NEUROLOGY AND NEUROSURGERY - Y. V. ALEKSEEENKO - 2014

SYRINGOMYELIA

Syringomyelia is a chronic progressive disease of The Nervous system characterized by The formation of cavities (cysts) within the Spinal Cord or Medulla Oblongata. The prevalence of this condition varies, ranging from 0.3 to 9 per 100,000 population across different countries. Syringomyelic cavities are most commonly detected in the lower cervical and upper thoracic segments of the spinal cord. Occasionally, they extend into the medulla oblongata, a condition referred to as syringobulbia. Much less frequently, cavities develop in the lumbosacral region.

The Etiology of syringomyelia remains incompletely understood, although it is clearly a heterogeneous disorder. Pathomorphological examination typically reveals longitudinally oriented cavities in the central Regions of the spinal cord spanning several segments, accompanied by glial tissue proliferation. Syringomyelia can be classified as communicating or non-communicating. The first variant involves communication between the cavity—or an abnormally dilated central canal of the spinal cord—and the normal pathways of CEREBROSPINAL FLUID (CSF) Circulation. This condition is most often attributed to a defect in the primary neural tube closure or fusion. The second variant is generally symptomatic, where cavity formation is the consequence of trauma, Hemorrhage, or other spinal cord lesions. Hydrodynamic CSF effects, impaired venous outflow, ischemia, and spinal cord edema play a significant role in cavity formation and enlargement. The Development of communicating syringomyelia may be associated with congenital Anomalies of the craniovertebral junction (such as Chiari and Dandy-Walker malformations); when CSF outflow from the Fourth ventricle into the subarachnoid space of the spinal cord is obstructed, conditions are created for the central canal to dilate under the pressure of the pulsating cerebrospinal fluid wave. Syringomyelia can also be familial and is frequently associated with certain developmental abnormalities.

The onset of syringomyelia is typically gradual. Most often, sensory disturbances are the initial manifestation of the disease. This is explained by damage to the posterior horns of the spinal cord and interruption of the spinothalamic tract fibers at the level of the affected segments, leading to impaired pain and Temperature Sensation while other modalities remain intact. These so-called dissociated sensory losses of a segmental type, which are highly characteristic of syringomyelia, are nevertheless not strictly pathognomonic for this disease. Frequently, the distribution of sensory impairment takes on a "jacket-like" or "half-jacket" pattern.

The gradual expansion of syringomyelic cavities leads to the appearance and progression of other symptoms—signs of a transverse spinal cord lesion involving the lateral and anterior horns, as well as the lateral and posterior columns. Consequently, bilateral segmental autonomic and trophic disorders emerge, along with signs of lower spastic paraparesis. Damage to the anterior horns of the spinal cord results in weakness and Atrophy of the hand, forearm, and other limb Muscles. The posterior columns of the spinal cord are affected last, which in the late Stages of the disease leads to impairments in proprioceptive and tactile sensation below the level of the lesion. Bladder and bowel dysfunctions are rare. Localization of the process in the medulla oblongata results in facial sensory disturbances. If the disease progresses in this region, bulbar symptoms (dysarthria, dysphagia, and dysphonia) may develop.

Sensory disturbances in the limbs and trunk account for The high frequency of accidental injuries, Burns, or other traumas that, in the Cytology/cytology/16.html">Early stages of the disease, typically defy logical explanation. Frequently, the initial manifestations of the condition are systematic burning pains, paresthesias, and hyperpathia in the arm or face. The progression of autonomic-trophic disorders leads to severe joint damage (neuro-osteoarthropathy) with joint enlargement and marked soft tissue Changes in the hands (cyanosis, hyperhidrosis, volumetric increase). Accidental soft tissue injuries under such circumstances heal slowly, often leading to purulent complications, bone necrosis in the distal PARTS OF THE limbs, and sequestration of the affected Phalanges. Nevertheless, segmental sensory deficits in syringomyelic patients may coexist with excruciating pain in the limbs or face (in cases of syringobulbia).

Patients with syringomyelia and their relatives may exhibit various developmental anomalies: chest deformations or Asymmetry, kyphoscoliosis, curvature of the fingers and toes, craniovertebral anomalies, Spina bifida occulta, etc. These abnormalities constitute signs of the so-called dysraphic status, confirming The Link Between the disease and underlying disruptions in tissue and systemic embryonic development.

In advanced stages of the disease, Diagnosis is generally straightforward. It is supported by the presence of segmental dissociated sensory disturbances, autonomic-trophic disorders in the same anatomical distribution, and potentially signs of long-tract spinal cord involvement. Evidence of a dysraphic status reinforces the suspicion of syringomyelia. Syringomyelia must most frequently be differentiated from intramedullary tumors, AMYOTROPHIC LATERAL SCLEROSIS, spinal vascular disorders, and certain other conditions accompanied by limb sensory deficits and Muscle hypotrophy.

The diagnosis of syringomyelia has improved significantly with the advent of MRI. This imaging modality allows for the detection and detailed Analysis of the primary pathomorphological substrate of the disease—the Location, size, and extent of the syringomyelic cavity. Equally important, MRI in most cases reliably differentiates syringomyelia from other spinal cord disorders with similar clinical presentations. Cerebrospinal fluid analysis in syringomyelia is typically normal.

In most cases, syringomyelia follows a chronic, slowly progressive course. In approximately 15% of observations, no substantial worsening of symptoms occurs. Patients generally maintain their working capacity for a long time.

Traditionally, a wide range of medications and Procedures are used in the Treatment of syringomyelia, the efficacy and pathogenetic rationale of which are frequently questionable. Analgesics and NSAIDs are used for pain management. For severe, intractable pain, antidepressants are occasionally prescribed. Vasoactive agents, Vitamins, and neurometabolic drugs are also employed. However, the heterogeneous nature of syringomyelia necessitates an individualized approach to managing each specific patient. For anomalies at the craniovertebral junction, surgical intervention is performed to relieve spinal cord compression and improve CSF circulation. Shunting of the syringomyelic cavities is also performed.

Clinical Problems

Option 1

A 26-year-old woman consulted a surgeon regarding a burn on her right shoulder, which she discovered after waking up. The burn presumably resulted from contact with a heating device. A neurological examination revealed a loss of pain and temperature sensation in the C₄ - T₆ dermatomes on the right and Horner's syndrome on the same side. Tendon Reflexes are somewhat brisk on the right. In childhood, she underwent surgery for a cleft palate.

   • List the main neurological disorders.

   • Determine The Nature of the sensory disturbances.

   • Describe the signs and explain THE ORIGIN OF Horner's syndrome.

   • Formulate the topical diagnosis.

   • Formulate the preliminary clinical diagnosis.

   • List other diseases that can cause similar symptoms.

   • Indicate the necessary instrumental examination Methods.

   • Describe the required somatic examination.

   • State the key elements of the ETIOLOGY AND Pathogenesis of the disease.

   • Explain The Significance of events in the remote medical history.

   • What autonomic disorders may occur in this condition?

   • What motor impairments can be observed as the pathological process progresses?

   • Name the main Clinical forms of the disease.

   • List the components of combination therapy.

   • Determine the prognosis of the disease.