in the lateral columns of the spinal cord. The clinical picture consists of subacute development of spastic paraplegia with impaired functions of pelvic organs. With the exclusion of the rank from food, a slow recovery is observed, often with residual paresis of the disease without atrophy and pelvic disorders. The diagnosis does not cause difficulties if anamnestic data are known. In the past, epidemics of lathyism were described.

Adrenoleukodystrophy. Adult ("spine-neuropathic") form of adrenoleukodystrophy is manifested between the ages of 20 and 30 years and is called adrenomyeloneuropathy. In these patients, adrenal insufficiency is present from early childhood (may be subclinical), but only in the third decade does progressive spastic paraparesis develop and relatively poorly expressed polyneuropathy (sometimes in combination with hypogonadism in men).

Differential diagnosis of the adult form is carried out with a chronically progressive form of multiple sclerosis, family spastic paraplegia, cervical myelopathy and a tumor of the spinal cord.

Paraneoplastic subacute necrotic myelopathy occurs with bronchogenic carcinoma or visceral lymphoma and manifests itself in rapidly progressive paraparesis with conduction sensory and pelvic disorders.

The rapidly progressing "unexplained" lower spastic paraparesis in the "absence" of obvious causes should serve as a pretext for a thorough oncological examination of the patient.

Autoimmune diseases (Sjogren's disease and especially systemic lupus erythematosus) sometimes lead to the development of inflammatory myelopathy with a picture of the lower spastic paraparesis.

Heroin myelopathy is characterized by the sudden development of paraplegia with conductive sensory and pelvic disorders. An extensive necrotizing myelopathy develops on the thoracic and sometimes cervical level.

Myelopathy of unknown etiology is diagnosed quite often (more than 25% of all cases of myelopathy), despite the use of all modern diagnostic methods, including myelography, MRI, study of cerebrospinal fluid, evoked potentials of different modalities and EMG.

It is also useful to remember some rare forms of myelopathy. In particular, myelopathy with lower paraparesis in combination with periphlete and hemorrhage in the retina can be observed in Eales disease (non-inflammatory occlusive disease of mainly retinal arteries, involvement of the brain vessels is rare) and Vogt-Koyanagi-Gerad syndrome (uveitis and meningitis). Paraparesis is also described for ectodermal dysplasia of the Bloch-Sulzberger type (combination of pigmentermatosis with congenital anomalies), hyperglycemia, Sjogren-Larson syndrome (hereditary anomalies), and hyperthyroidism (rarely).

Diagnosis with lower spastic parapaperesis

• MRI of the brain, spine and craniovertebral junction;
• Myelography;
• Investigation of cerebrospinal fluid;
• EMG;
• The evoked potentials of different modalities;
• General blood analysis;
• Blood chemistry;
• Serological diagnosis of HIV infection and syphilis;
• Determination of B12 and folic acid levels in the blood;
• Consultation of a geneticist;
• Oncop search.

[3], [4]