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

Lower spastic paraparesis (paraplegia) develops with bilateral damage to the upper motor neurons (in the area of the paracentral lobes of the cerebral hemispheres) or with damage to the corticospinal tract (pyramidal) at the level of the subcortical regions, the brainstem or (more often) the spinal cord. In acute processes in the initial period of acute damage, paraparesis can be flaccid, later replaced by typical spasticity and other manifestations of pyramidal syndrome.

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The main causes of lower spastic paraparesis (paraplegia):

A. Compression lesions.

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

B. Hereditary diseases.

1. Strumpell's familial spastic paraplegia.
2. Spinocerebellar degenerations.

C. Infections.

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

D. Vascular diseases.

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

E. Other reasons.

1. Parasagittal tumor or (rarely) cortical atrophic process.
2. Multiple sclerosis.
3. Syringomyelia.
4. Primary lateral sclerosis.
5. Radiation myelopathy.
6. Shay-Drager syndrome.
7. Vitamin B12 deficiency.
8. Lathyrism.
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.

Syndromic lower spastic paraparesis sometimes has to be differentiated from lower limb dystonia. For example, in Segawa disease ("dystonia sensitive to levodopa"), dystonia in the legs can manifest itself as dystonic hypertonia in the adductor muscles of the legs, hyperreflexia in them, and even a dystonic pseudo-symptom of Babinski; in this case, dysbasia can resemble spastic paraparesis. Analysis of the dynamics of dystonia helps in the diagnosis. Another name for Segawa disease is "dystonia with pronounced daily fluctuations."

A. Compression lesions.

Extramedullary and intramedullary tumors of the spinal cord. Lesions of the spinal cord above the lumbar and below the cervical spine, especially intramedullary space-occupying processes, lead to spastic lower paraparesis. Symptoms include pain, bilateral radicular disorders, spastic paraparesis with pyramidal signs, and urination disorders. Sensory disturbances in extramedullary processes are sometimes limited to the first symptom mentioned above; the level of sensory disturbances appears later. Such lesions are clarified by lumbar puncture and myelography. The first causes 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 for several days or weeks. When radiographing the spine, special attention should be paid to widening of the interpeduncular distances or deformation of the posterior contour of the vertebral bodies, destruction of the arches, or widening of the spinal canal.

Late traumatic compression of the spinal cord is manifested by neurological syndromes of various severity and manifestations (depending on the severity of the injury and the characteristics of surgical aggression), among which lower spastic paraparesis with sensory and pelvic disorders most often predominates. The history of trauma leaves no doubt for the diagnosis.

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

A herniated thoracic disc with spinal cord compression (especially with spinal canal stenosis) results in lower spastic paraplegia. CT or MRI confirms the diagnosis. Usually develops acutely during physical exertion. The differential diagnosis most often involves a spinal cord tumor.

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 transition of the process to the spinal cord or its mechanical compression.

Arnold-Chiari malformation is divided into four types: type I denotes herniation into the foramen magnum of only the cerebellar tonsils; type II - of the cerebellum and lower parts of the brainstem; type III - a rare variant of herniation of the brainstem in combination with cervical or occipital encephalocele; type IV - reflects pronounced cerebellar hypoplasia and caudal displacement of the contents of the posterior cranial fossa. The 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 palsy, paroxysmal intracranial hypertension, spasticity, nystagmus and other manifestations. Syringomyelic cavity in the cervical spinal cord, sleep apnea in adults (central type), dysphagia, progressive myelopathy, syncope, headaches and cervicoccipital pain (and trigeminal neuralgia), symptoms of hydrocephalus are often detected.

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

Differential diagnosis includes brain tumor and craniocervical junction, chronic meningitis, multiple sclerosis, cervical myelopathy, traumatic syringomyelia.

