This report summarizes neuropathological, clinical, and general autopsy findings in 105 individuals with nonneoplastic syringomyelia. On the basis of detailed histological findings, three types of cavities were distinguished: 1) dilations of the central canal that communicated directly with the fourth ventricle (47 cases); 2) noncommunicating (isolated) dilations of the central canal that arose below a syrinx-free segment of spinal cord (23 cases); and 3) extracanalicular syrinxes that originated in the spinal cord parenchyma and did not communicate with the central canal (35 cases). The incidence of communicating syrinxes in this study reflects an autopsy bias of morbid conditions such as severe birth defects. Communicating central canal syrinxes were found in association with hydrocephalus. The cavities were lined wholly or partially by ependyma and their overall length was influenced by age-related stenosis of the central canal. Non-communicating central canal syrinxes arose at a variable distance below the fourth ventricle and were associated with disorders that presumably affect cerebrospinal fluid dynamics in the spinal subarachnoid space, such as the Chiari I malformation, basilar impression, and arachnoiditis. These cavities were usually defined rostrally and caudally by stenosis of the central canal and were much more likely than communicating syrinxes to dissect paracentrally into the parenchymal tissues. The paracentral dissections of the central canal syrinxes occurred preferentially into the posterolateral quadrant of the spinal cord. Extracanalicular (parenchymal) syrinxes were found typically in the watershed area of the spinal cord and were associated with conditions that injure spinal cord tissue (for example, trauma, infarction, and hemorrhage). A distinguishing feature of this type of cavitation was its frequent association with myelomalacia. Extracanalicular syrinxes and the paracentral dissections of central canal syrinxes were lined by glial or fibroglial tissue, ruptured frequently into the spinal subarachnoid space, and were characterized by the presence of central chromatolysis, neuronophagia, and Wallerian degeneration. Some lesions extended rostrally into the medulla or pons (syringobulbia). Although clinical information was incomplete, simple dilations of the central canal tended to produce nonspecific neurological findings such as spastic paraparesis, whereas deficits associated with extracanalicular syrinxes and the paracentral dissections of central canal syrinxes included segmental signs that were referable to affected nuclei and tracts. It is concluded that syringomyelia has several distinct cavitary patterns with different mechanisms of pathogenesis that probably determine the clinical features of the condition.

Dysesthethic pain is a common complaint of patients with syringomyelia, traumatic paraplegia, and various myelopathic conditions. Because cavitary lesions of the spinal cord can be defined with good resolution by magnetic resonance imaging, syringomyelia provides a potential model for examining anatomic correlates of central pain. In this study, a syndrome of segmental dysesthesias, characterized by burning pain, hyperesthesia, and a variable incidence of trophic changes, was described by 51 of 137 patients (37%) with syringomyelia at the time of clinical presentation. Complete magnetic resonance scans, including axial images, demonstrated extension of the syrinx into the dorsolateral quadrant of the spinal cord on the same side and at the level of pain in 43 of 51 patients (84%). Surgical treatment of syringomyelia resulted in the relief or improvement of dysesthetic pain in 22 of 37 patients (59%), but 15 patients (41%) reported no improvement or an intensification of pain despite collapse of the syrinx. Postoperative dysesthetic pain was often a disabling complaint that responded poorly to medical therapy, including analgesics, sedatives, antiepileptics, antispasmodics, and anti-inflammatory agents. In most cases, there was a gradual improvement of symptoms, although six patients continued to complain of pain 24 to 74 months postoperatively. Prompt but transient relief was achieved in two of two patients with regional sympathetic blocks, and prolonged relief was achieved in one patient by stellate ganglionectomy. We conclude that painful dysesthesias can be caused by a disturbance of pain-modulating centers in the dorsolateral quadrant of the spinal cord and have certain causalgia-like features that respond in an unpredictable way to surgical collapse of the syrinx.

No abstract