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Headache and hand numbness in a young female

  • Prakash R. Paliwal
    Affiliations
    Division of Neurology, National University Hospital System, Singapore
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  • Vijay K. Sharma
    Correspondence
    Corresponding author at: Division of Neurology, Department of Medicine, National University Hospital, 5 Lower Kent Ridge Road, 119074, Singapore. Fax: +65 69723566.
    Affiliations
    Division of Neurology, National University Hospital System, Singapore

    Yong Loo Lin School of Medicine, National University of Singapore, Singapore
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Published:February 10, 2016DOI:https://doi.org/10.1016/j.ejim.2016.01.020

      Highlights

      • 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.
      Figure thumbnail gr1
      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.
      What is the diagnosis?

      2. Discussion

      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 [
      • Chiapparini L.
      • Saletti V.
      • Solero C.L.
      • Bruzzone M.G.
      • Valentini L.G.
      Neuroradiological diagnosis of Chiari malformations.
      ].
      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 [
      • George T.M.
      • Higginbotham N.H.
      Defining the signs and symptoms of Chiari malformation type I with and without 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 [
      • Clarke E.C.
      • Stoodley M.A.
      • Bilston L.E.
      Changes in temporal flow characteristics of CSF in Chiari malformation type I with and without syringomyelia: implications for theory of syrinx development.
      ]. Pressure-induced flow disturbances in the posterior spinal arteries further contribute to the formation of syrinx in the cervical spinal cord.

      Contributorship

      Prakash Paliwal wrote the report. Vijay K Sharma conceptualized, provided critical revision and final approval of the manuscript.

      Funding information

      This report received no specific funding.

      Conflict of interests

      The authors state that there are no competing interests.

      References

        • Chiapparini L.
        • Saletti V.
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        • Bruzzone M.G.
        • Valentini L.G.
        Neuroradiological diagnosis of Chiari malformations.
        Neurol Sci. 2011; 32: S283-S286
        • George T.M.
        • Higginbotham N.H.
        Defining the signs and symptoms of Chiari malformation type I with and without syringomyelia.
        Neurol Res. 2011; 33: 240-246
        • Clarke E.C.
        • Stoodley M.A.
        • Bilston L.E.
        Changes in temporal flow characteristics of CSF in Chiari malformation type I with and without syringomyelia: implications for theory of syrinx development.
        J Neurosurg. 2013; 118: 1135-1140