B. Hereditary diseases.

Familial spastic paraplegia of Strumpell may begin at any age from childhood to old age. The clinical picture consists of slowly progressing weakness in the legs and spasticity with increasing dysbasia. Tendon reflexes are increased, Babinski's symptom is revealed. At the onset of the disease in childhood, pseudocontractures of the gastrocnemius muscles with walking "on the big toes" may be observed. The knees are often slightly bent (sometimes completely straightened - genu recurvarum), the legs are adducted. The arms are involved to varying degrees. Such "plus symptoms" as dysarthria, nystagmus, optic atrophy, pigmentary degeneration of the retina, paralysis of the oculomotor nerves, ataxia (both cerebellar and sensory), sensorimotor polyneuropathy, epilepsy and dementia (in some families) are possible. With late onset (40-60 years), sensory and bladder disorders, as well as kinetic tremor, are more common.

Differential diagnosis includes diseases such as spinal cord or foramen magnum tumor, cervical spondylosis with myelopathy, multiple sclerosis, Arnold-Chiari malformation, primary lateral sclerosis and other diseases involving the spinal cord.

Spinocerebellar degenerations are a large group of hereditary and sporadic diseases that are united by the participation of neurons and conductors of the cerebellum and spinal cord in the degenerative process. The cardinal manifestation is progressive incoordination of movements. Initial symptoms in infants usually consist of hypotonia and delayed motor development. In older children, lower paraparesis, nystagmus, ataxia, spasticity, Babinski's sign, and often mental retardation appear. Tendon reflexes are variable from areflexia to hyperreflexia. In adolescents and adults, varying combinations of ataxia, dementia, ophthalmoplegia, retinitis, dysarthria, deafness, symptoms of damage to 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 ataxias, which are based on the so-called phenomenon of expansion of CAG repeats on various mutant chromosomes); ataxia-telangiectasia, abetalyproteinemia, some forms of familial spastic paraplegia, olivo-ponto-cerebellar atrophies of several types, Machado-Joseph disease, dentato-rubro-pallido-Lewis atrophy, progressive myoclonic ataxia, adrenoleukodystrophy. Some researchers include a number of other diseases in spinocerebellar degenerations (episodic ataxias, congenital cerebellar hypoplasia, sporadic forms of OPCA).

C. Infections.

1. Spinal neurosyphilis (in addition to the form tabes dorsalis) manifests itself in two more forms. We are talking about syphilitic meningomyelitis (Erb's spastic paraplegia) and spinal meningovascular syphilis. The latter is sometimes manifested by the syndrome of the anterior spinal artery. Gumma of the membranes of the spinal cord also occurs, but even more rarely. Syphilitic hypertrophic pachymeningitis with radicular pain, amyotrophy of the arms and pyramidal syndrome in the legs (syphilitic amyotrophy with spastic-ataxic paraparesis) has been 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 damage to the posterior and lateral columns of the spinal cord at the level of its upper thoracic region and is manifested by lower spastic paraparesis (paraplegia) and sensory ataxia. Serological tests for HIV infection are important for the diagnosis of this form.

Tropical spastic paraparesis is caused by the human T-lymphotropic virus (HTLV-I) and is characterized by slowly progressive paraparesis with hyperreflexia, abnormal foot signs, and pelvic dysfunction. Some patients also have symptoms of polyneuropathy. The cerebrospinal fluid shows a small lymphocytic pleocytosis (10 to 50 cells), normal protein and glucose levels, and increased IgG with antibodies to HTLV-I. The diagnosis is confirmed by detecting antibodies to the virus in the serum.

Transverse myelitis is caused by viruses, bacteria, fungi, parasites, and non-infectious inflammatory processes (post-infectious and post-vaccination, subacute necrotizing, idiopathic). The onset of these diseases is usually acute with fever and signs of meningomyelitis. Paresthesia or back pain at the level of the myelitic process, weakness in the legs and sphincter disorders are characteristic. At first, paresis is often flaccid, spasticity develops later. After reaching the peak of the disease, subsequent recovery is typical. Improvement is most pronounced in the first 3-6 months.

