Syringomyelia is a chronic, progressive and degenerative disorder of the spinal cord in the form of a cavity in the middle of the cervical spinal cord segment. This abnormality may extend caudally to the thoracic and lumbar segments or rostrally to the brainstem (syringobulbia). This disorder causes progressive neurologic disturbance, usually as brachial amyotrophy and segmental sensory dissociation [1,2]. This disorder is rare or following congenital abnormalities such as the Arnold-Chiari malformation. Therefore, the manifestation of this disorder can vary, depending on the location of the lesion, the extent of the lesion and the abnormalities it has. However, this disorder can be diagnosed because of the presence of characteristic symptoms such as amyotrophy and dissociation of sensibility. 

Generally, the symptoms are between the ages of 10-60 years or the age of 30-45 years. There is no difference in prevalence between men and women. Anatomical changes in the vertebrae can be in the form of kypho-scoliosis or subluxation. Most patients with types I and II of syringomyelia feel unilateral pain and burning at the edge of the area where there is a sensation of sensory disturbance. This disorder develops slowly, even a person with syringomyelia can stay in the same condition for years or even decades [3]. This case report was made because syringomyelia is a rare case with very varied clinical symptoms involving the motor, sensory and autonomic systems, which have a clinical symp similar to other disorders of the spinal cord [1,4].

We reported the case of a 29-year-old woman with the chief complaint of weakness in the lower limbs. The patient came to the emergency room in Moewardi General Hospital on January 8, 2022 with Complaints of Weakness of the lower limbs since 5 years. The initial complaint felt like numbness in both legs and hands felt stiff. The longe the hands and arms are getting heavier with twitching, feeling hot or cold the more insensitive and accompanied by weakness in both legs. Numbness in the legs followed by heavy walking movements. According to the patient sometimes felt jerky movements. If walking sometimes feels heavy, the pain radiates to both legs is denied, there is no additional unconscious movement, at this time the patient also complains of weakness in the right hand which has worsened to be lifted since 3 months ago, complaints of pain or tightness like being wrapped in a belt are not present. BAB and BAK within normal limits. Denied history of similar illness and family, denied history of trauma, denied old fever.

Examination of the muscle strength of the hands and feet of the four extremities experienced weakness compared to normal, the strength of the hands was stronger than the legs. Physiological reflexes are equally decreased, pathological reflexes are not found. Provisional diagnosis of tetraparese ec susp spinal cord tumor. Atrophy of the muscles of the hands and arms is less visible because the patient does seem thin. General physical examination results were within normal limits. Neurological examination showed a decrease in the movement of the left superior extremity and the strength was also decreased (4+). From the sensory examination, we found a sensory dissociation as high as left cervical dermatome 3 to thoracic 1 (C4-T1), proprioceptive function within normal limits.

Laboratory Examination

Hb 12.4g/d1. Al 7700, blood sugar at 84 mg/d1 and electrolytes Na+, K+, Cl- within normal limits. Because there was an impression of a progressive disease, an MRI was performed. The patient underwent a laminectomy and a shunt was placed. Operative action was carried out with the aim of stopping the progression of neurological deficit symptoms. Suboccipital decompression and upper cervical laminectomy C1-C3, syringotomy and syringosubduro shunt. Laminectomy at cervical level 1-3. The durameter was opened for approximately 1.7-2 cm, then a puncture was performed into the syringe. It was seen that the myelum was thin, clear liquid came out. Through the puncture hole, a semilunar tube (from silicone rubber) is inserted 3-4 cm long and fixed on the dura mater and partly inserted into the subdural space (+1.5 cm) then the dura is sewn back together. After the subduro-shunt is installed, the myelum appears to be deflated. Follow-up: 1 month after surgery, the left shoulder sometimes feels cramped. The strength of the right, left upper and lower limbs increased the same = 5; all physiological reflexes are still decreasing. Hypoesthesia in the left upper extremity.

Cervical Thoraxal MRI examination before surgery showed: There was no transverse myelitis or Hydrosyringomyelia mass as high as Corpus Cervical Vertebrae C2-7 herniation of the cerebellar tonsils through the foramina magnum as far as 1.13 cm leading to a chiari 1 malformation. Recovery and clinical improvement in this patient was very visible, on the 5th postoperative day, he showed an increase in muscle strength on motor examination. Evaluation through clinical conditions and post-operative MRI was evaluated for 1 month, there was improvement on both hands and feet. For mild gait disturbances are still felt but normal activities are still carried out. An MRI evaluation was performed with the results: Cerebral tonsillar herniation was seen with a peg like appearance through the foramina magnum as far as 1.13 cm with a Boogard's angle measured at 139° (Platybasia) leading to an Arnold-Chiari Malformation Type I image. Drainage syringotomy on the posterior aspect of the central canal at level C2-3. Cerebral tonsillar herniation with a peg like appearance through the foramina magnum 1.13 cm. Based on the Cervical Thoracal MRI after surgery (15 February 2021) the results of good drainage were found, the absence of cyst fluid in the spinal cord filled with cysts. Even so, cerebellar tonsillar hernia is still found but its height is reduced.

