Acute Idiopathic Syringomyelia
Ghazal Ahmad1, Mithilesh Siddu2
1Neurology, Medical College of Georgia, 2Neurology, Emory University
Objective:

To determine the etiology of syringomyelia that developed acutely in a patient without history of trauma, Chiari malformation, neoplasm, or infection.

Background:

Syringomyelia is a longitudinal fluid-filled cavity present in the spinal cord tissue or central canal. Most cases are associated with Chiari I malformations, but syrinx formation can also be associated with neoplasm, trauma, or postinfectious sequelae. When none of these factors are present, disruptions in cerebrospinal fluid (CSF) flow and microtrauma are considered as potential contributors to syrinx formation.

Design/Methods:

Case report

Results:

The patient is a 31 year-old-male who presented from a correctional facility to ER with altered mentation. His past medical history included hypertension, end-stage-renal disease, nephrotic syndrome, and type 2 diabetes. He had hypertensive emergency and respiratory distress secondary to pneumonia, requiring care in the medical intensive unit. Once initial metabolic insults were addressed, the patient complained of bilateral lower extremity weakness. On examination, he had complete loss of sensation below the T4 dermatome, and bilateral lower extremity paralysis with absent reflexes. MRI of cervical spine showed abnormal cord signal in the C7 to T2 area, favoring syringomyelia. MRI of the brain without contrast showed DWI signal in the splenium of the corpus callosum, and no Chiari malformation was seen. CSF studies, except for mildly elevated protein, were normal.  The patient received high-dose intravenous steroids for five days for acute myelopathy treatment. His motor strength improved slightly upon discharge but was insufficient for walking.

Conclusions:

Our patient likely experienced subtle microtrauma to the spinal cord while being hemodynamically unstable before the onset of syringomyelia symptoms. In the context of underlying CSF flow abnormalities, this microtrauma might have contributed to the development of acute syringomyelia.

10.1212/WNL.0000000000206253