If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
Corresponding author at: Division of Neurology, Department of Medicine, National University Hospital, 5 Lower Kent Ridge Road, 119074, Singapore. Fax: +65 69723566.
Syringomyelia is a fluid-filled cavity in the substance of the spinal cord.
•
A syrinx may expand and elongate over time and damage the spinal cord.
•
Weakness of upper limbs and stiffness in the back are common in syringomyelia.
•
A large syrinx may present with minimal symptoms and signs.
•
High index of suspicion is needed for early diagnosis and timely management.
1. Indication
A 21-year-old female presented with complaints of slowly progressive numbness and paresthesia of both hands for the past 4 months. She reported no diurnal variations in hand symptoms, relationship of symptoms to hand position or work, weakness in upper extremities, neck pain or difficulty in walking. For the past 2 months, she suffered from frequent mild occipital headaches, which were pressing in nature, mostly upon awakening and lasting for about one hour. These headaches were never associated with nausea or vomiting. She reported urinary incontinence on a couple of occasions during the past 2 months.
Neurological examination was remarkable for distinctly reduced pain and temperature sensation from the third cervical (C3) to the fourth thoracic (T4) dermatomes on both sides and generalized brisk tendon reflexes in all four extremities. No weakness was noted in the extremities and plantars were down-going. Examination of the spine did not reveal any deformity. Magnetic resonance imaging (MRI) findings of the cervico-thoracic region are shown in Fig. 1.
Fig. 1Magnetic resonance imaging of cervico-thoracic spine. A hyperintense intrinsic lesion is seen on T2-weighted sagittal sequences (A), which is hypointense on T1-weighted images (B) and centrally located in the spinal cord (C). The lesion extends from the second cervical to the tenth thoracic vertebra (D) and does not communicate with the fourth ventricle.
MRI of the cervico-thoracic spine showed a long saccular lesion (Fig. 1A and B), centrally located (C), which extended from the second cervical to the tenth thoracic vertebral level (D) and did not communicate with the fourth ventricle. These findings were suggestive of a large sacculated syringomyelia. Inferior displacement of cerebellar tonsil and mild retroversion of the odontoid process represented a Chiari type 1 malformation, suggestive of its congenital nature [
Absence of vascular risk factors, insidious onset and slow progression exclude the possibility of spinal cord infarction. MRI findings of the presence of fluids in the lesion are not seen in an acute demyelinating lesion. Although an intramedullary tumor may have a predominantly cystic component, no contrast enhancement was noted. Surgical decompression ameliorated her clinical disturbances and she has remained well during the past one year.
Paucity of clinical findings with extensive radiological features and various atypical clinical presentations can occur in patients with syringomyelia [
]. Syringomyelia in patients with Chiari malformation is usually located at the cervical region and may extend into the thoracic region. Delayed caudal cerebrospinal fluid flow in combination with altered spinal subarachnoid pressure are believed to be responsible for the development of syringomyelia and headache in patients with type 1 Chiari malformation [
Changes in temporal flow characteristics of CSF in Chiari malformation type I with and without syringomyelia: implications for theory of syrinx development.
Changes in temporal flow characteristics of CSF in Chiari malformation type I with and without syringomyelia: implications for theory of syrinx development.
00021-2&id=gr1.jpg){kind=link}