Figure 1 shows an MRI scan of the cervical region with sagittal and axial views. prior to surgery. The space in the spinal cord (syring) is as high as VC 2-7. 

Figure 1: Pre-Operation (08-11-2021)

Figure 2 MRI of the cervical region with sagittal and axial sections after shunting (I month after surgery) showed that the syring in the spinal cord had narrowed.

Syringomyelia is a potentially serious neurological condition with symptoms that vary from those of other neuromuscular disorders. Early detection and diagnosis with MRI is critical in preventing the development of spinal cord compression and potential neurologic injury. Symptomatic cases are generally treated with surgery.

Figure 2: Post Operation (15-02-2022)

Syringomyelia is a condition where cysts Containing Cerebrospinal Fluid (CSF) form in the spinal cord. Syringomyelia results from the blockage of normal cerebrospinal fluid flow through the spinal cord due to either a congenital anatomic anomaly or an acquired structural abnormality [5,6]. The syrinx usually develops as a result of focal widening of the central canal but can also form within the spinal cord parenchyma. These lesions can develop in association with a variety of congenital anatomic anomalies, acquired structural abnormalities, including scoliosis, spina bifida, Chiari malformation, tumors and bleeding, as well as post-infectious, post-inflammatory and posttraumatic conditions [3,7].

Patients with syringomyeli in the cervical region usually have atrophy of the arms and hands; In this patient, there was weakness of the limbs of the 4 extremities. The presence of complaints of segmental anesthesia with the type of dissociation and missing tendon reflexes from this patient are in accordance with previous findings. Syringomyeli here is syringomyeli without obstruction or Chiari malformation type 1, which occurs when the cerebellar tonsils expand into the foramen magnum, usually first seen in adulthood or adolescence and are generally asymptomatic [6-8]. The pathophysiology of syringomyelia is still debated and numerous reviews on the subject are available. Most articles agree that the syrinx is subject to continuous pulsatile pressure from outside the spinal cord and to sudden rises in pressure associated with coughing, sneezing and valsava maneuvers [9]. Shunting the contents of the syrinx to a low-pressure extraspinal site offers the best chance of maintaining the pressure within the syrinx at a reduced level and dissipating the effects of sudden pressure changes [10].

Neurological examination in this case will show a decrease in physiological reflexes in the arms, spasticity in the lower extremities which are often asymmetrical (paraparesis with lower extremity hyperreflexes) [11]. In addition, examination of the sensory system will reveal dissociation according to the location of the lesion. If syringomyelia affects the brain stem (e.g. due to Chiari malformation), then the brainstem reflex examination will also be disturbed. Based on the results of MRI in this patient, syringomyelia is accompanied by Chiari malformation type-1 [10,11] (Table 1).

Table 1: Classification of Chiari Malformation Based on Clinical Description [2]

There are no specific laboratory tests to make the diagnosis of syringomyelia. Examination of cerebrospinal fluid is not recommended because the risk of herniation is very large. Often there is an increase in intracranial pressure due to complete blockade of the subarachnoid space. There may be a slight increase in the amount of protein. In cases of total obstruction of the subarachnoid space, the amount of protein is around 100 mg/dl. Plain radiological examination can not see the presence of syringomyelia. The supporting examination recommended by clinicians is MRI (Magnetic Resonance Imaging) as the gold standard. This tool can take a detailed picture of body structures such as the brain and spinal cord [12].

Primary Management of syringomyelia is surgical but not all cases require surgical intervention. Asymptomatic incidental lesions are usually followed by periodic imaging. Syringomyelia cases with mild symptoms can remain stable and may gradually resolve spontaneously. Patients with overt symptoms are treated with surgical decompression to restore normal CSF flow. Some of the techniques include decompression of the posterior foramen magnum with or without dural opening, decompression of the anterior foramen magnum and shunting. Patients usually improve after surgical correction of the underlying cause of the syrinx. Relapse or residual syringomyelia after Chiari decompression in adults occurred in an average of 6.7% of cases [10-13].

