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Syringomyelia

Hydromyelia

Syringomyelia is a rare disease of the spinal cord characterized by the presence of a fluid-filled cavity, with resulting neurologic defects.


Presentation

The symptoms of syringomyelia usually present slowly and may eventually lead to permanent disabilities. Signs and symptoms of significant syrinx formation are mostly observed in ages 25 to 40 years old. It is imperative to identify the early signs of the disease to merit prompt medical or surgical attention.

In the head and neck areas, signs are often times unilateral. The tongue may present with weakness and hemiatrophy. Difficulty in deglutition may be experienced early. Facial paresis or diminished sensation of the face may be experienced up to the nape area. The eyes will noticeably have slow reaction to tactile stimulus or altered reflexes. Skin areas where supplying nerves are affected by the lesion may experience diminished pain and temperature sensation. Pain may be felt in certain dermatomes supplied by the affected nerves. The lower extremities are more prone to weakness or spasms during caudal compressions although arm fasciculation may be experienced in cervical lesions. Stiffness of back muscles, shoulders and arms are common. Spine deformities may be observable in large spinal tumors while scoliosis of the spine is not infrequent. The lower abdomen may be affected in syringes within the thoracic spinal cavity causing disruption in bowel activity and urinary bladder functions. Chronic constipation and bladder atony may be observable in these cases.

Intravenous Drugs
  • However, similar problems were described before the advent of HIV infection and have also been described in treatment of syphilis in intravenous drug users in the absence of HIV infection.[intranet.tdmu.edu.ua]
Fecal Incontinence
  • Unsteady gait, muscle atrophy, spasticity, urinary or fecal incontinence, or male impotence may also be described. 5,8 Physical examination The classical description of loss of pain and temperature sensation but preservation of light touch is rare.[now.aapmr.org]
Capillary Pulse
  • This reduces the efficiency of the pulse wave dampening, occurring within the cord parenchyma, increasing arteriolar and capillary pulse pressure.[ncbi.nlm.nih.gov]
Neurogenic Arthropathy
  • arthropathy NEC Nonsyphilitic neurogenic spondylopathy NEC Type 1 Excludes spondylopathy (in): syringomyelia and syringobulbia ( G95.0 ) tabes dorsalis ( A52.11 ) ICD-10-CM Diagnosis Code M49 Spondylopathies in diseases classified elsewhere 2016 2017[icd10data.com]
  • Neurogenic arthropathies (Charcot joints) may affect the shoulder, elbow, or wrist. [6] Scoliosis is seen sometimes. [7] Acute painful enlargement of the shoulder is associated with destruction of the head of the humerus.[physio-pedia.com]
Muscular Atrophy
  • Syringomyelia is marked clinically by pain and paresthesia, followed by muscular atrophy of the hands and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved; later marked by painless whitlow, spastic paralysis[medical-dictionary.thefreedictionary.com]
  • Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation).[fpnotebook.com]
  • Muscular atrophies are found especially in the lower neck, the shoulders, arms and hands. Neurological examination revealed diminished or abolished tendon reflexes.[medicaldb.blogspot.com]
Facial Numbness
  • Other symptoms which sometimes accompany the pain in the arms, back, and neck include bowel function problems , bladder problems, muscle spasms (especially in the legs), facial numbness or pain, and scoliosis (curvature of the spine).[disability-benefits-help.org]
Cape-like Loss of Pain and Temperature Sensation
  • The disorder generally leads to a cape-like loss of pain and temperature sensation along the back and arms. Each patient experiences a different combination of symptoms.[urmc.rochester.edu]
  • Patients with this condition demonstrate a wide variety of neurological symptoms depending on where exactly the syrinx is located, but classically patients present with: a cape-like loss of pain and temperature sensation along the back and arms, known[radiopaedia.org]
  • The disorder often leads to a cape-like loss of pain and temperature sensation along the back and arms. Each patient experiences a different combination of symptoms.[chrichmond.org]
Nystagmus
  • Numbness - may replace pain Signs - Horner's syndrome, Nystagmus, Muscle wasting, LE spasticity, Charcot UE joints, Pes Cavus, Short neck, Low hairline, Limb length inequality, Hand/foot asymmetry, Diplopia, Giddiness, Dysphagia, Dysphonia, Salivation[gait.aidi.udel.edu]
  • […] worse over time Pain down the arms, neck, or into the middle back or legs Weakness (decreased muscle strength) in the arms or legs Painless burn or injury of the hand Difficulty walking or toe walking in children Uncontrollable movements of the eyes ( nystagmus[medlineplus.gov]
  • […] reflexes in the arm Persistent headaches Decreased sensation and sensitivity or even numbness Radiating pain through the neck, shoulder, upper arms and upper trunk (also ventral) Uncoordinated movements, spasms and involuntary muscle contractions Dizziness Nystagmus[physio-pedia.com]
  • Vestibulocochlear nerve involvement causes vertigo and nystagmus. Facial, palatal and laryngeal nerve palsy can occur as the VIIth, IXth, Xth and XIth cranial nerves become involved.[patient.info]
Paresthesia
  • A 21-year-old man working as a carpenter presented with a 1-year history of paresthesias in his right arm.[ncbi.nlm.nih.gov]
  • Syringomyelia is marked clinically by pain and paresthesia, followed by muscular atrophy of the hands and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved; later marked by painless whitlow, spastic paralysis[medical-dictionary.thefreedictionary.com]
  • Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation).[fpnotebook.com]
  • Paresthesia Paresis and paresthesias followed by pain. And some studies suggest that sensory disturbance is the common manifestation of syringomyelia.[2]. Sensory disturbance Common presentation.[explainmedicine.com]
Areflexia
  • A complete physical examination will then be performed to look for signs of any areflexia, muscle weakness or other classic features of Syringomyelia.[epainassist.com]
  • References: [1] [4] [5] Clinical features Often asymptomatic Cape-like distribution (neck, shoulders, arms) of: Dissociated sensory loss Dysthetic pain Muscle atrophy , fasciculations , and areflexia (patients may present with a claw hand deformity )[amboss.com]
  • Areflexia, muscle weakness, and atrophy results, commonly involving the hand intrinsics. Conclusion Neuropathic arthropathy of the shoulder is rare, and if not evaluated carefully can be misdiagnosed.[jneuro.com]
  • The lesion produces a central medullary syndrome with dissociated loss of sensation and paresis of the upper limb areflexia. Thermal sensitivity is affected by painful, whose horses are injured at their place of crossing.[medicaldb.blogspot.com]
Cranial Nerve Involvement
  • nerve involvement: With syringobulbia Most often unilateral XII: Tongue weakness & hemiatrophy IX - X: Dysphagia; Dysarthria XI: Weakness & wasting of sternomastoid & trapezius VII: Facial paresis Descending tract of V Reduced pain & temperature on lateral[neuromuscular.wustl.edu]

