Best Syringomyelia Treatment In Delhi NCR.

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What Does Syringomyelia Mean?

Syringomyelia is the development of a fluid-filled cyst (syrinx) within your spinal cord. Syringomyelia develops when cerebrospinal fluid (CSF), which usually flows around the outside of your brain and spinal cord, collects inside your spinal cord and forms a fluid-filled cyst (syrinx). Over time, the cyst may enlarge, damaging your spinal cord and causing pain, weakness and stiffness, among other symptoms. Syringomyelia applies to any cavity within the substance of the spinal cord that may or may not communicate with the central canal (ependyma does not line the cavity wall). In syringomyelia, there is a dissection of the ependymal lining of the central canal and cerebrospinal fluid (CSF) collection within the cord itself. Therefore, the collection does not have an ependymal lining.

Characteristically, the syrinx is in the cervicothoracic cord, with C2 to T9 being the most common location, however the lesion may descend all the way down to the conus medullaris.

Causes of Syringomyelia
  • Congenital Causes-
    • Chiari Malformation: Developmental disorder causing displacement of the cerebellum into the foramen magnum, disturbing CSF circulation.
  • Acquired Causes
    • Trauma: Leads to progressive neurological deficits appearing months to years after the injury.
    • Inflammation: Chronic inflammation resulting from:
      • Meningitis
      • Hemorrhage
      • Surgery within the meninges (including arachnoiditis)
  • Tumors and Constrictions:
    • Extramedullary tumors
    • Osteophytes
    • Chronic large disc hernias compressing CSF drainage pathways
  • Chiari 1 Malformation: A specific malformation where the brain’s base protrudes into the spinal canal.
  • Other Causes:
    • Injury to the spinal cord
    • Spinal tumors
    • Meningitis
    • Tethered cord syndrome (a congenital condition)
Symptoms

Patients who have syringomyelia may experience symptoms like those listed below:

  • Headache
  • Weakness, numbness and stiffness in the back, legs, arms and shoulders
  • Intense pain in the neck and shoulders and face
  • Difficulty walking
  • Muscle weakness and signs of wasting
  • Reduced sensitivity (unable to feel hot or cold)
  • Loss of pain sensation
  • Bladder and bowel dysfunction
  • Scoliosis (spinal curvature)

Although symptoms do vary from patient to patient so it is important to watch out for signs. Usually patients experience early symptoms of weakness in their back, neck, arms and legs and loss of reflexes first.

The symptoms of syringomyelia usually develop slowly and depend more on whether the cavity is in the cervical or thoracic spine and less on its size. There is no typical symptomatology. The symptoms most often correspond to those of central spinal cord injury.

  • The leading symptom is a dissociated sensory disturbance. In this case, pain and temperature sensations are disturbed on one side or at least asymmetrically at the affected level of the spinal cord or are completely abolished while touch is still perceived. As a result, superficial injuries such as burns may not be noticed by the patient.
  • Deep sensitivity, i.e., the perception of certain stimuli from inside the body, is also sometimes impaired.
  • Furthermore, constant drilling pain may occur due to a disturbance of the sympathetic conduction.
  • In addition, a lesion of the central sympathetic fibers can lead to Horner’s syndrome or disorders of sweat production with absent or reduced sweat secretion.
  • At the motor level, damage to motor neurons results in muscle weakness and incomplete paralysis (paresis) of the upper extremities at the level of syringomyelia. In the lower extremities, incomplete bilateral paralysis (para paresis) may occur, usually manifested only in the form of increased reflex readiness (hyperreflexia) and pathological reflexes. Spasticity or even paralysis are rarer.
  • If syringomyelia also extends into the upper cervical medulla or brain stem, this is manifested by disturbances in breathing patterns (especially nocturnal apnea), eye movements (nystagmus), and lower cranial nerves (e.g., difficulty swallowing).
Types of Syringomyelia

  • Syringomyelia with fourth ventricle communication

    – About 10% of syringomyelia cases are of this type. This communication can be observed on MRI. In some cases, a blockage of CSF circulation occurs. A shunt operation may be the best therapeutic option for these patients.

  • Syringomyelia due to blockage of CSF circulation (without fourth ventricular communication)

    – Representing at least 50% of all cases, this is the most common type of syringomyelia. Obstruction of CSF circulation from the basal posterior fossa to the caudal space may cause syringomyelia of this type. The most common example is Arnold-Chiari malformation, which is also associated with communicating syringomyelia. Other causes include the following:

    • Basal arachnoiditis (postinfectious, inflammatory, postirradiation, blood in subarachnoid space)
    • Basilar impression or invagination
    • Meningeal carcinomatosis
    • Pathological masses (arachnoid cysts, rheumatoid arthritis pannus, occipital encephalocele, tumors)
  • Syringomyelia due to spinal cord injury

        – Fewer than 10% of syringomyelia cases are of this type. Mechanisms of injury include

    • spinal trauma,
    • radiation necrosis,
    • hemorrhage from aneurysm rupture or arteriovenous malformation or in a tumor bed,
    • infection (spinal abscess, human immunodeficiency virus, transverse myelitis), and
    • cavitation following ischemic injury or degenerative disease.
  • Syringomyelia and spinal dysraphism

     – Spinal dysraphism may cause syringomyelia through a variety of mechanisms, including those mentioned under the previous three categories. Identification and treatment of associated dysraphism has the greatest impact on arresting progression of syringomyelia.

