Acute brainstem dissection of syringomyelia associated with cervical intramedullary neurinoma

Intramedullary tumors and syringomyelia typically present with slowly progressing deficits. More rarely, they are characterized by acute presentation or worsening, at times mimicking other more common etiologies. The acute onset of syringomyelia is most likely attributable to an acute increase in cerebrospinal fluid and epidural venous pressure that results in impulsive fluid movement and, ultimately, in the rupture of the syrinx and dissection into the spinal cord or brainstem. Reported here is a case of acute presentation of a small cervical intramedullary neurinoma due to the upward dissection of its associated syrinx. Critical questions are: (1) how can a small tumor produce a large syrinx? and (2) in the absence of craniospinal interferences, which mechanism underlies the acute expansion of the cavity, resulting in a rapid onset? The authors examined the pathophysiology of syrinx formation and enlargement in intramedullary tumors and reviewed the literature, emphasizing the relationship between spinal cord movements and intramedullary pressure. On the basis of current pathogenetic concepts, the authors concluded that tumor-related syringomyelia might be caused by an association of mechanisms, both from within (obstruction of perivascular spaces; increase in extracellular fluid viscosity due to the tumor itself; intramedullary pressure gradients among different cord levels and between the cord and the subarachnoid space) and from without (the cerebrospinal fluid entering the tissue). All these factors may be amplified, as in the reported case, by a tumor located dorsally at the cervical level. Abnormal postures of the spine, such as a prolonged and excessive flexed neck position, may ultimately contribute to the acute dissection of the syrinx.