Workup

A detailed medical history and clinical examination is paramount in the diagnosis of syringomyelia. The following imaging techniques offer the most reliable diagnostic choice [6]:

  • Magnetic resonance imaging (MRI) is the most reliable tool to diagnose syringomyelia especially when it comes to the detailed magnetic image of a syrinx formation in spinal cord area. The entire spinal canal should be investigated with and without contrast. For a more detailed assessment of syringomyelia, flow-sensitive sequences are necessary.
  • Computed tomography (CT) may demonstrate the anomaly in the spine or brain effectively and may demonstrated inherent new growths in the area like tumors.

Treatment

Asymptomatic syringomyelia may be treated with constant monitoring and conservative treatment, in severe, symptomatic cases, surgery is necessary. Identification of the underlying etiology is paramount to identify the appropriate treatment. The conservative treatment includes analgesics and physical therapy. The following surgical approaches are available:

  • Suboccipital and cervical decompression [7]
  • Laminectomy with syringotomy
  • Lumboperitoneal or syringoperitoneal shunt placement
  • Fourth ventriculostomy
  • Percutaneous needling
  • Neuroendoscopic surgery

Successful surgery reduces the size of the syrinx. Regular follow-up with MRI is important to detect recurrence early.

Prognosis

The intensity and extent of the symptoms are not directly dependent on the size of the syrinx. In mild non-progressive asymptomatic cases of syringomyelia, an uneventful clinical course may be expected. In progressive and enlarging syrinx however, progressive loss of motor and sensory functions of the extremities may be observed. Unabated compression may cause severe disability to the patient in the long run. Surgical therapy and the underlying etiology may significantly affect prognosis and outcome of the disease [5].