Differential diagnosis of transverse myelitis is carried out with 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 rare and manifests itself differently depending on the size of the infarction. Typical symptoms are neck and back pain, weakness in the legs, sensory and pelvic disturbances. Symptoms develop immediately or within 1-2 hours. Sometimes radicular pain appears at the upper level of the lesion. The paralysis is usually bilateral, sometimes unilateral, and is rarely complete.

Epidural or subdural hemorrhage at the level of the spinal cord is observed much less frequently than ischemic infarctions and is manifested by suddenly developed compressive myelopathy.

Lacunar condition, developed as a result of cerebral multiple lacunar infarctions in hypertension, may manifest as pseudobulbar syndrome, pyramidal symptoms on both sides of the body, general weakness (mainly in the legs), dysbasia, and sometimes dementia. Impaired walking due to lower spastic paraparesis and falls sometimes become the main maladaptive factor of this form of discirculatory encephalopathy.

Cervical myelopathy is a serious complication of cervical spondylosis or, less commonly, calcification of the posterior longitudinal ligament at the cervical level, especially if they are combined with congenital narrowing of the spinal canal. Myelopathy develops in approximately 5-10% of patients with cervical spondylosis. Since it mainly affects the lateral and posterior columns of the spinal cord, typical complaints of these patients are numbness and clumsiness of the hands, deterioration of fine motor functions and gradual deterioration of gait.

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

1. transverse lesion syndrome involving the corticospinal, spinothalamic tracts and conductors of the posterior columns of the spinal cord with severe spasticity, sphincter disorders and Lhermitte's symptom;
2. syndrome of involvement of the anterior horns and pyramidal tracts with paresis, severe spasticity, but without sensory disorders (amyotrophic lateral sclerosis syndrome);
3. spinal cord injury syndrome with severe motor and sensory impairments, characterized primarily by weakness in the arms and spasticity in the legs;
4. Brown-Sequard syndrome with typical contralateral sensory deficit and ipsilateral motor deficit;
5. brachialgia with symptoms of lower motor neuron (anterior horns) involvement in the arms.

Many patients also report pain in the neck area. Pelvic disorders are generally uncommon. The first and subsequently steadily progressing symptom is often dysbasia.

Differential diagnosis includes 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 vertebrobasilar insufficiency, and sometimes Guillain-Barré syndrome, poliomyelitis, and peripheral neuropathy. Functional radiographs of the cervical spine, as well as computed tomography and magnetic resonance imaging are used to clarify the diagnosis.

E. Other reasons.

A parasagittal tumor or (rarely) a cortical atrophic process may be the cause of lower spastic paraparesis. The cortical atrophic process, mainly limited to the precentral gyrus, may manifest itself as unilateral (in the early stages) or bilateral motor disorders of varying severity from (para)paresis to tetraparesis, which slowly progress over the years. Brain atrophy may be detected by computed tomography (Mills' palsy).

Multiple sclerosis.

The spinal form of multiple sclerosis, manifested by lower spastic paraparesis, in the absence of clear ataxic and visual disorders can be difficult to diagnose. It is important to search for at least one more lesion, involve MRI, evoked potentials of different modalities and determine oligoclonal IgG groups in the cerebrospinal fluid. However, we must not forget that multiple sclerosis is primarily a clinical diagnosis. Transverse myelitis in the acute stage generally manifests itself with more severe clinical symptoms 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 its cervical region, and manifested by amyotrophy (of the arms) and dissociated segmental sensitivity disorders. Tendon reflexes in the amyotrophy zone are lost. Quite often, lower spastic paraparesis (not very severe) with hyperreflexia develops. Possible involvement of the posterior columns with ataxia. About 90% of syringomyelia are accompanied by symptoms of Arnold-Chiari malformation. Other dysraphic signs are often detected. Pain syndrome occurs in about half of 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 characterized by predominant upper motor neuron involvement in the absence of clinical signs of lower motor neuron involvement, and manifests itself initially as lower spastic paraparesis, then tetraparesis with hyperreflexia, and then with involvement of the oropharyngeal muscles. Sensory impairment is absent. Many researchers consider it 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 6 months (usually 12-15 months) after radiation therapy in the form of paresthesia in the feet and hands. Later, unilateral or bilateral weakness in the legs develops. Often, at the beginning, there is a picture of Brown-Sequard syndrome, but later a symptom complex of transverse spinal cord damage with spastic paraplegia, conductive sensory and pelvic disorders is formed. A slight increase in protein content is noted in the cerebrospinal fluid. MRI examination helps in the diagnosis.