Gardner proposed a new approach in patients with posterior fossa herniation (Chiari I malformation) based on the hydrodynamic theory of syringomyelia formation (Indirect Approach to Communicating Syringomyelia). The recommended surgery is a suboccipital craniectomy for decompression of herniated cerebellar tonsils and closure of the connection between the syrinx and the fourth ventricle by attaching the muscle to the obex. Overall, studies show that decompression and/or reconstruction of the posterior fossa through various techniques leads to effective neurologic improvement in 60 to 90% of cases [9,10,14].

In general, treatment depends on the neurological symptoms that arise. If it is mild then symptomatic therapy can be given alone but if symptoms worsen then surgical treatment is the main choice. Surgery may be classified into 2 types, depending on the purpose of the intervention: symptomatic surgery, aimed at emptying the syrinx by draining the CSF into other cavities through tubes; and etiological surgery, which aims to identify the exact location of the obstruction and restore normal CSF circulation. The prognosis of patients with syringomyelia is still uncertain due to the complexity of the disorder and its management [9-11]. Complications of syringomyelia are myelopathy and based on the modified Nurrick classification there are 7 grades (Table 2).

Table 2: Nurrick Classification for Myelopathy Examination [12]

Syringomyelia with Arnold Chiari malformation syndrome type I should be treated with surgery. In our case, surgical management of suboccipital decompression, laminectomy, syringotomy followed by a syringosubduro shunt was performed. In patients with cervical syringomyelia C2-C7, clinical and radiological improvement was noted at 1-month follow-up. Postoperative images of the syrinx cavity inevitably correlates with a patient's clinical neurological condition. The dysesthetic (neuropathic or chronic) effect of pain and loss of sensation does not disappear completely and needs to be evaluated post-operatively. Patients that have reduced-size but not complete disappearance of their syringes had similar clinical outcomes compared to patients with complete disappearance of their syringes.

1. Hankey, Graeme J. and Joanna M. Wardlaw. Syringomyelia. Clinical Neurology, Manson Publishing, 2002, pp. 541-533.

2. Minagar, Alireza and J.S. Alexander. “Arnold-Chiari Malformation and Syringomyelia.” Saunder’s Manual of Clinical Practice, edited by Randolph W. Evans, WB Saunders, 2003, pp. 903-909.

3. Ropper, Allan H. and Robert H. Brown. “Diseases of the Spinal Cord.” Adams and Victor’s Principles of Neurology, 8th ed., McGraw-Hill Publishing, 2005, pp. 1084-1087.

4. Mumenthaler, Mark and Heinrich Mattle. “Diseases of the Spinal Cord.” Fundamentals of Neurology, Georg Thieme Verlag, 2006, pp. 141-155.

5. Lin, J.W. et al. “Idiopathic Syringomyelia: Case Report and Review of the Literature.” Advances in Functional and Reparative Neurosurgery, Springer, 2006, pp. 117-120.

6. Massimi, L. et al. “History of Chiari Type I Malformation.” Neurological Sciences, vol. 32, no. 3, 2011, pp. 263-265.

7. Lucchetta, M. et al. “Syringomyelia Associated with Chiari I Malformation.” Neurological Sciences, vol. 30, no. 6, 2009, pp. 525-526.

8. Sarnat, H.B. “Disorders of Segmentation of the Neural Tube: Chiari Malformations.” Handbook of Clinical Neurology, vol. 87, 2007, pp. 89-103.

9. Atchley, T.J. et al. “Systematic Review and Meta-Analysis of Imaging Characteristics in Chiari I Malformation: Does Anything Really Matter?” Child’s Nervous System, vol. 36, no. 3, 2020, pp. 525-534. https://doi.org/10.1007/s00381-019-04398-4.

10. Aliaga, L. et al. “A Novel Scoring System for Assessing Chiari Malformation Type I Treatment Outcomes.” Neurosurgery, vol. 70, no. 3, 2012, pp. 656-665. https://doi.org/10.1227/ neu.0b013e31823200a6.

11. Gilmer, H.S. Chiari Malformation Program: Education, Treatment and Support. Michigan Head & Spine Institute, 2020. https://www2.mhsi.us/images/education/MHSI chiariBooklet.pdf.

12. Choi, Sung and Chang-Nam Kang. “Degenerative Cervical Myelopathy: Pathophysiology and Current Treatment Strategies.” Asian Spine Journal, vol. 14, 2020, pp. 710-720. https://doi.org/10.31616/asj.2020.0490.

13. Cesmebasi, A. et al. “The Chiari Malformations: A Review with Emphasis on Anatomical Traits.” Clinical Anatomy, vol. 28, no. 2, 2015, pp. 184-194.

14. Chern, J.J. et al. “Pediatric Chiari Malformation Type 0: A 12-Year Institutional Experience.” Journal of Neurosurgery: Pediatrics, vol. 8, no. 1, 2011, pp. 1-5.