Etiology

A syringomyelia may be congenital or acquired [1]. Congenital forms are common and in about 50% associated with malformations such as Arnold Chiari malformation, platybasia and Klippel-Feil syndrome. There are a number of diseases that can lead to the development of syringomyelia, including:

In about 20 to 30% of cases, no cause can be found, this is referred to as idiopathic syringomyelia.

Epidemiology

The prevalence of syringomyelia is relatively low and is estimated at about 84 cases per 1,000,000 inhabitants. In Japan only 19 cases per million population were described. In most cases the disease occurs in the third and fourth decade of life, childhood cases have been reported.

Sex distribution
Age distribution

Pathophysiology

The exact pathophysiology in syringomyelia remains unknown. However there are leading theories to explain it:

  • The hydrodynamic theory as postulated by Gardner states that the syrinx formation is created due to the pulsatile pressure created by the cerebrospinal fluid during its flow from the brain ventricles to the central canal of the spinal cord. The theory suggest that any impedance like the closure of the foramen of Magendie may initiate the process to form the cyst [2].
  • The difference of the intraspinal pressure and the cerebrospinal pressure gradient causes the cerebrospinal fluid to collect in the caudal area due to active regulation at the foramen magnum to prevent intracerebral accumulation of fluid during situations such as Valsalva or coughing [3]. Narrowing at the foramen magnum due to for example Chiari malformation causes syringomyelia.
  • The caudal movement of the cerebellar tonsils during systole forces cerebrospinal fluid into the lesion increasing intramedullary pressure [4].

Prevention

Patients diagnosed early with syringomyelia should avoid all occasion of straining and lifting to prevent the enlargement of the syrinx. For those with neurologic complications, physical therapy may significantly reduce pain symptoms. Chronic pain may be submitted for pain management to prevent aggravation of symptoms [8].

Summary

Syringomyelia is an acquired or congenital disease that involves the development of a fluid-filled cavity or “syrinx” within the spinal cord. Small, slowly progressive lesions may be asymptomatic. However, typical symptoms of the disease are dissociated sensory loss at the level of the lesion, pain, trophic and vegetative disorders. Since obstruction of CSF pulsation is the cause of the syringomyelia, a potential therapy is surgical removal of this cause. Although the syringomyelia is not operated directly, it will be smaller when the cause has been removed successfully. The risk of the surgical procedure has to be considered when discussing possible surgical treatment options. In some patients, in spite of all diagnostic possibilities, no cause of the disease can be found. Here, an operation is rarely indicated and conservative treatment options, such as physical therapy or antiepileptic medicatio ncan be considered.

Patient Information

Syringomyelia is the development of a fluid-filled cyst or cavity, called syrinx, within the spinal cord. The most common cause of syringomyelia is an abnormality of the brain (Arnold-Chiari malformation), other causes include tumors, injuries and inflammation. Common symptoms are pain, abnormal sensations, loss of sensation, weakness, stiffness, among others. If the condition is not causing severe symptoms, monitoring and conservative treatment (painkillers and physical therapy) may be sufficient. In other cases, surgery will be needed.

References

Article

  1. Lin JW, Lin MS, Lin CM, Tseng CH, Tsai SH, Kan IH. Idiopathic syringomyelia: case report and review of the literature. Acta Neurochir Suppl. 2006;99:117-20.
  2. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. 1965 Jun. 28:247-59.
  3. Williams B. Progress in syringomyelia. Neurol Res. 1986 Sep. 8(3):130-45.
  4. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg. 1994 Jan. 80(1):3-15.
  5. Williams B. Progress in syringomyelia. Neurol Res. Sep 1986;8(3):130-45
  6. Gruber DP, Crone KR. Neuroendoscopy. In: Grossman RG, Loftus CM, eds. Principles of Neurosurgery. 2nd ed. Philadelphia: Lippincott-Raven; 1998:757-62
  7. Prat R, Galeano I. Pain improvement in patients with syringomyelia and Chiari I malformation treated with suboccipital decompression and tonsillar coagulation. J Clin Neurosci. Apr 2009;16(4):531-4.
  8. Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. Oct 2006;29(4):251-63; discussion 264

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Last updated: 2018-06-22 12:27