Shy-Drager syndrome. Pyramidal signs in this disease sometimes take the form of a fairly pronounced predominantly lower spastic paraparesis. Concomitant symptoms of parkinsonism, cerebellar ataxia and progressive vegetative failure make the diagnosis of Shy-Drager syndrome not very difficult.

Vitamin B12 deficiency manifests itself not only by hematological (pernicious anemia), but also by neurological symptoms in the form of subacute combined degeneration of the spinal cord (damage to the posterior and lateral columns of the spinal cord). The clinical picture consists of paresthesia in the feet and hands, gradually accompanied by weakness and stiffness in the legs, instability when standing and walking. In the absence of treatment, ataxic paraplegia develops with a variable degree of spasticity and contracture. Tendon reflexes in the legs can change both downwards and upwards. Clonus and pathological plantar reflexes are possible. Sometimes there is neuropathy of the optic nerve with decreased visual acuity and changes in mental state (affective and intellectual disorders up to reversible dementia). Only timely treatment leads to the reverse development of symptoms.

Lathyrism develops with poisoning by a special type of lentil (pea) and is characterized by predominant damage to the pyramidal tracts in the lateral columns of the spinal cord. The clinical picture consists of subacutely developing spastic paraplegia with dysfunction of the pelvic organs. When pea is excluded from the diet, slow recovery is observed, often with residual paraperesis without atrophy and pelvic disorders. The diagnosis is not difficult if the anamnestic data are known. Epidemics of lathyrism have been described in the past.

Adrenoleukodystrophy. The adult ("spino-neuropathic") form of adrenoleukodystrophy manifests itself between the ages of 20 and 30 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 and relatively mild polyneuropathy develop (sometimes in combination with hypogonadism in men).

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

Paraneoplastic subacute necrotizing myelopathy occurs in association with bronchogenic carcinoma or visceral lymphoma and presents with rapidly progressive paraparesis with conductive sensory and pelvic impairment.

Rapidly progressing “unexplained” lower spastic paraparesis in the “absence” of obvious causes should serve as a reason 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 lower spastic paraparesis.

Heroin myelopathy is characterized by the sudden development of paraplegia with conductive sensory and pelvic disorders. Extended necrotizing myelopathy develops at 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, cerebrospinal fluid examination, 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 periphlebitis and retinal hemorrhage can be observed in Eales disease (non-inflammatory occlusive disease mainly of the retinal arteries; involvement of the cerebral vessels is rare) and Vogt-Koyanagi-Gerada syndrome (uveitis and meningitis). Paraparesis has also been described in ectodermal dysplasia of the Bloch-Sulzberger type (a combination of pigment dermatosis with congenital anomalies), hyperglycemia, Sjogren-Larson syndrome (hereditary anomalies), hyperthyroidism (rare).

Diagnostic studies for lower spastic paraparesis

• MRI of the brain, spine and craniovertebral junction;
• Myelography;
• Cerebrospinal fluid analysis;
• EMG;
• Evoked potentials of different modalities;
• Complete blood count;
• Blood biochemistry;
• Serological diagnostics of HIV infection and syphilis;
• Determination of the level of B12 and folic acid in the blood;
• Genetic consultation;
• Oncosearch.

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