  • Syringomyelia due to intramedullary tumors

       -Fluid accumulation is usually caused by secretion from neoplastic cells or hemorrhage. The tumors most often associated with syringomyelia are ependymoma and hemangioblastoma. Extramedullary intradural and extradural tumors are considered separately under the second category because the mechanism of syrinx formation is blockage of the CSF pathway.

  • Idiopathic syringomyelia

           – Idiopathic syringomyelia has an unknown cause and cannot be classified under any of the previous categories.Surgical decompression can help in some patients with remarkable neurologic deficit.

Physical Examination
  • A complete physical examination may reveal diminished arm reflexes, which are sometimes present early in the clinical course of syringomyelia.
  • Lower limb spasticity, which may be asymmetrical, appears with other long-tract signs such as paraparesis, hyperreflexia, and extensor plantar responses.
  • Rectal examination includes an evaluation of volitional sphincter control and sensory assessment of sacral dermatomes.
  • Dissociated sensory impairment may be noted.
  • The syrinx may extend into the brain stem, affecting cranial nerves or cerebellar function.
  • Brainstem signs are common in syringomyelia associated with Chiari malformations.
Differential Diagnosis
  1. Acute Inflammatory Demyelinating Polyradiculoneuropathy
  2. Amyotrophic Lateral Sclerosis
  3. Ankylosing Spondylitis
  4. Arteriovenous Malformations
  5. Atlantoaxial Instability in Individuals with Down Syndrome
  6. Brainstem Gliomas
  7. Central Pontine Myelinolysis
  8. Cervical Spondylosis: Diagnosis and Management
  9. Chronic Inflammatory Demyelinating Polyradiculoneuropathy
  10. Diabetic Neuropathy
  11. Ependymoma
  12. Hydrocephalus
  13. Limb-Girdle Muscular Dystrophy
  1. Medulloblastoma
  2. Meningioma
  3. Metastatic Disease to the Spine and Related Structures
  4. Motor Evoked Potentials
  5. Multiple Sclerosis
  6. Neural Tube Defects
  7. Neurological History and Physical Examination
  8. Spinal Cord Hemorrhage
  9. Spinal Cord Infarction
  10. Spinal Cord Trauma and Related Diseases
  11. Spinal Epidural Abscess
  12. Spinal Muscular Atrophy
Management

Medical Management:

  • There is no specific medical treatment for syringomyelia, but the origin of the disease can be met. For example the pressure caused by the Syrinx can be relieved by surgical intervention and thus reduce pain and stop further progression of neurological symptoms. Congenital syringomyelia may require that pieces of the cervical vertebrae in the area of the cavity be removed.

Physical Therapy

  • The physical therapy for patients suffering from Syringomyelia differs depending on the location and impact of the disease, unless presented with an MRI, a neurological examination should clear out at which level the syrinx occurs. The goals of the treatment are to stop the spinal cord damage from getting worse using the techniques explained in the following paragraph and to maximize functioning. They may require active physical therapy, passive mobilizations, occupational therapy or even speechtherapy.

Note: the following physical therapy techniques take into account the most frequent manifestations of this disease:

  • RoM
  • Muscle strength
  • Neck Stability
  • Balance,
  • Coping
  • Occupational Therapy

We try to focus the therapy on the following subjects:

  • Maintaining range of motion using stretching and mobilizations of the neck and upper limb. Do note: there is no evidence to support the use of manipulations, and since the spinal cord has been damaged, most authorities advise avoiding this.
  • Increasing their muscle strength (Usually upper extremity and paravertebral muscles, using training schemes individualized to the patients’ tolerance)
  • Training neck stability (best guided by physiotherapist at first)
  • Sitting and standing balance can be physical and occupational therapy (referring to daily activities as in getting dressed and grooming)
  • Educating the patients about their disease and it’s process over time. It is important to maintain an active lifestyle but there are several risks in high impact activities which should not be overlooked. (See topic “patient advice during follow up after surgery) They should also learn aboute adequate pain management and coping techniques
  • Improving and/or maintaining communication using speech therapy (when the syrinx is present in the lower brain stem, although that should be referred to as ‘syringobulbia’)
  • Maximizing functional capabilities by testing and asking the patient about daily activities and then acting upon the answers. This includes activities such as getting in and out of bed, walking, using a cane of crutches and such. Physical therapists may also refer a patient to the use of leg braces if they are deemed appropriate.

Patient advice during follow up after surgery:

  • Hospital Course: pain relief will be the most significant factor. Therefore it is important the patient receives a lot of bed rest and moves carefully.
    • During the first month after surgery: when the operation is successful, the pain and neurological symptoms should improve rapidly. The patient should try to maintain a modestly active lifestyle. It is important that these patients try to avoid tasks involving heavy lifting! Walking and performing daily activities at ease is highly recommended.
    • On the long term: an adequate follow-up is very important for these patients. If they do not deteriorate, they should try to maintain a relatively active lifestyle.
    • Avoid:
  • Activities that cause excessive strain on the nervous system and spinal cord.
  • Any activity in which high impact can be expected (e.g. football, rugby…)
  • Avoid straining due to heavy bowel movement
  • Avoid extensive coughing
  • Most important: avoid heavy lifting and straining

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